bluuebunny/crossref_metadata_2025_split huggingface dataset:
Nocturnal plasma prolactin rise in patients with complex partial seizures
Authors: Molaie Majid , Culebras Antonio , Miller Myron 
Published: January 2005

AbstractWe measured plasma prolactin and cortisol concentrations in 12 epileptic men with complex partial seizures during all‐night polygraphic recording, with continuous video monitoring and sequential blood sampling at 30‐minute intervals. There was a significant but moderate elevation of all‐night mean plasma prolactin concentration (p < 0.001), but not of cortisol, in epileptics when compared with normal subjects studied in a similar fashion. The data obtained in this environmentally controlled study of males with complex partial seizure disorder indicate a disruption of prolactin regulatory mechanisms resulting in a modest elevation of plasma concentrations independent of ictal electrographic discharges.
Anticerebellar antibodies in serum and cerebrospinal fluid of a patient with oat cell carcinoma of the lung and paraneoplastic cerebellar degeneration
Authors: Greenlee John E. , Lipton Howard L. 
Published: January 2005

AbstractA 56‐year‐old man was seen with subacute cerebellar degeneration and was found to have oat cell carcinoma of the lung. Antibodies to cerebellar Purkinje cells and granule cells were detected in both serum and cerebrospinal fluid (CSF), and intrathecal antibody synthesis was suggested by serum CSF antibody ratios, CSF IgG index, and CSF IgG synthesis rate. The patient's condition improved slightly with plasmapheresis, corticosteroids, and therapy of his underlying tumor, but he continued to exhibit a severe cerebellar deficit until his death more than one year later. Paraneoplastic cerebellar degeneration may be accompanied by synthesis of anticerebellar antibodies, both systemically and within the central nervous system.
Monoclonal antibody analysis of mononuclear cells in myopathies. III: Immunoelectron microscopy aspects of cell‐mediated muscle fiber injury
Authors: Arahata Kiichi , Engel Andrew G. 
Published: January 2005

AbstractWe have previously obtained light microscopical immunocytochemical evidence for cell‐mediated muscle fiber injury and destruction in polymyositis and inclusion body myositis [1, 2]. To evaluate further interactions of the different cell phenotypes with each other and with the muscle fibers, the T8, T4, and Leu 7 markers in 7 cases of polymyositis and in 9 cases of inclusion body myositis were localized by immunoelectron microscopy. In the early stages of the cell‐mediated process, T8+ cells and macrophages are apposed against, and/or send spikelike processes into, nonnecrotic muscle fibers. Leu‐7+ cells penetrate fibers infrequently, and T4+ cells do not penetrate muscle fibers. Subsequently, an increasing number of T8+ cells and macrophages traverse the basal lamina; focally replace, displace, or compress the fiber; and spikes from these cells honeycomb the adjacent muscle fiber regions. The macrophages contain only few heterophagic vacuoles and therefore act in a cytotoxic rather than a phagocytic capacity. The integrity of the muscle fiber surface membrane facing the invading cells is maintained, but the possibility also exists that the membrane is damaged and rapidly repaired, or that the damage cannot be detected by electron microscopy. Nearby fiber regions often show either degenerative or regenerative changes. Ultimately, segments of the entire muscle fiber are replaced by the invading cells.
A new syndrome of anosmia, ichthyosis, hypogonadism, and various neurological manifestations with deficiency of steroid sulfatase and arylsulfatase C
Authors: Sunohara Nobuhiko , Sakuragawa Norio , Satoyoshi Eijiro , Tanae Ayako , Shapiro Larry J. 
Published: January 2005

AbstractWe describe a family consisting of 3 affected men with congenital ichthyosis, anosmia, hypogonadism, nystagmus with decreased visual acuity, strabismus, hypopigmentation of the iris, and mirror movements of the hands and feet. Two of them had limitation of ocular movement and unilateral renal agenesis or hypoplasia. The condition appears to be inherited as an X‐linked recessive trait. Clinical, pathological, and biochemical evaluations were compatible with a diagnosis of X‐linked ichthyosis. Steroid sulfatase and arylsulfatase C activities in leukocytes and fibroblasts were markedly diminished in the affected patients. Their hypogonadism was due to decreased luteninizing hormone–releasing hormone secretion (hypogonadotropic). Hyposecretion of antidiuretic hormone was also recognized. Chromosome analysis of leukocytes and skin fibroblasts revealed a normal 46,XY male karyotype in all of the patients.
Topical therapy for oropharyngeal symptoms of myasthenia gravis
Authors: Dooley J. M. , Goulden K. J. , Gatein J. G. , Gibson E. J. , Brown B. St. J. 
Published: January 2005

AbstractInhibitors of acetylcholinesterase are commonly used in the treatment of myasthenia gravis. We studied a patient with mainly bulbar myasthenia gravis who did not tolerate oral pyridostigmine despite a clear clinical response. Treatment with nebulized pyridostigmine reduced her symptoms without systemic side effects. This route of therapy may benefit other patients.
Status epilepticus: Divergence of sympathetic activity and cardiovascular response
Authors: Benowitz Neal L. , Simon Roger P. , Copeland James R. 
Published: January 2005

AbstractTo study changes in plasma catecholamine concentrations and their relationship to changes in systemic and pulmonary vascular pressures, we induced status epilepticus by intravenous administration of bicuculline in 5 anesthetized, paralyzed, ventilated adult sheep. Status epilepticus was accompanied by marked elevations in plasma epinephrine and norepinephrine levels (390‐fold and 163‐fold, respectively) and by marked elevations in systemic and pulmonary vascular pressures. Vascular pressure returned to normal in 60 minutes, despite plasma catecholamine levels that remained elevated for the 3‐hour duration of the study. Mild metabolic acidosis and hyperkalemia were also noted. We discuss the implications of these findings with regard to the management of hypotension during status epilepticus.
Accumulation of glycogen in sural nerve axons in adult‐onset type III glycogenosis
Authors: Ugawa Yoshikazu , Inoue Kiyoharu , Takemura Tamiko , Iwamasa Teruo 
Published: January 2005

AbstractA 42‐year‐old man with adult‐onset type III glycogenosis (Cori's disease) developed a gradually progressive polyneuropathy with markedly reduced activity of muscle amylo‐1,6‐glucosidase and glycogen accumulation within all elements of biopsied sural nerve, including axons, as shown by ultrastructural assessment.
Long‐term follow‐up of early dopa treatment in Parkinson's disease
Authors: Markham Charles H. , Diamond Shirley G. 
Published: January 2005

AbstractA group of 19 patients with Parkinson's disease, who began dopa therapy 1 to 3 years after onset of symptoms, were followed for 12 years. They were evaluated every 3 to 4 months on the UCLA disability scale and the Hoehn and Yahr functional classification scale. This present group was compared to two other historical groups reported on earlier [8], one that began treatment with dopa 4 to 6 years after onset of symptoms and the other that began dopa treatment 7 to 9 years after onset of symptoms. Results on both scales showed that the mean disability scores of the three groups did not differ significantly when duration of disease was matched, even though duration of dopa therapy among the groups varied from 1 to 12 years. The highly significant differences between the groups prior to dopa treatment continued throughout the study. These longitudinal data support the conclusion that the worsening over time is the result of progression of Parkinson's disease and not a result of the duration of dopa treatment. Early treatment improves the beginning years of the disease and has no adverse effect on later years.Twelve years after beginning dopa therapy, 32% of the present group had died at a mean age of 76.0, in contrast to 50% (mean age, 74.7) and 57% (mean age, 71.5) in the groups who began dopa increasingly later in the course of their disease. Incidence of dementia increased with disease duration regardless of treatment duration. In the present group, 3 of 19 patients showed severe cyclinical fluctuations, but that complication as well as persistent chroreoathetosis and the rarely seen true on‐off effect also could not be related to duration of dopa treatment.
Oligoclonal immunoglobulin bands in cerebrospinal fluid of a patient with lymphocytic choriomeningitis
Authors: Jamieson Dara G. , Mehta Pankaj D. , Lavi Ehud 
Published: January 2005

AbstractWe describe a patient with meningitis caused by lymphocytic choriomeningitis virus. Oligoclonal IgG bands were found in the patient's cerebrospinal fluid during acute and convalescent stages of the illness, and results of liver function tests were abnormal. Acute attacks of lymphocytic choriomeningitis virus infection in humans can be added to the list of diseases associated with cerebrospinal fluid oligoclonal IgG.
Pathogenesis of diabetic neuropathy
Authors: Johnson Peter C. , Doll Susan C. , Cromey Douglas W. 
Published: January 2005

AbstractSamples of lumbosacral trunk, posterior tibial nerve, and sural nerve obtained at autopsy from diabetic and nondiabetic patients without mononeuropathy multiplex were evaluated using 1‐μ‐thick epoxy sections and teased nerve fiber preparations. Focal fascicular lesions characterized by reduced density of myelinated axons within fascicles were found predominantly in the specimens from diabetics, mainly in the posterior tibial nerve and lumbosacral trunk. In severe examples, the perineurium and even surrounding epineurium were damaged, stamping the lesions as ischemic. In addition, identical lesions were found in biopsies of nerves of nondiabetics with vascultis. Density of myelinated fibers at the three sites demonstrated a proximal–distal graded loss that was significantly greater in the diabetic samples. The loss from the lumbosacral trunk to the posterior tibial nerve was correlated with the density of focal lesions in the lumbosacral trunk in the diabetic (p = 0.025), indicating that distal fiber loss was partly due to the focal lesions. Teased nerve fiber abnormalities were common only in sural nerves of diabetics, suggesting that they are secondary. We conclude that beyond the possible metabolic abnormalities involved in the genesis of diabetic polyneuropathy, focal fascicular lesions, likely due to diabetic microangiopathy, are also important in the development of diabetic neuropathy.
Magnetic resonance imaging in subacute necrotizing encephalomyelopathy (Leigh's disease)
Authors: Koch Thomas K. , Yee Melvin H. C. , Hutchinson Harrol T. , Berg Bruce O. 
Published: January 2005

AbstractMagnetic resonance imaging (MRI) offers a sensitive alternative to computed tomography (CT) for lesion localization. Patients with subacute necrotizing encephalomyelopathy (SNE) may be diagnosed by finding focal lesions on CT that correspond to sites of anatomical involvement. We report serial CT and MRI scanning findings in a patient with clinical, radiographic, and laboratory evidence of SNE. MRI was more sensitive in detecting lesions involving the basal ganglia, brainstem, and cortex. We believe MRI is a valuable and sensitive means to establish an antemortem diagnosis of SNE.
Encephalitis caused by Coxiella burnetii
Authors: Brooks Robert G. , Licitra Carmelo M. , Peacock Marius G. 
Published: January 2005

AbstractAcute infection with Coxiella burnetii usually results in a self‐limited illness, but it can occasionally cause chronic endocarditis or hepatitis. Headache is a common presenting symptom of acute infection with this agent, but specific neurological abnormalities are rare. WE report the case of a patient with acute Q fever that caused frank encephalitis. We also review the literature on central nervous system disease attributable to C. burnetii.
Preferential loss of large lumbar primary sensory neurons in carcinomatous sensory neuropathy
Authors: Ohnishi Akio , Ogawa Michiko 
Published: January 2005

AbstractMorphometric studies at autopsy of a patient with sensory neuropathy associated with small‐cell lung carcinoma showed preferential loss of large‐diameter sensory nerve cell bodies (fifth lumbar dorsal root ganglion), marked decrease of large myelinated fibers in the dorsal root and sural nerve, and almost total loss of myelinated fibers in the fasciculus gracilis.
Combined 1H and 31P nuclear magnetic resonance spectroscopic studies of bicuculline‐induced seizures in vivo
Authors: Petroff Ognen A. C. , Prichard James W. , Ogino Takashi , Avison Malcolm , Alger Jeffry R. , Shulman Robert G. 
Published: January 2005

AbstractUsing a 1.89‐Tesla spectrometer, 1H and 31P nuclear magnetic resonance spectra were acquired from the brains of paralyzed rabbits ventilated with 30% oxygen in nitrous oxide. Intracellular pH and changes in lactate concentration in the cerebrum were monitored by nuclear magnetic resonance methods during and after bicuculline‐induced seizures, together with the electroencephalogram, heart rate, and arterial blood pressure. During seizures lasting more than an hour, cerebral intracellular pH became acidic, the cerebral lactate level rose rapidly, and both changes persisted as long as 2 hours without signs of recovery. After less prolonged seizures, lactate elevations were no less persistent, despite nearly complete recovery of intracellular pH and the electroencephalogram.
Parkinson's disease: Nigral receptor changes support peptidergic role in nigrostriatal modulation
Authors: Uhl George R. , Hackney Gail O. , Torchia Mary , Stranov Victoria , Tourtellotte Wallace W. , Whitehouse Peter J. , Tran Vinh , Strittmatter Steven 
Published: January 2005

AbstractAutoradiographic studies reveal densities of binding to somatostatin, neurotensin, μ‐opiate, and benzodiazepine receptors in substantia nigra specimens from neurologically normal human brains. Binding to nigral angiotensin converting enzyme is also dense, whereas more modest densities of κ‐opiate, dopamine, and serotonin receptors are noted. In nigral specimens taken from patients with idiopathic Parkinson's disease, substantial reductions in somatostatin, neurotensin, μ‐opiate and κ‐opiate receptors contrast with more modest reductions in dopamine and benzodiazepine I receptor subtypes. Angiotensin converting enzyme, serotonin, and benzodiazepine II binding are virtually unaltered. These results underscore the likelihood of strong peptidergic influences on normal and pathologically altered human nigrostriatal circuitry.
Selective saccadic palsy caused by pontine lesions: Clinical, physiological, and pathological correlations
Authors: Hanson Maurice R. , Hamid Mohamed A. , Tomsak Robert L. , Chou Samuel S. , Leigh R. John 
Published: January 2005

AbstractTwo patients suffered a selective deficit of voluntary saccades and quick phases of nystagmus after hypoxic–ischemic insults during open‐heart surgery. All voluntary saccades, in both horizontal and vertical planes, were slow, and quick phases of vestibular and optokinetic nystagmus were absent. Smooth pursuit, the vestibuloocular reflex, the ability to hold steady eccentric gaze, and vergence eye movements were all preserved. Pathological studies in 1 patient confirmed neuronal necrosis and gliosis, consistent with ischemic lesions involving the median and paramedian pontine reticular formation and median basis pontis but sparing the rostral mesencephalon and rostral interstitial nucleus of the medial longitudinal fasciculus. These findings, taken with data from experimental studies, support the hypothesis that each functionally defined class of horizontal eye movements is controlled by a separate neural substrate that projects independently to the abducens nuclei. In addition, these data suggest that the rostral interstitial nucleus of the medial longitudinal fasciculus is dependent on inputs from the paramedian pontine reticular formation for the programming of normal vertical saccades.
Familial myoclonic dementia masquerading as Creutzfeldt‐Jakob disease
Authors: Little Brian W. , Brown Paul W. , Rodgers‐Johnson Pamela , Perl Daniel P. , Gajdusek D. Carleton 
Published: January 2005

AbstractWe describe a kindred with 7 confirmed and 2 probable cases of subacute dementia accompanied by myoclonus. The inheritance pattern is consistent with autosomal dominance and shows anticipation. The pathological changes involve marked gliosis with neuronal loss of the dorsomedial and midline thalamic nuclei, with lesser involvement of the anterior, lateral, and posterior thalamic nuclei. Medullary olivary hypertrophy is prominent. Spongiform change is minimal or absent. Attempted disease transmission to primates from 3 affected family members has been unsuccessful; Western immunoblot tests have likewise failed to detect the presence of specifically reactive 27–30 kD Mr proteins in brain tissue from 2 of these patients. We conclude that this family has a rare inherited neurological systems degeneration with associated thalamic dementia, the clinical course of which is very similar to Creutzfeldt‐Jakob disease.
Peripheral neuropathy associated with mitochondrial myopathy
Authors: Yiannikas C. , McLeod J. G. , Pollard J. D. , Baverstock J. 
Published: January 2005

AbstractTwenty patients with mitochondrial myopathy were investigated for the presence of peripheral neuropathy. There were clinical features of a mild sensorimotor neuropathy in 5 patients (25%) and nerve conduction studies were abnormal in 10 patients (50%). Electrophysiological studies of the whole group showed significant impairment of motor and sensory conduction, compared with controls. Sural nerve biopsy and morphometric studies were performed on 4 patients with clinical neuropathy. There was a reduction in density of myelinated fibers and electron microscope features of axonal degeneration affecting myelinated and unmyelinated fibers. Abnormal mitochondria containing paracrystalline inclusions were seen in the Schwann Cell cytoplasm of two nerves.
Immunoglobulin abnormalities in cerebrospinal fluid and blood over the course of lymphocytic meningoradiculitis (Bannwarth's syndrome)
Authors: Henriksson Annemarie , Link Hans , Cruz Mabel , Stiernstedt Göran 
Published: January 2005

AbstractThe conditions of 5 patients with untreated lymphocytic meningoradiculitis (Bannwarth's syndrome, probably equivalent to Lyme disease) with serologically confirmed infection resulting from Borrelia spirochetes were followed with repetitive lumbar punctures up to 221 days after the onset of symptoms. Using a protein A plaque assay, high numbers of IgG‐, IgM‐, and IgA‐producing cells were found in the cerebrospinal fluid (CSF), whereas there were mostly normal numbers of immunoglobulin‐producing cells in peripheral blood. A markedly increased CSF IgM index and an elevated IgG index were observed in all patients during the early phase, reflecting production of these immunoglobulins within the central nervous system. All patients had oligoclonal IgG bands in the CSF that persisted during follow‐up; in the 2 patients tested, the bands contained Borrelia antibodies. Most serum immunoglobulin concentrations were normal and in only 1 patient was it possible to detect in serum some of the oligoclonal IgG bands present in CSF. Declining numbers of CSF cells producing immunoglobulin and decreasing immunoglobulin index values were observed during follow‐up, but 3 patients had an elevated CSF IgM index in the presence of normal IgG and IgA indices when examined during the later phases of disease. An intense and prolonged IgM response within the central nervous system seems to be a characteristic of the disease.
Evaluation of patients with multiple sclerosis by evoked potentials and magnetic resonance imaging: A comparative study
Authors: Cutler John R. , Aminoff Michael J , Brant‐Zawadzki Michael 
Published: January 2005

AbstractWe compared the diagnostic usefulness of evoked potential (EP) studies and magnetic resonance imaging (MRI) in the evaluation of 27 patients with definite or probable multiple sclerosis (MS). MRI scans demonstrated multiple lesions in 21 patients whereas EP studies showed multiple abnormalities in 14 patients (4 of whom had only somatosensory EP abnormalities). Eighteen patients has similar MRI and EP results (e.g., normal or multiple abnormalities), 8 had multiple abnormalities shown by MRI but normal or single‐modality abnormal EPs, and 1 had multiple abnormal EPs but a normal MRI scan. There was no significant difference in the sensitivity of the two techniques in detecting multiple lesions in the patients with definite MS, whereas among those with probable MS, MRI had a significantly higher yield. Seventeen patients showed clinical evidence of posterior fossa involvement, 6 patients had abnormal brainstem auditory evoked potentials (BAEPs), and 4 patients had areas of increased signal intensity revealed by MRI of the brainstem. There was no clinical evidence of brainstem involvement in 2 patients with BAEP abnormalities, 2 with an abnormal posterior fossa shown by MRI, and one patient with abnormalities shown by both BAEP and MRI. We conclude that MRI is more sensitive in detecting multiple lesions than are multimodality EP studies, but that BAEP assessment may be slightly more sensitive than MRI in detecting brainstem lesions.
The lennox‐gastaut syndrome: Metabolic subtypes determined by 2‐deoxy‐2 [18F]fluoro‐d‐glucose positron emission tomography
Authors: Chugani Harry T. , Mazziotta John C. , Engel Jerome , Phelps Michael E. 
Published: January 2005

AbstractWe employed positron emission tomography (PET) with 2‐deoxy‐2[18F]fluoro‐D‐glucose (FDG) to study local cerebral glucose utilization in 15 children who had Lennox‐Gastaut syndrome. Our results show that LGS can be classified into four predominant subtypes, each with a distinct metabolic pattern: (1) unilaterla focal hypometabolism, (2) unilateral diffuse hypometabolism, (3) bilateral diffuse hypometabolism, and (4) normal. Functional disturbances seen on FDG‐PET did not always correlate with abnormalities revealed by x‐ray computed tomographic scan. This classification of Lennox‐Gastaut syndrome into four major metabolic subtypes not only provides a new perspective toward understanding cerebral function in this complex syndrome, but may also prove useful in the clinical management of these patients.
Risk factors for complex partial seizures: A population‐based case‐control study
Authors: Rocca Walter A. , Sharbrough Frank W. , Hauser W. Allen , Annegers John F. , Schoenberg Bruce S. 
Published: January 2005

AbstractThis investigation is to our knowledge the first population‐based case‐control study of risk factors for complex partial seizures (CPS). Included in the study were all patients with onset of complex partial seizures before age 35, who were residents of Rochester, Minnesota, at the time of diagnosis between 1935 and 1979, and who were also born in Rochester (n = 82). Two control subjects were matched to each patient, and for both patients and control subjects, the unique records‐linkage system for residents of Rochester was used to obtain information about possible risk factors. A history of epilepsy or febrile seizures in the mother, febrile seizures, neonatal convulsions, cerebral palsy, head trauma, and viral encephalitis were significantly more common in patients than in control subjects (p < 0.05). None of the prenatal or perinatal factors investigated were found to be associated with complex partial seizures, except for being small for gestational age at birth. This factor lost significance after adjustment for cerebral palsy.