Answer 2 incorrect: Graves' disease is characterized by hyperthyroidism and one or more of the following features: goiter, exophthalmos and pretibial myxedema. Answer 4 incorrect: The most obvious symptom of subacute thyroiditis is neck pain. Sometimes, the pain may extend to the jaw or ears. Painful enlargement of the thyroid gland can last for weeks or months. And you usually have low serum thyroid stimulating hormone (TSH). High serum free T4 level. High serum thyroglobulin level. Incorrect answer 4: Very rare tumor.

Answer 2 [HIDDEN]: Graves' disease is characterized by hyperthyroidism and one or more of the following features: goiter, exophthalmos and pretibial myxedema. Answer 4 [HIDDEN]: The most obvious symptom of subacute thyroiditis is neck pain. Sometimes, the pain may extend to the jaw or ears. Painful enlargement of the thyroid gland can last for weeks or months. And you usually have low serum thyroid stimulating hormone (TSH). High serum free T4 level. High serum thyroglobulin level. [HIDDEN]: Very rare tumor.

A 30-year-old woman comes to the clinic reporting anxiety, weight loss of about 6 kg and a feeling of "nervousness" in the last three months. In the physical examination she has tachycardia, hyperreflexia and absence of goiter. In the blood test TSH values are < 0.01 microU/mL, T4 is elevated and thyroglobulin levels are low. A scintigraphy shows an absence of uptake in the thyroid region. What seems to you the most likely diagnosis?

Ovarian teratoma (ovarian stromal tumor).

RET/PTC3 rearrangement and thyroid differentiation gene analysis in a struma ovarii fortuitously revealed by elevated serum thyroglobulin concentration.

Struma ovarii (SO) is usually asymptomatic and only in a few cases it is associated with thyrotoxicosis. The presurgical diagnosis is very uncommon. In the majority of cases a pelvic mass is discovered at physical examination or by abdominal ultrasound. Only the hystopathologic examination is able to reveal the characteristic features of SO, with thyroid cells organized in follicles as the main tumoral tissue constituent. The histologic recognition of malignancy is not easy and usually requires an exhaustive sampling of the lesion to evaluate the extracapsular invasion. We report the case of a 59-year-old woman who came to our observation for the fortuitous finding of elevated serum thyroglobulin (Tg) levels (600-800 ng/mL). Because the thyroid function was normal and the ultrasound showed only a subcentrimetric nodule, the clinical suspicious of a SO was considered. Ultrasound examination of the abdomen showed a solid mass of 2 cm in the left ovary. A (131)I uptake was observed at scintiscan in the site of the solid mass. Three months after the resection of the left ovary serum Tg levels were markedly reduced (106 ng/mL), and its values continued to decrease down to 34 ng/mL at last control. The histology showed that the ovarian mass was mainly constituted of thyroid tissue (98%), with no malignant features. The molecular analysis of several thyroid differentiation gene mRNAs in the SO tissue showed an abundant expression of all genes but pendrin (PDS). A reduced PDS mRNA expression might explain the defective thyroxine (T(4)) production. Despite the absence of malignant features, the expression of RET/PTC3 rearrangement was found, raising the possibility of a potential malignant nature of the tumor. A cancer-free period of 3-4 years, as in our patient, is not long enough to definitively exclude a late onset metastatic disease but, unfortunately, the patient died of nonmedical reasons. In conclusion, we report a case of SO that, to our knowledge, is the first in which the clinical suspicion arose from the inappropriately elevated presurgical serum levels of Tg. A quite exhaustive molecular analysis of thyroid specific genes and oncogenes provided two interesting findings: the low PDS mRNA expression, which may explain the low hormonal production and the absence of thyrotoxicosis and the presence of a RET/PTC3 rearrangement, which prompts the possibility of a late malignant evolution.

First case report of papillary thyroid carcinoma arising within a functional teratoma in Graves' disease patient.

Mature cystic teratoma is the most common kind of ovarian germ tumor. However, malignant transformation is uncommon, differentiated thyroid carcinoma is even rare. Hyperthyroidism due to coexistence of Graves' disease (GD) and struma ovarii has been reported. Functional teratoma with papillary thyroid carcinoma (PTC) in GD case has never been reported in literature. A 48-year-old woman with GD for 4 years, who visited our hospital with complaints of severe abdominal pain for 1 day. Computed tomography of the abdominal revealed a large fat-containing lesion with dense calcification, measured 8.6 × 7.2 cm in size. Laparotomy right total oophorectomy was performed, and a huge gangrenous right ovary was noted during exploration. The final pathological diagnosis was teratoma with PTC change at right ovary. We performed thyroglobulin, TTF-1 and CK19 staining in the teratoma, the results were positive, suggesting the thyroid-hormone secretion in the PTC tissue. After resection of the ovarian lesion, euthyroidism was achieved. Adjuvant thyroidectomy is not performed for no evidence of thyroid lesion or distant metastases. No GD recurrence in the 2 years after operation. The patient also does not manifest any gynecological disease symptoms, whereas the other ovary, in the follow-up ultrasound examinations, shows normal size and echo structure. PTC can arise within ovarian teratoma and may have thyroid hormone production. Surgeries of unilateral oophorectomy or cystectomy are a reasonable treatment, and follow-up of thyroid image and data is necessary.

Hyperthyroidism due to struma ovarii: Diagnostic pitfalls and preventing thyroid storm.

We report struma ovarii in a case that had hyperthyroidism and was treated with laparoscopic tumor resection. A 40-year-old Japanese woman presented with tachycardia, finger tremor, and weight loss. Although blood examination showed hyperthyroidism, test results for thyroid stimulating hormone receptor antibody and thyroid stimulating antibody were negative, and thyroid scintigraphy showed no abnormal findings. Because she was diagnosed with an ovarian tumor, and whole-body scintigraphy showed that iodine uptake was detected in the pelvic space, we diagnosed her with an ovarian tumor, which caused excessive thyroid hormone secretion. After controlling the thyroid hormone level, we resected the ovarian tumor laparoscopically. The thyroid hormone level was within the normal range postoperatively without any medications. Based on our experience, physicians need to remember that ovarian tumors can cause hyperthyroidism. Controlling the thyroid hormone level preoperatively by using antithyroid drugs and performing minimally invasive laparoscopic surgery is considered useful for preventing thyroid storm.

Hyperthyroidism associated with <i>struma ovarii</i> - a case report and review of literature.

Hyperthyroidism is a state characterized by elevated serum level of thyroid hormones: thyroxine (T4) and triiodothyronine (T3). This is mainly related to the condition and functioning of the thyroid gland. In 60-80% of cases elevation of these hormones are caused by Grave's disease. Thyrotoxicosis is an extreme presentation of hyperthyroidism which can, in rare cases, be caused by excessive synthesis of thyroxine by tumor cells. Struma ovarii is a rare ovarian teratoma composed of thyroid tissue in more than 50%. To present a case of a 30-year-old female patient with a past history of Grave's disease treated by strumectomy 7 years prior; now presenting for the assessment of secondary amenorrhea. Pelvic ultrasound revealed bilateral solid tumors on the left and right ovary, respectively measuring 5 cm and 6 cm in diameter. Her clinical presentation was suggestive of overt hyperthyroidism, and she presented with a significantly elevated CA-125 (152.7 U/mL). The patient subsequently underwent a bilateral oophorectomy in which both masses were excised and histopathological examination confirmed teratoma maturum cysticum. Struma ovarii was noted as a component of the left ovary teratoma. Establishing a proper diagnosis of hyperthyroidism and elucidating its origin is often challenging. Struma ovarii is a rare cause of hyperthyroidism but should always be considered in case of treatment resistant hyperthyroidism. This case-report lends itself as an example of the value in maintaining gynecological-endocrinological knowledge in the setting if clinical gynecology.

The many causes of subclinical hyperthyroidism.

Subclinical hyperthyroidism, defined as serum thyroid hormone levels in the reference range with low serum TSH concentration, is a well recognized clinical entity, but little information is available concerning the prevalence of the disorders that produce it. We conducted a 24-month retrospective survey of subclinical hyperthyroidism patients referred to a university hospital nuclear medicine service for diagnostic studies. Twenty-four consecutive patients were evaluated (22 outpatients and 2 inpatients). All patients had highly sensitive TSH determination, thyroid hormone levels, radioiodine uptake and scan (except for 2 postpartum women), and, selectively, TSH-receptor antibody (TRAb), serum thyroglobulin, antithyroid antibodies, T3-suppression test, and erythrocyte sedimentation rate. A TSH value of about 0.1 microIU/mL was used as the cutoff. Only one patient in the group had a nonthyroidal disorder. In 14 patients (61%) subclinical hyperthyroidism was self-limited, due to silent thyroiditis (5 patients), iodine-induced hyperthyroidism (3 patients), postpartum thyroiditis (2 patients), subacute thyroiditis (2 patients), and probable hemorrhage into a functioning nodule (2 patients). Of the non-self-limited disorders (39%), Graves' disease accounted for 6 patients and solitary or multinodular goiter for 3. Graves' disease proved difficult to diagnose because the thyroid gland was normal in size in two of the six patients, TRAb was positive in only two of six, and the radioiodine uptake and gradient were normal in all six; the T3-suppression test was positive in two of two patients. We conclude that the causes of subclinical hyperthyroidism are the disorders that commonly produce overt thyrotoxicosis in medical practice, Graves' disease being the most frequent. However, the tests used to diagnose overt Graves' disease often fail in the setting of subclinical hyperthyroidism, except possibly the T3-suppression test.

Coexistence of malignant struma ovarii and Graves' disease.

To report an unusual case of hyperthyroidism from Graves' disease that was coexistent with malignant struma ovarii. We summarize the clinical history, physical findings, laboratory data, imaging studies, pathologic features, and treatment in a patient with recurrent hyperthyroidism and discuss the incidence of ovarian tumors of various histologic origins, including thyroid tissue (that is, struma ovarii). Five years after diagnosis of Graves' disease and resolution of symptoms with 1 year of antithyroid drug therapy, a 53-year-old woman had recurrence of palpitations, tremors, and weight loss. Results of thyroid function tests showed high total and free thyroxine levels and a low thyrotropin level. Thyroid radioiodine uptake was high (69% at 24 hours). Abdominal ultrasound studies disclosed a cystic mass in the right adnexal area. Total abdominal hysterectomy and bilateral oophorectomy revealed a 7.5-cm cystic right ovary that contained a 1.0-cm struma ovarii with a 0.4-cm nodule of follicular variant papillary thyroid carcinoma within it. The patient was treated with methimazole and radioiodine ablation of the thyroid. Three months later, a massive myocardial infarction resulted in her death. The concomitant presence of Graves' disease complicates the management of struma ovarii and raises interesting questions about treatment and prognosis.

[Particular evolution of the thyroid state in Grave's disease: two cases].

We report two cases of Grave's disease (GD) caracterized by the succession of hypothyroid and hyperthyroid states. Case 1: A 32 years old woman, has presented initially a typical GD with hyperthyroidism. Grave's ophtalmopathy and homogenous goiter. Four months later, she presented a spontaneous hypothyroidism necessiting treatment with thyroxine and a severe myasthenia gravis. More later (6 months), she experienced symptoms of hyperthyroidism after thymectomy. The level of anti-thyrotropin-receptor antibodies (TSab) was very high (141 UI/I, NV < 10). Case 2: A 29 years old woman has been treated by thyroxine (150 microg/day) for a primary hypothyroidism. Ten months later, she presented symptoms of hyperthyroidism even after stoppage of thyroxine. TSH value was decreased (TSH < 0.05 microU/ml) and FT4 level was raised (FT4 = 25.5 pmol/l). The thyroid antibodies were positive. We discuss, after review of the litterature, the physiopathological mecanisms of these changes in the thyroid state, particularly the role of the blocking and stimulating anti-thyrotropin-receptor antibodies.

Resolution of hyperthyroidism and thyroid antibodies following struma ovarii resection: an uncommon entity.

We report a case of 34-year-old clinically asymptomatic woman who had been followed for 6 years for hyperthyroidism with thyroid stimulating hormone <0.006 uIU/mL, free T4 1.98 ng/mL, free T3 5.3 pg/mL, elevated thyroid stimulating immunoglobulin 1.70 IU/L, thyroid peroxidase antibody 38 IU/mL and thyroglobulin antibody 9.3 IU/mL. Radioiodine thyroid scan showed minimal uptake in both thyroid lobes (24-hour uptake was 0.3%). She subsequently underwent evaluation for lower abdominal pain and menstrual irregularities, which revealed a large left ovarian cyst measuring 15.9 cm × 10.8 cm × 13.2 cm and right-sided ovarian cyst measuring 2.7 cm × 3.3 cm × 3.5 cm. Laparoscopic bilateral ovarian cystectomy was performed and the final pathology revealed struma ovarii of the left ovarian cyst with the entire ovarian tumour made up of benign thyroid tissue. Thyroid function tests performed 3 months after surgical removal of struma ovarii showed euthyroidism. We present a rare case with detailed laboratory and immunological data before and after ovarian extirpation with resolution of hyperthyroidism associated with functional struma ovarii.

Hyperthyroidism

Measuring specific antibodies, such as anti-TSH-receptor antibodies in Graves' disease, or anti-thyroid peroxidase in Hashimoto's thyroiditis—a common cause of hypothyroidism—may also contribute to the diagnosis. The diagnosis of hyperthyroidism is confirmed by blood tests that show a decreased thyroid-stimulating hormone (TSH) level and elevated T4 and T3 levels. TSH is a hormone made by the pituitary gland in the brain that tells the thyroid gland how much hormone to make. When there is too much thyroid hormone, the TSH will be low. A radioactive iodine uptake test and thyroid scan together characterizes or enables radiologists and doctors to determine the cause of hyperthyroidism. The uptake test uses radioactive iodine injected or taken orally on an empty stomach to measure the amount of iodine absorbed by the thyroid gland. Persons with hyperthyroidism absorb much more iodine than healthy persons which includes radioactive iodine which is easy to measure. A thyroid scan

Thyroid

An examination of the thyroid will also include observation of the person as a whole, to look for systemic signs such as weight gain or loss, hair loss, and signs in other locations – such as protrusion of the eyes or swelling of the calves in Graves' disease. Tests Thyroid function tests include a battery of blood tests, including the measurement of the thyroid hormones, as well as the measurement of thyroid stimulating hormone (TSH). They may reveal hyperthyroidism (high T3 and T4), hypothyroidism (low T3, T4), or subclinical hyperthyroidism (normal T3 and T4 with a low TSH).

Expression of benign and malignant thyroid tissue in ovarian teratomas and the importance of multimodal management as illustrated by a BRAF-positive follicular variant of papillary thyroid cancer.

The most common type of ovarian germ cell tumor is the teratoma. Thyroid tissue, both benign and malignant, may be a component of an ovarian teratoma. Here we review this topic and illustrate major features by presenting multimodal management of a patient with BRAF-positive disseminated follicular thyroid cancer arising in an ovarian teratoma. Malignant thyroid tissue is often difficult to distinguish from benign thyroid tissue arising in ovarian teratomas. Preoperatively, an elevated thyroglobulin (Tg) level, laboratory or clinical evidence of hyperthyroidism, or ultrasonography appearance of "struma pearl" should prompt referral to oncologist for surgical management of a possibly malignant ovarian teratoma. Postoperatively, tumor tissue should be referred to pathologists experienced with differentiating benign from malignant struma ovarii. Once diagnosed, treatment of this rare condition should be handled by a team of specialists with combined treatment modalities. We cared for woman with disseminated thyroid cancer arising in an ovarian teratoma whose history illustrates the complexity of managing ovarian teratomas with malignant thyroid tissue. At age 33 she had an intraoperative rupture of an ovarian cyst, thought to be struma ovarii. During her next pregnancy, pelvic masses were noted; biopsies revealed well-differentiated papillary thyroid carcinoma, follicular variant. She was euthyroid, but had elevated serum Tg levels. Surgical staging demonstrated widely metastatic intraabdominal dissemination. A thyroidectomy revealed no malignancy. A post-(131)I treatment scan revealed diffuse uptake throughout the abdomen. She then developed abdominal pain and, on computed tomography, was found to have multiple intraabdominal foci of disease. Serum Tg was 264 ng/mL while on L-thyroxine for hypothyroidism and to obtain thyrotropin suppression. A 18 fluorodeoxyglucose positron emission tomography scan showed no pathological uptake. The tumor was found to be BRAF mutation positive (K601E). She underwent extensive secondary debulking and a second course of (131)I with lithium pretreatment. Posttreatment scan revealed diffuse abdominal uptake. Six months posttherapy, the patient is asymptomatic with a serum Tg of 18.1 ng/mL. Aggressive multimodal management appears to be the most promising approach for malignant thyroid tissue arising in ovarian teratomas.

Malignant Struma Ovarii with Concurrent Thyroid Cancer: Outcomes during and after Pregnancy.

Struma ovarii (SO) is a rare ovarian teratoma characterized by the presence of thyroid tissue in more than 50% of the tumor. Malignant transformation is rare and the most common associated malignancy is papillary thyroid carcinoma (PTC). Pregnancy may represent a stimulus to differentiated thyroid cancer (DTC) growth in patients with known structural or biochemical evidence of disease, but data about malignant SO evolution during pregnancy are rare. We present the first reported case of a pregnant patient with malignant SO and biochemical evidence of disease. A previously healthy 35-year-old female diagnosed with a suspicious left pelvic mass on routine ultrasound was submitted to laparoscopic oophorectomy which revealed a malignant SO with areas of PTC. A 15-mm thyroid nodule (Bethesda V in the fine-needle aspiration cytology) was detected by palpation and total thyroidectomy was performed. Histology revealed a 15 mm follicular variant of PTC (T1bNxMx). Subsequently, she received 100 mCi of radioactive iodine therapy (RAIT) with the whole-body scan showing only moderate neck uptake. Her suppressed thyroglobulin (Tg) before RAI was 1.1 ng/mL. She maintained biochemical evidence of disease, with serum Tg levels of 7.6 ng/mL. She got pregnant 14 months after RAIT, and during pregnancy, Tg increased to 21.5 ng/mL. After delivery, Tg decreased to 14 ng/mL but, 6 months later, rose again and reached 31.9 ng/mL on the last follow-up visit. TSH was always suppressed during follow-up. At the time of SO diagnosis, a chest computed tomography scan showed 4 bilateral lung micronodules in the upper lobes which were nonspecific, and 9 months after diagnosis, a pelvic MRI revealed a suspicious cystic nodule located on the oophorectomy bed. These lung and pelvic nodules remained stable during follow-up. Neck ultrasonography, abdominal MRI, and fluorodeoxyglucose-positron emission tomography showed no suspicious lesions. As for DTC, pregnancy seems to represent a stimulus to malignant SO growth. This can be caused by the high levels of estrogen during pregnancy that may bind to receptors in malignant cells and/or by the high levels of hCG which is known to stimulate TSH receptors.

Hyperthyroidism

Graves' disease is the cause of about 50% to 80% of the cases of hyperthyroidism in the United States. Other causes include multinodular goiter, toxic adenoma, inflammation of the thyroid, eating too much iodine, and too much synthetic thyroid hormone. A less common cause is a pituitary adenoma. The diagnosis may be suspected based on signs and symptoms and then confirmed with blood tests. Typically blood tests show a low thyroid stimulating hormone (TSH) and raised T3 or T4. Radioiodine uptake by the thyroid, thyroid scan, and TSI antibodies may help determine the cause.

Hashimoto's thyroiditis

Given the relatively nonspecific symptoms of initial hypothyroidism, Hashimoto's thyroiditis is often misdiagnosed as depression, cyclothymia, premenstrual syndrome, chronic fatigue syndrome, fibromyalgia, and less frequently, as erectile dysfunction or an anxiety disorder. On gross examination, a hard goiter that is not painful to the touch often presents; other symptoms seen with hypothyroidism, such as periorbital myxedema, depend on the current state of progression of the response, especially given the usually gradual development of clinically relevant hypothyroidism. Testing for thyroid-stimulating hormone (TSH), free T3, free T4, and the antithyroglobulin antibodies (anti-Tg), antithyroid peroxidase antibodies (anti-TPO, or TPOAb) and antimicrosomal antibodies can help obtain an accurate diagnosis. Earlier assessment of the person may present with elevated levels of thyroglobulin owing to transient thyrotoxicosis, as inflammation within the thyroid causes damage to the integrity

InternalMed_Harrison

Laboratory Evaluation As depicted in Fig. 405-10, thyroid function tests characteristically evolve through three distinct phases over about 6 months: (1) thyrotoxic phase, (2) hypothyroid phase, and (3) recovery phase. In the thyrotoxic phase, T4 and T3 levels are increased, reflecting their discharge from the damaged thyroid cells, and TSH is suppressed. The T4/T3 ratio is greater than in Graves’ disease or thyroid autonomy, in which T3 is often disproportionately increased. The diagnosis is confirmed by a high ESR and low uptake of radioiodine (<5%) or 99mTc pertechnetate (as compared to salivary gland pertechnetate concentration). The white blood cell count may be increased, and thyroid antibodies are negative. If the diagnosis is in doubt, FNA biopsy may be useful, particularly to distinguish unilateral involvement from bleeding into a cyst or neoplasm.

Strumal Carcinoid Tumor of the Ovary: Report of Rare Occurrence with Review of Literature.

The primary ovarian carcinoid tumor is a very rare ovarian tumor, which accounts for approximately 0.5% to 1.7% of all carcinoids and 1% of ovarian cancer. According to its histopathological features, it can be divided into four categories: insular, trabecular, strumal, and mucinous, among which insular carcinoid is common in Western countries. By comparison, the chain-typed and trabecular carcinoid seem to be common in Asian countries. To date, about 150 cases have been reported in the world, and 40% of them are strumal carcinoid tumor of the ovary (SCTO), which is a highly specialized teratoma differentiated from the monomer, and often characterized by the coexistence of thyroid follicular tissue and carcinoid tissue with the neuroendocrine function. Preoperative diagnosis may be difficult due to the very insidious nature of the disease and its multiple imaging manifestations. We reported the case of a 39-year-old woman with a 5-year clinical history. Gynecologic examination and ultrasonic testing revealed an enlarged ovary with a diameter of about 60 mm, accompanied by a hypoechoic area, which was suspected to be a benign teratoma. Ca-125, AFP, free T4, TSH, and other diagnostic indicators were normal. During the laparoscopic oophorocystectomy of the left ovary, a smooth and solid tumor with the size of 6 × 6 × 5 cm was found in the right ovary. During the operation, a mature cystic teratoma containing a struma was frozen, then the oophorocystectomy of the left ovary was performed. According to the Federation International of Gynecology and Obstetrics (FIGO) in 2014, histopathological examination showed a mature teratoma with thyroid carcinoid stage Ic, and Douglas's cystic hygroma cytopathology was negative. One year after the operation, the patient was tumor-free, with Ca-125, FT4, and TSH being within the normal range. Specific diagnostic tools and serological monitoring of malignant tumors of the ovary have low specificity and sensitivity in the diagnosis of this rare malignant tumor of the ovary. Female patients with habitual constipation, chronic abdominal colic, diarrhea, and endocrine dysfunction also need to be alert to this rare malignant tumor of the ovary.

I-123 Uptake -- Normal and Critical Findings -- Thyrotoxicosis of Extrathyroidal Origin

There are multiple sources of extrathyroidal thyroid hormone, including exogenous thyroid hormone ingestion seen in factitious hyperthyroidism, thyroid hormone-producing metastatic thyroid cancer, and struma ovarii, a teratomatous ovarian mass that contains functional thyroid tissue. Another extrathyroidal cause of thyrotoxicosis includes TSH-induced thyrotoxicosis, which is caused by an autonomous TSH secreting pituitary adenoma, the only extrathyroidal cause associated with an elevated RAIU. Clinically, patients can have varying levels of thyrotoxicosis, depending on the level of excretion the exogenous source releases. Factitious hyperthyroidism can often be difficult to diagnose as the patients rarely disclose exogenous thyroid ingestion. Oftentimes the ingested thyroid hormone is T4, which may yield a higher than normal T4 to T3 ratio. Thyroid hormone production from metastatic thyroid malignancy is not very common as it requires a well-differentiated metastatic malignancy, which is usually less efficient at producing and excreting thyroid hormone. These metastases are usually seen in the setting of known thyroid cancer on I-131 imaging. Similar to metastasis, struma ovarii does not efficiently excrete thyroid hormone and rarely produces significant amounts of thyroid hormone. Struma ovarii is often found incidentally when pathology of an ovarian mass returns with thyroid tissue. RAIU of the overall thyroid gland in the neck is decreased, while the lesion may have elevated RAIU. [11]

InternalMed_Harrison

Primary thyrotoxicosis Features of Graves’ diseasea? T3 toxicosis Subclinical hyperthyroidism TSH low, unbound T4 high Measure TSH, unbound T4 Normal Measure unbound T3 TSH normal or increased, high unbound T4 TSH-secreting pituitary adenoma or thyroid hormone resistance syndrome Follow up in 6-12 weeks High TSH low, unbound T4 normal TSH and unbound T4 normal No further tests Graves’ disease Multinodular goiter or toxic adenomab? Yes No Toxic nodular hyperthyroidism Low radionuclide uptake? Yes No Destructive thyroiditis, iodine excess or excess thyroid hormone Rule out other causes including stimulation by chorionic gonadotropin

Thyroiditis

Diagnosis The most common and helpful way to diagnose thyroiditis is first for a physician to palpate the thyroid gland during a physical examination. Laboratory tests allow doctors to evaluate the patient for elevated erythrocyte sedimentation rates, elevated thyroglobulin levels, and depressed radioactive iodine uptake (Mather, 2007). Blood tests also help to determine the kind of thyroiditis and to see how much thyroid stimulating hormone the pituitary gland is producing and what antibodies are present in the body. In some cases, a biopsy may be needed to find out what is attacking the thyroid. Classification Thyroiditis is a group of disorders that all cause thyroidal inflammation. Forms of the disease are Hashimoto's thyroiditis, the most common cause of hypothyroidism in the US, postpartum thyroiditis, subacute thyroiditis, silent thyroiditis, drug-induced thyroiditis, radiation-induced thyroiditis, acute thyroiditis, and Riedel's thyroiditis.

InternalMed_Harrison

Differential Diagnosis Diagnosis of Graves’ disease is straightforward in a patient with biochemically confirmed thyrotoxicosis, diffuse goiter on palpation, ophthalmopathy, and often a personal or family history of autoimmune disorders. For patients with thyrotoxicosis who lack these features, the diagnosis is generally established by a radionuclide (99mTc, 123I, or 131I) scan and uptake of the thyroid, which will distinguish the diffuse, high uptake of Graves’ disease from destructive thyroiditis, ectopic thyroid tissue, and factitious thyrotoxicosis. Scintigraphy is the preferred diagnostic test; however, TRAb measurement can be used to assess autoimmune activity. In secondary hyperthyroidism due to a 2296 TSH-secreting pituitary tumor, there is also a diffuse goiter. The presence of a nonsuppressed TSH level and the finding of a pituitary tumor on CT or magnetic resonance scan (MRI) scan suggest this diagnosis. Clinical features of thyrotoxicosis can mimic certain aspects of other

Thyroid disease

In some types, such as subacute thyroiditis or postpartum thyroiditis, symptoms may go away after a few months and laboratory tests may return to normal. However most types of thyroid disease do not resolve on their own. Common hypothyroid symptoms include fatigue, low energy, weight gain, inability to tolerate the cold, slow heart rate, dry skin and constipation. Common hyperthyroid symptoms include irritability, anxiety, weight loss, fast heartbeat, inability to tolerate the heat, diarrhea, and enlargement of the thyroid. Structural abnormalities may not produce symptoms, however some people may have hyperthyroid or hypothyroid symptoms related to the structural abnormality or notice swelling of the neck. Rarely goiters can cause compression of the airway, compression of the vessels in the neck, or difficulty swallowing. Tumors, often called thyroid nodules, can also have many different symptoms ranging from hyperthyroidism to hypothyroidism to swelling in the neck and compression

Gynecology_Novak

Diffuse toxic goiter (Grave’s disease) is the most common cause of hyperthyroidism and results from abnormal stimulation of the thyroid gland by antithyroid antibodies. Other causes of hyperthyroidism include multinodular goiter, excess thyroid hormone, or thyroiditis. Any signs or symptoms suggestive of weight loss, tachycardia, atrial fibrillation, goiter, or proptosis should initiate a more extensive laboratory evaluation of thyroid function. Total thyroxin, free triiodothyronine (T3), free thyroxin (T4), and thyroid-stimulating hormone (TSH) tests are useful in diagnosis. In hyperthyroidism, the free T4 level will be elevated, and the TSH level will be suppressed (154). A new diagnosis of hyperthyroidism necessitates postponement of elective surgery until adequate treatment with antithyroid medication is received because of the risk of thyroid storm. Thyroid storm is associated with mortality of up to 40% (168). Stable thyroid conditions do not require any special preoperative

Hyperthyroidism

Hypersecretion of thyroid stimulating hormone (TSH), which in turn is almost always caused by a pituitary adenoma, accounts for much less than 1 percent of hyperthyroidism cases. Diagnosis Measuring the level of thyroid-stimulating hormone (TSH), produced by the pituitary gland (which in turn is also regulated by the hypothalamus's TSH Releasing Hormone) in the blood is typically the initial test for suspected hyperthyroidism. A low TSH level typically indicates that the pituitary gland is being inhibited or "instructed" by the brain to cut back on stimulating the thyroid gland, having sensed increased levels of T4 and/or T3 in the blood. In rare circumstances, a low TSH indicates primary failure of the pituitary, or temporary inhibition of the pituitary due to another illness (euthyroid sick syndrome) and so checking the T4 and T3 is still clinically useful.

Gynecology_Novak

Thyroid-Stimulating Receptor Antibody in Graves Disease The level of TSHRAb of the TBII class grossly parallels the degree of hyperthyroidism as assessed by the serum levels of thyroid hormones and total thyroid volume. Studies suggest that the combination of a small goiter volume (<40 mL) and a low TBII level (<30 U/L) results in a 45% chance of remission during the 5 years after completion of a 12-to 24-month course of antithyroid drug therapy (397). In contrast, the overall rate of relapse exceeded 70% in patients with a large goiter volume (>70 mL) and a higher TBII level (>30 U/L). The subgroup of patients with larger goiters and higher TBII levels had less than a 10% chance to remain in remission in the 5 years after treatment. Although it is not necessary for the diagnosis of Graves disease, except in some cases of multinodular goiter, a TSHRAb measurement may be a useful marker of disease severity. Used in combination with other clinical factors, it may contribute to initial

Graves' disease

Another sign of Graves' disease is hyperthyroidism; that is, overproduction of the thyroid hormones T3 and T4. Normal thyroid levels are also seen, and occasionally also hypothyroidism, which may assist in causing goiter (though it is not the cause of the Graves' disease). Hyperthyroidism in Graves' disease is confirmed, as with any other cause of hyperthyroidism, by measuring elevated blood levels of free (unbound) T3 and T4. Other useful laboratory measurements in Graves' disease include thyroid-stimulating hormone (TSH, usually undetectable in Graves' disease due to negative feedback from the elevated T3 and T4), and protein-bound iodine (elevated). Serologically detected thyroid-stimulating antibodies, radioactive iodine (RAI) uptake, or thyroid ultrasound with Doppler all can independently confirm a diagnosis of Graves' disease. Biopsy to obtain histiological testing is not normally required, but may be obtained if thyroidectomy is performed.

Gynecology_Novak

Because most disorders of hyperthyroidism and hypothyroidism are related to dysfunction of the thyroid gland and TSH levels are sensitive to excessive or deficient levels of circulating thyroid hormone, TSH levels are used to screen for these disorders. Current thyrotropin or TSH sandwich immunoassays are extremely sensitive and capable of differentiating low-normal from pathologic or iatrogenically subnormal values and elevations. TSH measurements provide the best way to screen for thyroid dysfunction and accurately predict thyroid hormone dysfunction in about 80% of cases (343). Reference values for TSH are traditionally based on the central 95% of values for healthy individuals, and some controversy exists regarding the upper limit of normal. Values in the upper limit of normal may predict future thyroid disease (342,344). In a longitudinal study, women with positive thyroid antibodies (TPOAbs or TgAbs), the prevalence of hypothyroidism at follow-up was 12.0% (3.0% to 21.0%; 95%

Thyrotoxicosis factitia

Patients present with hyperthyroidism and may be mistaken for Graves’ disease, if TSH receptor positive, or thyroiditis because of absent uptake on a thyroid radionuclide uptake scan due to suppression of thyroid function by exogenous thyroid hormones. Ingestion of thyroid hormone also suppresses thyroglobulin levels helping to differentiate thyrotoxicosis factitia from other causes of hyperthyroidism, in which serum thyroglobulin is elevated. Caution, however, should be exercised in interpreting thyroglobulin results without thyroglobulin antibodies, since thyroglobulin antibodies commonly interfere in thyroglobulin immunoassays causing false positive and negative results which may lead to clinical misdirection. In such cases, increased faecal thyroxine levels in thyrotoxicosis factitia may help differentiate it from other causes of hyperthyroidism. See also Foodborne illness Liothyronine References External links Thyroid disease Toxicology

InternalMed_Harrison

nonsuppressed TSH level and the finding of a pituitary tumor on CT or magnetic resonance scan (MRI) scan suggest this diagnosis. Clinical features of thyrotoxicosis can mimic certain aspects of other disorders, including panic attacks, mania, pheochromocytoma, and weight loss associated with malignancy. The diagnosis of thyrotoxicosis can be easily excluded if the TSH and unbound T4 and T3 levels are normal. A normal TSH also excludes Graves’ disease as a cause of diffuse goiter.

Thyroid-stimulating hormone

TSH concentrations in children are normally higher than in adults. In 2002, the NACB recommended age-related reference limits starting from about 1.3 to 19 µIU/mL for normal-term infants at birth, dropping to 0.6–10 µIU/mL at 10 weeks old, 0.4–7.0 µIU/mL at 14 months and gradually dropping during childhood and puberty to adult levels, 0.3–3.0 µIU/mL. Diagnosis of disease TSH concentrations are measured as part of a thyroid function test in patients suspected of having an excess (hyperthyroidism) or deficiency (hypothyroidism) of thyroid hormones. Interpretation of the results depends on both the TSH and T4 concentrations. In some situations measurement of T3 may also be useful. A TSH assay is now also the recommended screening tool for thyroid disease. Recent advances in increasing the sensitivity of the TSH assay make it a better screening tool than free T4. Monitoring The therapeutic target range TSH level for patients on treatment ranges between 0.3 and 3.0 μIU/mL.

A case of simultaneous occurrence of Graves' disease and Hashimoto's thyroiditis.

Simultaneous occurrence of Hashimoto's thyroiditis (HT), and Graves' disease (GD) is rare. We report a case of simultaneous occurrence of GD and HD, at presentation. A 60-year-old lady presented with tremulousness of hands, palpitation, and excessive sweating. She had a history of weight loss and neck-swelling. Her weight was 46 kg, BMI 17, afebrile, regular pulse rate of 110/min with fine tremor in hands. Thyroid gland was symmetrically enlarged, firm, without any bruit, but mildly tender with lobular surface. There were no occular manifestations. Initial thyroid function tests (TFT) revealed: T3: 3.80 ng/ml (0.80-2.10), T4: 12.40 ug/dl (5.10-12), thyroid stimulating hormone (TSH): 0.20 μU/L (0.70-5). Her anti thyroperoxidase (TPO) antibody: 374 IU/ml (normal [nl.] <35) and TSH receptor antibody: 15 U/L (nl. <1) were both strongly positive. Ultrasonogram of thyroid revealed a hypoechoic enlarged gland. 99mTc pertechnetate scan showed an enlarged gland with increased uptake of radiocontrast: 17% (nl. 0.4-4%) with some patchy defects in both lower poles. Thyroid fine needle aspiration cytology (FNAC) showed sheets of Hurthle cells with abdunce of lymphocytes indicating HT. She was observed on beta blockers. Repeat TFT, 3 months later showed: T3: 4.20 ng/ml, T4: 14.40 ug/dl, TSH: 0.001 μU/L, with increased uptake on repeat scan. HT rarely occurs following GD. Our case of an elderly lady with no eye signs, lobular, firm tender goiter with patchy uptake in both lower poles on Tc99m scan were odd points in diagnosing isolated GD. FNAC confirmed simultaneous HD with GD.

[A case of ovarian struma causing symptoms of hyperthyroidism].

The authors report the case of a 46-year old woman, who went with diffuse complaints to the endocrinology department. The laboratory date showed hyperthyroidism. She had hypogastric complaints, therefore a gynecological consultation was carried out. An infant--head sized elastic formation was found on the left hand side next to a female fist sized myomatous uterus. On sonographic examination the growth proved to have a size of 12 x 14 cm. Besides the myomatous uterus the histology of the operative specimen confirmed a left sided ovarious teratoma, which was composed solely of usual thyroid tissue. After the operation it the patient's endocrine disorders became spontaneously regulated. Our attention to called the fact that especially in case of therapy resistant hyperthyroidism we should think of this infrequent clinical picture.

[An ovarian tumor can hide another one].

We report the case of a 33-year-old woman who went under surgery for a cystic mature teratoma. The histological exam found two cysts, one was a mature teratoma and the other was a struma ovarii with a papillary carcinomatous element. Struma ovarii cancerization is seen in 5 to 10% of the cases usually under a papillary carcinoma type. Diagnosis is rarely made before surgery, the patients exceptionally show thyroid symptoms. Histologically, the tumour presents the same way as the one seen in the thyroid gland and BRAF mutations have been reported. The problem concerns ovarian metastases of a thyroid cancer. A normal thyroid check up and normal thyroid tissue close to the tumor in the ovary are in favor for a cancerize struma ovarii. The therapeutic care is not consensual, going from an annexectomy to hysterectomy and bilateral annexectomy. The patients must be followed on long-term with thyroglobulin quantitative analysis for at least 10 years and whole body scintigraphy with iodine 123 to detect relapse or metastases. The prognosis is usually good but precise criteria are still to define.


They describe a typical hemisection or Brown-Sequard picture. Ipsilateral posterior motor and chordal involvement with contralateral pain and temperature involvement.

A man presents on neurological examination with a thermoalgesic sensory deficit in the left leg associated with a loss of vibratory and positional sensitivity in the right leg. At the same time he presents with clumsiness and loss of distal strength in the right leg and a right plantar cutaneous reflex in extension. Which of the following statements is correct:

It is a centromedullary syndrome type syringomyelia.

It is a pattern of transverse medullary lesion.

It is a pattern of lateral bulbar lesion.

[Medial medullary infarction: report of three patients presented with central vestibular dysfunction without limb and lingual weakness].

The purpose of this article is to draw attention to atypical presentation of medial medullary infarction (MMI). With advanced imaging techniques, small infarctions occurring in the medulla are more easily identified. It is difficult, however, to make a clinical diagnosis of MMI if both hypoglossal nerve palsy and limb weakness are absent, because motor weakness is considered a cardinal manifestation of MMI. We describe here three patients who developed central vestibular dysfunction due to MMI without limb and lingual weakness. Case 1: A 44-year-old, diabetic woman developed vomiting and numbness on her left upper limb. Examination revealed unidirectional horizontal and rotatory nystagmus beating toward the right side. There were no Horner syndrome and hypoglossal nerve palsy. Barré arm and leg signs and limb ataxia were absent. Romberg sign was negative. Hypesthesia was present on her left forearm, hand, and fingers. Thumb-localizing test was normal. Cranial MRI demonstrated an infarction in the right paramedian region of the upper medulla. MR angiography demonstrated irregularity of the basilar and the left vertebral arteries. Case 2: A 69-year-old woman suffered from dizziness and nausea. She showed unidirectional, left-beating horizontal nystagmus. There were no Horner syndrome and hypoglossal nerve palsy, Barré arm and leg signs, and limb ataxia. MRI disclosed an infarction in the left upper medial medulla. Case 3: A 47-year-old man developed vertigo when turning over in bed. He showed left-beating nystagmus without latency, when lying down. Horner syndrome and hypoglossal nerve palsy were absent MRI showed bilateral MMI, with the right lesion being larger than the left. MR angiography demonstrated a stenosis in the distal portion of the left internal carotid artery but not in the vertebral and basilar arteries and their branches. This case represents central positional vertigo. Vestibular syndrome seen in cases 1 and 2 was incomplete and incongruent, suggesting dysfunction of the central vestibular system. There have been only nine cases of MMI with horizontal nystagmus in primary position, including unidirectional horizontorotatory nystagmus. In these cases, horizontal nystagmus beats toward the side of the lesion. In sharp contrast, horizontal nystagmus typically beats away from the lesion side in cases of Wallenberg syndrome, suggesting different underlying mechanism. Unidirectional horizontal and rotatory nystagmus is generally associated with peripheral vestibular dysfunction. There has been no reported case of MMI presenting with vestibular dysfunction preserving motor power. Thus, this "benign" form of MMI might have been misdiagnosed as peripheral vestibular dysfunction before the era of MRI.

[Sensory disturbance of crossed oral-pedal topography in a case of lateral medullary infarction].

We report a 40-year-old man with hypertension and diabetes mellitus, who had crossed oral-pedal sensory disturbance in lateral medullary infarction. He suddenly developed dysesthesia in the right mount and the left leg. His blood pressure was 150/90 mmHg. Neurological examination showed Horner's sign in the right eye and horizontal nystagmus. Sensory function revealed decreased temperature, hypalgesia and dysesthesia in the right mouth and the left leg. Vibratory and position sense were normal. T1- and T2-weighted images disclosed a low and high signal intensity area in the lateral portion of the right medulla oblongata, respectively. Brain and neck MRA using time-of-flight sequence revealed no obvious abnormal structures. We have diagnosed him as lateral medullary infarction. The unique topography of sensory dysfunction thought to be attributed to a far-lateral lesion in the medulla oblongata. Our patient suggests that lateral medullary infarction causes variable patterns of sensory disturbance. Thus, lateral medullary infarction should be warranted when we encounter patients with miscellaneous distribution of sensory impairment, such as crossed mouth-foot hypalgesia.

Lateral medullary syndrome

Lateral medullary syndrome is a neurological disorder causing a range of symptoms due to ischemia in the lateral part of the medulla oblongata in the brainstem. The ischemia is a result of a blockage most commonly in the vertebral artery or the posterior inferior cerebellar artery. Lateral medullary syndrome is also called Wallenberg's syndrome, posterior inferior cerebellar artery (PICA) syndrome and vertebral artery syndrome. Signs and symptoms This syndrome is characterized by sensory deficits that affect the trunk and extremities contralaterally (opposite to the lesion), and sensory deficits of the face and cranial nerves ipsilaterally (same side as the lesion). Specifically a loss of pain and temperature sensation if the lateral spinothalamic tract is involved. The cross body finding is the chief symptom from which a diagnosis can be made.

InternalMed_Harrison

Signs and symptoms: Structures involved 1. Medial medullary syndrome (occlusion of vertebral artery or of branch of vertebral or lower basilar artery) On side of lesion Paralysis with atrophy of one-half half the tongue: Ipsilateral twelfth nerve On side opposite lesion Paralysis of arm and leg, sparing face; impaired tactile and proprioceptive sense over one-half the body: Contralateral pyramidal tract and medial lemniscus 2. Lateral medullary syndrome (occlusion of any of five vessels may be responsible—vertebral, posterior inferior cerebellar, superior, middle, or inferior lateral medullary arteries) On side of lesion

Brown-Séquard syndrome

Pure Brown-Séquard syndrome is associated with the following: Interruption of the lateral corticospinal tracts: Ipsilateral spastic paralysis below the level of the lesion Babinski sign ipsilateral to lesion Abnormal reflexes and Babinski sign may not be present in acute injury Interruption of posterior white column: Ipsilateral loss of tactile discrimination, vibratory, and position sensation below the level of the lesion Interruption of lateral spinothalamic tracts: Contralateral loss of pain and temperature sensation. This usually occurs 2–3 segments below the level of the lesion. Treatment Treatment is directed at the pathology causing the paralysis. If the syndrome is caused by a spinal fracture, this should be identified and treated appropriately. Although steroids may be used to decrease cord swelling and inflammation, the usual therapy for spinal cord injury is expectant.

[Intermediate medullary infarction: a case report].

A 68-year-old man presented with right eye pain and vertigo. Thereafter, he gradually leaned rightward, then laid down. He felt nausea and vomited. His right upper eyelid drooped and he felt dysethesia of the right hand. On neurological examination, ptosis of his right eye with slightly miotic right pupil, paresis of the right soft palate and hoarseness were noted. Arm deviation test demonstrated rightward deviation. He presented sensory ataxia of the right upper and lower extremities: finger nose test showed mild dysmetria of the right upper extremity, heel knee test demonstrated dysmetria of right lower extremity and these findings worsened when he closed his eyes. He showed mild bending of his bilateral ring and little fingers when he did rapid alternative movement. He leaned rightward when he sat and closed his eyes. Position sense of his right upper and lower extremities was decreased and sometimes he could not answer correctly when asked on which direction his finger pointed. Pinprick sensation was mildly decreased on the left side not including the face. Touch and vibration sense were normal. SEP findings on upper and lower extremity stimulation were normal. MRI of the brain showed T2 high intensity and partially T1 low intensity lesion at the right medulla (Figure). MR angiography showed no apparent lesion of major arteries such as dissection of the vertebral arteries. He complained and presented with hiccup initially. On MRI, the lesion was thought to involve the spinothalamic tract, medial lemniscus and inferior olivary nucleus. Ambiguus nucleus was in the lesion and solitary nucleus near the lesion. There is no report that seems to describe clinical features of a lesion like that in this case. Intermediate medullary infarction may present dissociated sensory disturbance like Brown-Sequard syndrome and position sensory disturbance without disturbance of vibration sense.

[Subacute myeloneuropathy after abuse of nitrous oxide: an electron microscopic study on the peripheral nerve].

We have experienced a case of myeloneuropathy following habitual abuse of nitrous oxide. We report clinical and pathological findings of this case with review of literatures. A 36-year-old dentist was first admitted to our hospital on August 17, 1983 because of numbness of both lower legs and unsteady gait. He had recreationally inhaled nitrous oxide 30 to 60 minutes everyday since a year ago. Neurological examination showed ataxic broad-based gait, moderate loss of pain and touch sensation in the lower limbs up to patella. Position and vibratory senses were more severely impaired. Deep tendon reflexes increased in the upper extremities and at the knees, but diminished at ankle jerks. Muscle strength was normal. Prominent Lhermitte's sign was present. Except for reduced serum vitamin B12 level, laboratory results were normal. Needle electromyography showed high amplitude and long duration NMU in the right quadriceps and anterior tibial muscles. Motor nerve conduction velocity was 34 m/sec at the right posterior tibial nerve and could not be detected at the left. Sural nerve biopsy was performed. The density of the myelinated fibre measured on transverse section was in normal range but degenerated fibres were occasionally recognized. Single teased nerve fibre showed various degree of myelin ovoid along the fibre. Myelin loss was shown to occur in some parts of the fibre. Electron microscopy showed myelin splitting and formation of intramyelinic vacuoles containing myelin debris. Axon was almost normal at least in the early stage of degeneration. Later, axon disappeared with destruction of myelin sheath. These nerve changes largely demonstrated demyelination but it was occasionally accompanied with axonal degeneration.(ABSTRACT TRUNCATED AT 250 WORDS)

Neurology_Adams

be more rapid or more leisurely. The initial disturbance may be of motor or sensory function and the distribution may be asymmetrical. High cervical or foramen magnum lesions produce special clinical syndromes, as described in Chap. 3 and below. With thoracic lesions, one leg usually becomes weak and stiff before the other one. Subjective sensory symptoms of the dorsal column type (tingling paresthesias) assume similar distributions. Pain and thermal senses are more likely to be affected than tactile, vibration, and position senses. Nevertheless, the posterior columns are frequently involved as the process progresses. Initially, the sensory disturbance is contralateral to the maximum motor weakness, but a sharply defined Brown-Séquard hemicord syndrome is rarely observed. The bladder and bowel usually become paralyzed coincident with paralysis of the legs. If the compression is relieved, there is recovery from these sensory and motor symptoms, often in the reverse order of their

Syringo-subarachnoidal shunting to correct unilateral leg deformity in a child with terminal syringomyelia: case report.

Among cases with terminal syringomyelia, 25% are associated with tethered cord syndrome. As it can be difficult to determine whether the neurological deficits are attributable to a syrinx or to a coexisting occult spinal dysraphism, it is not easy to determine the correct surgical strategy. We report a 19-month-old girl with an underdeveloped right leg and pes varus detected when she was 1 month old; lumbosacral magnetic resonance imaging (MRI) revealed syringomyelia. She developed recurrent urinary tract infections and consulted our department with a diagnosis of congenital neurogenic bladder. She presented with clubbed equine position, wore a short brace on her underdeveloped right leg, and exhibited limping gait due to shortening of the right leg. There was no anal reflex. The skin on her lower back was normal. MRI study showed that the lower end of the conus medullaris existed at the L3/4 level; central-type syringomyelia was recognized in the conus medullaris at the T12/L1-L2/L3 level. Computed tomography myelography detected no trabeculae causing tethering effects or influx of contrast medium into the syrinx. There was no comorbid disease like hydrocephalus or Chiari malformation. We performed syringo-subarachnoidal shunt by L1-L2 hemilaminectomy. Postoperative MRI confirmed shrinkage of the syrinx. One year later, although her perianal sensory loss and bowel and bladder dysfunction remained, her right leg had caught up with the left and at 1 year and 9 months after the procedure there is no discrepancy in her legs and she is able to run without limping.

Tibialis anterior R-1 response: physiological behaviour, normative data and clinical utility in L4-L5 radicular compression.

A modified H-reflex, known as R-1 response, was recorded in healthy subjects by averaging 200 responses from the moderately contracting tibialis anterior muscle (TA) after repetitive submaximal stimulation of common peroneal nerve. Physiological changes to different modes of stimulation and recording were studied and normative data obtained at the most reliable parameters. Subsequently, the test is applied to 36 patients with clinical and myelographic evidences of L5 or L4-L5 radicular compression (with or without S1 radicular compression). Increasing the force of muscle contraction resulted in increase in amplitude up to a certain limit, with no change in latency. Increasing the current from subliminal to submaximal stimulation caused initial increase and subsequent fall in the amplitude without change in the latency. The change in the stimulus frequency from 0.5 to 3 Hz did not alter latency or amplitude but the higher frequencies sometimes did. On changing the position of the foot from plantarflexed to dorsiflexed, an increase in amplitude and reduction in duration of the response was noted. Fifty subjects were studied using stimulus intensity just enough to produce 200-400 microV M-response, stimulus duration of 1.0 msec, stimulus rate of 3 Hz and the muscle force sufficient to keep the foot in complete dorsiflexion. The tibialis anterior R-1 response (TAR-1) could be recorded reliably from both legs in all the subjects. The take-off point was sharp and in no subject was there any difficulty in measuring the latency to onset point. On repeating the test, the onset point of the response was superimposable. Latency showed linear correlation with height and age. Among the patients, 21 out of 25 cases of unilateral (84.0%) and 10 out of 11 cases of bilateral (90.9%) radicular compression had abnormal findings. Abnormalities included significant a right-left latency difference within the normal range of absolute latencies, unilateral or bilateral prolongation of latency to onset point and attenuation or absence of the response. It was concluded that the TAR-1 is an important and reliable test for the study of functional integrity of L4-L5 spinal segments in lumbo-sacral sensorimotor root lesions.

Neurology_Adams

Fig. 10-3) and between the fourth and fifth lumbar vertebrae (compressing the L5 root). Lesions of the fifth lumbar root (L5) produce pain in the region of the hip and posterolateral thigh (i.e., sciatica) and, in more than half such cases, lateral calf (to the lateral malleolus), and less often, the dorsal surface of the foot and the first or second and third toes. Pain is elicited by the straight-leg raising test or one of its variants, and protective nocifensive reflexes come into play, limiting further elevation of the leg. Paresthesia may be felt in the entire territory or only in its distal parts. The tenderness is in the lateral gluteal region and near the head of the femur. Weakness, if present, involves the extensors of the big toe and foot and the foot invertors (a distinguishing feature of foot drop originating from peroneal nerve damage, which spares inversion because it is a tibial nerve function). The ankle jerk may be diminished (more often it is normal), but the knee

Trigeminal nerve

Clinical significance Trigeminal neuralgia Cluster headache Migraine Wallenberg syndrome Wallenberg syndrome (lateral medullary syndrome) is a clinical demonstration of the anatomy of the trigeminal nerve, summarizing how it processes sensory information. A stroke usually affects only one side of the body; loss of sensation due to a stroke will be lateralized to the right or the left side of the body. The only exceptions to this rule are certain spinal-cord lesions and the medullary syndromes, of which Wallenberg syndrome is the best-known example. In this syndrome, a stroke causes a loss of pain-temperature sensation from one side of the face and the other side of the body.

[A 64-year-old man with recurrent blurred vision and an abdominal mass].

We report a 64-year-old man with recurrent bouts of blurred vision who died after developing an abdominal mass. He was well until June of 1985 when he was 59-years-old when he had an acute onset of loss of vision in his right eye. He was treated by prednisolone with a complete remission. In August of 1986, he had another bout of blurring of vision in his left eye. Once he lost his left vision completely, from which he showed slow recovery. In January of 1987, he developed blurring of his right eye and loss of pain and touch sensation in his right leg. Since then he repeated loss of vision in his right or left eye five times, and he was admitted to our hospital in May of 1990. On admission, he was alert and oriented. General physical examination was unremarkable. Neurologic examination revealed bilateral optic nerve atrophy. He could not discriminate light or dark by either eye. Other cranial nerves were unremarkable. He could walk in a wide-base only with support; spasticity was noted in his left leg. Muscle strength was preserved. Deep reflexes were exaggerated in both legs with extensor plantar reflex bilaterally. Pain and touch sensation was decreased in the left leg by 30%, and vibration was diminished in both feet. Position sense was preserved. Routine blood counts and chemistries were unremarkable. Cranial MRI scans revealed multiple high-signal intensity lesions in both pontine bases, basal ganglia, thalami, and in the deep cerebral white matters. He was treated with oral prednisolone, plasmapheresis, lymphocytapheresis, and then immuran. His vision showed only slight recovery to discriminate light and dark. In October of 1990, slight weakness appeared in his both legs. In December of that year, he developed nausea, and a fiber colonoscopic study revealed a stenosis in the transverse colon. In March of 1991, he developed anemia and liver dysfunction. In July of that year, jaundice appeared, and his serum bilirubin was increased. In October, his leg weakness became more prominent, and his cranial CT scans at that time revealed a low density change in the right cerebellum in the right superior cerebellar artery territory; in addition, multiple low density spots were scattered to be seen in both cerebral hemispheres including the basal ganglia and thalamic areas with ventricular dilatation and cortical atrophy.(ABSTRACT TRUNCATED AT 400 WORDS)

Comparison of the electrically evoked leg withdrawal reflex in cerebellar patients and healthy controls.

The aim of this study was to analyze the contribution of the cerebellum in the performance of the lower limb withdrawal reflexes. This has been accomplished by comparing the electrically evoked responses in cerebellar patients (CBL) with those in sex- and age-matched healthy control subjects (CTRL). The stimulus was applied to the subjects' medial plantar nerve in four blocks of ten trials each with switching the stimulus from one leg to the other after each block. Responses of the main muscle groups (tibial muscle: TA; gastrocnemius muscle: GA; rectus femoris muscle: RF; biceps femoris muscle: BI) of both legs were recorded during each stimulus. The group of CBL patients consisted of both focally lesioned patients (CBLf) and patients presenting a diffuse degenerative pathology (CBLd). (1) For the withdrawal reflex in CTRL subjects, responses were observed in distal and proximal muscles of the ipsilateral side and corresponding concomitant responses on the side contralateral to the stimulation, whereas in CBL patients responses were restricted primarily to distal muscles, particularly the TA of the ipsilateral, i.e. the stimulated, side. (2) The sequence of activation of the different distal and proximal muscles ipsilateral to the stimulation, derived from latencies and times-to-peak, was for the CTRL group: TA-GA-BI-RF. This sequence was found also in the CBLf patients on their unaffected side. However, on their affected side CBLf patients showed very early GA activation, almost simultaneously with TA and RF activations and before BI activation. RF activation before BI activation was also found in CBLd. In the latter group, GA was activated after RF but before BI with all responses typically delayed. (3) The general pattern of the electrically evoked lower limb reflex consisted of an early, excitatory F1 component and a later, excitatory F2 component of larger amplitude observed in the CTRL subjects and the CBLd patients. In contrast to this pattern CBLf patients exhibited large F1 components followed by small F2 components. (4) The characteristic differences in the withdrawal reflex responses of cerebellar patients depended on the type of the lesion, providing evidence for an important involvement of the cerebellum in the control of the performance of withdrawal reflexes.

[A man with systemic lupus erythematosus presenting with spastic paraplegia].

We report a 38-year-old man systemic lupus erythematosus who presented with an acute onset of paraplegia and urinary retention. The man had a 12-year history of nodular cutaneous mucinosis and arthralgia. In 1994, he was admitted to our hospital with a sudden onset of weakness and numbness of the right leg followed by an emergence of similar symptoms in the left leg. His elder sister had died at 16 years of age after suffering from systemic lupus erythematosus for 6 years. On examination, the patient had skin rash on his chest, back, head, forehead, and extremities. The neurological examination revealed that his tongue deviated to the right on protrusion. The muscle power was reduced to 2-3/5 in the right leg and to 4/5 in the left leg. The sensory disturbance was noted in the lower extremities with predominant involvement of the right leg. Reflexes were increased in the right biceps, triceps, both patellas, and Achilles tendons. Babinski sign was noted bilaterally. Urinary retention and constipation were also noted. The results of the blood cell count and hepatic and renal function tests were normal. Serum levels of C-reactive protein and complements (C3, C4, CH50) were also normal. Serological examinations showed increased anti-DNA antibody (14 U/ml, [normal, < 6]). Antinuclear antibody was positive at a titer of 1:1380. CSF study showed an increased protein concentration of 83 mg/dl and an IgG level of 14 mg/dl with a normal number of cells. MR images revealed a T1-low, T2-high signal lesion at the upper part of the left ventral medulla. MR images of the brain and spinal cord were normal. The patient was diagnosed as having SLE. High-dose intravenous methylprednisolone (1 g/day) pulse treatment that was started 25 days after the onset of neurological symptoms, produced partial relief. Our case presented with paraplegia with a focal lesion in the left upper ventral part of the medulla on MR images. The incidence of male SLE is low, and paraplegia is a rare complication of SLE. Thus, the medullary lesion in SLE observed in our case appears to be rare. SLE should be considered as a cause of acute onset paraplegia or myelopathy.

Pure Conus Medullaris Syndrome without Lower Extremity Involvement Caused by Intradural Disc Herniation at L1/2: A Case Report.

Conus medullaris syndrome (CMS) is a rare pathology. The conus medullaris is located at the end of the spinal cord and continues to the cauda equina. Conus medullaris lesions can cause variable symptoms and neurological deficits, usually involving the lower extremities; CMS that does not affect the lower limbs is extremely rare. No reports have described isolated CMS caused by intradural disc herniation (IDH). This report describes a case of CMS without lower extremity involvement associated with IDH at L1/2. A 52-year-old man with a 10-year history of lower back pain complained of dysuria and lumbago with no leg symptoms at his first visit to the urology department. Neurological examination revealed mild perineal hypoalgesia; however, motor function and lower extremity sensation were normal with except for left ankle dorsiflexion weakness (manual muscle test, 4/5). Magnetic resonance imaging revealed conus medullaris compression by a mass, continuous with the L1/2 disc, and severe spinal canal stenosis at vertebral levels L3/4 and L4/5. Postmyelographic computed tomography indicated direct conus medullaris compression by an intradural and extramedullary mass continuous with the L1/2 disc. Without recovery of his dysuria, the patient underwent surgery, including partial laminectomy of the L1/2, incision of the dura mater, and removal of the herniated disc. Immediately after surgery, his dysuria completely resolved. More than one year postoperatively, the patient remained active with no change in his neurological condition. Although CMS without lower limb symptoms is extremely rare, we experienced an isolated case of CMS associated with IDH causing direct conus medullaris compression. Without lower extremity involvement, the CMS diagnosis was relatively easy. Surgical treatment for CMS without lower extremity involvement caused by IDH was effective.

Lumbosacral Plexopathy -- History and Physical

Physical examination may be normal in mild cases. Bruises may be seen in cases of trauma. A straight leg raise test is positive in more than half of the patients. Asymmetric lower limb muscle weakness may be seen with asymmetrically absent or reduced deep tendon reflexes. Knee jerk reflex is affected in lumbar plexopathy and ankle jerk is affected in sacral plexopathy. Muscle weakness in hip flexion, knee extension, or adduction suggests a possible injury to the lumbar plexus. Sensory loss may be present in a dermatomal pattern in cases of proximal LS plexopathy involving the roots, or in the nerve distribution. Sensory changes to the medial thigh, anterior thigh, and medial leg can suggest lumbar plexus involvement; posterior thigh, dorsum of the foot, and perineum are likely related to sacral plexus involvement. Spinal point tenderness may be present, especially in cases of sacral fracture or infection. A rectal exam should be performed to assess rectal tone. Saddle anesthesia and bowel or bladder incontinence are rare and may be present, making it difficult to differentiate from cauda equina and conus medullaris syndromes. The inguinal region should also be palpated for suspected hematomas.

[A 74-year-old man with urinary incontinence, right leg weakness and multiple cranial nerve palsies].

We report a 74-year-old man with a lung cancer, who developed right leg weakness, neurogenic bladder, and multiple cranial nerve palsies. The patient was well until December of 1992, when he was 74-year-old, when he noted transient double vision; in February of 1993, he noted numb sensation and weakness in his right leg. Later in the same month, he developed overflow incontinence of urine and weakness in his right face. He also noted deafness in his left ear (he had a marked loss of hearing in his right ear since childhood because of otitis media). His weakness in his right leg had progressed, and he was admitted to our service on March 19, 1993. On admission, he was afebrile and BP was 130/50 mmHg. General physical examination was unremarkable. On neurologic examination, he was alert and oriented to all spheres; no dementia was noted nor were detected aphasia, apraxia, and agnosia. His optic fundi were unremarkable; ocular movement appeared normal, however, he complained of diplopia in far vision. Sensation of the face was intact. He had right facial palsy of peripheral type; he was unable to close his right eye, and Bell's phenomenon was observed on attempted eye closure. On the left side, he had facial spasm. He had marked bilateral deafness. He had no dysarthria or dysphagia. The remaining of the cranial nerves were intact. Motor wise, he was unable to stand or walk alone; weakness did not appear to account for his difficulty in gait; manual muscle testing revealed 4/5 weakness in his tibialis anterior muscle, 1/5 in the peroneus longus, 0/5 in his extensor hallucis longus and extensor digitorum longus, all on the right side. Brachioradial and quadriceps femoris reflexes were increased to 3/4; plantar response was equivocal on the right side, and flexor on the left. Sensory examination revealed loss of touch and pain sensation in the L5 and S1 distributions in his right leg: vibration and position sensations were also diminished in his right foot. He had overflow urinary incontinence with loss of bladder sensation. Marked nuchal stiffness was noted, however, no Kernig's sign or eye ball tenderness was present. Pertinent laboratory findings were as allows; WBC 8,100/microliters, Ht 42.5%, platelet 326,000/microliters, TP 6.8 g/dl, BUN 16 mg/dl, creatinine 0.54 mg/dl, glucose 95 mg/dl, Na 136 mEq/l, K 4.4 mEq/l, Cl 100 mEq/l; liver profile was normal; CEA 436.6 ng/ml, CA19-93 U/ml; urinalysis was normal.(ABSTRACT TRUNCATED AT 400 WORDS)

[Disturbance of deep sensation in medial medullary syndrome. Topographical localization of medial lemniscus in the medulla oblongata].

Medial medullary infarction is characterized by ipsilateral hypoglossal nerve palsy with contralateral hemiparesis and disturbance of deep and discriminative sensory perception. We examined the extent and distribution of disturbances in deep sensation and compared the findings with the lesion location in the medial lemniscus detected by MRI in 3 patients with medial medullary infarction. We classified the lesion location into 2 groups; type I and type II. Type I was ventral to the middle medial lesion of the medial lemniscus, and type II was ventral to the dorsal medial lesion. In our series, type I (Case 1) impairment of the three kinds of deep sensations was more severe in the lower extremities than in the up-per extremities. In type II (Cases 2, 3) the severity or impairment in the upper extremities was moderate or severe and nearly equal to that in the lower extremities. There was no difference in the severity of impairment for the four kinds of discriminative sensations. In the literature, type I (8 patients) impairment of position sense in deep sensation was found in 1 of 7 patients in the upper extremities and 5 of 7 patients in the lower extremities. Impairment of vibration sense was found in 1 of 7 patients in the upper extremities and in all patients in the lower extremities. In type II (14 patients) severe impairment of position and vibration sense in deep sensation was found in 3 patients in the upper extremities equal to that in the lower extremities. There was no tendency in the severity of impairment of four kinds of discriminative sensations. Including our 3 cases and 22 in the literature, impairment of deep sensation was more severe in the lower extremities than in the upper extremities in type I (9 patients) and the extent was none (7), mild or moderate (2) in the upper extremities, mild (2), moderate (1), severe (2), obscure (4) in the lower extremities, while in type II (16 patients) the severity in the upper extremities was nearly equal to that in the lower extremities and the extent was none (1), mild or moderate (1), severe (5), obscure (9) in the upper extremities, none (2), mild or moderate (1), severe (6), obscure (7) in the lower extremities. It is concluded that hemiparesis appeared with lesions located in the pyramidal tract of the medulla, hemiparesis and disturbance of deep sensation in the upper and lower extremities, predominantly in the lower extremities with the lesion of the pyramidal tract to the middle of medial lemniscus in the medulla, hemiparesis and disturbance of the upper and lower extremities deep sensation with lesions of the pyramidal tract to the whole of the medial lemniscus in the medulla. Evaluating deep sensation of the upper and lower extremities is useful for speculation of the lesion location in the medial lemniscus in medial medullary infarction.

Sensory symptoms in ipsilateral limbs/body due to lateral medullary infarction.

To characterize the incidence, topography, and radiologic and pathophysiologic findings of ipsilateral sensory symptoms in the limbs/body in patients with lateral medullary infarction. Between 1994 and 2001, the author identified 12 patients with lateral medullary infarction (6.7% of all lateral medullary infarction patients) who presented with ipsilateral sensory symptoms in the limbs/body in addition to typical lateral medullary syndrome. Brain MRI, nerve conduction velocity, and electromyographic studies were performed. Twenty-four patients without ipsilateral sensory symptoms were included as a control group. Clinical and radiologic findings were compared between the two groups. The ipsilateral sensory symptoms were generally described as numbness or tightness, predominantly affecting the upper extremities, especially distal fingers. Vibration and proprioceptive sensation were occasionally impaired. None showed evidence of peripheral neuropathy or radiculopathy. The patients with ipsilateral sensory symptoms significantly more often had vertigo, nausea/vomiting, severe gait ataxia, hiccup, ipsilateral hemiparesis, and caudally located lesions than those without. The caudal lesions producing ipsilateral sensory symptoms tended to extend dorsomedially. Lateral medullary infarction associated with ipsilateral sensory symptoms in the limbs/body is an uncommon but distinct variant caused by caudal lesions extending dorsomedially that probably involve the ipsilateral dorsal column or decussating lemniscal fibers.

[Two cases of familial amyotrophic lateral sclerosis with a SOD1L126S mutation showing high age at onset and slow progression].

An 80-year-old man (patient 1) was admitted to our hospital with numbness of the right leg and weakness of the lower extremities, predominantly in the right leg, for 1 year previously. Neurological examination revealed moderate weakness without atrophy, and fasciculation in the bilateral lower extremities. No hyperreflexia was noted, and the plantar response was flexor. Neither bulbar palsy nor sensory disturbance was observed. Electromyography (EMG) showed a chronic neurogenic pattern, including giant motor unit potentials and reduced interference in all four limb muscles. MRI images of the cervical and lumbar spines showed severe age-related spondylosis. The clinical and laboratory findings led to a diagnosis of spinal progressive muscular atrophy. Motor paralysis progressed slowly for the following four years, culminating in respiratory failure. A 79-year-man, the younger brother of patient 1 (patient 2), suffered from gait disturbance for 3 years before the admission to our hospital. During the following 3 years, bilateral leg weakness developed, causing difficulty walking. Neurological examination revealed a diffuse mild weakness with atrophy and fasciculation in the bilateral lower extremities; the right deltoid muscle was also mildly weak. Mild hyperreflexia was also noted on the left side, and the plantar response was extensor on the left. EMG showed acute and chronic neurogenic patterns in the four limb muscles. Because the missense mutation c.377 T > C; p.L126S was found on exon 5 of the superoxide dismutase (SOD) 1 gene in this patient, a diagnosis of familial ALS was made. Eight patients reported as familial ALS with the SOD1L126S mutation, including the present cases, all developed an onset of weakness in the lower extremities, but showed few upper motor neuron signs. It is important to consider the possibility of this type of familial ALS in a case of spinal progressive muscular atrophy with late-onset and mild progression, if family history is positive.

Medial Medullary Syndrome -- History and Physical -- Symptoms and Signs

Contralateral paralysis of the upper and lower limb of the body due to lateral corticospinal tract involvement is a common finding. [10] [11] A contralateral decrease in proprioception, vibration, and/or fine touch sensation may occur if medial lemniscus involvement is present in medial medullary syndrome. Some patients report paresthesias or less commonly dysesthesias in the contralateral trunk and lower limb. There are no objective signs of position, touch, or vibration sense loss in many patients with sensory symptoms. In certain patients, a slight loss of position and vibration sense with proprioceptive dysfunction in the contralateral foot is observed. [12]

Zoster myelitis in sickle cell anemia.

A 17-year-old female patient, known case of sickle cell anemia was admitted to our hospital with 10 days history of fever, vomiting, and epigastric pain. On examination, her temperature was 38°C. There was a vesicular type of rash below the nipple and over the left chest involving the back. She was diagnosed as herpes zoster and was started on acyclovir with good hydration and analgesia. Three days later, she developed weakness and decreased sensation of the right leg. On the fifth day, examination revealed power of 1/5 on the right leg, and 4/5 on the left leg, there was proximal and distal increased tone and brisk reflexes and up going toe on the right side with sensory level at T4-T6. An MRI of the thoracolumbar spine showed high signal intensity at T4-T6. The CSF analysis revealed positive polymerase chain reaction for varicella zoster. She was treated with intravenous (IV) dexamethasone 4 mg, 4 times per day. After 3 days she developed left leg weakness, urine incontinence, and power in the left leg was 3/5. Reflex plantar was up going bilaterally with sensory level at the nipple, T4-T6. She was then stared on IV methylprednisolone one gm for 3 days followed by a tapering dose of prednisolone 50 mg for 2 weeks, after a week of starting medication she was able to walk. This case of transverse myelitis is related to varicella zoster infection, with sickle cell anemia, and was successfully treated with high dose IV methylprednisolone, IV acyclovir, and physiotherapy.

Cutaneomeningospinal Angiomatosis (Cobb Syndrome) in a Young Patient.

Cobb Syndrome (Spinal Arteriovenous Metameric Syndrome 1-31 (SAMS 1-31)) is a rare, non-hereditary disorder. Approximately 100 cases of CS have been described to date. The actual incidence may be much higher since only symptomatic patients were documented. In particular, post mortem studies suggest a possibly higher incidence of this syndrome. The main clinical manifestations of this syndrome include skin stains of vascular nature on the torso, in combination with spinal vascular malformations localized in one and the same metameric or spinal segment. A rare diagnosis of this syndrome in patients over 18 is probably related to the fact that the disease may be asymptomatic throughout a long period of time, while patients may tend to disregard the skin lesions. As a result, most publications on this pathology are based on separate case reports. Significant variability of clinical manifestations as well as prolonged progress of the disease often cause errors in diagnosis. What follows is a case report of a young patient with Cobb Syndrome, who was admitted to a regional vascular centre with a misdiagnosis of stroke. 20 patients of young age (from 20 to 35 years old), with a diagnosis of stroke, who were admitted to a University Clinic (of the Russian National Research Medical University Named After Pirogov N.I., Moscow). Among this group of patients, a patient with Cobb syndrome was identified. Patient P., of 22 years, presented with acute, intensive cervical spinal pain, predominantly on the right, numbness and weakness in the arms and legs. About 3 weeks before admission to the hospital, the patient had ARVI with a fever of up to 37.5°C: two weeks before the onset of symptoms, he had undergone extirpation of 2 teeth, for which reason he spent over 2 hours in a forced position with his head thrown back (prolonged overextension in the cervical spine). Multiple skin angiomas on the chest spreading to the shoulder and scapula region. Tetraparesis up to 4 points: tetraparesis in hands with low muscle tone, low reflexes, tetraparesis in legs with high muscle tone, high reflexes, foot clonus when causing Achilles reflexes, tremor in the extremities and no plantar reflex pathology were detected, sensitivity disorders in the hands "the high gloves" and no pelvic disorders were detected. Given the presence and exacerbation of neurological symptoms and cutaneous angiomas, MRI with a contrast agent of the cervical spine was recommended. MR-image of an advanced arteriovenous malformation (AVM) of the cervical spinal cord with signs of gliosis and spinal cord oedema at the C2-C7 level. Endovascular embolization of the AVM in the cervical spinal cord was performed. The treatment led to the complete reversal of neurological symptoms. In the presence of skin lesions, the diagnosis of CS does not present particular difficulties, so in children and young patients with skin angiomatosis, it is advisable to conduct a comprehensive examination using selective spinal angiography or MR angiography to exclude arteriovenous malformations in the spinal cord.

The central loop of H-reflex in the S1 spinal nerve: normal values and constitutional influencing factors.

To investigate the central loop of H reflex in the S1 nerve and the constitutional influencing factors in normal healthy individuals. The study was performed on 39 apparently healthy volunteers. To obtain central H-reflex, the cathode electrode was inserted at a point 1 cm medial to the posterior superior iliac spine, perpendicular to the frontal plane. After the needle touched the sacrum, it was slightly retracted to avoid direct contract to the sacrum. The anode electrode was placed over the anterior iliac spine. The active pick-up electrode was placed at the middle of the line connecting the popliteal crease to the medial malleolus. The reference electrode was placed 2 cm distal to it. The ground eletrode was placed near the active pick-up electrode over the calf muscles. Mean +/- SD for minimum necessary stimulation to obtain central H-reflex was 23 +/- 13 mA. This was significantly higher than the minimum necessary stimulation to obtain peripheral H reflex (p < 0.0001 using paired t test). Mean central loop of H reflex was 6.9 msec with SD of 0.4 msec. There were no significant differences between the two sides. Mean amplitude of M-wave was 2.2 mV with SD of 1.5 in central loop study. Mean amplitude of H-wave was 2.4 mV with SD of 1.5 in central loop study. The Mean time interval between the onset of M-wave and the end of H-wave (M-H duration) was 18.5 msec with SD of 2.3. After stepwise reduction of variables, considering the correlation between leg length and other variables, the leg length was the only variable strongly correlating with central loop of H-reflex. Lack of major influencing factors after correction for leg length, elimination of a major fraction of the afferent segment of the loop, and its ability to differentiate peripheral from central lesions make central H-reflex studies an invaluable diagnostic tool.

Neurology_Adams

Sensorimotor spinal tract syndrome The clinical picture is related predominantly to compression and less often to invasion and destruction of spinal cord tracts. The signs of compression consist of a combination of (1) an asymmetrical spastic weakness of the legs with thoracolumbar lesions and of the arms and legs with cervical lesions, (2) a sensory level on the trunk below which perception of pain and temperature is reduced or lost, (3) posterior column signs, and (4) a spastic bladder under weak voluntary control. The onset of the compressive symptoms is usually gradual and the course progressive over a period of weeks and months, frequently with back pain. With extradural tumors, paralysis usually develops over a period of days to several weeks, but the tempo of progression may be more rapid or more leisurely. The initial disturbance may be of motor or sensory function and the distribution may be asymmetrical. High cervical or foramen magnum lesions produce special clinical

InternalMed_Harrison

Superior cerebellar peduncle Midpontine syndrome: Medial longitudinal fasciculus 5th n. sensory nucleus 5th n. motor nucleus Spinothalamic Medial lemniscus FIGURE 446-12 Axial section at the level of the midpons, depicted schematically on the left, with a corresponding magnetic resonance image on the right. Approximate regions involved in medial and lateral midpontine stroke syndromes are shown. Signs and symptoms: Structures involved 1. Medial midpontine syndrome (paramedian branch of midbasilar artery) On side of lesion Ataxia of limbs and gait (more prominent in bilateral involvement): Pontine nuclei On side opposite lesion Paralysis of face, arm, and leg: Corticobulbar and corticospinal tract Variable impaired touch and proprioception when lesion extends posteriorly: Medial lemniscus 2.

[Short-latency somatosensory evoked potentials and magnetic resonance imaging in the medial medullary syndrome].

A 57-year-old woman was admitted to Kakeyu Hospital complaining of paresis of the left upper and lower extremities which suddenly developed three years ago. Neurological examination revealed spastic paresis of the left upper and lower limbs without facial and lingual paresis. The reflexes were abnormally brisk on both sides but they were more remarkable on the left side, which also showed Babinski's sign. Sensitivity to light touch and vibration was moderately decreased and sensitivity to pinprick and joint position was minimally decreased in the left upper and lower extremities. No cerebellar sign was observed. Needle EMG disclosed large motor units with an amplitude of 4-5 mV and a duration of 8 msec in the light half of the tongue during weak contraction. MRI using a 0.5-T superconducting magnetic resonance unit detected a small, wedge-shaped infarction in the anterior medial portion of the medulla just below the pontomedullary junction. Somatosensory evoked potentials (SEPs) after median nerve stimulation with a non-cephalic reference were recorded. After stimulation of the left side, the scalp-recorded P13 was recognized at the normal latency, but the later components, N16 and N18, were apparently absent. On the other hand, SEPs in another case with thalamic hemorrhage revealed normal N16 potential with absence of N18 on the affected side. From these SEP findings and the reports on SEPs in lesions of the brain stem or thalamus, it was suggested that P13 is abnormal in lower medullary lesions and is preserved in upper medullary lesions, and that N16 is abnormal in brain stem lesions and is preserved or changed a little in thalamic lesions.

A case of reversible paraparesis following celiac plexus block.

Permanent and acute reversible paraplegia following celiac plexus block (CPB) have been reported. We report a case of prolonged reversible paraparesis after alcohol celiac plexus block. A 72-year-old man with primary multicentric pancreatic tumor and multiple hepatic metastases underwent alcohol celiac plexus neurolysis for severe abdominal pain radiating to the back. The patient had complete pain relief after the block but developed paresthesia of the left leg, which then spread to the right leg. Subsequently, loss of flexion and extension of the muscles supplying the left hip, knee, and foot developed. Deep tendon reflexes were brisk on the left compared to the right, and both plantar reflexes gave flexor responses. Magnetic resonance imaging and myelography were normal. Motor-evoked potential recordings showed a spinal cord lesion with involvement of the pyramidal and spinothalamic tracts. Somatosensory-evoked potentials indicated a relative sparing of dorsal column pathways. Physiotherapy was started, the sensory changes gradually subsided, and the patient was discharged 30 days after the block with clinically insignificant neurological deficit. Paraparesis following alcohol celiac plexus block may be reversible over an extended period of time.

Comparison of the classically conditioned withdrawal reflex in cerebellar patients and healthy control subjects during stance: I. electrophysiological characteristics.

The aim of this study was to demonstrate the involvement of the human cerebellum in the classically conditioned lower limb withdrawal reflex in standing subjects. Electromyographic activity was recorded from the main muscle groups of both legs of eight patients with cerebellar disease (CBL) and eight control subjects (CTRL). The unconditioned stimulus (US) consisted of electrical stimulation of the tibial nerve at the medial malleolus. The conditioning stimulus (CS) was an auditory signal given via headphones. Experiments started with 70 paired conditioning stimulus-unconditioned stimulus(CSUS) trials followed by 50 US-alone trials. The general reaction consisted of lifting and flexing the stimulated (stepping) leg with accompanying activation of the contralateral (supporting) leg. In CTRL, the ipsilateral (side of stimulation) flexor and contralateral extensor muscles were activated characteristically. In CBL, the magnitudes of ipsilateral flexor and contralateral extensor muscle activation were reduced comparably. In CTRL, the conditioning process increased the incidence of conditioned responses (CR), following a typical learning curve, while CBL showed a clearly lower CR incidence with a marginal increase, albeit, at a shorter latency. Conditioning processes also modified temporal parameters by shortening unconditioned response (UR) onset latencies and UR times to peak and, more importantly in CBL, also the sequence of activation of muscles, which became similar to that of CTRL. The expression of this reflex in standing subjects showed characteristic differences in the groups tested with the underlying associative processes not being restricted exclusively to the CR but also modifying parameters of the innate UR.

Middle cerebral artery syndrome

Signs and Symptoms Hemiparesis or hemiplegia of the lower half of the contralateral face Hemiparesis or hemiplegia of the contralateral upper and lower extremities* Sensory loss of the contralateral face, arm and leg* Ataxia of contralateral extremities* Speech impairments/aphasia: Broca's area, Wernicke's or Global aphasia as a result of a dominant hemisphere lesion (usually the left brain) Perceptual deficits: hemispatial neglect, anosognosia, apraxia, and spatial disorganization as a result of a non-dominant hemisphere lesion (usually the right brain) Visual disorders: déviation conjuguée, a gaze preference towards the side of the lesion; contralateral homonymous hemianopsia Note: *faciobrachial deficits greater than that of the lower limb Diagnosis Diagnosis can be confirmed by CT Scan or MRI for advanced investigations. References External links Stroke Syndromes affecting the nervous system

Medial medullary syndrome. Report of 18 new patients and a review of the literature.

With advanced imaging techniques, infarctions occurring in the medulla are now more easily identified. To date, however, only approximately 30 cases of medial medullary infarction syndrome (MMS) have been reported, and the clinical and radiological characteristics of MMS remain to be studied. We studied 18 patients (15 men, 3 women; mean age, 62 years) who had compatible clinical and MRI findings of MMS and reviewed the previously reported cases. Seventeen patients had a unilateral lesion usually involving the upper medulla, and 1 had bilateral lesions. Fifteen patients had unilateral sensorimotor stroke, while 2 presented with pure motor stroke. The face was usually but not always spared. The degree of hemiparesis varied, and a tingling sensation with decreased vibration and position sense was the most common sensory manifestation. Two patients had lingual paresis, and none suffered respiratory difficulties. One patient presented with bilateral gait ataxia without sensorimotor dysfunction. Angiography or MR angiography performed in 9 patients showed vertebral artery disease in 6. Three patients had concurrent lateral medullary infarction, and 1 had a previous history of lateral medullary syndrome. The prognosis was generally good, although residual hemiparesis remained in patients with initially severe hemiparesis. Review of 26 previously reported cases showed that they frequently had bilateral lesions, often presenting with quadriplegia, lingual paresis, respiratory symptoms, and a grave prognosis. Our data illustrate that MMS is most often manifested as benign hemisensorimotor stroke frequently associated with tingling sensation and impaired deep sensation. This benign form of MMS should be much more common than MMS with poor prognosis and may have been frequently misdiagnosed as capsular or pontine stroke before the era of MRI.