abstract：:Autopsy cases of two brothers with bulbo-spinal muscular atrophy associated with gynecomastia, testicular atrophy and sensory neuropathy are reported. The disease started with finger tremor, proximal muscle weakness and facial muscle twitching at the second and fourth decades, accompanied by bulbar signs and glove-sto...

journal_title：Journal of the neurological sciences

authors： Nagashima T,Seko K,Hirose K,Mannen T,Yoshimura S,Arima R,Nagashima K,Morimatsu Y

更新日期：1988-11-01 00:00:00

abstract：:We report a 2-year-old girl who presented with delayed development, weakness and persistent vomiting. She had a demyelinating peripheral neuropathy. The activity of cytochrome oxidase in skeletal muscle from the patient was 10% of controls. Immunochemical studies using antibodies to holo-cytochrome oxidase and the ind...

journal_title：Journal of the neurological sciences

authors： Shepherd IM,Birch-Machin MA,Johnson MA,Sherratt HS,Aynsley-Green A,Droste M,Kadenbach B,Stephenson JB,King MD,Dick DJ

更新日期：1988-11-01 00:00:00

abstract：:Two transgenic strains of mutant mice lacking hypoxanthine-guanidine phosphoribosyltransferase (HPRT) activity were examined behaviorally and neurochemically for phenotypic similarity to the human Lesch-Nyhan syndrome. In this syndrome, male children markedly deficient in the enzyme HPRT develop self-mutilation and se...

journal_title：Journal of the neurological sciences

authors： Finger S,Heavens RP,Sirinathsinghji DJ,Kuehn MR,Dunnett SB

更新日期：1988-09-01 00:00:00

abstract：:We recorded saccadic, pursuit and fixation eye movements in patients (n = 5) with moderately advanced Huntington's disease (HD), offspring of HD patients (n = 22) and control subjects (n = 15), using the scleral sensor coil technique. Saccadic slowing was seen in all patients, no controls and (marginally) in a few at-...

journal_title：Journal of the neurological sciences

authors： Collewijn H,Went LN,Tamminga EP,Vegter-Van der Vlis M

更新日期：1988-09-01 00:00:00

abstract：:The production of interleukin-1 (IL-1) and interleukin-2 (IL-2) by peripheral blood mononuclear cells (PBM) was assessed in multiple sclerosis (MS) patients in relapse, chronic progressive MS patients, patients with other neurological diseases (OND) and healthy subjects. Production was defined as the level of IL-1 and...

journal_title：Journal of the neurological sciences

authors： Selmaj K,Nowak Z,Tchórzewski H

更新日期：1988-05-01 00:00:00

abstract：:Electron microscopical (EM) studies were carried out on the retinas of 2-3-(baby), 12-, 14- and 21-28-day-old (adult) mice infected with avirulent (A774) Semliki Forest virus (SFV). Virions (mature virus), spherules and advanced stages of virus replication, cytopathic vacuoles type II (CPV II), were seen in the retina...

journal_title：Journal of the neurological sciences

authors： Pathak S,Webb HE

更新日期：1988-05-01 00:00:00

abstract：:Etiology and source of amyloid deposition in senile plaques of Alzheimer's disease (AD) are still unknown. In order to know whether or not fibronectin (Fn), an adhesive glycoprotein, is related to the amyloid deposition in the senile plaque, we conducted immunohistochemical studies using polyclonal anti-Fn and affinit...

journal_title：Journal of the neurological sciences

authors： Koike F,Kunishita T,Nakayama H,Tabira T

更新日期：1988-05-01 00:00:00

abstract：:Infantile Refsum's disease (IRD) is a peroxisomal deficiency disease which is closely related to neonatal adrenoleukodystrophy (NALD) and the Zellweger syndrome (ZS). Recent observations suggest that NALD and ZS are separate genetic disorders but the delimitation towards IRD remains uncertain. We present here the firs...

journal_title：Journal of the neurological sciences

authors： Torvik A,Torp S,Kase BF,Ek J,Skjeldal O,Stokke O

更新日期：1988-05-01 00:00:00

abstract：:Weanling male Wistar rats fed a diet containing 0.5-2% cuprizone developed intramyelinic edema of the cerebellar white matter, hilum of the dentate nucleus and superior cerebellar peduncle. Oligodendrocytes in these regions showed hyperchromatic nuclei and abnormally dense cytoplasm, with an increase in the number of ...

journal_title：Journal of the neurological sciences

authors： Love S

更新日期：1988-04-01 00:00:00

abstract：:Platelet aggregation (PA) induced by (-)-epinephrine and adenosine diphosphate (ADP) was studied in 16 patients with myotonic dystrophy (MyD) and 14 healthy subjects. Plasma beta-thromboglobulin level (beta-TG), a useful marker of in vivo platelet release reaction, as well as in vitro 5-[14C]hydroxytryptamine (5-HT) r...

journal_title：Journal of the neurological sciences

authors： Bornstein NM,Zahavi M,Korczyn AD,Zahavi J

更新日期：1988-04-01 00:00:00

abstract：:In view of recent reports on lower brainstem dysfunction in Huntington's chorea, we studied respiration during sleep in 12 patients with Huntington's chorea (HC) and in controls. There were no statistically significant differences between patients and controls with respect to apnea periods, respiratory frequency and t...

journal_title：Journal of the neurological sciences

authors： Bollen EL,Den Heijer JC,Ponsioen C,Kramer C,Van der Velde EA,Van Dijk JG,Roos RA,Kamphuisen HA,Buruma OJ

更新日期：1988-03-01 00:00:00

abstract：:In Creutzfeldt-Jakob disease (CJD), there are prominent ultrastructural alterations of the plasma membrane, which contains many glycolipids and glycoproteins. Glycosidases can degrade glycolipids and glycoproteins. Gangliosides, a subset of glycolipids, are decreased in amount at the terminal stages of CJD, and CJD in...

journal_title：Journal of the neurological sciences

authors： Kim JH,Manuelidis L,Manuelidis EE

更新日期：1988-03-01 00:00:00

abstract：:Single channel recording patch-clamp technique was used in the mouse to compare the acetylcholine (ACh)-induced channel behaviour between normal and dystrophic myotubes. While open time and slope conductance were equivalent, ACh-induced channel opening frequency was more than 4-fold reduced in dystrophic compared to n...

journal_title：Journal of the neurological sciences

authors： Grassi F,Cossu G,Eusebi F

更新日期：1988-03-01 00:00:00

abstract：:Observations on streptozotocin-diabetic rats have confirmed overhydration of peripheral nerve. As in previous studies, the sorbitol and fructose content, when expressed in terms of wet weight of nerve was found to be increased and myo-inositol decreased. The reduction in myo-inositol content was less, although still s...

journal_title：Journal of the neurological sciences

authors： Anand P,Llewelyn JG,Thomas PK,Gillon KR,Lisk R,Bloom SR

更新日期：1988-02-01 00:00:00

abstract：:Six clones obtained from the neoplastic, astrocytic murine cell line VMDk P497 were injected intracerebrally into syngeneic hosts and the tumorigenicity of each clone was established. Five of the 6 clones produced tumours with incidences ranging from 25% to 100% and mean latencies of 43-100 days, according to the clon...

journal_title：Journal of the neurological sciences

authors： Koppel H,Pilkington GJ,Lantos PL

更新日期：1988-02-01 00:00:00

abstract：:gamma-Aminobutyric acid (GABA), substance P and dopamine concentrations and choline acetyltransferase (ChAT) activity were measured in post-mortem cerebrocortical and basal ganglial areas of 14 controls and 4 patients with pathologically verified Pick's disease (1 classic case and 3 cases of the generalized form). GAB...

journal_title：Journal of the neurological sciences

authors： Kanazawa I,Kwak S,Sasaki H,Muramoto O,Mizutani T,Hori A,Nukina N

更新日期：1988-01-01 00:00:00

abstract：:Thromboxane-prostacyclin imbalance may be an important determinant of platelet-vessel wall interactions that are vital in circulatory homeostasis. In experimental spinal cord injury, the vascular damage contributes substantially to the process of progressive secondary injury culminating in post-traumatic myelopathy. I...

journal_title：Journal of the neurological sciences

authors： Hsu CY,Halushka PV,Spicer KM,Hogan EL,Martin HF

更新日期：1988-01-01 00:00:00

abstract：:The hippocampus is generally believed to be only rarely affected in Creutzfeldt-Jakob disease. In a systematic study of the hippocampus in 6 cases of Creutzfeldt-Jakob disease, the stratum moleculare-lacunosum showed a definite spongiform change with gliosis in 5 cases. The stratum radiatum et pyramidale showed spongi...

journal_title：Journal of the neurological sciences

authors： Mizusawa H,Hirano A,Llena JF

更新日期：1987-12-01 00:00:00

abstract：:The physiological and histological changes following injection of 10 microliter of proteinase K into the rat tibial nerve have been compared with those induced by proteinase K and specific inhibitor phenylmethylsulphonyl fluoride (PMSF) and also by rabbit experimental allergic neuritis (EAN) serum. Proteinase K and PM...

journal_title：Journal of the neurological sciences

authors： Westland K,Pollard JD

更新日期：1987-12-01 00:00:00

abstract：:Somatosensory evoked potentials (SEPs) following median nerve stimulation were abnormal in 7 patients with sensory impairment due to vitamin B12 deficiency. Extensor plantar reflexes indicated a central sensory pathway lesion in 4 cases and absent tendon jerks suggested peripheral neuropathy in 4, but median nerve SEP...

journal_title：Journal of the neurological sciences

authors： Jones SJ,Yu YL,Rudge P,Kriss A,Gilois C,Hirani N,Nijhawan R,Norman P,Will R

更新日期：1987-12-01 00:00:00

abstract：:By observing the effect of 0.08 and 0.02 mg/ml linoleic acid (LA on the electrophoretic mobility of fresh red blood cells derived from the parents of a subject with DMD, it has been shown that all of 16 fathers as well as 15 mothers, consistently differ from normal. In normal subjects, whilst 0.08 mg/ml LA causes incr...

journal_title：Journal of the neurological sciences

authors： Field EJ,Joyce G

更新日期：1987-12-01 00:00:00

abstract：:Cerebrospinal fluid (CSF) was studied in 2 cases of Devic's syndrome (DS). In one of these cases autopsy was carried out. The main CSF feature in DS is the association of blood-brain barrier damage and intrathecal IgG synthesis. These findings are in keeping with our neuropathological observation of diffuse central ne...

journal_title：Journal of the neurological sciences

authors： Leonardi A,Arata L,Farinelli M,Cocito L,Schenone A,Tabaton M,Mancardi GL

更新日期：1987-12-01 00:00:00

abstract：:Five patients with acute polyneuritis following treatment with rabies vaccine prepared in suckling mouse brains are reported. These patients had severe and protracted disease. The extensive cranial nerve involvement, high mortality rate, and disabling longterm sequelae were in contrast to cases of the Guillain-Barré s...

journal_title：Journal of the neurological sciences

authors： Cabrera J,Griffin DE,Johnson RT

更新日期：1987-11-01 00:00:00

abstract：:Idiopathic Parkinson's disease (PD) may possibly be caused by one or more unidentified neurotoxins present in the environment, or formed endogenously, which progressively damage dopaminergic nigrostriatal neurons. N-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) is an experimental neurotoxin which produces biochemi...

journal_title：Journal of the neurological sciences

authors： Perry TL,Yong VW,Hansen S,Jones K,Bergeron C,Foulks JG,Wright JM

更新日期：1987-11-01 00:00:00

abstract：:Recently it has been reported that the C57BL mice can be used as a model of benign monoclonal gammopathy (MG). Since experimental models have until now failed to reproduce specific lesions of human dysglobulinemic polyneuropathies, we decided to investigate the peripheral nerve of these mice. The sciatic nerve and the...

journal_title：Journal of the neurological sciences

authors： Dib M,Vital A,Vital C,Georgescault D,Baquey A,Bezian J

更新日期：1987-10-01 00:00:00

abstract：:Sequential pairs of cerebrospinal fluid (CSF) and serum samples from 10 patients followed for 2.5-12 years after onset of unilateral optic neuritis (ON) were studied. Eight patients developed definite multiple sclerosis (MS) during the observation period. All patients had normal CSF protein patterns on agar or agarose...

journal_title：Journal of the neurological sciences

authors： Sandberg-Wollheim M,Vandvik B,Nadj C,Norrby E

更新日期：1987-10-01 00:00:00

abstract：:An epidemiological survey of multiple sclerosis (MS) in the State of Queensland was undertaken with its prevalence day being the national census day on June 30th, 1981, 20 years after a regional survey within the State. The relationship between increasing prevalence of MS and increasing south latitude within the State...

journal_title：Journal of the neurological sciences

authors： Hammond SR,de Wytt C,Maxwell IC,Landy PJ,English D,McLeod JG,McCall MG

更新日期：1987-09-01 00:00:00

abstract：:In two different studies, monkeys were fed diets with 50% and 30% of calories replaced with alcohol for 5 and 3 years, respectively. Nerves were studied with electrophysiological and quantitative histological methods, but no deleterious effect of alcohol was identified. ...

journal_title：Journal of the neurological sciences

authors： Hallett M,Fox JG,Rogers AE,Nicolosi R,Schoene W,Goolsby HA,Landis DM,Pezeshkpour G

更新日期：1987-08-01 00:00:00

abstract：:We have used light microscopic histomorphometry to quantify the developmental histopathological changes induced by muscular dystrophy in the soleus and extensor digitorum longus (EDL) muscles of the mdx mouse. We find that this X-linked disease exhibits early fibre necrosis with foci of invasive cells, clustering of a...

journal_title：Journal of the neurological sciences

authors： Carnwath JW,Shotton DM

更新日期：1987-08-01 00:00:00

abstract：:Administration of retinoic acid (RA) prevented the development of experimental allergic encephalomyelitis (EAE) in Lewis rats. RA suspended in corn oil was given for 3 days before the expected onset of the disease to rats immunized with myelin and to controls. The drug suppressed the neurological symptoms as well as t...

journal_title：Journal of the neurological sciences

authors： Massacesi L,Abbamondi AL,Giorgi C,Sarlo F,Lolli F,Amaducci L

更新日期：1987-08-01 00:00:00

abstract：:In five patients with cerebral manifestation of Wilson's disease, copper was measured in CSF, serum, urine and liver, and ceruloplasmin was determined in CSF and serum. CSF copper was found to be elevated in all cases, especially in the four examined before therapy. Two patients were followed up for a period of 3 year...

journal_title：Journal of the neurological sciences

authors： Weisner B,Hartard C,Dieu C

更新日期：1987-06-01 00:00:00

abstract：:The analysis of the firing intervals of motor units has been suggested as a diagnostic tool in patients with neuromuscular disorders. Part of the increase in number of turns seen in patients with myopathy could be secondary to the decrease in motor unit firing intervals at threshold force of the motor units, as noted ...

journal_title：Journal of the neurological sciences

authors： Fuglsang-Frederiksen A,Smith T,Høgenhaven H

更新日期：1987-03-01 00:00:00

abstract：:Long-term effects of MPTP on striatal dopamine (DA) content and the influence of aging on the recovery were investigated in mice. Male C57BL/6J mice, young (2-month-old) and mature (10-month-old), were used. Two different dosage schedules of MPTP, i.e., 4 doses subcutaneous injections of 20 mg/kg each, and 4 doses sub...

journal_title：Journal of the neurological sciences

authors： Saitoh T,Niijima K,Mizuno Y

更新日期：1987-02-01 00:00:00

abstract：:Functional and metabolic recovery of a female cat is described which survived for 1 year following 1 hour global cerebro-circulatory arrest at normothermia. Ischemia was produced by intrathoracal occlusion of the innominate, the left subclavian and both mammary arteries. Following ischemia the animal was kept under in...

journal_title：Journal of the neurological sciences

authors： Hossmann KA,Schmidt-Kastner R,Grosse Ophoff B

更新日期：1987-02-01 00:00:00

abstract：:Vitamin B1- and B-deficient encephalopathy was produced in adult Wistar rats and the effect of methylcobalamin was studied. These experiments were performed in vitamin B1- or B-deficient rats with or without the administration of guanidine, a magnesium antagonist. These encephalopathic rats showed symmetrical lesions ...

journal_title：Journal of the neurological sciences

authors： Goto I,Nagara H,Tateishi J,Kuroiwa Y

更新日期：1987-01-01 00:00:00

abstract：:In an attempt to obtain insight into the pathogenesis of proteolipid apoprotein (PLP)-induced experimental allergic encephalomyelitis (EAE) in Lewis rats (Yamamura et al. 1986), PLP-sensitized lymph node cells or spleen cells were passively transferred into normal or irradiated (400 rad) recipients after incubation wi...

journal_title：Journal of the neurological sciences

authors： Yamamura T,Namikawa T,Endoh M,Kunishita T,Tabira T

更新日期：1986-12-01 00:00:00

abstract：:Alterations in short- and long-latency components of median and tibial somatosensory evoked potentials (SEPs) were studied in patients with lesions in the thalamus and thalamo-cortical radiations. When the lesions were located primarily in the ventro-posterior thalamus, the SEP changes consisted of the following combi...

journal_title：Journal of the neurological sciences

authors： Chu NS

更新日期：1986-12-01 00:00:00

abstract：:A monoclonal antibody (MAb) raised against central nervous system (CNS) myelin (212) and a MAb (308) raised against brain with Semliki Forest virus (SFV) were both found to react against the same CNS glycolipids. Both these MAbs were also found to react strongly with SFV and against certain brain glycolipid fractions ...

journal_title：Journal of the neurological sciences

authors： Khalili-Shirazi A,Gregson N,Webb HE

更新日期：1986-11-01 00:00:00

abstract：:Ophthalmological examination and visual evoked responses (VERs) were repeated at 6-120 (mean 46) months after the first attack of acute optic neuritis in 80 patients who had abnormal VERs in 98 symptomatic eyes at the initial examination. The wide field VER returned to within the normal range in 19/98 (19%) symptomati...

journal_title：Journal of the neurological sciences

authors： Hely MA,McManis PG,Walsh JC,McLeod JG

更新日期：1986-10-01 00:00:00

abstract：:Identification of the Negri bodies in the brain of an 8-year-old boy who died 8 days after a paralytic illness and 20 days after a dog bite, and who had received 9 injections of Semple's anti-rabies vaccine, provided evidence that he died of acute rabies encephalitis and not of post-vaccinal allergic encephalomyelitis...

journal_title：Journal of the neurological sciences

authors： Manghani DK,Dastur DK,Nanavaty AN,Patel R

更新日期：1986-09-01 00:00:00