bluuebunny/crossref_metadata_2025_split huggingface dataset:
Antiserum‐mediated demyelination: Relationship between remyelination and functional recovery
Authors: Saida Kyoko , Sumner Austin J. , Saida Takahiko , Brown Mark J. , Silberberg Donald H. 
Published: January 2005

AbstractA focal demyelinative lesion of peripheral nerve was produced by intraneural injection of either antiserum from rabbits with experimental allergic neuritis or experimental allergic encephalomyelitis or antiserum to galactocerebroside. We studied the relationship between clinical and electrophysiological recovery from this lesion and the morphological pattern of remyelination. Foot muscles on the injected side weakened within an hour of injection and remained paralyzed for 7 days; strength gradually returned to normal by 16 days after injection. Electrophysiological conduction block, apparent within a few hours of injection, persisted for about 7 days. At 8 days we detected dispersed, very low amplitude muscle action potentials with long latency. Morphologically, demyelinated axons were surrounded by Schwann cells at 7 days after injection, but compacted myelin was not present. After 8 days, remyelinating axons became surrounded by thickening compacted myelin. The time of onset of remyelination and the rate of remyelination up to 14 days following the injection were independent of axon size. The onset of clinical and electrophysiological recovery from the lesion corresponded to the appearance of 2 to 8 myelin lamellae around each remyelinating axon. At 37 days after injection, when conduction velocities had returned to preinjection values, myelin thickness of remyelinating fibers had increased to approximately one‐third that of control nerves.
Effects of stroke on local cerebral metabolism and perfusion: Mapping by emission computed tomography of 18FDG and 13NH3
Authors: Kuhl David E. , Phelps Michael E. , Kowell Arthur P. , Metter E. Jeffrey , Selin Carl , Winter James 
Published: January 2005

AbstractBy means of emission computed tomography (ECT), we used 18F‐fluorodeoxyglucose (18FDG) and 13N‐ammonia (13NH3) as indicators of abnormalities in local cerebral glucose utilization (LCMRglc) and relative perfusion, respectively. The ECAT positron tomograph was used to scan normal control subjects and 10 stroke patients at various times during recovery. In normal subjects, mean CMRglc was 5.28 ± 0.76 mg per 100 gm tissue per minute (mean ± SD; N = 8). In patients with stroke, mean CMRglc in the contralateral hemisphere was moderately decreased during the first week, profoundly depressed in irreversible coma, and normal after clinical recovery.Quantification was restricted by incomplete understanding of tracer behavior in diseased brain, but relative local distributions of 18FDG and 13NH3 trapping qualitatively reflected the increases and decreases as well as coupling and uncoupling expected for local alterations in glucose utilization and perfusion in stroke. Early after cerebrovascular occlusion there was a greater decrease in local trapping of 13NH3 than 18FDG within the infarct, probably because of increased anaerobic glycolysis. Otherwise, 18FDG was a more sensitive indicator of cerebral dysfunction than was 13NH3. Hypometabolism, due to deactivation or minimal damage, was demonstrated with the 18FDG scan in deep structures and broad zones of cerebral cortex that appeared normal on x‐ray computed tomography and technetium 99m pertechnetate scans.In its present state of development, the 18FDG ECT method should aid in defining the location and extent of altered brain in studies of disordered function after stroke. With improved knowledge of tracer behavior in diseased brain, the method has promise for mapping the response to therapeutic intervention and increasing our understanding of how the human brain responds to stroke.
Blindness following tentorial herniation
Authors: Keane James R. 
Published: January 2005

AbstractIn seven patients, permanent bilateral visual loss was the chief neurological residual following relief of tentorial herniation. The causes of herniation were unilateral subdural hematoma in three patients and bilateral subdural hematomas, bilateral subdural empyemas, traumatic intracerebral hematoma, and postoperative infarction in one patient each. Three patients, including two with occipital infarction documented by computed tomographic scan, developed optic atrophy, indicating that both pregeniculate visual pathway damage and posterior cerebral artery compression occurred.Increasing availability of computed tomographic scanning should help eliminate this rare cause of blindness by facilitating earlier diagnosis of supratentorial masses.
Multiple sclerosis in a migrant population: 2. Half‐orientals immigrating in childhood
Authors: Kurtzke John F. , Bui‐Quoc‐Huong  
Published: January 2005

AbstractWe studied offspring of Vietnamese mothers and French fathers who had been born in Vietnam and brought to France to be raised. All but 72 of 3,451 such individuals completed background and neurological questionnaires. Those with positive answers on the latter were examined neurologically. The median year of birth for the 3,379 respondents was 1948, the median year of arrival in France was 1957, and they had resided in France a median of 18 years before interview in 1975. Twenty‐five persons had neurological symptoms, and 6 others reported a history of seizures alone. A total of 16 reported seizures: 7 with infantile febrile fits and 9 with afebrile seizure disorders.Three cases of exacerbating‐remitting multiple sclerosis (MS) were discovered as well as 3 persons in whom MS was suspected: 1 with cervical myelopathy, 1 with progressive paraparesis, and 1 with bilateral optic neuropathy. The 3 MS cases provided a crude prevalence rate (or 18‐year cumulative risk) of 89 per 100,000. Their age‐specific prevalence rate was 169 per 100,000 population aged 20 through 29 years. Each measure was similar to such rates for Denmark and had lower 95% confidence limits that clearly exceeded expectations for Vietnam. The results provide further evidence that the risk of MS can be increased by migration in childhood from low‐ to high‐risk regions.
Diminished epidermal growth factor binding by neurofibromatosis fibroblasts
Authors: Zelkowitz Marvin , Stambouly Joseph 
Published: January 2005

AbstractNeurofibromatosis (NF) is an autosomal dominant disease characterized by growth abnormalities of epithelial, mesothelial, and endothelial elements. We recently reported abnormal growth and morphology of NF fibroblasts in tissue culture. Because epidermal growth factor (EGF) is known to stimulate the growth of fibroblasts in tissue culture, we studied the binding of commercial iodine 125‐labeled EGF to age‐ and passage‐matched confluent NF (N = 6) and normal (N = 4) fibroblasts. Fibroblasts were maintained at 37°C for 2, 30, 60, 120, and 240 minutes in a medium in which the cells grow slowly (Dulbecco's Eagle medium) and one in which they grow normally (Ham's F‐12 medium). Binding assays were done in both serum‐free media according to accepted procedures. The EGF binding did not differ in the two media, and pooled data are presented. These data demonstrate no significant differences in the early binding of EGF to normal and NF fibroblasts (4,682 ± 1,092 versus 3,441 ± 826 cpm/106 cells; 20,000 cpm/ng; p > 0.15 at 30 minutes). At one hour, however, differences suggestive of abnormal EGF binding become apparent (12,495 ± 1,989 versus 3,172 ± 853 cpm/106 cells; 20,000 cpm/ng; p < 0.0025). We conclude that there may be a membrane defect in NF which is reflected by diminished EGF binding.
Torsion dystonia in israel
Authors: Korczyn Amos D. , Kahana Esther , Zilber Nelly , Streifler Max , Carasso Raphael , Alter Milton 
Published: January 2005

AbstractA country‐wide search for idiopathic torsion dystonia (ITD) in Israel between 1969 and 1975 revealed 42 patients (41 Jewish and 1 Druze Arab). Prevalence of ITD per million population, age‐adjusted to the United States population in 1970, was 10.8 in the total Jewish population (22.0 among Jews of European extraction contrasted with 1.5 among Jews with Afro‐Asian forebears). Among Europeans, the highest prevalence was among Jews from Eastern Europe.The average age‐adjusted annual incidence rates per million population were 0.43 in the total Jewish population, 0.98 in the Europeans, and 0.11 in the Afro‐Asians. Among the 40 patients for whom familial data were available, the majority of cases (26) were sporadic. The other 14 belonged to four unrelated European families, all of Russian‐Polish origin. The pattern of inheritance in these four families fits an autosomal dominant model with incomplete penetrance.
Periodic movements in sleep (nocturnal myoclonus): Relation to sleep disorders
Authors: Coleman Phd Richard M. , Pollak Charles P. , Weitzman Elliot D. 
Published: January 2005

AbstractPeriodic movements in sleep (PMS) are stereotyped, repetitive, nonepileptiform movements of the lower extremities. A total of 409 sleep disorder patients were studied with all‐night polysomnogram recording, and 53 (13%) had PMS. Such movements occurred in a wide variety of sleep‐wake disorders in addition to insomnia. The prevalence and magnitude of PMS were not statistically greater in patients with insomniac disorders than in those with syndromes of excessive daytime sleepiness or other sleep‐wake disorders. The results suggest that although PMS is responsible for disturbed sleep in relatively few patients, chronic sleep‐wake disturbance is associated with PMS and may lead to the development of these movements.
Review of 38 cases of subacute sclerosing panencephalitis: Effect of amantadine on the natural course of the disease
Authors: Robertson William C. , Clark David B. , Markesbery William R. 
Published: January 2005

AbstractThirty‐eight cases of subacute sclerosing panencephalitis (SSPE) were reviewed. Deterioration in school performance, personality changes, and seizures were common early symptoms. Initial examination frequently showed myoclonus, spasticity, and extrapyramidal dysfunction, and in two‐thirds of the patients these findings were asymmetrical or focal. Retinitis or papilledema was present on initial examination in 50% of the patients. At last follow‐up 24 children had died, with a mean survival of 42 months. Most patients reached a state of severe neurological impairment within 13 months. Subsequent evidence of improvement was noted in 10 children and was sustained in 4. Fifteen patients received antiviral treatment. Ten treated patients died from 5 to 133 months (mean, 58) from onset of their illness, while 15 untreated patients survived a mean of 33 months.Duration of survival appeared to be affected most by treatment with amantadine. Three patients treated with the drug were alive 97 to 139 months after onset of SSPE, and 5 died with a mean survival of 78 months. Five of 6 individuals treated with rifampin died after a mean survival of 27 months. Prolonged remissions occurred only in patients treated with amantadine. Although the number of treated individuals was small, our data suggest that amantadine may affect the natural course of SSPE.
Range of variations in motor unit potentials during reinnervation after traumatic nerve lesions in humans
Authors: Borenstein S. , Desmedt J. E. 
Published: January 2005

AbstractSuccessive stages in the reinnervation of denervated muscle fibers following a complete or partial motor nerve lesion in humans were analyzed with the concentric needle electrode during voluntary contraction. Motor unit potential (MUP) patterns were displayed by coherent EMG, in which the potential triggers an oscilloscope sweep and a digital delay line enables the early part of the MUP to be displayed. After a complete nerve lesion, MUPs showing the response of as few as one to three muscle fibers can be observed. MUPs become highly polyphasic as more muscle fibers are incorporated. Marked latency jitter and intermittent blocking of components as well as the occurrence of linked potentials result in complex patterns that may be confused with a “myopathic” pattern of brief small spikes if conventional observation of free‐running sweeps is used alone. After a partial nerve lesion, the healthy motor axons achieve extensive collateral reinnervation of the denervated muscle fibers whereby linked potentials of various latencies are added after the original MUP.Adequate understanding of these characteristic features can help clarify some current issues in electromyography. The mechanisms by which long‐latency linked potentials and desynchronized components are produced during remodeling of motor units in the course of regeneration involve a number of factors such as slow conduction along regenerated axon branches, ectopic motor end‐plates, insecure neuromuscular transmission, and muscle fiber atrophy.
Restoration of dopaminergic function by grafting of fetal rat substantia nigra to the caudate nucleus: Long‐term behavioral, biochemical, and histochemical studies
Authors: Freed William J. , Perlow Mark J. , Karoum Farouk , Seiger Ake , Olson Lars , Hoffer Barry J. , Wyatt Richard Jed 
Published: January 2005

AbstractMotor deficits produced in rats by unilateral substantia nigra lesions have been found to be reduced by grafts of fetal rat substantia nigra to the dopamine‐denervated caudate nucleus. In the present study these grafts were examined behaviorally, histochemically, and biochemically over six‐ to 10‐month periods. The grafts were found to survive in a healthy condition and contain catecholaminergic cells and fibers after eight to ten months. Concentrations of dopamine in adjoining parts of the caudate nucleus were increased when examined six months after grafting. Apomorphine‐induced rotation was reduced by the grafts, and these reductions persisted for at least six months. Although signs of aging were observed in the brains of the host animals when sacrificed eight to ten months after grafting, the grafts remained healthy and showed no signs of aging or deterioration. It is concluded that substantia nigra grafts can become permanent, functional constituents of the brains of host animals with prior substantia nigra lesions.
Lymph nodes—A possible site for sympathetic neuronal regulation of immune responses
Authors: Giron Louis T. , Crutcher Keith A. , Davis James N. 
Published: January 2005

AbstractNoradrenergic fibers were identified in rat cervical lymph nodes by fluorescence histochemistry and confirmed by radioenzymatic determination of norepinephrine. After superior cervical ganglionectomy, lymph node norepinephrine and noradrenergic fluorescence were strikingly decreased. The α‐adrenergic radioligand [3H] dihydroergocryptine bound to saturable sites on lymph node membranes (0.20 pmol/mg protein; affinity constant, 8 nM) with characteristics expected for α‐adrenergic receptor binding. The α‐adrenergic binding sites were present in high concentration in membranes from lymph node stroma but were negligible in membranes of lymphocytes teased from nodes. In lymph nodes the ratio of α‐adrenergic binding sites to norepinephrine content was higher than ratios of many other sympathetically innervated tissues such as iris or vas deferens. The presence of sympathetic innervation and the high concentration of α‐adrenergic binding sites relative to that innervation suggest that the sympathetic nervous system modulates an intrinsic lymph node function through α‐adrenergic mechanisms.
Treatment of periodic alternating nystagmus
Authors: Michael Halmagyi G. , Rudge Peter , Gresty Michael A. , John Leigh R. , Zee David S. 
Published: January 2005

AbstractTwo patients with longstanding acquired periodic alternating nystagmus (PAN) were treated with baclofen, 30 mg/day. Baclofen abolished the PAN and relieved oscillopsia in both patients but was ineffective in another patient with congenital PAN.
Epilepsy in adults
Authors: So Elson L. , Penry J. Kiffin 
Published: January 2005

AbstractThe past decade has seen advances in the management of patients with epilepsy. The development of practical long‐term electroencephalographic techniques, with or without simultaneous video recording, has increased the accuracy of diagnosis of seizure types. The technique also provides clinicians and investigators with a method for establishing the clinical efficacy of antiepileptic drugs and determining their therapeutic serum concentrations. Computerized tomography has enhanced the identification of structural brain lesions. Most of the reported CT abnormalities consist of diffuse and focal atrophies, mild ventricular dilatations, and porencephalies. CT has detected tumors in 8 to 10% of the patients regardless of age or type of seizure involved. New concepts of antiepileptic drug therapy have developed from the recognition of pharmacological properties peculiar to each agent. Determination of serum antiepileptic drug levels has to be utilized to reduce the problem of pharmacokinetic variability from one patient to another and in the same patient at different times, so that dosage can be individualized to achieve maximum therapeutic effects with least toxicity. Review of the literature on pregnancy in epileptic women shows that a third to half experienced more seizures during gestation. Reduced serum levels of most antiepileptic drugs have recently been observed during gestation. Infants of epileptic women taking antiepileptic drugs have a two to three times greater risk for congenital anomalies than infants of nonepileptic women. However, with the exception of oxazolidinediones, evidennce to date has not proved the teratogenicity of antiepileptic drugs. The role of genetic factors and the effect of seizures during pregnancy have not been determined.Modest progress has been made in epilepsy rehabilitation, but serious problems still remain. The unemployment rate of persons with epilepsy is twice the national average. Half of those who are successfully employed did not disclose their disorder at the time of employment. Several prognostic indicators have been reported, but the validity of many of these indicators is questionable. For example, does shorter life expectancy apply to all subgroups, or does it vary according to seizure type and cause? The life expectancy, treatment response, and proability of remission in epileptic persons must be reevaluated after consistent applications of current methods of epilepsy management.
Diabetic thoracoabdominal neuropathy: Clinical and electrodiagnostic features
Authors: Sun Sallie F. , Streib Erich W. 
Published: January 2005

AbstractFive diabetic patients developed upper back or abdominal pain associated with substantial weight loss in three. Electrophysiological evidence of associated thoracoabdominal somatic neuropathy was found in all cases. Signs of a generalized neuropathy were present in two patients, and four had asymmetrical proximal leg weakness. At least two have improved spontaneously. The syndrome is closely related to diabetic amyotrophy; it is probably more common than is recognized.
Clinical and electroencephalographic characteristics of midline parasagittal foci
Authors: Pedley Timothy A. , Tharp Barry R. , Herman Kathleen 
Published: January 2005

AbstractWe report the clinical and electroencephalographic (EEG) characteristics of 14 patients with midline parasagittal epileptogenic foci. Montages not including the vertex electrodes demonstrated the abnormal activity poorly or not at all. The clinical manifestations were diverse and included tonic‐clonic, atypical absence, and partial seizures. In the majority, no cause could be established. The interictal EEG abnormality did not predict the pattern of clinical attack. Half the patients had evidence of structural brain disease. Four patients had no history of seizures.
An ultramicroscopic study of skin and conjunctival biopsies in chronic neurological disorders of childhood
Authors: Arsenio‐Nunes M. L. , Goutières F. , Aicardi J. 
Published: January 2005

AbstractOne hundred twenty‐seven skin and 59 conjunctival biopsies were performed in 136 pediatric patients with chronic neurological disorders. Ultrastructural abnormalities were found in 48 of 72 patients with unequivocally progressive encephalopathies (67 of which could not be diagnosed by biochemical means) and in 4 patients with miscellaneous diseases (fetal rebulla syndrome and hereditary motor‐sensory neuropathy), but in none of 55 patients with static or dubiously progressive encephalopathies. Of the 48 positive biopsies in the group with progressive encephalopathies, 39 showed abnormalities characteristic enough to permit a specific diagnosis (lysosomal storage disorders, neuroaxonal dystrophy, ophthalmoplegia‐plus) in conjunction with a compatible clinical picture. In 9 cases no diagnosis could be made despite the finding of lamellar or vacuolar inclusions. In no case were discrepancies observed between the results of skin and conjunctival biopsies. Although biochemical analysis is the simplest and most effective technique for the diagnosis of most progressive encephalopathies, skin or conjunctival biopsy is a valuable procedure in patients without detectable enzyme deficiency.
Blepharospasm and oromandibular dystonia (Meige's syndrome) in sisters
Authors: Nutt John G. , Hammerstad John P. 
Published: January 2005

AbstractIsolated idiopathic oromandibular dystonia and blepharospasm (Meige's syndrome) has generally been described as a nonfamilial disorder. This report describes Meige's syndrome in 2 sisters free of other neurological and psychiatric disease.
GM1 Gangliosidosis: Phenotypic variation in a single family
Authors: Farrell Donald F. , Ochs Ute 
Published: January 2005

AbstractInfantile, juvenile, and adult forms of GM1 gangliosidosis have been well characterized. Certain genetic and biochemical studies have suggested that the phenotypic variation found in GM1 gangliosidosis results from different allelic mutations affecting the GM1 ganglioside β‐galactosidase locus and that different combinations of these mutations account for the clinical heterogeneity of this illness. A family in which both the infantile and juvenile forms of GM1 gangliosidosis occurred, the children sharing a common mutation of their acid β‐galactosidase activity, supports the allelic nature of these different clinical forms of the disease. From the observations made in this unique family, additional phenotypes of GM1 gangliosidosis might be anticipated.
GM1 Gangliosidosis: Enzymatic variation in a single family
Authors: Farrell Donald F. , Macmartin Marian P. 
Published: January 2005

AbstractAcid β‐galactosidase activity can be separated into multiple molecular forms by isoelectric focusing on cellulose acetate membranes. The residual acid β‐galactosidase in the juvenile form of GM1 gangliosidosis has three bands of enzyme activity with an apparent isoelectric pH (pI) range from 4.9 to 5.2, whereas that in the infantile form has a single band with an apparent isoelectric pI of 5.2. Separation of residual acid β‐galactosidase into multiple molecular forms by analytical isoelectric focusing demonstrates enzymatic differences that can be correlated with the allelic mutations that affect the GM1 ganglioside β‐galactosidase locus.