bluuebunny/crossref_metadata_2025_split huggingface dataset:
Activation of cortical circuits during interictal spikes
Authors: Collins Robert C. , Caston Torris V. 
Published: January 2005

AbstractPenicillin, bicuculline, and strychnine were used as topical convulsants in unanesthetized rats to study aspects of interictal spiking. Bicuculline (0.1 mM) was more potent than penicillin (3.0 mM) or strychnine (1.5 mM) in initiating simple discharges, and at only slightly higher doses caused complex spikes and afterdischarges with motor convulsions. Penicillin caused similar types of discharges at much higher concentrations (> 50 mM), but strychnine failed to produce either complex spikes or afterdischarges. Penicillin and bicuculline were similar in causing an increase in utilization of carbon 14‐deoxyglucose in cortical layers I to III and V. Strychnine increased metabolism in layer V, with individual columns running from this base to the surface. Areas of decreased metabolism highlighted the strychnine focus and are probably related to a “pericolumnar inhibition” mediated by γ‐aminobutyric acid. Such inhibition would be blocked in the penicillin and bicuculline focus‐an effect which might be responsible for the genesis of complex spikes and afterdischarges and a more uniform activation of cortical metabolism.
Hereditary myoclonus and progressive distal muscular atrophy
Authors: Jankovic Joseph , Rivera Victor M. 
Published: January 2005

AbstractMyoclonus occurs in a variety of pathological conditions, some inherited. We recently evaluated 3 members of a Louisiana‐Texas family with an autosomal dominant disorder manifested by adult‐onset, generalized, stimulus‐sensitive myoclonus and slowly progressive distal muscle weakness and wasting. The analyses of cerebrospinal fluid homovanillic acid and 5‐hydroxyindoleacetic acid before and after probenecid provided some evidence of impaired turnover of central dopamine and serotonin. Treatment with clonazepam resulted in complete and lasting improvement of the myoclonus. A postmortem examination in 1 member of the family revealed chiefly neuronal degeneration of the anterior horn cells, Clarke's nucleus, and the lower cranial nerve nuclei. A similar syndrome has not previously been reported.
Treatment of chronic relapsing inflammatory polyradiculoneuropathy by plasma exchange
Authors: Server Alfred C. , Lefkowith James , Braine Hayden , McKhann Guy M. 
Published: January 2005

AbstractA 58‐year‐old man developed recurrent episodes of symmetrical motor weakness associated with acroparesthesias and areflexia. The cerebrospinal fluid protein was elevated and nerve conduction velocities were slowed. There was no evidence of systemic disease or toxic exposure, and a diagnosis of chronic relapsing inflammatory polyradiculoneuropathy was made. The patient improved during treatment with corticosteroids, but steroid‐induced complications necessitated discontinuance. A recrudescence of symptoms prompted a search for alternative therapy, and plasma exchange was tried. A marked improvement in strength was noted following each course of plasma exchange, suggesting that further similar trials are justified.
Multiple endocrine neoplasia, type 2b: Phenotype recognition; neurological features and their pathological basis
Authors: James Dyck Peter , Carney J. A. , Sizemore G. W. , Okazaki H. , Brimijoin W. S. , Lambert E. H. 
Published: January 2005

AbstractSixteen patients affected with multiple endocrine neoplasia, type 2b (MEN 2b), were evaluated by clinical, neurological, nerve conduction and electromyographic, and postmortem examinations. Eight of the 11 patients examined clinically had symptoms: 5, neurogenic constipation; 1, failing vision due to hypertrophied corneal nerves; 1, neuromuscular symptoms and pes cavus; and 1, facial disfigurement. Expression of the dominantly inherited MEN 2b gene is more variable than previously known. When neuromuscular findings are present alone, the features may be those of peroneal muscular atrophy. Because 10 of the 11 patients had sufficiently full expression of the dominantly inherited gene—“Marfanlike” body build, full and fleshy lips, whitish yellow nodules (neuromas) on the tip and edges of the tongue, pes cavus, or peroneal muscular atrophy the presence of MEN 2b was recognized and a search for the usually associated medullary thyroid carcinoma was instigated. In addition to the recognized involvement of autonomic nerves, we have confirmed that somatic motor and sensory neurons may be involved. Findings at postmortem evaluation indicate that symptoms can be attributed to neuroma formation: a characteristic adventitious plaque of tissue composed of hyperplastic, interlacing bands of Schwann cells and myelinated fibers overlay the posterior column of the spinal cord.
Deficiency of arylsulfatase B in 2 brothers aged 40 and 38 years. (Maroteaux‐Lamy syndrome, type B)
Authors: Pilz H. , von Figura K. , Goebel H. H. 
Published: January 2005

AbstractTwo brothers, aged 40 and 38 years, suffered from dysplastic features, coarse facies, bone and skeletal abnormalities, deformities of spine, and joint impairments. Body heights were 168 and 164 cm, respectively. Enlargement of liver and spleen, cardiac insufficiency, marked corneal clouding, and hernias were absent. Both patients had signs of cervical and lumbar radiculopathy and cervical myelopathy (tetraspastic syndrome).Vacuoles, acid phosphatase‐positive granules, and metachromatic inclusions were found in peripheral lymphocytes; granulocytes and monocytes contained azurophilic hypergranulation. By electron microscopy, clear membrane‐bound vacuoles were noted in lymphocytes (but not in neutrophils), fibroblasts, Schwann cells, mural cells of the vasculature, and epidermal cells.Leukocytes, urine, and cultured skin fibroblasts revealed a deficiency of arylsulfatase B (N‐acetylgalactosamine 4‐sulfate sulfatase). The 6‐year‐old daughter of one of the patients has an intermediate level of this enzyme. Fibroblasts exhibited a constant intracellular accumulation of 35S‐labeled mucopolysaccharides. The urine of one of the brothers showed an abnormal mucopolysacchariduria; in both, the presence of urinary dermatan sulfate could be demonstrated. These findings conform to the mild B variant of Maroteaux‐Lamy syndrome with high longevity.
Extraocular muscle biopsy in chronic progressive external ophthalmoplegia
Authors: Ringel Steven P. , Bruce W. Wilson , Barden Michael T. 
Published: January 2005

AbstractA quantitative assessment of the pathological changes in extraocular muscle is presented in 8 patients with chronic progressive external ophthalmoplegia (CPEO). Serial cross‐sections of extraocular muscle were stained with a battery of histochemical and immunohistochemical techniques and compared with 36 normal extraocular muscles and 1 muscle from a patient who had longstanding third nerve palsy with anomalous reinnervation.Several of the patient had a striking increase in the number of ragged‐red fibers in extraocular muscle, particularly if frequent ragged‐red fibers also were found on limb muscle biopsy. One patient demonstrated extrajunctional acetylcholine receptor (AChR) in a small percentage of fibers, although this finding was not present in the reinnervated muscle. Numerous darkly staining central regions were noted in the ocular muscle fibers of a patient with Stephens syndrome (CPEO, peripheral neuropathy, and cerebellar disease) and in the reinnervated muscle. A patient with myotubular myopathy had single central nuclei in both limb and ocular muscle. All patients demonstrated in their extraocular muscles variation in both the size and distribution of each of the three histochemical fiber types.Extraocular muscule biopsy proved to be a safe, reliable technique. As a similar quantitative analysis is applied to the study of further patients, a better understanding of the pathogenesis of CPEO should be possible.
Creutzfeldt‐Jakob disease in France: II. Clinical characteristics of 124 consecutive verified cases during the decade 1968–1977
Authors: Brown Paul , Cathala Françoise , Sadowsky Doris , Gajdusek D. C. 
Published: January 2005

AbstractOne hundred twenty‐four consecutive cases of Creutzfeldt‐Jakob disease (CJD) in France, verified by biopsy or autopsy between 1968 and 1977, were analyzed with respect to their clinical characteristics. The series comprised equal numbers of men and women, with the most frequent age at onset being 60 to 64 years and the most frequent duration, two to three months. A prodromal illness was observed in more than one‐third of the patients. Clinical presentations and symptom frequencies are tabulated, and a multifactor analysis has been performed to obtain those combinations of symptoms and signs which occurred at least as often as the triad of dementia, myoclonus, and a positive electroencephalogram. Two groups of atypical cases are also emphasized: one with sudden, strokelike presentation and rapidly evolving illness of less than 2 moths' duration, and the other with a long clinical course of between 2 and 10 years.
Creutzfeldt‐Jakob disease in France: III. Epidemiological study of 170 Patients dying during the decade 1968–1977
Authors: Brown Paul , Cathala Francoise , Gajdusek D. C. 
Published: January 2005

AbstractSystematic search for Creutzfeldt‐Jakob disease (CJD) in France yielded a total of 170 patients dying between 1968 and 1977. The overall annual mortality rate from CJD for France was 0.32 case per million persons, with by far the highest rate (1.33 cases per million) in the most densely populated parts of Paris. A highly significant correlation between population density and the mortality rate of CJD in the Paris urban agglomeration was consistent with the hypothesis of human‐to‐human disease transmissison, although in the rest of France CJD occurred at equally low rates among rural and urban populations. Temporospatial case clustering was not observed, nor could occupation, surgery, or potential exposure to scrapie be associated with an increased risk of contracting CJD. Six to 9% of cases were familial, analysis of which discouraged the notion of a familial “common exposure” in favor of genetic transmission of susceptibility to the virus or of the virus itself. A prospective epidemiological study of newly occurring cases of CJD, especially familial and geographically isolated cases, is being undertaken in the hope of identifying the means by which CJD is naturally acquired.
Clinical correlations of CT scan‐detected calcifications of the basal ganglia
Authors: Murphy Martin J. 
Published: January 2005

AbstractA review of CT scans of 7,081 patients demonstrated calcifications of the basal ganglia in 53. The calcifications were evident in the skull roentgenograms of only 4 patients out of 40 in whom both CT scans and plain roentgenograms were available, demonstrating the superior resolution of this new method. Seventy‐five percent of the patients were older than 50 years of age. Of the younger patients, 5 had had prior cranial irradiation; 1 had received cranial irradiation and intrathecal methotrexate therapy for meningeal leukemia; and 2 others had deep‐seated arteriovenous malformations. Serum concentrations of calcium and phosphorus were normal in all 46 patients in whom they were measured.We conclude that the detection of small calcifications of the basal ganglia in persons above 50 years of age is infrequently associated with either clinical signs of basal ganglia dysfunction or calcium and phosphorus abnormalities. Calcium deposition in these patients may be related to vascular changes associated with aging. In younger patients a specific pathogenetic factor or underlying process is frequently found.
Failure of electroencephalography to diagnose brain death in comatose children
Authors: Ashwal Stephen , Schneider Sanford 
Published: January 2005

AbstractTwo isoelectric electroencephalograms obtained 24 hours apart support a clinical diagnosis of brain death in prolonged coma. Without documentation of electrocerebral silence, physicians are reluctant to discontinue vital support systems. A radionuclide bolus technique has been developed that documents the absence of cerebral blood flow in suspected brain death and supplements the flat EEG.In a recent review of this technique, all 27 adults who had EEG activity maintained the integrity of their cerebral blood flow. This contrasts to our studies of 5 children, all of whom demonstrated persistent EEG activity but had no evidence of cerebral blood flow by either the isotope bolus technique (5 patients) or cerebral angiography (4 patients). These children (mean age, 7 months) lacked cephalic reflexes and were maintained on assisted ventilation for an average of 15 days. Multiple electroencephalographic tracings persistently demonstrated low‐voltage cortical activity over this time. Despite the lack of cerebral blood flow, all patients were continued on respiratory support. At autopsy, extensive brain liquefaction necrosis was noted. In comatose children, EEG monitoring may be of limited value while cerebral blood flow measurements can provide more practical and prognostic information.
Central nervous system maturation in the stressed premature
Authors: Holmes Gregory L. , Logan William J. , Kirkpatrick Barry V. , Meyer Edwin C. 
Published: January 2005

AbstractThe developmental sequence of sleep cycles has been found to be a useful index of central nervous system maturation in premature infants. To determine the effects of severe reversible stress on the maturation of sleep cycles, 6‐ to 8‐hour sleep studies were done on 10 premature infants with severe hyaline membrane disease (HMD) and 10 healthy premature babies. The studies were done in the neonatal intensive care unit and included patients from 30 weeks' gestation to term and in all stages of the disease.Prior studies were confirmed showing that percentage of quiet sleep increases, transitional sleep changes little, and active sleep decrease from 30 weeks' gestation to term. In infants with severe HMD, the percentage of quiet sleep was less and active sleep higher than in unstressed infants of similar age who acted as controls. Once the infants revovered from HMD, sleep patterns became similar to those found in the control group. These data suggest that when respiratory distress occurs in the premature infant, there is a transient delay in central nervous system maturation as measured by sleep cycle analysis.
Cerebellar control of ocular gaze stability
Authors: Zee David S. , Leigh R. John , Mathieu‐Millaire Francine 
Published: January 2005

AbstractThe ability to hold an eccentric position of gaze has been attributed to a brainstem network termed the ocular motor neural integrator. For this integrator to function properly, an intact cerebellum is necessary. This report describes a patient with cerebellar dysfunction who showed an unusual form of nystagmus: each slow phase had a waveform of increasing velocity. This contrasts with gaze paretic nystagmus, the more typical manifestation of cerebellar disorder, in which each slow phase has a waveform of decreasing velocity. Based on these observations and results from basic research, we propose that (1) the cerebellum controls neural integration in the brainstem by a positive feedback loop, and (2) pathological alterations in the strength of transmission (or gain) through the feedback loop cause the eyes to drift off target, with either an exponentially increasing (gain too high) or decreasing (gain too low) velocity.
Immunocytochemical method to identify myelin basic protein in oligodendroglia and myelin sheaths of the human nervous system
Authors: Itoyama Yasuto , Sternberger Nancy H. , Kies Marian W. , Cohen Steven R. , Richardson Edward P. , Webster Henry DeF. 
Published: January 2005

AbstractTo study the distribution of basic protein (BP) and other myelin constituents immunocytochemically in human nervous tissue, we modified the unlabeled antibody enzyme (peroxidase‐antiperosidase) method. The technique is described here. Because the availability of unfixed tissue from human central nervous system is limited, we tested the method on blocks that had been fixed in formalin and embedded in paraffin, fixed and stored in 4% formalin, or frozen at autopsy and stored. We obtained the best results with paraffin blocks. BP antiserum scained oligodendroglia and myelin sheaths in the developing human nervous system. In the adult, myelin sheaths were well stained. Also, abnormalities associated with myelin breakdown could be identified in multiple sclerosis lesions. The results suggest that this method will be useful in studying the cellular distribution of myelin components in human demyelinating diseases.
Immunocytochemical observations on the distribution of myelin‐associated glycoprotein and myelin basic protein in multiple sclerosis lesions
Authors: Itoyama Yasuto , Sterhberger Nancy H. , Webster Henry DeF. , Quarles Richard H. , Cohen Steven R. , Richardson Edward P. 
Published: January 2005

AbstractTo study the distribution of myelin‐associated glycoprotein (MAG) in human nervous tissue and in multiple sclerosis (MS) lesions, we used paraffin sections and our modification of the peroxidase‐antiperoxidase technique. Sections of MS lesions also were treated with antiserum to basic protein (BP) and with histological stains for axons and myelin sheaths. In tissue from normal developing central nervous system, oligodendroglia, their processes, and newly formed myelin sheaths were intensely stained by MAG antiserum. In adults, MAG was found in periaxonal regions of myelinated fibers of the central and peripheral nervous system. The most striking finding in MS lesions was the extension of decreased MAG immunostaining into white matter that appeared normal when treated with BP antiserum or luxol fast blue. In acute early MS lesions the decrease in MAG immunostaining extended far beyond the margin of acute demyelination, where the BP staining of degenerating sheaths often was increased. In chronic inactive plaques, this decrease in periaxonal MAG immunostaining was limited to relatively few fibers in a thin rim around each lesion. These observations suggest that in MS, immunoreactivity of periaxonal MAG is altered before myelin breakdown begins. Early in degeneration, myelin sheaths and their fragments often were more intensely stained by BP antiserum than normal sheaths; later the staining intensity decreased. In shadow plaques, BP antiserum stained some oligodendroglia. Their appearance and location among thinly myelinated axons suggested that these oligondendroglia were forming new sheaths around previously demyelinated axons.
Effects of manipulation of the sensorimotor system on focal motor seizures in the monkey
Authors: Hosokawa Shinichi , Iguchi Takahiko , Caveness William F. , Kato Motohiro , O'Neill Raymond R. , Wakisaka Shinichiro , Malamut Barbara L. 
Published: January 2005

AbstractDuring focal motor seizures induced by injecting penicillin into the face‐hand area of the right motor cortex of 24‐month‐old monkeys, the sensorimotor system was manipulated by three methods. Elimination, by a paralytic agent, of proprioceptive input from contracting muscles and joints did not alter the electrographic expression of the seizure or the pattern of local glucose utilization in cortical or subcortical components of the sensorimotor system. An overall increase in the rate of energy metabolism occurred in the paralyzed monkeys with electrographic seizures. Cryogenic destruction of up to 90% of the ipsilateral ventral caudal globus pallidus had no effect on electrographic or clinical expression of the seizure. Electrical stimulation of the ipsilateral ventral caudal globus pallidus caused reproducible maximum expression of electrographic and clinical seizure phenomena for the 90‐second duration of the stimulus.
Neurotonia: Impulse‐induced repetitive discharges in motor nerves in peripheral neuropathy
Authors: Warmolts John R. , Mendell Jerry R. 
Published: January 2005

AbstractDelayed relaxation of muscle following voluntary contraction was an unusual feature of a mild chronic sensorimotor peripheral neuropathy in an adult. This abnormality resulted from rapid repetitive discharges in motor nerves occurring only in response to passing impulses; there was no spontaneous nerve discharge. Voluntary contraction of affected muscles generated involuntary high‐frequency discharges of motor unit potentials, which persisted briefly after attempted relaxation. Nerve blocks localized independent zones of hyperexcitability in distal and proximal sections of nerve from which such repetitive discharges could be triggered. Intravenous administration of phenytoin diminished the discharge. Examination of intramuscular nerve bundles revealed loss of myelinated nerve fibers with numerous sprouting and remyelinating axons. These findings, along with muscle biopsy changes of neurogenic atrophy and type grouping, strongly favor an axonal neuropathy. An explanation for the repetitive nerve discharge is slow waning of heightened excitability of a motor nerve after passage of an impulse.
Transient global amnesia
Authors: Shuping J. Ross , Rollinson Russell D. , Toole James F. 
Published: January 2005

AbstractTransient global amnesia (TGA) was found in 41 of 76 patients admitted to the North Carolina Baptist Hospital with temporary disturbances of memory. Risk factors for stroke were present in 26(63%) of these 41 patients, and additional risk factors developed in 9 (27%) of the 33 patients available for follow‐up. Follow‐up evaluation in 33 patients (80%) for periods ranging from 1 to 17 years (mean, 60.3 months) revealed a low rate of recurrence (18%) and a still lower rate of progression to stroke (6%). No transient ischemic attacks were reported during this period. In 2 patients, multiple recurrences of TGA ceased after treatment of the underlying condition (polycythemia in 1 case and myxomatous degeneration of the mitral valve in the other).
Glutamate dehydrogenase deficiency in three patients with spinocerebellar syndrome
Authors: Plaitakis Andreas , Nicklas William J. , Desnick Robert J. 
Published: January 2005

AbstractFour nicotinamide‐adenine dinucleotide phosphate‐requiring enzymes were measured in disrupted cultured skin fibroblasts from a 19‐year‐old patient with juvenile onset of a spinocerebellar and extrapyramidal syndrome. There was marked reduction in the activity of glutamate dehydrogenase (GDH) (22% of mean control activity); GDH activity was also decreased in homogenates of leukocytes from this patient (38% of mean control activity). GDH activity was measured in the leukocytes of two siblings afflicted with adult‐onset spinocerebellar syndrome and found to be decreased in both (29% and 31% of mean control activity); an unaffected sibling had normal GDH activity. Mixing experiments with control fibroblast and leukocyte homogenates did not show the presence of a GDH inhibitor in cells from these patients. This allosterically regulated enzyme was stimulated by adenosine 5′‐diphosphate (10−3 M) and inhibited by guanosine 5′‐triphosphate (10−3 M) in both fibroblast and leukocyte homogenates; these changes occurred in equal proportions in the patients and controls. The decreased fibroblast and leukocyte GDH activity persisted at different concentrations of the enzyme's substrates and with successive passages of cultured fibroblasts. GDH may have an important role in the metabolism of glutamate, a putative neurotransmitter in cerebellum, brainstem, and spinal cord. A genetic deficiency of GDH may underlie some forms of spinocerebellar ataxias.