It seems that they want to present us with precocious puberty (or premature telarche) but they do not provide any analytical data and the ultrasound data are ambiguous (we should assume that by a small uterus they are referring to a prepubertal uterus, but they do not provide any data on ovarian size). We are presented with the case of a three-year-old girl with advanced mammary development, in principle without any associated cause (in principle she does not take drugs that can increase the level of estrogen in the blood, she does not seem to use body creams or eat a lot of chicken meat). If we follow the diagnostic scheme for a premature telarche or suspicion of precocious puberty, we request bone age and abdominal ultrasound (the EO is not advanced as in precocious puberty, and we assume that with a small uterus they mean a prepubertal uterus); according to the complementary examinations that we are given, it does not seem to be precocious puberty, except for the clinical (Tanner IV). Strictly speaking, without analytical hormonal data, it seems that we could mark option 1, being necessary to follow the girl closely. If we take all the above data for granted, we could rule out option 4, which would be the treatment of a central precocious puberty. Regarding the option of mammography, breast ultrasound is used in pediatrics, and in this case it would be indicated if we were told that there is breast asymmetry (we discard option 3). Regarding breast biopsy, it would only be indicated if there are warning signs.

It seems that they want to present us with precocious puberty (or premature telarche) but they do not provide any analytical data and the ultrasound data are ambiguous (we should assume that by a small uterus they are referring to a prepubertal uterus, but they do not provide any data on ovarian size). We are presented with the case of a three-year-old girl with advanced mammary development, in principle without any associated cause (in principle she does not take drugs that can increase the level of estrogen in the blood, she does not seem to use body creams or eat a lot of chicken meat). If we follow the diagnostic scheme for a premature telarche or suspicion of precocious puberty, we request bone age and abdominal ultrasound (the EO is not advanced as in precocious puberty, and we assume that with a small uterus they mean a prepubertal uterus); according to the complementary examinations that we are given, it does not seem to be precocious puberty, except for the clinical (Tanner IV). Strictly speaking, without analytical hormonal data, it seems that we could mark option 1, being necessary to follow the girl closely. If we take all the above data for granted, we could rule out option 4, which would be the treatment of a central precocious puberty. Regarding the option of mammography, breast ultrasound is used in pediatrics, and in this case it would be indicated if we were told that there is breast asymmetry ([HIDDEN]). Regarding breast biopsy, it would only be indicated if there are warning signs.

A woman comes to the office with her 3 year old daughter because she has detected a slight mammary development since 3 months without taking any medication or any relevant history. Indeed, the physical examination shows a Tanner stage IV, with no growth of pubic or axillary hair. The external genitalia are normal. Ultrasonography reveals a small uterus and radiology reveals a bone age of 3 years. What attitude should be adopted?

Follow-up every 3-4 months, as this is a temporary condition that often resolves on its own.

Thelarche

Premature thelarche Premature thelarche is a benign condition in which a young female has breast development before the age of 8 without any accompanied pubertal changes. Individuals undergoing isolated premature thelarche do not experience menstruation, pubic hair growth (pubarche), or the bone growth characteristic of puberty. Initial breast development can be bilateral or unilateral and usually begins with a firm, disc-like area of tissue under the areola which can be mistaken for a mass but is almost always a normal, physiologic process. The breast is often tender and palpation is sometimes painful but breast discharge is absent. Usually, the breasts do not develop past stage 3 on the Tanner Scale, hence maintaining adolescent nipples. Moreover, in 90% of patients with isolated premature thelarche, breast enlargement will resolve 6 months to 6 years after diagnosis.

[True hermaphroditism with bilateral ovotestis].

A nineteen years old woman with ambiguous external genitalia was studied. This condition had been previously identified as a newborn, but her parents refused medical attention and it was reared as a girl. At 12-years, she began spontaneous mammary development, appearing pubic and axillary hair, and clitoral enlargement. The menarche occurred at 15-years and it was followed by irregular periods. Physical examination, showed absence of hirsutism and acne, normal mammary development equivalent to grade V of Tanner. The external genitalia showed fused labio-scrotal folds with an small introitus. The urethral meatus was absent and was later located inside the introitus. There was a big phallus similar to an adult penis with a normal glans, flexed by a chordee. Hormonal determinations discarded congenital adrenal hyperplasia. The karyotype was 46,XX and testosterone levels were in adult male range. Pelvic ultrasonography disclosed a normal uterus and both gonads in confirmed by laparoscopy identifying bilateral ovotestis. Testicular tissue was removed and plastic reconstruction of female genitals was done.

Puberty

Pubic hair Pubic hair is often the second noticeable change in puberty, usually within a few months of thelarche. It is referred to as pubarche. The pubic hairs are usually visible first along the labia. The first few hairs are described as Tanner stage 2. Stage 3 is usually reached within another 6–12 months, when the hairs are too numerous to count and appear on the pubic mound as well. By stage 4, the pubic hairs densely fill the "pubic triangle." Stage 5 refers to spread of pubic hair to the thighs and sometimes as abdominal hair upward towards the navel. In about 15% of girls, the earliest pubic hair appears before breast development begins.

Underarm hair

Underarm hair, also known as axillary hair, is the hair in the underarm area (axilla). Development and function Underarm hair, as human body hair, usually starts to appear at the beginning of puberty, with growth usually completed by the end of the teenage years. Axillary hair goes through four stages of development, driven by weak androgens produced by the adrenal in males and females during adrenarche, and testosterone from the testicle in males during puberty. Like Tanner Staging for pubic hair, axillary hair can be staged according to the Wolfsdorf Staging system, named for pediatric endocrinologist Joseph Wolfsdorf, as follows: Wolfsdorf Stage 1 – no axillary hair Wolfsdorf Stage 2 – scant axillary hair (usually coinciding with onset of adrenarche) Wolfsdorf Stage 3 – coarse axillary hair, less than full-adult Wolfsdorf Stage 4 – full adult axillary hair Staging a patient's axillary hair will allow the physician to track the child's development longitudinally.

Precocious Puberty -- History and Physical

Linear growth acceleration is one of the important features of early puberty. So the exact height, weight, growth velocity (cm/year) and BMI should be documented.  In females, accurate Tanner staging of the breast should take place, which is particularly challenging in obese or overweight girls to differentiate between adipose tissue and the glandular breast tissue. In males, an orchidometer should be used to determine the testicular volume. Volumes of more than 4 ml confirm pubertal development.  In males and females with pubic hair and body odor, the absence of increased testicular volume and breast development should prompt investigation of peripheral causes. Unilateral testicular enlargement is likely due to testicular tumors.

Well-woman examination

Breast ultrasound is a complementary study of mammography. In many women the tissue that makes up the breast is very dense, representing fibrous tissue and glandular tissue, which produces milk during lactation. This limits the radiologist interpreting the study, so, in these cases, the ultrasound is helpful, since this is capable of distinguishing tumors in women with dense breast tissue, where identification is otherwise difficult. Additionally, it is advisable to follow up a mammogram that shows indications of tumors with an ultrasound, to confirm, before more invasive procedures are undertaken. Pelvic exam The pelvic exam is part of the physical examination of the internal pelvic organs (uterus, cervix, ovaries), vagina, and external genitalia. This exam often includes three parts: Inspection of the external genitalia Bimanual examination Inspection of the cervix and vagina using a speculum.

Tanner Stages -- Function

In females, the normal onset of puberty ranges from 8 to 13 years old, averaging age 10 years in White Americans and age 8.9 years in African-Americans. Puberty in females begins with the development of breast buds under the areola, also known as thelarche, and represents entry into Tanner Stage 2. As puberty progresses, the glandular tissue of the breast increases in size and changes in contour. In females, thelarche is followed in 1 to 1.5 years by the onset of sexual hair (pubic and axillary), known as pubarche. Menarche, the onset of menses, arrives on average at age 12.5 years, regardless of ethnicity, following thelarche on average by 2.5 years (range 0.5 to 3 years).  Between Tanner Stage 2 and 3 breast development, females experience peak height velocity. African-American females have closer to 3 years between their thelarche and menarche, accounting for greater height potential.

Puberty

In the months and years following the appearance of pubic hair, other areas of skin that respond to androgens may develop androgenic hair. The usual sequence is: underarm (axillary) hair, perianal hair, upper lip hair, sideburn (preauricular) hair, periareolar hair, and the beard area. As with most human biological processes, this specific order may vary among some individuals. Arm, leg, chest, abdominal, and back hair become heavier more gradually. There is a large range in amount of body hair among adult men, and significant differences in timing and quantity of hair growth among different racial groups. Facial hair is often present in late adolescence, but may not appear until significantly later. Facial hair will continue to get coarser, darker and thicker for another 2–4 years after puberty. Some men do not develop full facial hair for up to 10 years after the completion of puberty. Chest hair may appear during puberty or years after, though not all men develop it.

Female Development -- Definition/Introduction

As females develop, a universal means to classify where they are in puberty is known as Tanner stages. Stage one is prepubertal females. They will have no breast tissue or pubic hair. Stage two is when the breast bud begins to protrude with enlargement of the areola and the sparse presence of pubic hair. [2] Female typically experience their ‘growth spurt’ during Tanner stage two. [1] Continued breast and areola enlargement without distinct separation of the contour and darkening pubic hair along the mons pubis is known as Tanner stage three. Stage four is classified when a secondary mound above the breast forms and the pubic hair thickens but is not on the thigh. Menarche occurs in stage three or four. Stage five is the adult female body. It is when the nipple projects out of the areola and the pubic hairs reach the medial thigh. [3]

Surgery_Schwartz

accounts for the greatest num-ber of malpractice claims for errors in diagnosis and for the largest number of paid claims. Litigation often involves younger women, whose physical examination and mammogram may be misleading. If a young woman (≤45 years) presents with a palpable breast mass and equivocal mammographic findings, ultrasound examination and biopsy are used to avoid a delay in diagnosis.ExaminationInspection. The clinician inspects the woman’s breast with her arms by her side (Fig. 17-18A), with her arms straight up in the air (Fig. 17-18B), and with her hands on her hips (with and without pectoral muscle contraction).135,136 Symmetry, size, and shape of the breast are recorded, as well as any evidence of edema (peau d’orange), nipple or skin retraction, or erythema. With the arms extended forward and in a sitting position, the woman leans forward to accentuate any skin retraction.Figure 17-18. Examination of the breast. A. Inspection of the breast with arms at sides. B.

47,Xxx in an adolescent with premature ovarian failure and autoimmune disease

Background: Premature ovarian failure (POF) is often associated with autoimmune disorders. The 47,XXX karyotype has also been associated with POF and other genitourinary abnormalities. Following is a case of a 17 year old with immune thrombocytopenic purpura (ITP), POF, 47, XXX and a positive antinuclear antibody (ANA).Case Report: A 17 year old Caucasian female was referred to the Adolescent Health Clinic for evaluation of oligomenorrhea with secondary amenorrhea. Thelarche occurred at 12 years, and menarche at 13 years of age. Since then she had a total of five menstrual periods, spaced 1-15 months apart and lasting 3-5 days. Her last menstrual period was six months prior to presentation. Past medical history was significant for chronic ITP diagnosed seven months prior to presentation, when she developed easy bruising. She was treated with IV gamma globulin and had a moderate response, but relapsed several weeks later. She was started on oral prednisone and had a good response, but continued to relapse whenever steroids were tapered. She was therefore maintained on prednisone 10 mg QOD. There was no family history of irregular menses or autoimmune disease. Physical exam revealed a well-appearing, slightly Cushingoid 17 year old. Physical and cognitive development were age-appropriate. There were no stigmata of Turner Syndrome. The thyroid was normal. Breasts were Tanner 5; public hair was Tanner 3. The external genitalia were normal and appeared well-estrogenized. The remainder of the exam was unremarkable. Pelvic ultrasound demonstrated a normal uterus and ovaries. Laboratory evaluation was significant for elevated gonadotropins and nondetectable estradiol. ANA was positive at 1:320 with a speckled pattern. Blood counts, serologies, complement levels, and coagulation studies were otherwise normal. Cytogenetic studies revealed a 47,XXX karyotype. The patient was placed on an estrogen/norethindrone hormone replacement patch for premature ovarian failure. To date, she has developed no further symptoms, and does not meet criteria for a diagnosis of systemic lupus erythematosis.Conclusions: A 47,XXX karyotype was found in a 17 year old with POF and ITP with a positive ANA. The presence of known autoimmune disease in a woman with POF should not dissuade the physician from evaluating for a potential genetic cause.

[Not Available].

Sexual ambiguities are due to varied congenital or hormonal causes. A retrospective study carried out from January 1995 to December 2000 and followed by a prospective study from January 2001 to September 2002 sought to describe the clinical aspects of sexual ambiguities in Internal Medicine in a tertiary hospital Mali. Among 12 patients out of 2223 consultants identified (0.54 percent), 10 of them were phenotypically feminine and 2 phenotypically masculine. The average age of the patients was 14.3+- 8.9 years. Clinically, 3 out of the 10 phenotypically feminine patients presented an anomaly of the external genital organs; 5 out of the 10 had low axillary and pubic hair growth; 4 out of the 10 had delayed puberty; 6 out of the 10 had primary amenorrhoea; 7 out of 10 had hypoplasia of the mammary glands; and3 out of 10 had an inguinal mass. For the the 2 phenotypically masculine patients, one had a bilateral gynecomastia, a macroskelia of 2m04, a low axillary and pubic hair growth and the other had an anomaly of the external genital organs.

Trichotillomania: Bizzare Patern of Hair Loss at 11-Year-old Girl.

Trichotillomania (TTM) is defined by the Diagnostics and Statistic Manual of Mental Disorders, 4th edition (DMS-IV) as hair loss from a patient`s repetitive self-pulling of hair. The disorder is included under anxiety disorders because it shares some obsessive-compulsive features. Patients have the tendency towards feelings of unattractiveness, body dissatisfaction, and low self-esteem (1,2). It is a major psychiatric problem, but many patients with this disorder first present to a dermatologist. An 11-year-old girl came to our department with a 2-month history of diffuse hair loss on the frontoparietal and parietotemporal area (Figure 1). She had originally been examined by a pediatrician with the diagnosis of alopecia areata. The patient`s personal history included hay fever and shortsightedness, and she suffered from varicella and mononucleosis. Nobody in the family history suffered from alopecia areata, but her father has male androgenetic alopecia (Norwood/Hamilton MAGA C3F3). The mother noticed that the child had had changeable mood for about 2 months and did not want to communicate with other persons in the family. The family did not have any pet at home. At school, her favorite subjects were Math and Computer Studies. She did not like Physical Education and did not participate in any sport activities during her free time. This was very strange because she was obese (body-mass index (BMI) 24.69). She was sometimes angry with her 13-year-old sister who had better results at school. The girl had suddenly started to wear a blue scarf. The parents did not notice that she pulled out her hair at home. Dermatological examination of the capillitium found a zone of incomplete alopecia in the frontoparietal and parietotemporal area, without inflammation, desquamation, and scaring. Hairs were of variable length (Figure 1). There was a patch of incomplete alopecia above the forehead between two stripes of hair of variable length (Figure 2). The hair pull test was negative along the edges of the alopecia. Mycological examination from the skin capillitium was negative. The trichoscopy and skin biopsy of the parietotemporal region of the capillitium (Figure 3) confirmed trichotillomania. Laboratory tests (blood count, iron, ferritin, transferrin, selenium, zinc, vitamin B12, folic acid, serology and hormones of thyroid gland) were negative. We referred the girl for ophthalmologic and psychological examination. Ophthalmologic examination proved that there was no need to add any more diopters. The psychological examination provided us with a picture in which she drew her family (Figure 4). The strongest authority in the family was the mother because she looked after the girls for most of the day. She was in the first place in the picture. The father had longer working hours and spent more time outside the home. He worked as a long vehicle driver. He was in the second place in the picture. There was sibling rivalry between the girls, but the parents did not notice this problem and preferred the older daughter. She was successful at school and was prettier (slim, higher, curly brown hair, without spectacles). Our 11-years-old patient noticed all these differences between them, but at her level of mental development was not able to cope with this problem. She wanted to be her sister's equal. The sister is drawn in the picture in the third place next to father, while the patient's own figure was drawn larger and slim even though she was obese. Notably, all three female figures had very nice long brown hair. It seemed that the mother and our patient had better quality of hair and more intense color than the sister in the drawing. The only hairless person in the picture was the father. The girl did not want to talk about her problems and feelings at home. Then it was confirmed that our patient was very sensitive, anxious, willful, and withdrawn. She was interested in her body and very perceptive of her physical appearance. From the psychological point of view, the parents started to pay more interest to their younger daughter and tried to understand and help her. After consultation with the psychiatrist, we did not start psychopharmacologic therapy for trichotillomania; instead, we started treatment with cognitive behavioral therapy, mild shampoo, mild topical steroids (e.g. hydrocortisone butyrate 0.1%) in solution and methionine in capsules. With parents' cooperation, the treatment was successful. The name trichotillomania was first employed by the French dermatologist Francois Henri Hallopeau in 1889, who described a young man pulling his hair out in tufts (3-5). The word is derived from the Greek thrix (hair), tillein (to pull), and mania (madness) (5). The prevalence of TTM in the general adult population ranges from 0.6% to 4%, and 2-4% of the general psychiatric outpatient population meet the criteria for TTM (2-5). The prevalence among children and adolescents has been estimated at less than 1% (5). The disease can occur at any age and in any sex. The age of onset of hair pulling is significantly later for men than for women (3). There are three subsets of age: preschool children, preadolescents to young adults, and adults. The mean age of onset is pre-pubertal. It ranges from 8 to 13 years (on average 11.3 years) (2-5). The occurrence of hair-pulling in the first year of life is a rare event, probably comprising <1% of cases (5). The etiology of TTM is complex and may be triggered by a psychosocial stressor within the family, such as separation from an attachment figure, hospitalization of the child or parent, birth of a younger sibling, sibling rivalry, moving to a new house, or problems with school performance. It has been hypothesized that the habit may begin with "playing" with the hair, with later chronic pulling resulting in obvious hair loss (2). Environment is a factor because children usually pull their hair when alone and in relaxed surroundings. The bedroom, bathroom, or family room are "high-risk" situations for hair-pulling (5). Men and women also differed in terms of the hair pulling site (men pull hair from the stomach/back and the moustache/beard areas, while women pull from the scalp) (3). Pulling hair from siblings, pets, dolls, and stuffed animals has also been documented, often occurring in the same pattern as in the patient (5). Genetic factors contributing to the development of TTM are mutations of the SLITRK1 gene, which plays a role in cortex development and neuronal growth. The protein SAPAP3 has been present in 4.2% of TTM cases and patients with obsessive-compulsive disorder (OCD). It may be involved in the development of the spectrum of OCD. A significantly different concordance rate for TTM was found in monozygotic (38.1%) compared with dizygotic (0%) twins in 34 pairs (3). The core diagnostic feature is the repetitive pulling of hairs from one`s own body, resulting in hair loss. The targeted hair is mostly on the scalp (75%), but may also be from the eyebrows (42%), eyelashes (53%), beard (10%), and pubic area (17%) (3,5). There are three subtypes of hair pulling - early onset, automatic, and focused. Diagnostic criteria for TTM according to DSM-IV criteria are (2,3,5): 1) recurrent pulling of one`s hair resulting in noticeable hair loss; 2) an increasing sense of tension immediately prior to pulling out the hair or when attempting to resist the behavior; 3) pleasure, gratification, or relief when pulling out the hair; 4) the disturbance is not better accounted for by another mental disorder and is not due to a general medical condition (e.g., a dermatologic condition); 5) the disturbance causes clinically significant distress or impairment in social, occupational, or other important areas of functioning. The differential diagnosis includes alopecia areata (Table 1) (6), tinea capitis, telogen effluvium, secondary syphilis, traction alopecia, loose anagen syndrome, lichen planopilaris, alopecia mucinosa, and scleroderma (2-5). Biopsy of an involved area (ideally from a recent site of hair loss) can help to confirm the diagnosis (5). On histologic examination, there are typically increased numbers of catagen and telogen hairs without evidence of inflammation. Chronic hair pulling induces a catagen phase, and more hairs will be telogen hairs. Pigment casts and empty anagen follicles are often seen. Perifollicular hemorrhage near the hair bulb is an indicator of TTM (2). Complications of TTM are rare, but they comprise secondary bacterial infections with regional lymphadenopathy as a result of picking and scratching at the scalp. Many patients play with and ingest the pulled hairs (e.g. touching the hair to lips, biting, and chewing). Trichophagia (ingestion of the hair) can lead to a rare complication named trichobezoar (a "hair ball" in stomach). This habit is present in approximately 5% to 30% of adult patients, but it is less frequent in children. Patient with trichophagia present with pallor, nausea, vomiting, anorexia, and weight loss. Radiologic examination and gastroscopy should not be delayed (2,4,5). The management of the disease is difficult and requires strong cooperation between the physician, patient, and parents. The dermatologist cannot take part in the therapy, strictly speaking, but without the psychological, psychopharmacologic, and topic dermatologic treatment a vicious circle will be perpetuated.

Germaine Greer

A Paladin paperback followed, with cover art by British artist John Holmes, influenced by René Magritte, showing a female torso as a suit hanging from a rail, a handle on each hip. Clive Hamilton regarded it as "perhaps the most memorable and unnerving book cover ever created". Likening the torso to "some fibreglass cast on an industrial production line", Christine Wallace wrote that Holmes's first version was a faceless, breastless, naked woman, "unmistakably Germaine ... hair fashionably afro-frizzed, waist-deep in a pile of stylised breasts, presumably amputated in the creation of a 'female eunuch' based on an assumed equivalence of testicles and mammary glands". The book was reissued in 2001 by Farrar, Straus & Giroux at the instigation of Jennifer Baumgardner, a leading third-wave feminist and editor of the publisher's Feminist Classics series. According to Justyna Wlodarczyk, Greer emerged as "the third wave's favorite second-wave feminist". Arguments

Gynecology_Novak

History and physical examination remain the most effective methods of follow-up in patients treated for endometrial cancer (392–394). Patients should be examined every 3 to 4 months during the first 2 years and every 6 months thereafter. About one-half of patients discovered to have recurrent cancer have symptoms, and 75% to 80% of recurrences are detected initially on physical examination. Particular attention should be given to peripheral lymph nodes, the abdomen, and the pelvis. Very few asymptomatic recurrences are detected by vaginal cytology. Chest x-ray every 12 months is an important method of posttreatment surveillance. Almost one-half of all asymptomatic recurrences are detected by chest x-ray. Other radiologic studies, such as CT scans, are not indicated for routine follow-up of patients who do not have symptoms.

Gynecomastia

Diagnosis To diagnose gynecomastia, a thorough history and physical examination are obtained by a physician. Important aspects of the physical examination include evaluation of the male breast tissue with palpation to evaluate for breast cancer and pseudogynecomastia (male breast tissue enlargement solely due to excess fatty tissue), evaluation of penile size and development, evaluation of testicular development and an assessment for masses that raise suspicion for testicular cancer, and proper development of secondary sex characteristics such as the amount and distribution of pubic and underarm hair. Gynecomastia usually presents with bilateral involvement of the breast tissue but may occur unilaterally as well.

The association of primary hyperparathyroidism and primary ovarian failure: a de novo t(X; 2) (q22p13) reciprocal translocation.

A 40-year-old female presented with primary amenorrhoea at 17 years of age. She was tall at 98th centile for height with eunuchoidal body habitus. Her breast development was Tanner stage 3, pubic and axillary hair Tanner stage 4 with normal external genitalia. Her bone age was 13.4 years at a chronological age of 17.8 years. Gonadotrophins were elevated indicating primary ovarian failure. A diagnostic laparotomy revealed hypoplastic, infantile uterus with bilateral streak gonads. Chromosomal analysis showed a balanced reciprocal translocation 46X, t(X; 2) (q22 p13). She became pregnant by in vitro fertilization with egg donation at the age of 36 years. At 13 weeks of gestation, she presented with intractable vomiting. She had raised corrected serum calcium and parathyroid hormone concentrations consistent with the diagnosis of primary hyperparathyroidism (PHPT). She underwent parathyroidectomy at 24 weeks of gestation with removal of a large left inferior parathyroid adenoma which normalized her serum calcium. Multipoint linkage from a genome-wide screen has identified a region of suggestive linkage on chromosome 2p13.3-14 in some cases of familial isolated hyperparathyroidism (FIHP). To our knowledge, this is the first case of primary amenorrhoea due to reciprocal translocation involving chromosome 2 and the X chromosome associated with PHPT. PHPT in this case is most likely to be as a result of chromosome 2 involvement where a locus for FIHP has been identified. Identification of the gene involved on chromosome 2p13.3-14 will be of considerable interest.

New Breast Mass -- History and Physical -- Physical Examination

Clinical examination of a breast lump is the first stage in the triple-assessment approach. Both breasts and axillae should be examined meticulously by the clinician, as well as carrying out a physical examination of other body systems as indicated by the history. Although it can be tempting to bypass the physical examination in favor of other, more targeted investigation modalities such as mammography or sonography, the findings of the physical examination are crucial for the effective diagnosis and management of breast disease. [16] Repeated studies have indicated that only by combining all three assessments can optimal sensitivity and specificity be achieved. [4] [16]

Clinical breast examination.

An astute clinician has an appreciation of the wide variation possible in normal breasts, but anticipates that the palpation of the breasts of an individual woman will be determined by basic facts concerning typical relative distribution of gland tissue, breast symmetry, the influence of life history, and possibly previous surgery. If the findings of a CBE are not as anticipated, the clinician must find out why this is so. This is a different frame of reference from asking whether a given lump or area should be considered suspicious for cancer. The basic questions are whether the findings of a CBE are consistent with typical breast structure and anatomy and in the context of the woman's life history. If these questions can be answered in the affirmative, the examination is complete; if not, further evaluation is necessary.

Case 308.

An 11-year-old girl presented to the pediatric gastroenterology outpatient department of our institution with gradually increasing painless abdominal distention. The distention started 2 years earlier and was not associated with any other constitutional symptoms, vomiting, diarrhea, jaundice, hematemesis, or melaena. She reported early satiety and heaviness in the lower abdomen. The abdominal swelling was predominantly in the infraumbilical region and was soft at palpation. She was the first child of nonconsanguineous parents and had an uneventful perinatal course after a normal vaginal delivery. Her developmental milestones were normal. She had an average scholastic performance at school. There was no history of visual problems, seizures, or inappropriate behaviors. She had an early menarche 2 years previously. Her menstrual cycles were regular, and there was no abnormal vaginal discharge. Her breast development was normal (Tanner stage III), while pubic and axillary hair were absent (Tanner stage I). She was short for her age (104 cm; normal range, 120-154 cm). There was no history of short stature among her siblings or parents. Laboratory investigations were performed to measure thyroid-stimulating hormone (1354.34 μIU/mL; normal range, 0.35-5.5 μIU/mL), triiodothyronine (<2.5 ng/dL [0.0385 pmol/L]; normal range, 100-200 ng/dL [1.54-3.08 pmol/L]), thyroxine (1.35 μg/dL [17.37 nmol/L]; normal range, 5-12 μg/dL [64.35-154.44 nmol/L]), β-human chorionic gonadotropin (<1.2 mIU/mL; normal, <5 mIU/mL), luteinizing hormone (0.08 mIU/mL; normal range, 0.1-6.0 mIU/mL), and follicle-stimulating hormone (6.93 mIU/mL; normal range, 0.3-2.0 mIU/mL) levels. Complete blood count was normal. An abdominal mass was suspected, and abdominopelvic CT was performed and followed by US; these examinations revealed multiple large cysts in both ovaries (Figs 1, 2A, 2B). The uterus was pubertal in shape, and endometrial thickness was 9 mm, representing normal follicular phase measurement. Serum CA-125 and inhibin levels were normal. To evaluate short stature, radiographs of the hand (Fig 3) and pelvis (Fig 3B) were obtained as part of a limited skeletal survey, keeping in mind the possible skeletal changes associated with hypothyroidism. In view of the hypothyroidism, US of neck was also performed (Fig 4). Treatment was started based on the clinical and radiologic parameters, and the child's condition improved with medical treatment.

Gynecomastia -- History and Physical

A careful review of family history, genetic history, medications, and recreational drug use should also be taken into consideration. A complete and thorough physical exam should be done. The head and neck exam should evaluate for any abnormal masses or thyroid abnormalities. Assess breasts for the nature of the tissue, masses, skin changes, nipple discharge, asymmetries, and tenderness, along with an axillary examination. The testes should be examined to look for asymmetry, masses, enlargement, or atrophy. Those males with feminizing characteristics should have endocrine testing and genetic testing. Any other positive findings on physical examination should be treated in an appropriate manner.

Precocious puberty in Turner Syndrome: report of a case and review of the literature.

Turner Syndrome (TS) is caused by monosomy or structural abnormalities of the X chromosome, with a prevalence of about 1/2000 females live birth. Most important clinical features of TS are short stature and gonadal failure. Approximately one third of girls with TS may undergo spontaneous puberty. Here we report on the case of a girl with a rare 45X0/47XXX mosaic TS exhibiting a precocious puberty. The patient was diagnosed with TS at the age of 4 years, upon a diagnostic work-up for dysmorphic features. Chromosome analysis revealed a mosaic karyotype (45X0/47XXX). She presented with normal height and normal growth velocity so that Growth Hormone (GH) therapy was not started. She was referred to our Department at the age of 7 years and 10 months, because of vaginal bleeding. A physical examination revealed a Tanner stage III for breast and Tanner stage III for pubic hair development. Height and weight were within the normal range for age. Psychological evaluation showed moderate global developmental delay, together with emotional and social immaturity and reading difficulties. The growth rate was accelerated. Her bone age was 10 years. Pelvic ultrasound demonstrated increased size for age of both the uterus and the ovaries, with bilateral ovarian follicles. GnRH stimulation test revealed pubertal response of gonadotropins (peak LH 22.5 mIU/ml). MRI of the brain was normal. These clinical, radiologic and laboratory findings were consistent with a diagnosis of idiopathic central precocious puberty; therefore, GnRH analog therapy was started, in order to slow pubertal progression and to preserve adult stature. Furthermore, GH treatment was added to further improve adult height. Our case highlights the possibility of precocious puberty as an atypical clinical feature of TS. Thus, precocious puberty may occur in TS girls when a dosage compensation by the cell line with more than two X chromosomes allows normal ovarian function. GnRH analog therapy in addition to GH treatment should be recommended in TS girls with precocious puberty in order to slow pubertal progression and to preserve adult stature.

InternalMed_Harrison

Breast cancer is about 1/150th as frequent in men as in women; 1720 men developed breast cancer in 2006. It usually presents as a unilateral lump in the breast and is frequently not diagnosed promptly. Given the small amount of soft tissue and the unexpected nature of the problem, locally advanced presentations are somewhat more common. When male breast cancer is matched to female breast cancer by age and stage, its overall prognosis is identical. Although gynecomastia may initially be unilateral or asymmetric, any unilateral mass in a man older than age 40 years should receive a careful workup including biopsy. On the other hand, bilateral symmetric breast development rarely represents breast cancer and is almost invariably due to endocrine disease or a drug effect. It should be kept in mind, nevertheless, that the risk of cancer is much greater in men with gynecomastia; in such men, gross asymmetry of the breasts should arouse suspicion of cancer. Male breast cancer is best managed

An Unusual Presentation of 46,XY Pure Gonadal Dysgenesis: Spontaneous Breast Development and Menstruation.

46,XY pure gonadal dysgenesis (Swyer syndrome) is characterized by normal female genitalia at birth. It usually first becomes apparent in adolescence with delayed puberty and amenorrhea. Rarely, patients can present with spontaneous breast development and/or menstruation. A fifteen-year-old girl presented to our clinic with the complaint of primary amenorrhea. On physical examination, her external genitals were completely female. Breast development and pubic hair were compatible with Tanner stage V. Hormonal evaluation revealed a hypergonadotropic state despite a normal estrogen level. Chromosome analysis revealed a 46,XY karyotype. Pelvic ultrasonography showed small gonads and a normal sized uterus for age. SRY gene expression was confirmed by multiplex polymerase chain reaction. Direct sequencing on genomic DNA did not reveal a mutation in the SRY, SF1 and WT1 genes. After the diagnosis of Swyer syndrome was made, the patient started to have spontaneous menstrual cycles and therefore failed to attend her follow-up visits. After nine months, the patient underwent diagnostic laparoscopy. Frozen examination of multiple biopsies from gonad tissues revealed gonadoblastoma. With this report, we emphasize the importance of performing karyotype analysis, which is diagnostic for Swyer syndrome, in all cases with primary or secondary amenorrhea even in the presence of normal breast development. We also suggest that normal pubertal development in patients with Swyer syndrome may be associated with the presence of a hormonally active tumor.

The breasts can most easily be palpated by asking the patient to lie back at approximately 30 degrees and rest their palm up underneath their head. Palpation of the breast must proceed in a structured manner; generally, clinicians will use a four-quadrant approach (upper outer, upper inner, lower outer, and lower inner quadrants), followed by palpating the areola and then the axillary tail. Particular attention should focus on the inframammary fold and the axillary tail. The normal breast is examined first, and the tissue is assessed for its overall consistency. Masses are most often detected in the upper outer quadrant, as most breast tissue is located here.

Growing Up Fast: Managing Autism Spectrum Disorder and Precocious Puberty.

John is a 4-year-old boy with autism spectrum disorder (ASD) and developmental delay who presented with concerns about increasing aggressive behavior at a follow-up visit with his developmental-behavioral pediatrician. Diagnosis of ASD was made via Diagnostic and Statistical Manual of Mental Disorders, 5th version criteria at initial evaluation at 34 months. Medical history at that time was pertinent for rapid linear growth since the age of 1 and recent pubic hair growth and penile enlargement. Family history was significant for early puberty in a maternal uncle and 4 distant maternal relatives. Standardized testing included administration of the Childhood Autism Rating Scale 2-Standard, which was consistent with severe symptoms of ASD, and the Mullen Scales of Early Learning, which indicated moderate delay in fine motor skills and expressive language and severe delay in receptive language and visual receptive skills.At initial assessment, John's parents also reported a pattern of aggressive behavior, which included frequent hitting of other children at childcare, consistently forceful play with peers and family members, and nightly tantrums with hitting and throwing at bedtime. Triggers of aggressive behavior included other children taking his toys, transition away from preferred activities, and being told "no."John was concurrently evaluated by a pediatric endocrinologist at 34 months. At that assessment, his height Z-score was +2.5, and he had Tanner 2 pubic hair, Tanner 3 genitalia, and 6 cc testicular volumes. Radiograph of the hand revealed a bone age of 6 years (+7.8 S.D.). Laboratory studies revealed a markedly elevated testosterone level and low gonadotropin (luteinizing hormone [LH] and follicle-stimulating hormone) levels and a normal dehydroepiandrosterone sulfate, suggestive of peripheral precocious puberty. Targeted genetic testing with sequencing of the LHCGR gene revealed a heterozygous D578G mutation resulting in the rare condition Familial Male-Limited Precocious Puberty (FMPP), characterized by constitutive activation of the LH receptor. FMPP, also referred to as testotoxicosis, was attributed as the cause of John's peripheral precocious puberty.By the age of 4, John's height Z-score was +3.1, his genitalia larger, and his bone age 10 years (+10.3 S.D.). His parents elected to start off-label therapy with bicalutamide (a nonsteroidal antiandrogen) and anastrazole (an aromatase inhibitor), recommended by the endocrinologist. Unexpectedly, as John's hyperandrogenism was treated, John's family reported intensified aggression toward other children and adults, especially at school, in addition to multiple daily instances of biting when upset. What is your next step in John's treatment of his challenging behavior? 1. Shenker A, Laue L, Kosugi S, et al. A constitutively activating mutation of the luteinizing hormone receptor in familial male precocious puberty. Nature. 1993;365:652-654.

Papal armorial

|} Late Middle Ages and Renaissance Note that some of the images of the coats of arms shown below anachronistically include the external adornments of the papal tiara and the keys of Peter. These ornaments were not in use before the 1450s. Popes of the Early Modern period Most popes of the 16th to 18th centuries came from Italian noble families, but there were some exceptions, such as Sixtus V (1585-1590), who was of low birth. Popes of the modern period The last person elected as pope who was not already an ordained priest or monk was Leo X (Giovanni di Lorenzo de' Medici) in 1513. Thus, throughout the Early Modern period, the elected pope already had a coat of arms: if he did not have a family coat of arms to begin with, he would have adopted one upon being made bishop. Upon his election as pope, he would continue using his pre-existing coat of arms, in some cases with heraldic augmentations. This tradition was continued into the modern period.

Hetero- and isosexual pseudoprecocity associated with testicular sex-cord tumors in an 8 year-old male.

Enlargement of the right breast, axillary hair, and acceleration of linear growth rate were first noted at 8 years of age in an otherwise healthy male with no known exposure to exogenous hormones. At 9.5 years of age the right subareolar mass was excised; histologic examination revealed fibrous breast tissue. Subsequently pubic hair appeared. At 10.7 years of age, the patient complained of right inguinal pain after a minor injury. Examination revealed a tall (height age 12.7 years), mature, muscular boy with enlarged (R: 5 x 3 x 2 cm; L: 3 x 2 x 3 cm) firm, irregular testes, Tanner stage II pubic hair, and modest axillary hair. No perioral pigmentation was present. Testicular ultrasonography revealed multilobular echogenic foci with calcifications. Bone age was 13 years, the LH and FSH secretory responses to GnRH were minimal (LH: < 0.038-->0.28 mIU/ml; FSH: < 0.063-->0.11 mIU/ml), and basal serum testosterone (< 10 ng/dl) and estradiol (< 10 pg/ml) values were undetectable. Following administration of human chorionic gonadotropin (hCG), the serum testosterone concentration increased to 275 ng/dl, while estradiol remained unmeasurable. Spermatic vein concentrations of testosterone were undetectable in the basal state and increased after hCG administration. After bilateral orchiectomy, pathologic examination revealed multifocal tumors composed of brightly eosinophilic, large polygonal cells arranged in nests, cords, and clusters within dense connective tissue or mucinous stroma with lamellar calcifications of varying sizes. These pathologic findings were compatible with a large cell calcifying Sertoli cell (sex-cord)tumor of the testes. Testosterone, estradiol, immunoreactive and bioactive aromatase activity were not detectable in the tumor. Thus, both heterosexual (gynecomastia) and isosexual (increased musculature, pubic and axillary hair) precocious puberty may occur in boys with testicular sex-cord tumors.

Elling Woman

Examination The Elling Woman is believed to have been hanged, like the Tollund Man. The estimated year of death was dated to approximately 280 BCE in the Nordic Iron Age, also around the time of the Tollund Man; however, it is not possible to confirm whether or not they were both killed at exactly the same time. It also initially might have been impossible to tell the sex of her body, if the hair had not been preserved, although X rays were taken of her pelvis, which proved she was female. In 1978, the body was reexamined with radiographs, from which the sex was determined to be female and the original age-at-death estimate of 25 years was found to be accurate. This body is often identified by the braid on her head, which was tied into an elaborate knot. Elling Woman is believed to have been a human sacrifice. Demineralization, which often occurs with bog bodies, was found to be the initial cause of what was first understood as apparent osteoporosis in the remains. References

Partial hypogonadotropic hypogonadism associated with the Leu266Arg and Gln106Arg mutation of the gonadotropin-releasing hormone receptor.

We describe a patient with partial hypogonadotropic hypogonadism caused by a compound heterozygous GnRH-R mutation. She is a 20-year-old tall, eunuchoid female referred for evaluation of primary amenorrhea. Spontaneous thelarche occurred at the age of 15 years. Breast and pubic hair were at Tanner stages 3 and 4, respectively. Evaluation revealed low plasma estradiol level and absence of withdrawal bleeding after progestin challenge. Pelvic ultrasonography showed a small uterus and ovaries. Bone age was delayed at 14.5 years. Bone mineral density showed osteopenia. Endogenous LH secretory pattern was abnormal with low amplitude and frequency, but responded to pulsatile GnRH administration. The coding exons of the GnRH-R gene were amplified and the PCR products were sequenced bidirectionally. Two different mutations were identified: one in exon 1 (Gln106Arg) and the other in exon 3 (Leu266Arg).

Breast Lymphatics -- History and Physical

Physical examination of the breast lymphatics occurs during the breast exam. The examiner can best palpate the axillary nodes with the patient sitting up and the examiner supporting the arms to the side, or the hands resting on the hips. The examiner should palpate the supraclavicular and infraclavicular lymph nodes as well.

Estrogen insensitivity syndrome

The patient had a small uterus, with an endometrial stripe that could not be clearly identified. At the age of 15 years, 5 months, her bone age was 11 or 12 years, and at the age of 17 years, 8 months, her bone age was 13.5 years. Her bone mass was lower than expected for her age, and levels of osteocalcin and C-terminal telopeptide were both elevated, suggesting an increased rate of bone turnover. She was 162.6 cm tall, and her growth velocity indicated a lack of estrogen-induced growth spurt at puberty. The patient had normal pubic hair development (Tanner stage IV) and severe facial acne, which could both be attributed to testosterone. Her ovarian pathology was attributed to the elevated levels of gonadotropins. In addition to her absence of breast development and areolar enlargement, the patient also appeared to show minimal widening of the hips and a lack of subcutaneous fat deposition, which is in accordance with the established role of estrogen and ERα in the development of


In this question we are presented with a case to deduce a diagnosis and then indicate which would be the treatment of choice. Both the characteristics of the case and the answers suggest that it is a trigemino-autonomic headache (intense, periocular pain with lacrimation and rhinorrhea). The idea is to make a differential diagnosis mainly between a cluster headache (whose treatments include answers 2, 3, 4 and 5), a paroxysmal hemicrania (whose treatment of choice is the indomethacin of answer 1) and a SUNCT (unilateral neuralgiform headache with conjunctival injection and lacrimation). Cluster headache predominates in males whose duration can vary between 15-180 minutes, between once every 2 days, up to 8 times a day. Paroxysmal hemicrania predominates in women, with episodes of pain similar to cluster headache, but with a shorter duration (2-30 min), and a higher frequency (5-30 episodes per day). As for SUNCT, the crises are much shorter, lasting seconds (5-240 seconds) and are usually refractory to treatment. Therefore the diagnosis would be of a paroxysmal hemicrania and its treatment of choice is indomethacin (which also the answer is a diagnostic criterion). Therefore correct answer 1 (Indomethacin). All data provided are based on the Diagnostic Criteria of the International Headache Society.

In this question we are presented with a case to deduce a diagnosis and then indicate which would be the treatment of choice. Both the characteristics of the case and the answers suggest that it is a trigemino-autonomic headache (intense, periocular pain with lacrimation and rhinorrhea). The idea is to make a differential diagnosis mainly between a cluster headache (whose treatments include answers 2, 3, 4 and 5), a paroxysmal hemicrania (whose treatment of choice is the indomethacin of [HIDDEN]) and a SUNCT (unilateral neuralgiform headache with conjunctival injection and lacrimation). Cluster headache predominates in males whose duration can vary between 15-180 minutes, between once every 2 days, up to 8 times a day. Paroxysmal hemicrania predominates in women, with episodes of pain similar to cluster headache, but with a shorter duration (2-30 min), and a higher frequency (5-30 episodes per day). As for SUNCT, the crises are much shorter, lasting seconds (5-240 seconds) and are usually refractory to treatment. Therefore the diagnosis would be of a paroxysmal hemicrania and its treatment of choice is indomethacin (which also [HIDDEN]). Therefore [HIDDEN]. All data provided are based on the Diagnostic Criteria of the International Headache Society.

A 40-year-old woman consults for approximately 20 episodes per day of intense left periocular pain lasting 15 minutes, accompanied by intense tearing and rhinorrhea. Her examination and MRI are normal. His treatment of choice would be:

The usual treatment of trigeminal autonomic cephalalgias.

Trigeminal autonomic cephalalgias include cluster headache, paroxysmal hemicrania, and short-lasting unilateral neuralgiform headache attacks with conjunctival injection, tearing, and rhinorrhea (SUNCT). Conventional pharmacological therapy can be successful in the majority of trigeminal autonomic cephalalgias patients. Most cluster headache attacks respond to 100% oxygen inhalation, or 6 mg subcutaneous sumatriptan. Nasal spray of sumatriptan (20 mg) or zolmitriptan (5 mg) are recommended as second choice. The bouts can be brought under control by a short course of corticosteroids (oral prednisone: 60-100 mg/day, or intravenous methylprednisolone: 250-500 mg/day, for 5 days, followed by tapering off the dosage), or by long-term prophylaxis with verapamil (at least 240 mg/day). Alternative long-term preventive medications include lithium carbonate (800-1600 mg/day), methylergonovine (0.4-1.2 mg/day), and topiramate (100-200 mg/day). As a rule, paroxysmal hemicrania responds to preventive treatment with indomethacin (75-150 mg/day). A short course of intravenous lidocaine (1-4 mg/kg/hour) can reduce the flow of attacks during exacerbations of SUNCT. Lamotrigine (100-300 mg/day) is the preventive drug of choice for SUNCT. Gabapentin (800-2700 mg/day), topiramate (50-300 mg/day), and carbamazepine (200-1600 mg/day) may be of help.

[Three Japanese cases of hypnic headache].

We described three cases of hypnic headache with successful treatment by lithium carbonate or caffeine. This is the first detail report of Japanese cases. An endocrinological test and rhythm analyses of ambulatory blood pressure (ABP) and heart rate variability in a case suggested possible association between hypnic headache and hypothalamic-pituitary dysfunction. Case 1: A 48-year-old female migraineur complained of new-onset nocturnal headaches. Her headache awakened her from sleep between 1 AM and 2 AM. The headache occurred 3-4 times per week and lasted from 1 hour to 2 hours. The headache were moderate intensity and bilateral dull throbbing pain that located in the forehead to temples. There was no accompanying symptoms such as nausea, phonophobia, photophobia, nor the other autonomic features including conjunctival injection or tearing during the headache attacks. Physical and neurological examinations showed normal results except slight weakness and mild dysesthesia of the left arm due to a vertebral disk herniation at C5/6 level. In the pituitary endocrinological test, the prolactin level remarkably increased in response to the TRH loading. The single cosinor analysis demonstrated significant circadian rhythm of ABP parameters. However, the analysis did not demonstrate any significant circadian rhythm of Holter ECG parameters of time domain analysis and frequency analysis. Receiving 200 mg lithium bicarbonate before sleep, her nocturnal headache completely disappeared. Case 2: A 68-year-old woman had been followed up by her chronic tension-type headache since her forties. At her 66-years, she suffered from a new nocturnal headache. She awoke from sleep by the headache about 3 AM and the headache lasted 30 min. Moderate, dull headache located on her left temple to parietal head, 3-4 times/week. She was able to go back asleep without any medication after spontaneous headache cessation. She first complained the nocturnal headache at the 10 months later of the new headache appearance. She received 200 mg caffeine just before sleep and her headache has been disappeared. Case 3: 70-year-old women had been regularly visited our clinics for her migraine and chronic tension-type headache. She received amitriptyline and her headaches was well controlled. At her 69 years, she complained nocturnal headache. It occured every other day. The headache was moderate pulsative dull pain on the occipital region and lasted 90 minutes without any autonomic symptoms. Headache began between midnight and 1 AM. She told us her new nocturnal headache one year later of the onset. Oral caffeine (200 mg) just before sleep did not improve her headache and caused insomnia. Receiving 100 mg lithium before sleep, her hypnic headache disappeared completely. These three cases are compatible with the diagnostic criteria proposed in ICHD-II. There were some patients with hypnic headache in Japan and neurologists should pay attentions to this form of benign headache, because some beneficial treatments are currently available.

[Therapy of trigeminal autonomic headaches].

Trigeminal autonomic cephalgias (TAC) are characterized by severe and strictly unilateral headaches with a frontotemporal and periorbital preponderance in combination with ipsilateral cranial autonomic symptoms, such as lacrimation, conjunctival injection, rhinorrhea, nasal congestion, and restlessness or agitation. One main differentiating factor is the duration of painful attacks. While attacks typically last 5 s to 10 min in SUNCT syndrome (short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing), paroxysmal hemicrania lasts 2-30 min and cluster headaches 15-180 min. Hemicrania continua represents a continuous TAC variant. From a therapeutic view, TACs differ substantially. Lamotrigine is used as first-choice prevention in SUNCT syndrome and indometacin in paroxysmal hemicrania. For cluster headaches, acute therapy with inhaled pure oxygen and fast-acting triptans (sumatriptan s.c. and intranasal zolmitriptan) is equally important to short-term preventive therapy with methysergide and cortisone and long-term prophylactic treatment comprising verapamil as drug of first choice and lithium carbonate and topiramate as drugs of second choice. In refractory cases of chronic cluster headache, neuromodulatory approaches such as occipital nerve stimulation and sphenopalatine ganglion stimulation are increasingly applied.

Chronic Cluster Headache with an Atypical Presentation and Treatment Response.

The management of cluster headache (CH) may be challenging. We report a 50-year-old male with recurrent attacks of dull and severe unilateral periorbital pain, lasting 30-45 minutes, twice a day, exclusively during sleep, and accompanied by ipsilateral rhinorrhea and lacrimation. The pain switched sides within every attack. CH treatment was initiated but the patient maintained recurrence rates compatible with chronic CH, even after increasing verapamil to 460 mg/day. Afterwards we decided to add lithium (800 mg/day). With this treatment the severity and recurrence of CH substantially decreased, despite the patient's autonomous decision to take lithium only during the acute phase of the cluster. The exclusively alternating location and the excellent response to short cycles of lithium represent two unique features of CH.

Headache

Primary Ninety percent of all headaches are primary headaches. Primary headaches usually first start when people are between 20 and 40 years old. The most common types of primary headaches are migraines and tension-type headaches. They have different characteristics. Migraines typically present with pulsing head pain, nausea, photophobia (sensitivity to light) and phonophobia (sensitivity to sound) Tension-type headaches usually present with non-pulsing "bandlike" pressure on both sides of the head, not accompanied by other symptoms. Such kind of headaches maybe further classified into-episodic and chronic tension type headaches Other very rare types of primary headaches include: cluster headaches: short episodes (15–180 minutes) of severe pain, usually around one eye, with autonomic symptoms (tearing, red eye, nasal congestion) which occur at the same time every day. Cluster headaches can be treated with triptans and prevented with prednisone, ergotamine or lithium.

Pharmacotherapy for Cluster Headache.

Cluster headache is characterised by attacks of excruciating unilateral headache or facial pain lasting 15 min to 3 h and is seen as one of the most intense forms of pain. Cluster headache attacks are accompanied by ipsilateral autonomic symptoms such as ptosis, miosis, redness or flushing of the face, nasal congestion, rhinorrhoea, peri-orbital swelling and/or restlessness or agitation. Cluster headache treatment entails fast-acting abortive treatment, transitional treatment and preventive treatment. The primary goal of prophylactic and transitional treatment is to achieve attack freedom, although this is not always possible. Subcutaneous sumatriptan and high-flow oxygen are the most proven abortive treatments for cluster headache attacks, but other treatment options such as intranasal triptans may be effective. Verapamil and lithium are the preventive drugs of first choice and the most widely used in first-line preventive treatment. Given its possible cardiac side effects, electrocardiogram (ECG) is recommended before treating with verapamil. Liver and kidney functioning should be evaluated before and during treatment with lithium. If verapamil and lithium are ineffective, contraindicated or discontinued because of side effects, the second choice is topiramate. If all these drugs fail, other options with lower levels of evidence are available (e.g. melatonin, clomiphene, dihydroergotamine, pizotifen). However, since the evidence level is low, we also recommend considering one of several neuromodulatory options in patients with refractory chronic cluster headache. A new addition to the preventive treatment options in episodic cluster headache is galcanezumab, although the long-term effects remain unknown. Since effective preventive treatment can take several weeks to titrate, transitional treatment can be of great importance in the treatment of cluster headache. At present, greater occipital nerve injection is the most proven transitional treatment. Other options are high-dose prednisone or frovatriptan.

Management of cluster headache.

The prevalence of cluster headache is 0.1% and cluster headache is often not diagnosed or misdiagnosed as migraine or sinusitis. In cluster headache there is often a considerable diagnostic delay - an average of 7 years in a population-based survey. Cluster headache is characterized by very severe or severe orbital or periorbital pain with a duration of 15-180 minutes. The cluster headache attacks are accompanied by characteristic associated unilateral symptoms such as tearing, nasal congestion and/or rhinorrhoea, eyelid oedema, miosis and/or ptosis. In addition, there is a sense of restlessness and agitation. Patients may have up to eight attacks per day. Episodic cluster headache (ECH) occurs in clusters of weeks to months duration, whereas chronic cluster headache (CCH) attacks occur for more than 1 year without remissions. Management of cluster headache is divided into acute attack treatment and prophylactic treatment. In ECH and CCH the attacks can be treated with oxygen (12 L/min) or subcutaneous sumatriptan 6 mg. For both oxygen and sumatriptan there are two randomized, placebo-controlled trials demonstrating efficacy. In both ECH and CCH, verapamil is the prophylactic drug of choice. Verapamil 360 mg/day was found to be superior to placebo in one clinical trial. In clinical practice, daily doses of 480-720 mg are mostly used. Thus, the dose of verapamil used in cluster headache treatment may be double the dose used in cardiology, and with the higher doses the PR interval should be checked with an ECG. At the start of a cluster, transitional preventive treatment such as corticosteroids or greater occipital nerve blockade can be given. In CCH and in long-standing clusters of ECH, lithium, methysergide, topiramate, valproic acid and ergotamine tartrate can be used as add-on prophylactic treatment. In drug-resistant CCH, neuromodulation with either occipital nerve stimulation or deep brain stimulation of the hypothalamus is an alternative treatment strategy. For most cluster headache patients there are fairly good treatment options both for acute attacks and for prophylaxis. The big problem is the diagnosis of cluster headache as demonstrated by the diagnostic delay of 7 years. However, the relatively short-lasting attack of pain in one eye with typical associated symptoms should lead the family doctor to suspect cluster headache resulting in a referral to a neurologist or a headache centre with experience in the treatment of cluster headache.

[Cluster-tic syndrome: two case reports].

Two patients with cluster-tic syndrome are reported. The first, a 43-years-old man, complaining of trigeminal pain in the right side of the face, accompanied by homolateral autonomic signs, such as ocular injection, sweating and drooped eyelid. The cluster attack was triggered by chewing, shaving and washing the face. The periodicity of bouts was six months. The pain was relieved by carbamazepine (800 mg/day). The second patient, a 43-year-old man, with an excruciant, neuralgic pain in the left side of the face, accompanied by tearing, conjuntival injection, drooped eyelid, rhinorrhea, photophobia and phonophobia. The neurologic examination showed triggered points in the first and second division of the trigeminal nerve. The patient was treated with verapamil (160 mg/day) and prednisone (60 mg/day), with relief of his symptoms. The periodicity of bouts was once a year. The literature was reviewed and 37 cases previosly reported are considered. We conclude that there are two different groups of patients. In the first group, the patients had cluster and trigeminal bouts in different time. In the second group, with only nine cases, the patients presented both cluster and trigeminal type of pain at the same time, as in the two cases reported here.

Hypnic headache: A review of 348 cases published from 1988 to 2018.

Hypnic headache (HH) is a rare benign disorder described initially by Raskin in 1988. It is characterized by recurrent nocturnal episodes of headache that periodically awaken the sleeping patient and usually occur in the elderly. This review aimed to describe the clinical features of the HH cases published in the literature from 1988 to 2018. Based on literature search in the major medical databases (LiLacs, SciELO, Bireme, Medline, Embase, Current Contents, Scopus, EBSCO and PubMed), we have analyzed the case reports on HH that have been published from 1988 to 2018. We described 343 adults (69.0% women and 31.0% men) and 5 children (3 girls and 2 boys) diagnosed with HH. Average age for adults and children was, respectively, 58.0 ± 13.1 years (ranging from 15 to 85 years) and 9 years (ranging from 7 to 11 years). The diagnosis was made 7.6 ± 14.2 years (range 0.1 to 39 years) after onset of headache. Pain occurred during nocturnal sleep (94.8%), with an average duration of 90 min, bilaterally located (55.5%), having a dull character (74.4%), and moderate intensity (61.5%). In 94.5% of the patients, headache occurred for 10 or more days per month (mean of 21 days). Autonomic manifestations occurred in 7.6% of the patients, predominantly lacrimation (61.1%) and rhinorrhea (16.7%). Caffeine presented the best therapeutic response in acute treatment. In prophylaxis, lithium, caffeine and indomethacin were effective drugs in 77.8% of the patients. In 56.7% of the patients there was remission with treatment and in 72.7% of them, without recurrence. HH is a rare disease that usually occurs for the first time in older women but may begin in childhood. Lithium and caffeine are effective drugs for pain prophylaxis, but randomized clinical trials are required.

Hypnic headache

Treatments Lithium carbonate 200–600 mg at bedtime is an effective treatment for most patients but for those that can not tolerate Lithium, Verapamil, indomethacin, melatonin or methysergide may be tried. Two patients have also responded to flunarizine 5 mg. It has also been shown that 1–2 cups of coffee or 100–200 mg of caffeine before bed can prevent hypnic headaches. A recent review of 348 cases available in the literature has been recently published. References External links Headaches

Chronic paroxysmal hemicrania

Treatments A ten-patient study conducted by Pareja et al. found that all patients diagnosed with CPH were responsive to indomethacin and were able to completely control their symptoms. Doses of the drug ranged from 25 mg per day to 150 mg per day with a median dose of 75 mg per 24-hour period. Almost all cases of CPH respond positively and effectively to indometacin, but as much as 25 percent of patients discontinued use of the drug due to adverse side effects, namely complications in the gastrointestinal tract. According to a case study by Milanlioglu et al., 100mg of lamotrigine, an antiepileptic drug, administered twice daily alleviated all painful symptoms. No side effects were noted after two months of treatment. Dosage of lamotrigine was decreased to 50mg a day after the first two months, and no symptoms or side-effects were recorded after a three-month followup.

InternalMed_Harrison

Many experts favor verapamil as the first-line preventive treatment for patients with chronic cluster headache or prolonged bouts. While verapamil compares favorably with lithium in practice, some patients require verapamil doses far in excess of those administered for cardiac disorders. The initial dose range is 40–80 mg twice daily; effective doses may be as high as 960 mg/d. Side effects such as constipation and leg swelling can be problematic. Of paramount concern, however, is the cardiovascular safety of verapamil, particularly Prednisone 1 mg/kg up to Verapamil 160–960 mg/d 60 mg qd, tapering over 21 days Gabapentinb 1200–3600 mg/d Melatoninb 9–12 mg/d aNot available worldwide. bUnproven but of potential benefit.

[Coexistance of cluster headache and hemicrania continua: a case report].

We reported a 36-year-old man, who suffered from cluster headache (CH) associated with hemicrania continua (HC). The continuous, dull or pressure-type headache appeared on the same side of the CH during the third month of a prolonged cluster period, and fluctuated in the severity of pain. This headache was aggravated when the CH was ameliorated by the administration of lithium carbonate. This converse relationship between CH and HC persisted during an on-off trial of the lithium carbonate, and the HC was exacerbated again after the complete cessation of CH. Retrobulbar pain and nasal congestion were present as components of HC similarly to CH, but they subsided gradually and the pressure-type vascular headache over the temporal area predominated later. The continuous headache lasted more than 3 months, and responded significantly to the indomethacin at a dose of 75mg/d. The clinical course of this patient suggests that HC and CH have a common pathomechanism including hyperactivation of the trigemino-vascular reflex, and may be different in the involvement of other central pathway of pain generation. Indomethacin may deserve consideration for the treatment of continuous headache that appears during an atypical course of other primary headaches.

Shortlasting unilateral neuralgiform headache attacks with conjunctival injection, tearing, sweating, and rhinorrhea.

Three grown-up males with a long-lasting history of rather uniform, unilateral headache in the ocular-periocular area, in cluster fashion, are examined. Pain paroxysms of short duration (15-60 sec) appear up to 5-30 times per h. The headache is unilateral without side shift. Conjunctival injection appears at the very beginning of the attack and is partly massive, lasting the entire duration of the attack, and fading away at the end of it. Tearing (massive), forehead sweating (subclinical) and rhinorrhea, all on the symptomatic side, accompany the attack. In the youngest patient, the headache became chronic after clustering for six months initially, and after approximately 3 1/2 years it became bilateral. However, even in this patient, a clear unilateral pain preponderance prevails, and the autonomic disturbances are all on the original pain side. Attacks can partly be precipitated by chewing, eating (e.g. citrus fruits), moving the head, etc. The headache is completely refractory to drug therapy, including indomethacin.

[Lithium treatment of chronic Horton's headaches].

The authors used lithium carbonate in treatment of 7 patients with Horton's headaches of primarily or secondarily chronic character. In all patients the blood level of lithium was determined and it was found to reach therapeutic levels. Disappearance of attacks was achieved in 3 cases, significant improvement in 2, and in 2 cases treatment was ineffective. The mechanism of lithium action in this disease is discussed. The authors recommend lithium as worthy of use since other drugs are ineffective in this disease or they cannot be used, eg. steroids or indomethacin, in view of frequent coexistence of paptic ulcer.

Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing syndrome treated with microvascular decompression of the trigeminal nerve: case report.

Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing syndrome is a very rare disorder characterized by short-lasting neuralgiform unilateral pain affecting the orbital-periorbital area and associated with autonomic phenomena consisting mainly of conjunctival injection, tearing, and rhinorrhea. Treatment of this condition is difficult; many drugs and surgical procedures have been tried with variable results. In the literature, two cases have been described with short-term response to microvascular decompression of the trigeminal root. We present the case of a patient with short-lasting unilateral neuralgiform headache with conjunctival injection and tearing syndrome who remains asymptomatic 2 years after microvascular decompression. A 56-year-old woman was referred to our clinic because she had experienced pain in the distribution of the first left trigeminal branch during the previous 2 years. She experienced paroxysms lasting from a few seconds to 1 to 2 minutes superimposed over a dull sensation of pain involving the same territory. The paroxysms had no refractory period and were triggered by touching the eye or the left side of the face, chewing, yawning, washing her hair, and even by light. Although the paroxysms were triggered by light touch or chewing, she was able to talk or touch herself while having the paroxysm. During pain attacks, she experienced tearing and ipsilateral conjunctival injection, eyelid edema and rhinorrhea, as well as intense photophobia. A magnetic resonance imaging scan revealed a vascular structure distorting and compressing the trigeminal root. The patient underwent microvascular decompression of the trigeminal root. At surgery, there was clear compression of the trigeminal root by a superior cerebellar artery loop that was resolved by interposing a Teflon patch. The patient awoke from the operation without pain, and all the accompanying signs and symptoms, such as photophobia, disappeared. The postoperative course was uneventful, and 2 years after treatment, the patient remains asymptomatic. Microvascular decompression could be an alternative therapeutic approach to this rare syndrome.

Episodic paroxysmal hemicrania with seasonal variation: case report and the EPH-cluster headache continuum hypothesis.

Episodic paroxysmal hemicrania (EPH) is a rare disorder characterized by frequent, daily attacks of short-lived, unilateral headache with accompanying ipsilateral autonomic features. EPH has attack periods which last weeks to months separated by remission intervals lasting months to years, however, a seasonal variation has never been reported in EPH. We report a new case of EPH with a clear seasonal pattern: a 32-year-old woman with a right-sided headache for 17 years. Pain occurred with a seasonal variation, with bouts lasting one month (usually in the first months of the year) and remission periods lasting around 11 months. During these periods she had headache from three to five times per day, lasting from 15 to 30 minutes, without any particular period preference. There were no precipitating or aggravating factors. Tearing and conjunctival injection accompanied ipsilaterally the pain. Previous treatments provided no pain relief. She completely responded to indomethacin 75 mg daily. After three years, the pain recurred with longer attack duration and was just relieved with prednisone. We also propose a new hypothesis: the EPH-cluster headache continuum.

[Carotidynia as a form of presentation of paraxysmal hemicrania].

Paroxysmal hemicrania is a well-defined clinical condition about which many articles have been published. Attempts have been made to explain the response of this illness to indomethacin, suggesting its possible cervical origin. In some patients it is set off by stimulation of certain trigger zones situated in this region. The exceptional radiation of the pain seen in our patient clearly supports this theory. A 34 year old man with a past history of a similar but briefer episode 5 years previously presented to us. He complained of repeated episodes of stabbing pain with no obvious cause. The pain started at the base of the neck and radiated along the right carotid vessels to the cheek, base of the nose and ipsilateral eye. This was accompanied by injection of the conjunctivae, tears, nasal congestion and nasal discharge. Each episode lasted 15 to 30 minutes and was repeated 20 to 25 times a day without any particular relation to the time of day. The neurological examination, MR and angio-MR were normal. Before being seen by us he had been treated with prednisone and verpamil without effect. Indomethacin at a dose of 100 mg/day controlled the problem completely. We report a case of paroxysmal hemicrania with a spontaneous description of pain starting at the base of the neck and radiating along the carotid vessels. We consider this clinical description to be of interest since it supports the theories of a cervicogenic origin of this type of headache.

Lithium-induced headache.

A 23-year old woman developed headache and papilledema due to benign intracranial hypertension (BIH) while taking lithium carbonate for only seven months because of manic-depressive disease. Having discarded other causes, drug ingestion was the most likely etiology of the syndrome since it was observed that symptoms improved upon lithium withdrawal and worsened when the treatment was restarted. This report shows that BIH may appear as a side-effect of relatively short-term therapy with lithium and, therefore, funduscopic exams should be performed in every patient receiving this drug.

Treatment of the elderly patient with headache or trigeminal neuralgia.

The elderly as a whole suffer fewer headaches than the young. For the majority headache will represent a minor annoyance to be endured or treated with any available drug in the medicine chest. For some, migraine headaches or tension-type headaches become entwined with every daily activity. With the advent of modern pharmacology, headache can often be treated successfully. Trigeminal neuralgia is a source of particularly high morbidity among the elderly, but may be treated very satisfactorily with carbamazepine or baclofen. Paroxysmal hemicrania is exquisitely sensitive to indomethacin, while cluster headache patients receive relief from oxygen inhalation, corticosteroids or lithium. Headache may be the signature of the disease which leads to serious morbidity and mortality. The 'sentinel' headache of subarachnoid haemorrhage is evaluated by a physician in 15% of patients who will eventually rupture an intracranial aneurysm. Morning headache with nausea and vomiting may represent increased intracranial pressure caused by a tumour, haematoma or abscess. The elderly patient with a new headache needs emergency evaluation for temporal arteritis and rapid corticosteroid treatment if the diagnosis is confirmed, to prevent blindness. The broad spectrum of headache, at times a benign aggravation, while at others the harbinger of death, makes the careful evaluation of each headache imperative. This article attempts to make the difficult evaluation of head pain a little easier.

SUNCT syndrome associated with pituitary tumor: case report.

For twelve years, the subject of this report, a 38-year-old man, presented a clinical condition compatible with the SUNCT (short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing) syndrome. He presented a stabbing and intense daily pain located in the left pre-auricular and temporal regions. Each of these intense pain attacks lasted around one minute and presented a frequency of two to eight times per day. The pain was associated with ipsilateral lacrimation, conjunctival injection and rhinorrhea. MRI revealed a pituitary tumor with little suprasellar extent. The subjects serial assays of prolactin, GH, TSH and ACTH were within normal levels. Following transsphenoidal hypophysectomy, with complete removal of the tumor, the subject no more presented pain. The pathological diagnosis was non-secreting adenoma. Fourteen months after the surgery, he remains symptom-free.

Double-blind placebo-controlled trial of lithium in episodic cluster headache.

Lithium is widely used in the prophylaxis of episodic cluster headache without formal evidence of efficacy. Placebo-controlled clinical trials are not easy in conditions characterized by frequent severe pain. In this study, it was assumed that lithium would work quickly if at all, and placebo response would be zero. Strict diagnostic criteria excluded uncertain or atypical cases. Patients were male in so-far untreated episodes expected to last for at least 3 weeks more. In a double-blind, placebo-controlled comparison of matched parallel groups, treatment was either slow-release lithium carbonate, 800 mg/day, or placebo. After 7 days, compliance was estimated by tablet count, blood was taken for lithium assay, efficacy was assessed (attacks stopped or substantially improved) and adverse reactions were recorded. The study was stopped after planned sequential analysis of the 27th patient (13 on lithium, 14 on placebo). Estimated compliance was usually but not always good. Plasma lithium levels were mostly in the range 0.5-0.6 mmol/l on lithium, zero on placebo. Cessation of attacks within 1 week occurred in two patients in each group, substantial improvement in 6/14 (43%) on placebo, 8/13 (62%; NS) on lithium. Only minor adverse events were reported. Lithium treatment was therefore associated with a useful subjective improvement rate but the assumptions made at outset had proved wrong. The trial was stopped because superiority over placebo could not be demonstrated. There were lessons for future trials.

[Amnesic presentations of the compulsive obsessional confusions (about 3 patients appearing in a consultation of memory)].

Disorders or complaints of memory are a frequent cause of consultation in depression, major anxiety and psychiatry disease with personality disorders. We report 3 patients with obsessive compulsive disorder (OCD), without diagnosis and treatment, examined in a specialized memory consultation. They always had OCD with cognitive checking. Diagnosis of transient global amnesia and temporal complex seizure were discussed in 2 cases. Psychometric impairment only was observed in first free recall of a verbal memory task and was no specific. Behavioural during testing seemed to be very important to analyse. First, a 49-year-old man consulted because he had stereotyped transient amnesia lasted one minute, 2 or 3 times a week, since 6 months. He was a teacher. Transient amnesia always occurred during lessons. Suddenly he didn't know where he was or what he was speaking about. Episodes lasted one minute. After them, he had no confusion and no difficulty in concentration but intense anxiety. In an another hand, when he was in his car, after lessons, he could forget where he was during some minutes. CT scan and EEG were normal. Neuropsychological tests only objectived impairment in first free recall of Grober and Buschke's words. Patient explained that he could not prevent to check responses. He told us checking obsessive compulsive disorder during since long time ago. We discussed clear differences which existed between seizure and ruminations or preoccupations. Secondly, a 55-year-old woman was afraid of her memory performances. She was medical secretary and had no problem in her work but she would like a memory consultation to reassure herself. She was neither depressed nor anxious. She presented curious production in fluency task. She had to produce as many animals's names as possible: she could say 35 names which was an excellent performance but only in alphabetic order! Neuropsychological tests objectived impairment in her first free recall of Grober and Buschke's words. She tried in her first free recall to remember words in alphabetic order. She explained how she was bound to range everything in alphabetic order! She had a lot of rituals. She thought that she had an obsessive compulsive disorder but never consulted about this. The observation illustrated suspiscions about memory operations which could be observed in patients group with obsessive compulsive disorders. Finally, a 62-year-old man told us that he had presented a transient global amnesia during 4 hours. He had an important appointment and was upset about that. He didn't go to it and wandered in his flat. He always asked the same questions and forgot everything. He had no neurological deficit. He was anxious, sad and cried several times. He perfectly remembered the episod and thought that he had a panic attack! Verbal memory tests only objectived difficulties in his first free recall of Grober and Buschke words as the two others patients. He had a story of obsessive compulsive disorder with checking and rituals. In this observation, we discussed clear differences which existed between panic attacks and global transient amnesia. We analyzed patterns of neuropsychological performances which illustrated clinical features of obsessive compulsive disorder. These three patients impaired in their first free recall of verbal memory task. It is not a specific result. We observed during psychometric evaluation, strategic processing which impaired episodic memory: patients tried to check their performances. Memory complaints only were observed in checking obsessive compulsive disorder. It is a difficulty or a doubt about memory capacities. Difficulties could be due to particular cognitive processes who pertubate normal memory capacities.

[Lithium treatment and hyperparathyroidism].

Lithium treatment, which is extensively used in bipolar affective disorders, may give rise to hypercalcaemia and sometimes to irreversible hyperparathyroidism. We present a patient who developed hyperparathyroidism following long-term treatment with lithium. After 15 years on lithium the patient was diagnosed with hypercalcaemia; at the same time the patient stopped her lithium medication. Two years later she developed depression with psychotic symptoms and was given electroconvulsive treatment. Measurements of serum calcium and parathormon showed that she had developed hyperparathyroidism. Neck exploration was performed, and two parathyroid adenomas (weight 650 mg and 880 mg), which had been detected by scintigraphy, were removed. Lithium treatment was restarted. One year later she was normocalcaemic and her mood was normal. In lithium-induced hyperparamyroidism, lithium should be replaced with other mood stabilizers, preferably an antiepilepticum. If cessation of lithium therapy does not lead to normocalcaemia, parathyroidectomy is indicated.

Side-locked headache as the chief complaint of inflammatory orbital pseudotumor (myositic form): a case report.

The case of a 38-year-old woman with continuous unilateral side-locked headache is reported. She had continuous right-sided periorbital pain of mild to moderate intensity for the past 5 months. She also reported a few episodes of pain exacerbations every day. She had no autonomic features. Based on a normal CT scan ordered by her general physician, we started indomethacin (150 mg/day) as well as celecoxib (400 mg/day) for 2 weeks, without relief. Oral prednisone for 6 days provided important relief, and she stayed on daily use of steroids, refusing other forms of therapy. After 5 months she developed orbital and eyelid edema, with painful restrictions to eye movement. Orbital MRI and pathological exam demonstrated inflammatory orbital pseudotumor (myositic form).

[Cluster headache and other trigeminal-autonomic headaches].

Cluster headache is a primary headache with a male predominance that presents in two forms: episodic and chronic, occurring at 45-to 60-day intervals with one to three headaches a day lasting 45 min to 2 h. An attack starts by a violent unilateral retro-ocular pain with sympathetic signs such as tearing and rhinorrhea. Diagnosis is made by questioning and therefore requires no complementary tests. Treatment for the attack consists of injectable sumatriptan or oxygen therapy, with long-term treatment with verapamil, lithium salts, or Topiramate; in certain cases in which the number of attacks is greater than two, injections of corticosteroids at the emergence of the Arnold nerve can be used, or in cases of attacks resistant to all treatments, hypothalamus stimulation surgery can be useful.

SUNCT syndrome responsive to gabapentin (Neurontin).

A 48-year-old male suffering with SUNCT (severe unilateral neuralgiform headache with conjunctival injection and tearing, rhinorrhea and sub-clinical sweating) presented in 1996 after a 10-year history of multiple failed therapies. The symptoms included strictly left-sided ocular, as well as facial and temple pain. The pain attacks were burning, sharp, shooting and occurred 25 times daily, lasting 2 to 3 minutes with tearing and conjunctival injection. There was no associated nausea or vomiting, but there was photophobia. No other autonomic changes were reported and the pain was not triggerable. Initially Indocin (indomethacin) was tried without significant benefit. Gabapentin (Neurontin) was then started with improvement at 1800 mg per day. The patient was then lost to follow-up for 3 years, as he moved from the Los Angeles area. He returned in 1999 having stopped the gabapentin after his prescription ran out in 1996, reporting the pain returned immediately. Again gabapentin was prescribed and at 900 mg three times daily he has been pain free for 12 months.

[Lithium therapy in Horton's neuralgia: preliminary results].

Prophylactic use of Lithium salts in patient suffering from cluster headaches has been evaluated looking at the mean number of headache attacks in one critical period and the mean weekly duration of the period itself. These two elements have been compared to those observed on other drugs treatments. Plasma Lithium monitoring has been performed weekly during the trial. Authors discuss the results reported and the hypothetic basis of them.

Neurology_Adams

Raskin described a headache syndrome in older patients that shares with cluster headache a nocturnal occurrence (hypnic headache). It also may occur with daytime naps. However, it differs in being bilateral and unaccompanied by lacrimation and rhinorrhea. He has successfully treated a number of his patients with 300 mg of lithium carbonate or 75 mg of sustained-release indomethacin at bedtime. The nosologic position of this hypnic headache syndrome is undetermined. Despite these considerations, the most hazardous cause of headache in the elderly is temporal (cranial) arteritis with or without polymyalgia rheumatica, as discussed further on.

[Recurrent aseptic osteonecrosis in Crohn's disease - extraintestinal manifestation or steroid related complication?].

A 57-year-old woman complained about increasing pain and weakness in her hips and legs. 7 months earlier active (Crohn's) ileocolitis had been diagnosed. She had received several bouts of steroids and had been in clinical remission for 12 weeks under a dosage of 40 mg/d prednisone. Clinical examination, laboratory work up, x-rays and MRI of the pelvis, bone scan, neurologic examination and muscle biopsy showed unspecific results. THERAPY AN COURSE: Steroides were tapered and replaced by weekly intramuscular methotrexate 20 mg which resulted in long lasting clinical remission. Pain and weakness persisted. 6 months later MRI revealed osteonecrosis of both femoral heads. 4 1/2 years after the initial diagnosis of Crohn's disease the patient complained about pain in her lower legs without evidence of osteonecrosis in MRI. Another 2 years later avascular osteonecrosis was diagnosed by tibial bone biopsy. Now MRI verified patchy osteonecrosis of the tibiae. Further osteonecrosis of the left foot were diagnosed by MRI ten years after initial diagnosis of Crohn's disease in the now 67-year-old patient. She is still in remission on weekly intramuscular 15 mg methotrexate. The long interval between steroid treatment and recurrent avascular bone necrosis as well as the unusual pattern of bone involvement indicate that osteonecrosis is an extraintestinal manifestation of Crohn's disease. More reports and comparative studies are necessary to give more evidence that avascular osteonecrosis is an extraintestinal manifestation of inflammatory bowel disease.

Pseudotumor cerebri secondary to lithium carbonate.

Three patients were initially seen with headache, blurred vision, and papilledema while taking lithium carbonate for their respective bipolar affective disorder. A diagnosis of pseudotumor cerebri was made in each case when a thorough evaluation revealed only elevated intracranial pressure. Two of the patients had complete resolution of their symptoms and papilledema after discontinuing use of the drug. Increased intracranial pressure with papilledema persisted in the third patient when she failed to adjust psychiatrically, necessitating continuance of the lithium carbonate therapy. A history of lithium carbonate ingestion should be sought in patients with the syndrome of pseudotumor cerebri. All patients receiving this drug should have a regular funduscopic examination.

[Complete atrioventricular block during lithium therapy within  therapeutic range].

A 69-year-old man came to the emergency unit because of vertigo and presyncope. A bipolar disorder - known since an age of 15 years - has been treated with 2 × 450 mg lithium and 100 mg perazine per day for several years (no other medications). With the exception of a low heart rate (36/min) clinical examination findings were unremarkable. Electrocardiography revealed a permanent complete atrioventricular block with a heart rate of 36/min. Echocardiography showed a normal left ejection fraction (EF 65 %). Laboratory tests were mainly unremarkable, particularly the lithium levels (0,7 mmol/l) were within the therapeutic range. Continuous treatment with orciprenaline stabilized the heart rate at an average of 52/min. After pacing with a provisional pacemaker a permanent pacemaker was implanted without complications, and the symptoms of vertigo and dizziness disappeared. Pacemaker checkup on the following day still showed a complete atrioventricular block with a heart rate of 28/min. Complete atrioventricular block secondary to chronic lithium therapy even in therapeutic levels is a rare complication with poor prognosis. Therefore it should be treated consequently.