bluuebunny/crossref_metadata_2025 huggingface dataset:
Intensive monitoring of interictal psychosis in epilepsy
Authors: Ramani Venkat , Gumnit Robert J.
Published: January 2005

Ten epileptic patients developed interictal psychosis while being treated in hospital for seizure control. They were subjected to intensive behavioral, video‐electroencephalographic, and serum anticonvulsant monitoring for an average of 7.1 weeks in a specialized epilepsy unit. In 9 patients, the interictal psychosis was indistinguishable from acute schizophrenia. Only 5 of these patients had complex partial seizures; the other 4 showed evidence of generalized epilepsies. Thus a “unique” association between schizophreniform psychosis and complex partial seizures, noted by previous authors, could not be confirmed. Only 1 patient showed normalization of the electroencephalogram during psychosis and an inverse relationship between psychosis and seizure frequency. In most cases the emergence of psychosis could not be explained. Interictal psychosis in epilepsy appears to be a spectrum of disorders that may be multifactorially determined.
Recurrent paraplegia with total recovery from spontaneous spinal epidural hematoma
Authors: Hernandez D. , Vinuela F. , Feasby R. T. E.
Published: January 2005

A 51‐year‐old man had three episodes of complete paraplegia and anesthesia, each lasting 30 to 120 minutes, over the course of five hours. Myelography and computerized tomographic scanning showed an epidural hematoma at T11–T12 for which no cause was found. Complete spontaneous recovery occurred.
Central neurogenic hyperventilation in an awake patient with brainstem astrocytoma
Authors: Rodriguez Moses , Baele Philippe L. , Marsh H. Michael , Okazaki Haruo
Published: January 2005

A patient had the rare combination of central neurogenic hyperventilation (Pa of 9 torr) and a normal level of consciousness for eight days. Morphine attenuated but never corrected the hyperventilation. Experimental effects of hypocapnia, which decreases both cerebral blood flow and metabolism in humans, are at odds with the normal mentation initially seen in this patient despite her marked and persistent hypocapnia. Death occurred after progressive brainstem dysfunction. Pathological study showed a well‐differentiated astrocytoma involving primarily the medulla and pons, with scattered tumor foci throughout the entire neuraxis. Possible mechanisms for central neurogenic hyperventilation are discussed briefly in relation to the pathological findings and the observed response to morphine.
Biopsy‐proved Alzheimer disease presenting as a right parietal lobe syndrome
Authors: Crystal Howard A. , Horoupian Dikran S. , Katzman Robert , Jotkowitz Seymour
Published: January 2005

A progressive right parietal lobe disorder charcterized by astereognosis and pseudoathetosis developed in a 57‐year‐old woman. Two years later, the patient began to show signs of intellectual deterioration. Biopsy of the right frontal lobe revealed numerous plaques and tangles. The patient's right parietal lobe dysfunction was probably the first manifestation of Alzhemer disease. On rare occasions, Alzhemier disease can present as a focal syndrome with findings referable to a localized area of maximal involvement.
Catencholamine neuron systems in brain
Authors: Moore Robert Y.
Published: January 2005

The organization of catecholamine neuron systems in brain systems in brain is described. Dopamine neuron systems form components of larger, more general neuron systems and are organized into conventional topographic projectoins. Norepinephirne neuron systems innervate widespread areas of the neuroxais and appear to function in the regulation of other neuronal systems.
Acute neurological dysfunction associated with destructive lesions of the basal ganglia in children
Authors: Goutières F. , Aicardi J.
Published: January 2005

Three children ranging in age from 1 to 41/4 years suffered an acute illness with disturbance of consciousness followed by motor rigidity, loss of spontaneous movements and language, axial hypotonia, and a stereotyped response to any kind of stimulus. In two cases, images on computerized tomography were consistent with necrosis of the lenticular and caudate nuclei. The acute onset was followed by later improvement and stabilization. One child died, probably not as a direct consequence of the neurological disorder; autopsy showed bilateral necrosis of the putamina.

We suggest that three patients, together with two previously reported cases, constitute a clinically and radiologically recognizable subgroup among the heterogeneous disorders that produce bilateral striatal necrosis in children. The cause of the syndrome is unknown.
Cerebral metabolism and atrophy in huntington's disease determined by 18FDG and computed tomographic scan
Authors: Kuhl David E. , Phelps Michael E. , Markham Charles H. , Metter E. Jeffrey , Riege Walter H. , Winter James
Published: January 2005

Patterns of local cerebral glucose utilization were measured with positron emission computed tomography using the 18F‐fluorodeoxyglucose method in 13 patients with Huntington's disease (HD), 15 subjects at risk for HD, and 40 normal control subjects. These data were compared with computed tomographic measures of cerebral atrophy, with age, and with duration and severity of symptoms. The results indicate that in HD there is a characteristic decrease in glucose utilization in the caudate and putamen and that this local hypometabolism appears early and precedes bulk tissue loss. In contrast to patients with senile dementia, in these HD patients glucose utilization typically was normal throughout the rest of the brain, regardless of the severity of symptoms and despite apparent shrinkage of brain tissue. Our results suggest the possibility that the caudate may be hypometabolic in some asymptomatic subjects who are potential carriers of the autosomal dominant gene for HD.
Correlation of isotopic cisternographic patterns in multiple sclerosis with CSF IgG values
Authors: Bartolini S. , Inzitari D. , Castagnoli A. , Amaducci L.
Published: January 2005

Thirty‐eight patients with multiple sclerosis (MS) were examined with isotopic cisternography (IC) in order to study cerebrospinal fluid (CSF) dynamics. Cisternography was also performed in 15 patients with amyotrophic lateral sclerosis and in 14 with senile dementia of the Alzheimer type as controls. IC pattern of “mixed” type was found in 18 MS patients and all those with Alzheimer senile dementia examined, while the IC examination did not show abnormality in any of 15 patients with amyotrophic lateral sclerosis. In MS patients with amyotrophic lateral sclerosis. In MS Patients, the abnormal IC picture proved to be significantly correlated with the CSFIgG values as calculated by Link's and Tourtelotte's formulas. The abnormal IC in MS may be due to altered CSF reabsorption or increased transependymal flow, or it may be related to the abnormal concentration of IgG.
Pathological findings underlying focal temporal lobe hypometabolism in partial epilepsy
Authors: Engel Jerome , Brown W. Jaan , Kuhl David E. , Phelps Michael E. , Mazziotta John C. , Crandall Paul H.
Published: January 2005

Histopthogical studies were carried out on temporal lobe tissue from 25 patients with partial complex seizures who were studied by interictal positen computed tomography (PCT) with 18F‐fluorodeoxyglucose and subsequently underwenty anterior lobe resection. Abnormalities were identified on x‐ray computed tomographic scane in 7 patients, but none indicated the site of a pathologically confirmed structural lesion. Hypometabolic zones were observed on PCT scans of 22 patients and corresponded to focal pathological abnormalities in 19 (15 mesial temporal sclerosis, 2 small neoplasms, 1 angioma, 1 heterotopia). In 1 patient with a foclly abnormal PCT scan and no pathological changes, the lesion may have been located posterior to the resection. In the remaining 2 patients, the hypometabolic zones later disappeared and may have represented a transient response reponse induced by depth electrode implantation. Three patients with normal PCT scans had no pathological abnormalities in their resected tissue. The degree of relative hypometabolism measured by PCT correlated well with the severity of the pathological lesion, byt the size of the hypometabolic zone was generally much larger than the area of pathological involvement. This discrepancy could not be considered an artifact of techique and muist represent either structural abnormalities below the resolution of routine histopathological studies (e.g., loss of synapses) or functional incativation of neuronal elements associated with the epileptogenic lesion.
Abnormal brainstem auditory evoked potentials in chronic paint sniffers
Authors: Metrick Scott A. , Brenner Richard P.
Published: January 2005

Two young adults developed neurological disorders consisting of cerebellar dysfunction, optic neuropathy, and dementia secondary to chronic inhalation of spray paint (toluene). Computed tomographic brain scans showed pontomedullary atrophy. Both patients had abnormal brainstem auditory evoked potentials. Although chronic toluene abuse can cause various neurological abnormalities, until now there has been little evidence that the brainstem can also be affected.
Self‐grasping: A focal neurological sign
Authors: Ropper Allan H.
Published: January 2005

A physical sign is described in patients with typical bilateral grasp responses, whereby one hand graps the contralateral forearm (self‐grasping). Of 37 patients with strong bilateral grasp responses, unilateral self‐grasping occurred in 8; of these 8 patients, 7 were hemiparetic and 5 showed extinction of double simultaneous stimulation. Two patients had bilateral self‐grasping, and 27 avoided self‐grasping. In contrast, only 4 of the 27 patients without self‐grapsing were hemiparetic and 1 had associated sensory extinction. In 2 confused, uncooperative patients, unilateral self‐grasping was the most easily demonstrable focal or asymmetrical sign.

The sign indicates a contralateral frontal or parietal lobe lesion. It may be related to the inability to recognize onself.
Phosphoglycerate kinase deficiency: Another cause of recurrent myoglobinuria
Authors: DiMauro Salvatore , Dalakas Marinos , Miranda Armand F.
Published: January 2005

A 14‐year‐old boy had myoglobinuria and renal failure after intense exercise; a year earlier he had experienced a milder episode. There was no consanguinity and no family history of neuromuscular diseases or hemolytic anemia. Strength was normal. Forearm ischemic exercise caused prolonged contracture with no rise of venous lactate. Muscle morphology showed only a mild increase of lipid droplets. Glycogen concentration was normal. Muscle phosphoglycerate kinase (PGK) activity was 5% of the normal mean, and all other glycolytic enzymes were normal. The residual PGK activity of muscle was heat stable but showed slower than normal electrophoretic mobility and decreased Michaelis constants for 3‐phosphoglycerate and adenosine triphosphate. The enzyme defect was also expressed in erythrocytes and in fibroblast and muscle cultures. PGK activity was decreased in tissues from the patient's mother but normal in the father. PGK deficiency is an X‐linked recessive trait usually associated with hemolytic anemia, mental retardation, and seizures; myopathy had not been recognized previously. Muscle PGK deficiency is now added to two other newly recognized glycolytic defects, phosphoglycerate mutase and lactate dehydrogenase deficiencies, as a cause of recurrent myoglobinuria.
Occurrence of oligoclonal bands in multiple sclerosis and other CNS diseases
Authors: Miller James R. , Burke Alan M. , Bever Christopher T.
Published: January 2005

The results of cerebrospinal fluid agarose gel electrophoresis in 300 consecutive patients were correlated with neurological examinations and diagnoses, other cerebrospinal fluid studies, and the results of evoked potential examinations. The presence of oligoclonal bands was the most sensitive test for multiple sclerosis (MS); bands were present in from 100% (11/11) of patients with definite MS to 82% (27/33) of those with possible MS (classified by McAlpine criteria). The visual evoked response was the next most sensitive study. Thirty‐eight patients without MS or related disorders had bands in the IgG region. Three patients had plasma cell dyscrasias. Seven patients had thick single bands. Single bands did not correlate with chronic polyneuropathy but appeared to be an artifact of storage. Twenty‐eight patients had active neurological disease, including cerebral infarction (in 5), viral infection (in 4), remote effect of carcinoma (in 4), and acute and chronic polyneuropathies (in 6). In acute illnesses (i.e., vascular insults), repeat electrophoresis showed disappearance of bands. In continually active disease, bands persisted. These results indicate that the presence of oligoclonal bands provides sensitive supporting evidence for the diagnosis of MS but that bands may be present in other disorders, including those not directly related to infection or abnormal immune response. The data suggest that oligoclonal bands may represent an immune reponse to neurological injury that is prominent in disorders with a particularly intense or continuous antigenic stimulus.
Pyridoxine‐dependency seizure: Report of a rare presentation
Authors: Krishnamoorthy K. S.
Published: January 2005

A child developed minor motor seizures at the age of 14 months accompanied by an abnormal electroencephalogram showing single spikes and polyspikes over the vertex and frontocentral regions. Seizures continued until the age of 22 months despite administration of several standard anticonvulsants. At age 22 months, pyridoxine, 75 mg daily, was initiated and anticonvulsants were discontinued. Both the seizures and the electroencephalographic abnormality have disappeared over the ensuing 20 months with pyridoxine therapy.
Epilepsies of infancy and childhood
Authors: Gomez Manuel R. , Klass Donald W.
Published: January 2005

This review deals with selected forms of epilepsy beginning after the neonatal period, with special emphasis on those that are highly age dependent for their expression. Experience accumulated in the past decade provides helpful data concerning the clinical‐electroencephalographic correlations, remission rate, differential response to medications, and prognosis, although many questions remain unanswered.
Immunocytochemical studies of cathepsin D in human skeletal muscle
Authors: Whitaker John N. , Bertorini Tulio E. , Mendell Jerry R.
Published: January 2005

The distribution of cathepsin D, an acidic endopeptidase, was localized by immunocytochemistry in human skeletal muscle obtained from 34 persons with a variety of neuromuscular disorders. Normal human skeletal muscle contained small amounts of cathepsin D, all of which was found close to the sarcolemmal membrane. Immunoreative cathepsin D was present in the cytoplasm of many infiltrating phagocytic cells and was increased in skeletal muscle fibers from patients with muscular dystrophies, inflammatory myopathies, rhabdomyolysis, acid maltase deficiency, and neurogenic atrophy. In cases of Duchenne type muscular dystrophy, the increase in cathepsin D was especially prominent in small regenerating fibers, in which it was visualized at the ultrastructural level in lysosome‐like organelles anextralysosomal locations. The function of cathepsin D in skeletal muscle is unclear, but the present findings suggest a possible role in muscle regeneration and repair. Such a role would necessitate careful selection of drugs which interfere with proteolytic activity if they are to be used as therapeutic agents in treating neuromuscular diseases.
The comparative effects of organic brain disease on cerebral blood flow and measured intelligeence
Authors: Butler Robert W. , Dickinson William A. , Katholi Charles , Halsey James H.
Published: January 2005

Raw scores for cerebral blood flow (CBF), determined by xenon 133 inhalation, and for Wechsler Adult Intelligence Scale (WAIS) were measured in five groups of 12 subjects each: young normals, aged normals, and patients with cerebrovascular disease without dementia, dementia with cerebrovascular disease, and degenerative brain disease. Important differences were present in the raw data according to age and sex. When these were adjusted a quadratic model revealed a highly significant (p < 0.0001, r = 0.86) correlation between CBF and measured intelligence score. We interpret these findings to indicate that in dementing illnesses the WAIS raw score reflects the severity of the brain disorder, regardless of cause, and that CBF is reduced as a function of the severity rather than the cause of the abnormality.
Amitriptyline potentiates morphine analgesia by a direct action on the central nervous system
Authors: Botney Mitchell , Fields Howard L.
Published: January 2005

Trycyclic antidepressants are often effective in the management of neuropathic pains. To elucidate the mechanism of tricyclic‐induced analgesia, amitriptyline and other drugs were injected into lightly anesthetized rats either systemically or via lumbar intrathecal cannulas. Analgesia was assessed by measuring the latency of the tail flik reflex. Using this model, intrathecal amitriptyline (30 μg) significantly enhanced the analgesic effect of an intraperitoneal dose of morphine (0.5 mg/kg) that by itself produced no measurable effect. Given systemically, amitriptyline (30 or 100 μg intraperitoneally) was ineffective. Cocaine (30 μg) also potentiated morphine analgesia, but iprindole, a tricyclic antidepressant with a very weak inhibitory effect on monoamine uptake, was ineffective. This enhancement of analgesia by intrathecal amitriptyline was prevented by pretreating the rats with p‐chlorophenylalanine (300 mg/kg). These results are consistent with the hypothesis that amitriptyline produces analgesia by blocking serotonin uptake and therefore enhancing the action of serotonin at the spinal terminals of an opioid‐mediated intrinsic analgeisa system.
Electrophysiological effects of myasthenic serum factors studied in mouse muscle
Authors: Lerrick A. J. , Wray D. , Vincent A. , Newsom‐Davis J.
Published: January 2005

Miniature end‐plate potential (mepp) amplitudes were investigated in mouse diaphragm exposed in vitro to different serum fractions from seven patients with myasthenia gravis who had elevated serum anti‐acetylcholine receptor antibody levels and from controls. The mepp amplitudes were significantly reduced by whole myasthenic sera, restored by washing, and not reduced by heated (56°C) myasthenic sera, which would inactivate complement but not antireceptor antibody. Immunoglobulin G (IgG)‐depleted myasthenic sera also significantly reduced mepp amplitudes, while the IgG fraction alone or with normal serum did not. The results indicate that in vitro reduction of mepp amplitudes in mouse muscle by myasthenic sera is not dependent on the IgG fraction alone, and requires a heat‐sensitive factor.
Thirst, resetting of the osmostat, and water intoxication following encephalitis
Authors: Howe J. G. , Penney M. D. , Currie S. , Morgan D.
Published: January 2005

A young man developed pathological thirst and hyperdipsia, hyperphagia, disordered temperature regulation, a lowered threshold for aggressive behavior, apathy, impaired memory, and seizures following encephalitis. He had marked hyponatremia. Bouts of water drinking produced water intoxication and precipitated status epilepticus. Studies of water handling with measurements of plasma osmolality and arginine vasopressin (AVP) revealed a very low thirst threshold (below 242 mOsm/kg) with resetting of the osmostat to a new level (255 mOsm/kg) but normal control of plasma osmolality at that level with adequate AVP release.
Progressive supranuclear palsy: Clinical features and response to treatment in 16 patients
Authors: Jackson Jeffrey A. , Jankovic Joseph , Ford Janet
Published: January 2005

Among 415 patients with parkinsonism, 16 (3.9%) had findings of progressive supranuclear palsy (PSP). This report reviews the clinical features and response to drug therapy in those 16 patients. Anticholinergic drugs failed to benefit any of the 5 patients treated, while presynaptic dopaminergic drugs (Sinemet or amantadine) were beneficial in only 5 of 22 patient trials. Alternatively, dopamine agonists (bromocriptine and pergolide) caused improvement in 9 of 14 patient trials despite the fact that all but 1 of these patients had previously failed to respond to presynaptic dopaminergic drugs. Dopamine agonists such as bromocriptine and pergolide may be useful in some patients with PSP.
Tonic status epilepticus presenting as confusional state
Authors: Somerville Ernest R. , Bruni Joseph
Published: January 2005

A 17‐year‐old epileptic was admitted to the hospital in a confused state. Electroencephalography (EEG) with polygraphic recording revealed tonic status epilepticus. Therapy with phenytoin, phenobarbital, valproic acid, and methyl phenidate was unsuccessful. Intravenous diazepam exacerbated the seizures. EEG with polygraphic recording may detect clinically inapparent tonic seizures in epileptics with unexplained confusion, and when generalized paroxysmal fast activity occurs during EEG recording.
Benign infantile mitochondrial myopathy due to reversible cytochrome c oxidase deficiency
Authors: Dimauro Salvatore , Nicholson John F. , Hays Arthur P. , B Abraham B. , Papadimitriou Alexandros , Koenigsberger Richard , Devivo Darryl C.
Published: January 2005

A 2‐week‐old boy had profound generalized weakness, hypotonia, hyporeflexia, macroglossia, and severe lactic acidosis. The infant improved spontaneously: he held his head at 4 1/2 months, rolled over at 7 months, and walked by 16 months. At 33 month of age, he had mild proximal weakness. Macroglossia disappeared by age 4 months. Blood lactic acid declined steadily and was normal by 14 months of age. Histochemical and ultrastructural studies of muscle biopsy specimens obtained at 1 and 7 months of age showed excessive mitochondria, lipid, and glycogen; a third biopsy at age 36 months showed only atrophy of scattered fibers. Cytochrome c oxidase stain was positive in fewer than 5% of fibers in the first biopsy, in approximately 60% of fibers in the second biopsy, and in all fibers in the third biopsy. Biochemical analysis showed an isolated defect of cytochrome c oxidase activity, which was only 8% of the lowest control level in the first biopsy; the activity increased to 47% in the second biopsy and was higher than normal in the third. In contrast to that in the fatal infantile form of cytochrome c oxidase deficiency, the enzyme defect in this condition is reversible. The biochemical basis for this difference remains to be explained.
Sural nerve immune deposits in polyneuropathy as a remote effect of malignancy
Authors: de Visser Bram W. Ongerboer , Feltkamp‐Vroom Thea M. , A Constance A.
Published: January 2005

Sural nerves from six patients with polyneuropathy as a remote accompaniment of malignancy were were studied by light microscopy, immunofluorescence, electron microscopy, and immuno‐electron microscopy. Sural nerves also were studied in twelve control patients with malignant diseases without peripheral neuropathy and in fifteen with neuropathy associated with nonmalignant conditions. Only nerves from the first group of six patients showed immune deposits of immunoglobulin M, C3, and C1q as granular or confluent layers in the inner laminae of the perineurium as well as in and around endoneural capillaries. The findings are similar to those in published studies of Waldenström's macroglobulinemia, chronic relapsing polyneuropathy, and neuropathy of monoclonal gammopathy and extend the list of conditions to include several forms of carcinoma and Hodgkin's disease.
Suppressor T‐lymphocytes in multiple sclerosis: Analysis of patients with acute relapsing and chronic progressive disease
Authors: Paty Donald W. , Kastrukoff Lorne , Morgan Norma , Hiob Lydia
Published: January 2005

T cell‐specific antigens as identified by the anti‐Leu 1, anti‐Leu 2a, and anti‐Leu 3a monoclonal antibodies were quantitated in 31 normal control subjects, 21 adults with spinal cord injury, 58 patients with stable multiple sclerosis, 31 patients with chronic progressive multiple sclerosis, and 15 patients with multiple sclerosis in acute relapse. The only significant deviations from normal values were a slightly low mean T cell (Leu 1‐positive) value in patients in acute relapse and a low suppressor T cell (Leu 2a‐positive) mean value in patients with chronic progressive multiple sclerosis. We were unable to confirm previous studies using the OK series of monoclonal antibodies that reported low suppressor T cell levels (OKT5 and OKT8 positive) in patients with acute relapses. We suggest that modulation (a spontaneous or induced variability in the expression) of individual cell surface antigens may explain the differences seen between our study and other reported series.
Mitochondrial cytochrome deficiency presenting as a myopathy with hypotonia, external ophthalmoplegia, and lactic acidosis in an infant and as fatal hepatopathy in a second cousin
Authors: Boustany Rosemary N. , Aprille June R. , Halperin John , Levy Harvey
Published: January 2005

Fatal infantile mitochondrial myopathy with lactic acidosis, morphologically abnormal mitochondria, deficient cytochromes aa3 and b, and a Fanconi‐like aminoaciduria has been described. We report two infants, second cousins, with a similar fatal mitochondrial disorder, the cytochrome deficiency limited to skeletal muscle in one child and to liver in the other. The first child at 3 months of age had weight loss, hypotonia, external ophthalmoplegia, and a severe lactic acidosis with a high lactate/pyruvate ratio. Electron microscopy of muscle showed marked proliferation of enlarged mitochondria, many containing concentric rings of cristae. In skeletal muscle mitochondria, cytochromes aa3 and b were not detectable but cytochrome cc was found to be normal by spectroscopy. Cytochrome c oxidase activity was less than 1% of normal. Mitochondria from kidney, liver, heart, lung, and brain examined postmortem had normal cytochromes and preserved cytochrome c oxidase activity. The second cousin at 5 months of age had weighty loss and hepatomegaly but no systemic lactic acidosis. Liver biopsy showed hepatocytes packed with enlarged mitochondria. The liver mitochondria showed deficient cytochromes aa3 and b postmortem, and cytochrome c oxidase activity was less than 10% of normal. Kidney mitochondria had normal cytochromes. Muscle was not studied. The mitochondrial abnormality in the two cousins presumably is related. Unexplained are the mode of genetic transmission or environmental exposure and the apparent involvement of a single different organ in each child.
Genetic linkage evidence for heterogeneity in Charcot‐Marie‐Tooth neuropathy (HMSN type I)
Authors: Bird T. D. , Ott J. , Giblett E. R. , Chance P. F. , Sumi S. M. , Kraft G. H.
Published: January 2005

A genetic linkage study performed on a large family with autosomal dominant Charcot‐Marie‐Tooth neuropathy (HMSN type I) showed affected family members to have slow motor nerve conduction velocities, hypoactive tendon reflexes, and distal muscle weakness and atrophy. Results excluded close linkage of the neuropathy in this family to the Duffy blood group locus on chromosome 1. Previous studies in other families have shown positive linkage of HMSN type I to the Duffy locus. The present results provide support for the concept of genetic heterogeneity in HMSN type I. Comparison of this new family with the previous families showing linkage to Duffy reveals that the hereditary neuropathy not linked to the Duffy locus may have less severe slowing of motor nerve conduction velocities and less prominent onion bulb change evident on sural nerve biopsy.
Demyelination induced by serum form patients with Guillain‐Barré syndrome
Authors: Harrison B. M. , Hansen L. A. , Pollard J. D. , McLeod J. G.
Published: January 2005

Sera from 16 patients with acute Guillain‐Barré syndrome (GBS) and 14 healthy control subjects were injected into rat sciatic nerve and assessed for demyelinating activity by electrophysiological and histological techniques. Only fresh GBS serum, and not GBS serum stored at−20°C or −70°C, blocked conduction to a significantly greater extent than did fresh control serum. Conduction block developed gradually, starting within 24 hours of injection and reaching a maximum between days 3 and 6. Recovery of conduction commenced thereafter, and conduction returned to normal by day 33. Quantitative histological studies on day 6 showed that fresh GBS serum produced significantly more widespread demyelination than did stored GBS serum (p < 0.01). Stored GBS serum showed residual demyelinating activity When compared with fresh control serum (p < 0.01). Fresh serum obtained from 4 patients after recovery from GBS did not produce conduction block, despite it having done so during the acute phase of the disease.
Enzyma‐linked immunosorbent assay for antibody against the nicotinic acetylcholine receptor in human myasthenia gravis
Authors: Kawanami Sachiko , Tsuji Rieko , Oda Kenichiro
Published: January 2005

Antibody against acetylcholine receptor (AChR) of human skeletal muscle was measured using enzyme‐linked immunosorbent assay and found in 23 (74%) of 31 Japanese patients with generalized myasthenia gravis. In 15 patients with generalized myasthenia gravis who had not undergone thymectomy and who were not receiving adrenocorticosteriods, the antibody was found in 13 (87%). Antibody was also found in 13 (54%) of 24 patients with myasthenia gragis against AChR fractions obtained from fetal calf thymus. Based on the subunit structures of the AChR protein, the double precipitation assay using iodine 125–α‐bungarotoxin is also capable of detecting antibody against the toxin binding site, by cross reactivity. This is among the first reports of experiments in which enzyme‐linked immunosorbent assay was used to measure the antibodies in human myasthenia gravis and provides evidence of anti‐AChR antibody against antigens from fetal calf thymus.
Use of a heparinoid in patients with hemorrhagic stroke and thromboembolic disease
Authors: Ten H. , Henny C. P. , Büller H. R. , Ten J. W. , Magnani H. N.
Published: January 2005

A new heparinoid, Org 10172, was given to five patiens with hemorrhagic stroke with thromboembolic complications. Treatment did not cause progression of cerebral bleeding, and thrombotic processes were inhibited. No side effects were encountered.
Tranfer of language dominance
Authors: Lee Henry , Nakada Tsutomu , Deal Jon L. , Lin Stan , Kwee Ingrid L.
Published: January 2005

It has been suggested that recovery from aphasia may depend on the ability of the nondominant hemisphere to subserve language functions. We describe a patient who illustrates a possible “transfer” of language dominance. The patients sustained two infarcts two years apart, one in each hemisphere, involving mirror image territories in the temporoparietal area. The clinical course of each infract was almost identical, consisting of an initial Wernicke's aphasia which evolved into anomic aphasia during recovery.
Alzheimer's disease: A study of epidemiological aspects
Authors: Heyman Albert , Wilkinson William E. , Stafford Judith A. , Helms Michael J. , Sigmon Alverta H. , Weinberg Tina
Published: January 2005

A case‐control study was performed to determine the possible roles of various environmental factors, prior illnesses, drug use, and personal habits in the development of Alzheimer's disease. Such information was collected from 40 patients with onset of dementia prior to age 70 and from 80 community control subjects matched for age, sex, and race. No significant differences were found between patients and control subjects in toxic environmental exposures, animal contacts, smoking, drinking, or unusual dietary habits. A significantly higher frequency of prior thyroid disease was found in women patients than in women control subjects (25.0% and 7.1%, respectively). A history of severe head injury was also obtained significantly more often among the patients than among the controls (15.0% and 3.8%, respectively). Aside from these differences, which may prove to be important associative factors in this illness, there appeared to be no major premorbid demographic or clinical factors associated with this form of dementia. There was evidence, however, of a genetic factor that was manifested in an excess of dementia and mental retardation (including Down's syndrome) in families of patients with Alzheimer's disease.
Severe neurological impairment: Legal aspects of decisions to reduce care
Authors: Beresford H. Richard
Published: January 2005

Decisions to reduce care for patients with severe neurological impairment may raise legal questions. The laws of most states now authorize physicians to stop care for those who have suffered irreversible cessation of all functions of the brain (“brain death”). Where state law is not explicit, it is nevertheless probably lawful to regard brain death as death for legal purposes so long as currently accepted criteria are satisfied. Several courts have ruled that it is lawful to reduce care for patients in vegetative states, but have prescribed differing standards and procedures for implementing such decisions. The issue of whether parents can authorize physicians to reduce care for neurologically impaired children is the focus of current litigation. Implicit in this litigation is the question of how severe neurological impairment must be before parents and physicians may lawfully agree to reduce care. For severely impaired but not vegetative adults, there is some legal authority to justify certain decisions to reduce care. The issue of whether withholding feeding from a severely demented patient with life‐threatening medical problems constitutes criminal behavior is now being considered by a state supreme court.
Regional blood flow in compression‐induced brain edema in rats: Effect of dietary vitamin E
Authors: Busto Raul , Yoshida Shinichi , Ginsberg Myron D. , Alonso Ofelia , Smith David W. , Goldberg William J.
Published: January 2005

Regional cerebral blood flow (rCBF) was studied autoradiographically in a murine model of focal epidural brain compression, and the effect of vitamin E administration was investigated. Mean cortical CBF was reduced to 0.48 to 0.50 ml/gm/min following 2 or 24 hours of compression. Early (2 hours) following subsequent decompression, a mixed pattern of hypoperfusion and hyperperfusion was observed. Twenty‐four hours later, rCBF heterogeneities were less marked. Comparisons among animal groups raised on vitamin E–supplemented, vitamin E–normal, and vitamin E–deficient diets 2 hours after decompression revealed marked reductions in rCBF in the previously compressed cortex of the last two groups and hyperemia of the underlying hippocampus. The vitamin E–supplemented rats showed increased flow in the previously compressed cortex. In addition, vitamin E supplementation tended to eliminate rCBF gradients between subjacent zones. These data may help explain our previous observations of the beneficial effects of vitamin E on compression‐induced brain edema.
Severe neurological disease associated with hyperparathyroidism
Authors: Patten Bernard M. , Pages Michel
Published: January 2005

Two patients, one with ataxia, internuclear ophthalmoplegia, muscle weakness, atrophy, fasciculations, and bilateral Babinski's signs, the other with dysarthria, dysphagia, muscle weakness, atrophy, fasciculations, and hyperreflexia, had elevated serum calcium and parathyroid hormone levels, establishing the diagnosis of primary hyperparathyroidism (HPT). Removal of a parathyroid adenoma in one patient and three hyperplastic parathyroid glands in the other resulted in remission of the hyperparathroidism but left both patients with residual neurological damage. Postmortem examination of the second patient showed typical features of amyotrophic lateral sclerosis. The findings in these patients show that hyperparathyroidism may be associated with signs of severe central nervous system disease and that patients with unexplained neurological signs or symptoms should be checked for hyperparathyroidism.
DA strain of Theiler's murine encephalomyelitis virus induces demyelination in nude mice
Authors: Roos Raymond P. , Wollmann Robert
Published: January 2005

DA strain of Theiler's murine encephalomyelitis virus produces a chronic, progressive demyelinating disease in mice that resembles multiple sclerosis. An immunopathological mechanism for demyelination has been postulated, because there is a brisk immune response with low virus titers at the time of demyelination and because immunosuppression lessens the degree of demyelination. We inoculated Nude mice with DA virus to clarify the role of immune‐mediated demyelination. Animals became paralyzed 3 weeks to 2 months after inoculation, usually dying within a week of appearance of signs. Demyelinated foci were present in the spinal cords, with evidence of degenerating myelin around intact axons as well as completely demyelinated, naked axons. Occasional macrophages were present, but none was seen actively stripping intact myelin lamellae. These results suggest that DA virus lytic infection, without a contribution from the T lymphocyte immune system, is sufficient to produce demyelination. It is likely that DA virus demyelination has varying mechanisms that may be active at different times.
Chronicity of hyponatremia as a factor in experimental myelinolysis
Authors: Norenberg Michael D. , Papendick Rebecca E.
Published: January 2005

A recent analysis of hyponatremic patients disclosed that those who were chronically hyponatremic prior to rapid correction subsequently developed central pontine myelinolysis. To test the view that chronicity of hyponatremia is a factor in myelinolysis, we studied a group of rats that were hyponatremic for 1 day and compared it with another group of animals maintained in a hyponatremic state for 3 days prior to the administration of hypertonic saline solution. The 3‐day hyponatremic rats developed more numerous and more severe demyelinative lesions than the 1‐day rats. The findings support the view that duration of hyponatremia is a factor in myelinolysis and that the electrolyte disturbance of chronically hyponatremic patients should be corrected cautiously and relatively slowly.
The protective influence of the locus ceruleus on the blood‐brain barrier
Authors: Harik Sami I. , McGunigal Thomas
Published: January 2005

The functions of the putative noradrenergic innervation of cerebral microvessels from the nucleus locus ceruleus remain ambiguous. Although most evidence indicates that such innervation does not have a major role in the control of cerebral blood flow, there are increasing indications that it modulates transport and permeability functions of the blood‐brain barrier. In this study we investigated the effect of unilateral chemical lesioning of the locus ceruleus on the leakage of radioiodinated human serum albumin across the blood‐brain barrier. Experiments were performed in awake and restrained rats under steady‐state conditions and during drug‐induced systemic arterial hypertension, and in anesthetized and paralyzed rats during bicuculline‐induced seizures. Both hypertension and seizures are known to be associated with increased leakage of macromolecules across the blood‐brain barrier. Albumin leakage into norepinephrine‐depleted forebrain structures ipsilateral to the locus ceruleus lesion was compared with that of the contralateral side. There were no side‐to‐side differences in blood‐brain barrier permeability to albumin under steady‐state conditions, the stress of restraint, or angiotensin‐induced hypertension, or after isoproterenol administration. Norepinephrine‐induced hypertension and seizures, however, caused significant increases in albumin leakage into forebrain structures ipsilateral to the lesion. These results suggest that noradrenergic innervation of cerebral microvessels from the locus ceruleus helps preserve the integrity of the blood‐brain barrier during pathophysiological states associated with hypertension and increased circulating catecholamines.
Rapid alterations of the axon membrane in antibody‐mediated demyelination
Authors: Saida Kyoko , Saida Takahiko , Kayama Hisae , Nishitani Hiroshi
Published: January 2005

Alterations of nodal and paranodal axolemma of the rat sciatic nerve were investigated in antigalactocerebroside serum–induced demyelination. A ferric ion–ferrocyanide (FeFCN) stain that appears to stain the regions with a high sodium channel density in nerve fibers was applied. When acute conduction block was initiated 20 to 180 minutes after the antiserum injection, myelin terminal loops began to be detached from the paranodal axolemma and reaction product of FeFCN stain originally localized at the nodes decreased in density and extended to the paranodal axolemma. By the time that complete conduction block was established, 5 hours after the injection, FeFCN stain was barely detectable around the nodal area. The loss of staining was associated with detachment and vesiculovacuolar degeneration of the paranodal myelin. This rapid deterioration and disappearance of normal cytochemical characteristics of the axolemma in the presence of only modest paranodal demyelination could be a morphological correlate of the loss of excitability of the axon membrane.
Serum neuronal growth factor levels in von Recklinghausen's neurofibromatosis
Authors: Riopelle Richard J. , Riccardi Vincent M. , Faulkner Shizuye , Martin Mary C.
Published: January 2005

A single neuronal cell biological assay was used to quantitate neuronal growth factors in 87 serum samples from 69 patients in 48 families with von Recklinghausen's neurofibromatosis, plus 16 samples from 16 comparison subjects. Mouse nerve growth factor was used as a standard for the bioassay, and results of serum assays were expressed as nerve growth factor equivalents. Antiserum to mouse nerve growth factor inhibited fractionally serum‐induced neurite outgrowth, while kinetics of neurite outgrowth and maximal cellular response to serum differed from those induced by mouse nerve growth factor. The mean values (± SD) of neuronal growth factors for the patients were 20.5 ± 15.7 pg/mg serum protein, while mean values for the comparison group as a whole were 22.3 ± 15.6 pg/mg serum protein. Sex, race/ethnicity, patient age, and date of sample collection did not significantly influence serum levels among patients or comparison subjects. Three to six serial samples taken from women before pregnancy, during the course of pregnancy, during delivery, and in the postpartum period did not show significant differences from one period to another. These data suggest that human serum does contain non‐nerve‐growth‐factor neuronal growth factors, but that levels of the factors do not contribute to the identification of patients with von Recklinghausen's neurofibromatosis.
Acute Kingella kingae endocarditis with recurrent cerebral emboli in a child with mitral prolapse
Authors: Lee Wei Ling , Dooling Elizabeth C.
Published: January 2005

An 11‐year‐old girl had sudden onset of right hemiplegia and dysphasia with fever. Blood cultures grew Kingella kingae. Echocardiography showed a prolapsed mitral valve vegetation, which became larger during a prolonged course of antibiotics. After seven weeks of therapy, when surgery was being planned, the child deteriorated abruptly. A repeat echocardiogram showed that the vegetation had disappeared. Conservative medical management is usually inadequate in treating vegetations associated with bacterial endocarditis; surgery is advised.
A new form of sea‐blue histiocytosis associated with progressive anterior horn cell and axonal degeneration
Authors: Ashwal Stephen , Thrasher Thais V. , Rice David R. , Wenger David A.
Published: January 2005

A 15‐year‐old girl evidenced a slowly progressive central nervous system degenerative disorder. The illness had begun and progressed between ages 1 and 12 years, with ataxia, spasticity, choreoathetosis, early‐onset seizures (which later ceased), and mild retardation. At age 13 she had developed rapidly progressive generalized weakness and atrophy, indicating peripheral nervous system involvement. Laboratory investigation revealed the presence of sea‐blue histiocytes in the bone marrow without evidence of a disorder of sphingolipid metabolism or neuronal ceroid lipofuscinosis. Muscle biopsy showed large‐ and small‐group atrophy, and sural nerve biopsy demonstrated axonal degeneration. This patient's illness appears to be a hitherto undescribed form of “sea‐blue histiocytosis” associated with neurological dysfunction in children.
Monoclonal antibody analysis of mononuclear cells in myopathies. II: Phenotypes of autoinvasive cells in polymyositis and inclusion body myositis
Authors: Engel Andrew G. , Arahata Kiichi
Published: January 2005

In 6 cases of polymyositis and 6 of inclusion body myositis, phenotypes of mononuclear cells focally surrounding and invading muscle fibers were analyzed. By localizing the T8, T4, and Ia markers with direct immunofluorescence and acid phosphatase enzyme cytochemically in the same sections, five different phenotypes were simultaneously identified in a given section: T8+ and T4+ cells that were either activated (Ia+) or not activated (Ia−), and acid phosphatase—reactive and Ia+ macrophages. This approach permitted the separate and quantitative assessment of the distributions of the different phenotypes among the invading versus the surrounding cells. In both polymyositis and inclusion body myositis, the invading cells were selectively enriched in the T8+ cells were more abundant among the surrounding than the invading cellsm and only a small proportion of the T4+ cells were activated. These findings are especially significant in view of the cytotoxic capability of the T8+ cells and because histocompatibility factors permit T8+ but not T4+ cells to recognize an antigen on muscle fibers. Macrophages accounted for 21 to 31% of the cells invading or surrounding nonnecrotic fibers. For purposes of comparison, we also analyzed mononuclear cells in necrotic fibers: 80% of these cells were macrophages, and only 20% were T cells. The findings indicate that in polymyositis and inclusion body myositis, nonnecrotic muscle fibers are injured by autoinvasive T8+ cells that act in concert with macrophages. Further, the findings strongly imply previous sensitization of clones of T cells to muscle fiber—associated surface antigen(s).
Cholesterol and phospholipids in cultured skin fibroblasts from patients with dystonia
Authors: Maltese William A. , De Vivo Darryl C.
Published: January 2005

Cultured skin fibroblasts from patients with hereditary (recessive) torsion dystonia or adult‐onset sporadic dystonia and from healthy volunteers were compared with respect to total cellular cholesterol, total phospholipid, and phospholipid composition. Differences that could be attributed specifically to dystonia were not detected. The data suggest that the reported cell membrane abnormality in recessive torsion dystonia does not involve a generalized defect in cholesterol or phospholipid metabolism.
Descending paralysis resulting from occult wound botulism
Authors: Rapoport Samuel , Watkins Paul B.
Published: January 2005

A 30‐year‐old male drug absuser developed ophthalmoplegia, bulbar paralysis, and limb weakness responsive to edrophonium. However, potentiation of a lowamplitude evoked muscle action potential was produced with repetitive nerve stimulation at 10 Hz, and the clinical and electrophysiological dat suggested the diagnosis of botulism. The source of botulism type B toxin was a clinically obscure cyst produced by subcutaneous infiltration of cocaine two weeks prior to the onset of symptoms. The patienat improved with chronic administration of pyridostigmine bromide and plasmapheresis. Wound botulism may be underdiagnosed because of confususion with inflammatory neuropathy or myasthenia gravis. Neuromuscular transmission studies in patients with acute craniosomatic paralysis can prevent such oversights.