abstract：:Genetic, biochemical and morphological investigations were conducted on skeletal muscle mitochondria from 6 cases of ocular myopathy: 4 cases with Kearns-Sayre syndrome (KSS) and 2 with chronic progressive external ophthalmoplegia. All of these 6 cases showed mitochondrial DNA (mtDNA) deletions in addition to normal s...

journal_title：Journal of the neurological sciences

authors： Degoul F,Nelson I,Lestienne P,Francois D,Romero N,Duboc D,Eymard B,Fardeau M,Ponsot G,Paturneau-Jouas M

更新日期：1991-02-01 00:00:00

abstract：:Duchenne muscular dystrophy (DMD) is a fatal X-linked recessive disorder of muscle in children. The DMD gene product, "dystrophin", is absent from DMD, while the allelic disease, Becker muscular dystrophy (BMD), exhibits dystrophin of abnormal size and/or quantity. But we are still uncertain about the scenario that in...

journal_title：Journal of the neurological sciences

authors： Arahata K,Beggs AH,Honda H,Ito S,Ishiura S,Tsukahara T,Ishiguro T,Eguchi C,Orimo S,Arikawa E

更新日期：1991-02-01 00:00:00

abstract：:The glucocorticoid methylprednisolone (Mepd) increased dystrophin and myosin heavy chain levels in differentiated cultures of cloned human myoblasts. Mepd increased the number of myotubes per area by preventing myotube death and detachment during myogenesis in vitro. Myotube death was the result of an endogenous proce...

journal_title：Journal of the neurological sciences

authors： Sklar RM,Brown RH Jr

更新日期：1991-01-01 00:00:00

abstract：:A single micro-injection of Tumour Necrosis Factor alpha (TNF) or gamma Interferon (IFN-gamma) into the lumbosacral spinal cord of the rat produced meningitis and mononuclear cuffs within the cord, an inflammatory response remarkably similar in pattern to that observed during experimental autoimmune encephalomyelitis ...

journal_title：Journal of the neurological sciences

authors： Simmons RD,Willenborg DO

更新日期：1990-12-01 00:00:00

abstract：:Genetic linkage studies were performed in 12 British families with von Hippel-Lindau disease (VHL) using RFLPs at three loci (DNF15S2, THRB, RAF1) on the short arm of chromosome 3. Linkage was detected between the VHL disease locus and RAF1 with a maximum lod score of 3.88 at a recombination fraction of 0.05 (confiden...

journal_title：Journal of the neurological sciences

authors： Maher ER,Bentley E,Yates JR,Barton D,Jennings A,Fellows IW,Ponder MA,Ponder BA,Benjamin C,Harris R

更新日期：1990-12-01 00:00:00

abstract：:Menkes' disease is an inherited disturbance of copper metabolism. Addition of copper to the medium of cultured fibroblasts and lymphoblasts from patients with Menkes' disease results in an increased induction of metallothionein. We investigated the metallothionein induction in response to copper and zinc in muscle cel...

journal_title：Journal of the neurological sciences

authors： Herzberg NH,Wolterman RA,van den Berg GJ,Barth PG,Bolhuis PA

更新日期：1990-12-01 00:00:00

abstract：:On December 31, 1988 there were 201 registered multiple sclerosis patients in Kuwait, an overall prevalence rate (PR) of 10.2 per 100,000; among them were 186 Arabs, of whom 72 were Palestinians and 51 Kuwaitis. Comparison of these two subgroups, who had a similar age distribution revealed that the disease was 2 1/2 t...

journal_title：Journal of the neurological sciences

authors： al-Din AS,Khogali M,Poser CM,al-Nassar KE,Shakir R,Hussain J,Behbahani K,Chadha G

更新日期：1990-12-01 00:00:00

abstract：:The occurrence of neuropathy in 5 cases of inclusion body myositis (IBM) was studied. The intramuscular nerve branches showed variable ultrastructural changes in all cases. The observed changes were loss of axons, wallerian degeneration and axon terminal atrophy. EMG with concentric needles showed myopathic motor unit...

journal_title：Journal of the neurological sciences

authors： Lindberg C,Oldfors A,Hedström A

更新日期：1990-11-01 00:00:00

abstract：:We studied the production within the CNS of anti-HIV antibodies, of non-specific IgG, and the presence of HIV antigens in the serum and CSF of 28 HIV infected patients belonging to group IV in the Center for Disease Control classification. CSF and serum were diluted under optimal conditions to equalize their IgG conte...

journal_title：Journal of the neurological sciences

authors： Lolli F,Colao MG,De Maio E,Di Pietro M,Galli E,Grippo A,Mazzotta F,Pinto F

更新日期：1990-11-01 00:00:00

abstract：:Structural alterations of endoneurial microvessels occur in diabetic neuropathy and are statistically associated with severity of nerve fiber loss and teased fiber abnormality. It is therefore hypothesized that the microvessel alterations may cause or contribute to pathologic alterations of nerve fibers in diabetic ne...

journal_title：Journal of the neurological sciences

authors： Benstead TJ,Sangalang VE,Dyck PJ

更新日期：1990-10-01 00:00:00

abstract：:Ninety-four asymptomatic patients with internal carotid artery occlusion were followed for a mean of 44 months, 16% suffered strokes and 11.7% reported transient ischemic attacks (TIA). The annual stroke and TIA rates were 4.4% and 3.2%, respectively, the annual mortality was 11.3%. In 27 asymptomatic patients progres...

journal_title：Journal of the neurological sciences

authors： Rautenberg W,Mess W,Hennerici M

更新日期：1990-09-01 00:00:00

abstract：:Arteriolosclerotic leucoencephalopathy in the elderly (ALE) is characterized by white matter lesions associated with atherosclerosis and arteriolosclerosis. Mild lesions are focal and probably represent early status cribosus or incomplete lacunar infarcts. Moderate and severe lesions are diffuse areas of demyelination...

journal_title：Journal of the neurological sciences

authors： Ferrer I,Bella R,Serrano MT,Martí E,Guionnet N

更新日期：1990-08-01 00:00:00

abstract：:In human and subhuman primates, 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) produces irreversible clinical, biochemical and neuropathological alterations highly reminiscent of those observed in Parkinson's disease. The MPTP model has provided the best available tool to date for the assessment of efficacy and s...

journal_title：Journal of the neurological sciences

authors： Bloem BR,Irwin I,Buruma OJ,Haan J,Roos RA,Tetrud JW,Langston JW

更新日期：1990-07-01 00:00:00

abstract：:The muscarinic acetylcholine receptors were identified in membrane preparations from human tissues by the specific binding of 1-[benzilic-4,4'-3H] quinuclidinyl benzilate. Saturation binding isotherms of this radioligand yielded a total amount of receptors of 435 +/- 208, 159 +/- 65 and 913 +/- 89 fmol/mg protein, res...

journal_title：Journal of the neurological sciences

authors： Vanderheyden P,Gies JP,Ebinger G,De Keyser J,Landry Y,Vauquelin G

更新日期：1990-06-01 00:00:00

abstract：:A method has been adapted to produce membrane vesicles suitable for routine membrane patch clamping from neonate rat skeletal muscle. Single K+ channel activity was recorded from cell-free inside-out patches. Most Ca2(+)-activated voltage sensitive channels had large conductances of up to 300 pS, as determined from th...

journal_title：Journal of the neurological sciences

authors： Wareham AC,Rowe IC,Whittle MA

更新日期：1990-05-01 00:00:00

abstract：:We describe a 56-year-old man who had a progressive pseudobulbar palsy, spastic tetraparesis, forced laughing and disturbance of voluntary eyelid closure, and was clinically compatible with chronic progressive spinobulbar spasticity. Magnetic resonance images (MRI) revealed atrophy of the bilateral motor cortices and ...

journal_title：Journal of the neurological sciences

authors： Nishimura M,Tojima M,Suga M,Hirose K,Tanabe H

更新日期：1990-05-01 00:00:00

abstract：:We studied the clinical features of 47 patients with a non-hereditary degenerative disease and with atrophy of brainstem or cerebellum or both in CT scanning. There was no relation between the CT findings and duration or severity of the disease, nor with the kind of the neurological signs which comprised ataxia, a hyp...

journal_title：Journal of the neurological sciences

authors： Staal A,Meerwaldt JD,van Dongen KJ,Mulder PG,Busch HF

更新日期：1990-03-01 00:00:00

abstract：:Quantitative analysis of motor unit potentials (MUPs) with spike triggering and averaging was performed from the mildly affected brachial biceps muscle of 18 patients with amyotrophic lateral sclerosis (ALS) and of 18 age-matched controls. The total MUP duration was only 6% higher in ALS patients than in controls (P l...

journal_title：Journal of the neurological sciences

authors： Partanen J,Nousiainen U

更新日期：1990-02-01 00:00:00

abstract：:The diagnostic value of analogue frequency analysis of EMG from patients with neuromuscular disorders has not been convincing. Using fast Fourier transformation it is today possible to obtain the EMG power spectrum on-line and with a better resolution. We examined the power spectrum of the EMG pattern of the brachial ...

journal_title：Journal of the neurological sciences

authors： Rønager J,Christensen H,Fuglsang-Frederiksen A

更新日期：1989-12-01 00:00:00

abstract：:Two siblings are presented with late onset, rapidly progressive truncal ataxia, paralysis of down-gaze and loss of up-gaze saccades in association with other oculomotor dysfunctions as well as dementia. Electron microscopic muscle studies revealed abnormal distribution and form of the mitochondria, probably being the ...

journal_title：Journal of the neurological sciences

authors： al-Din AS,al-Zuhair AG,al-Salem MK,al-Nassar KE,Rudwan MA,Khafaja S,Hamawi T

更新日期：1989-11-01 00:00:00

abstract：:The effect of aluminum (Al) was studied in an in vitro system of cultured hippocampal neurons from fetal rats in a chemically defined medium. Neuronal aggregates interconnected by neurites were detected light and electron microscopically in control and Al-treated cultures. In the Al-treated cultures disruptions of the...

journal_title：Journal of the neurological sciences

authors： van Welsum RA,van der Voet GB,Marani E,van Keep JP,de Wolff FA

更新日期：1989-11-01 00:00:00

abstract：:Standardised skin biopsies followed by immunohistochemical examination for the presence of terminal nerve fibres reacting for neuropeptides substance P (SP) and calcitonin gene-related peptide (CGRP) were evaluated. Healthy subjects regularly displayed free nerve endings of both fibre types in the papillary and reticu...

journal_title：Journal of the neurological sciences

authors： Lindberger M,Schröder HD,Schultzberg M,Kristensson K,Persson A,Ostman J,Link H

更新日期：1989-11-01 00:00:00

abstract：:Plasma-induced release of platelet-serotonin and Met-enkephalin was studied by incubating platelets from migraineurs, tension headache patients and healthy volunteers with water, plasma collected during migraine attacks and with plasma collected during attack-free periods. Migraine-attack plasma, but not migraine atta...

journal_title：Journal of the neurological sciences

authors： Ferrari MD,Odink J,Frölich M,Tapparelli C,Portielje JE

更新日期：1989-10-01 00:00:00

abstract：:Increased resistance to ischemic nerve conduction failure (RINCF) has been demonstrated in the spontaneously diabetic BB Wistar rat as well as in non-insulin dependent but hyperglycemic litter-mates. There is marked variability in the severity of the ischemic resistance in insulin dependent rats but not in the non-ins...

journal_title：Journal of the neurological sciences

authors： Parry GJ,Kohzu H

更新日期：1989-10-01 00:00:00

abstract：:Human carnitine palmitoyltransferase (CPT) deficiency results in 2 clinical forms: a more common "muscular form" with myoglobinuria with or without delayed or impaired ketogenesis and a rare "hepatic form" with hypoketotic hypoglycemia, encephalopathy and seizures without muscular manifestations. We present 2 patients...

journal_title：Journal of the neurological sciences

authors： Tein I,Demaugre F,Bonnefont JP,Saudubray JM

更新日期：1989-09-01 00:00:00

abstract：:Muscle electrical activity has been studied in mice after intraperitoneal injection of sera from myasthenia gravis (MG) patients. Myasthenic serum did not modify the electrical properties of innervated muscle fibres. The resting membrane potential and the action potential parameters remained unchanged. However, tetrod...

journal_title：Journal of the neurological sciences

authors： Losavio A,Muchnik S,Sica RE,Panizza M

更新日期：1989-07-01 00:00:00

abstract：:Myositis was proven histopathologically in 4 patients (age range 36-66 years) who suffered from early or late stages of Borrelia burgdorferi infection. Muscle weakness was present in 3 patients, 1 complaining of additional myalgias. One man came to medical attention because of skin discoloration and swelling of one le...

journal_title：Journal of the neurological sciences

authors： Reimers CD,Pongratz DE,Neubert U,Pilz A,Hübner G,Naegele M,Wilske B,Duray PH,de Koning J

更新日期：1989-06-01 00:00:00

abstract：:In order to determine if free (F) and bound (B) levels of autoantibodies to myelin basic protein (anti-MBP) are present from the onset of multiple sclerosis (MS), 201 patients referred to our clinic were clinically divided into a group diagnosed as having an initial MS relapse and a group of non-MS controls. Ninety-fo...

journal_title：Journal of the neurological sciences

authors： Warren KG,Catz I

更新日期：1989-06-01 00:00:00

abstract：:In our study, mitochondria were isolated from skeletal muscle in 2-, 3-, 4-, 6-, 8-, and 12-week-old normal (C57BL6j dy/+), and 4-, 8-, and 12-week-old dystrophic (C57BL6j dy/dy) mice and in normal subjects and patients with Duchenne or Becker muscular dystrophy. A deficit was observed in a calcium-specific mitochondr...

journal_title：Journal of the neurological sciences

authors： Lucas-Héron B,Mussini JM,Ollivier B

更新日期：1989-05-01 00:00:00

abstract：:Ischemic brain lesions were induced in adult Wistar and Fischer rats by temporary occlusion of the bilateral common carotid artery. The severity of ischemic lesions depended on the duration of carotid occlusion. While 2 h occlusion resulted in 15 deaths among 40 rats and the development of ischemic lesions in 16 of 25...

journal_title：Journal of the neurological sciences

authors： Iwasaki Y,Ito S,Suzuki M,Nagahori T,Yamamoto T,Konno H

更新日期：1989-04-01 00:00:00

abstract：:The excitotoxin kainic acid (10 nmol/microliter) was used to produce bilateral lesions in the nucleus basalis magnocellularis (NBM) of rats which provides extensive cholinergic innervation to the cerebral cortex. The behavioral effects of physostigmine, THA (9-amino-1,2,3,4-tetrahydroacridine hydrochloride) and NIK-24...

journal_title：Journal of the neurological sciences

authors： Ueki A,Miyoshi K

更新日期：1989-03-01 00:00:00

abstract：:The retrovirus that causes the acquired immunodeficiency syndrome (AIDS) has two targets: the immune system and the nervous system. Central nervous system (CNS) manifestations of AIDS are protean. This review describes the opportunistic infections and malignancies that affect the nervous system in AIDS, and discusses ...

journal_title：Journal of the neurological sciences

authors： Tucker T

更新日期：1989-02-01 00:00:00

abstract：:In order to develop a greater understanding of the importance of peptide catabolism in the intracellular protein degradation process in normal and pathological human brain, we have undertaken a systematic investigation of the aminopeptidase group of enzymes. Although a wide range of aminoacyl-7-amino-4-methylcoumarin ...

journal_title：Journal of the neurological sciences

authors： Mantle D,Lauffart B,Perry EK,Perry RH

更新日期：1989-02-01 00:00:00

abstract：:Three of 4 adult patients with Marinesco-Sjögren syndrome (MSS; 2 males and 2 females, aged 26-31 years) in 2 families became non-ambulant because of slowly progressive muscular weakness rather than cerebellar ataxia. Other clinical features in these 4 patients were typical for MSS: bilateral cataracts from infancy, m...

journal_title：Journal of the neurological sciences

authors： Komiyama A,Nonaka I,Hirayama K

更新日期：1989-01-01 00:00:00

abstract：:The activities of the two polyamine biosynthetic decarboxylases (PBD), L-ornithine decarboxylase (ODC) and S-adenosyl-L-methionine decarboxylase (SAMD), have been measured in quadriceps femoris of rats killed at different times after the induction of calciphylaxis- or serotonin(5-HT)-induced myopathy. Decreases in bot...

journal_title：Journal of the neurological sciences

authors： Lorenzini EC,Colombo B,Ferioli ME,Scalabrino G,Canal N

更新日期：1989-01-01 00:00:00

abstract：:Although peroxisomes were initially believed to play only a minor role in mammalian metabolism, it is now clear that they catalyse essential reactions in a number of different metabolic pathways and thus play an indispensable role in intermediary metabolism. The metabolic pathways in which peroxisomes are involved inc...

journal_title：Journal of the neurological sciences

authors： Wanders RJ,Heymans HS,Schutgens RB,Barth PG,van den Bosch H,Tager JM

更新日期：1988-12-01 00:00:00

abstract：:The progression of clinical disease and serum creatine kinase (CK) levels in canine X-linked muscular dystrophy (CXMD) was studied in 7 dogs from birth to 12-14 months and in 18 dogs at varying intervals from birth to 8 weeks. One affected male was studied from age 3.5 to 6 years, and all pups were descendants of this...

journal_title：Journal of the neurological sciences

authors： Valentine BA,Cooper BJ,de Lahunta A,O'Quinn R,Blue JT

更新日期：1988-12-01 00:00:00

abstract：:The effect of membrane plasma exchange on the course of chronic experimental allergic neuritis in rabbits is described. Using miniature membrane plasma separators conscious animals were treated with 4 exchanges over 5 days removing one plasma volume per procedure and using a non-immunogenic gelatin plasma solution as ...

journal_title：Journal of the neurological sciences

authors： Harvey GK,Schindhelm K,Antony JH,Pollard JD

更新日期：1988-12-01 00:00:00

abstract：:Autoantibodies to myelin basic protein (anti-MBP) can be detected in the cerebrospinal fluid (CSF) of MS patients using a solid phase radioimmunoassay. Acute relapses of MS are characterized by an elevated, above unity, free (F) to bound (B) anti-MBP ratio; while in contrast, patients with chronic progressing disease ...

journal_title：Journal of the neurological sciences

authors： Warren KG,Catz I

更新日期：1988-12-01 00:00:00

abstract：:Twelve patients with neurobrucellosis and 17 patients with systemic brucellosis without neurological involvement underwent a brainstem auditory evoked potentials (BAEP) study. All neurobrucellosis patients (100%) showed abnormalities in their BAEP recordings, suggestive of brainstem lesions at various levels. On the o...

journal_title：Journal of the neurological sciences

authors： Khuraibet AJ,Shakir RA,Trontelj JV,Butinar D,al-Din AS

更新日期：1988-11-01 00:00:00