Abstract：
:We report three related patients with autosomal dominant hereditary motor and sensory neuropathy (HMSN). An unusual and characteristic feature was calf enlargement, caused by muscle fiber hypertrophy predominantly of type 1 fibers. None of the family members showed atrophy of the legs. Sural nerve pathology disclosed marked loss of myelinated fibers and numerous onion bulb formations. While cases of HMSN with calf muscle hypertrophy have been reported, the present pedigree was different from that in any of the previous cases in that no family member showed clinically apparent leg atrophy.
journal_name
J Neurol Scijournal_title
Journal of the neurological sciencesauthors
Sakashita Y,Sakato S,Komai K,Takamori Mdoi
10.1016/0022-510x(92)90274-osubject
Has Abstractpub_date
1992-11-01 00:00:00pages
118-22issue
1eissn
0022-510Xissn
1878-5883pii
0022-510X(92)90274-Ojournal_volume
113pub_type
杂志文章abstract：BACKGROUND:Parkinson's disease (PD) and multiple system atrophy (MSA) are neurodegenerative diseases that can be difficult to diagnose and distinguish from each other. STUDY AIMS AND METHODS: Patients with PD and MSA and controls were studied with magnetic resonance imaging (MRI) using tissue segmentation and outlining...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2005.07.013
更新日期：2005-12-15 00:00:00
abstract：:The Clausen modification of the peripheral blood buffy coat leukocyte migration test was used to test patients with multiple sclerosis, normal subjects and patients with other neurologic diseases for cell-mediated immunity to commercial measles virus, purified nuclear core material from a human neurotropic strain of m...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(76)90026-5
更新日期：1976-07-01 00:00:00
abstract：:Chronic relapsing experimental allergic encephalomyelitis has been induced in juvenile strain 13 guinea pigs with isologous spinal cord in Freund's complete adjuvant. Retention of antigen at the injection site and in the draining lymph nodes was studied by immunocytochemical staining with antiserum to myelin basic pro...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(84)90145-x
更新日期：1984-10-01 00:00:00
abstract：BACKGROUND:Neuromyelitis optica (NMO) is a severe autoimmune inflammatory disorder of the central nervous system. NMO and its abortive forms are referred to as NMO spectrum disorders (NMOSD). NMOSD are mostly associated with antibodies to aquaporin-4 (AQP4-IgG). However, recent studies have demonstrated antibodies to m...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2017.08.3254
更新日期：2017-10-15 00:00:00
abstract：:Lipopolysaccharide (LPS) induces a strong immune response, and pretreatment with low dose of LPS suppresses the production of proinflammatory mediators. In the present study, we investigated the effect of LPS preconditioning on the delayed neuronal death in the gerbil hippocampal CA1 region after 5 min of transient ce...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2010.06.004
更新日期：2010-09-15 00:00:00
abstract：:A suspension of kaolin was injected into the cisterna magna of 44 rats at 2 weeks of age. Animals killed at intervals from 5--19 weeks of age showed varying degrees of hydrocephalus. Light microscopy, scanning and transmission electron microscopy revealed stretching and flattening of the ependymal cells but no signifi...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(78)90046-1
更新日期：1978-05-01 00:00:00
abstract：BACKGROUND:We recently reported that left versus right hemisphere cerebral infarctions patients more frequently have worse outcomes. However our clinical experience led us to suspect that the incidence of malignant middle cerebral artery infarctions (MMCA) was higher in the right compared to the left hemispheric stroke...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/j.jns.2015.04.036
更新日期：2015-01-01 00:00:00
abstract：:Complement-dependent gliotoxic antibody activity was determined in 22 patients with multiple sclerosis (MS) and 19 normal control persons. Peripheral blood serum was collected from MS patients at about 4-week intervals for one year, and the results of cytotoxicity tests correlated with the course of disease. For 10 MS...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(79)90096-0
更新日期：1979-05-01 00:00:00
abstract：BACKGROUND:The likelihood of severe disability and death increases with each recurrent stroke. Repeated intravenous (IV) thrombolysis remains one of the therapeutic options when secondary prevention fails; however, its effects after recurrent stroke are largely unknown. The aim of the present review was to assess the r...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/j.jns.2014.07.039
更新日期：2014-10-15 00:00:00
abstract：:Multiple studies have reported that adult cells of bone marrow origin can differentiate into muscle, skin, liver, lung, epithelial cells, and neurons. To determine whether such cells might produce neurons and other cells in the human brain, we examined paraffin sections from female patients who had received bone marro...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/j.jns.2005.03.017
更新日期：2005-06-15 00:00:00
abstract：:We present an uncommon case of a 38-year-old man presented with bilateral subacute weakness of intrinsic hand muscles, manifesting as bilateral claw-hand, without sensory deficits and absent tendon reflexes in upper arms. Nerve conduction studies showed findings consistent with demyelinating GBS. During the fourth day...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2013.07.009
更新日期：2013-11-15 00:00:00
abstract：:We herein report a 12-year-old girl with a basal ganglia germinoma who presented with right-sided hemiparesis after a minor head trauma. Magnetic resonance (MR) imaging revealed a minimally enhanced lesion involving the left putamen, thalamus, and corona radiata. The lesion showed low-signal intensity on T1-, and high...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2008.02.006
更新日期：2008-07-15 00:00:00
abstract：:Forty-seven spinal cords from subjects between 13 and 95 years of age have been examined; in the majority death occurred suddenly or rapidly from a state of previous good health. The limb motor neurons in the lumbospinal segments have been estimated by counting nucleoli in characeteristic cells in every fifth group of...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(77)90069-7
更新日期：1977-11-01 00:00:00
abstract：:The internodal length of remyelinated internodes was examined by observations on teased CNS nerve fibres following primary demyelination induced by intraspinal injections of lysolecithin into the white matter of cats. A remyelinated internode was identified as a thinly-myelinated internode, where a node of Ranvier was...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(81)90084-8
更新日期：1981-02-01 00:00:00
abstract：:Altered functional connectivity has been associated with the influence of epileptic activity. Abnormalities in connectivity, particularly in dorsal attention (DAN), salience (SN) and default mode (DMN) networks, might contribute to the loss of consciousness during seizures and cognitive deficits in patients with child...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2015.04.054
更新日期：2015-07-15 00:00:00
abstract：:Opsoclonus-myoclonus syndrome (OMS) is characterized by opsoclonus and arrhythmic-action myoclonus that predominantly involves the trunk, limbs, and head. Human herpes virus-6 (HHV-6) can rarely cause encephalitis in immunocompetent children and adults. Here we report on a case of OMS associated with HHV-6 rhomboencep...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2014.04.013
更新日期：2014-06-15 00:00:00
abstract：:Reperfusion following ischemia increases ischemic fiber degeneration (IFD) in diabetic nerves compared to control normoglycemic nerves. The mechanism of this excessive susceptibility is unclear. Since reperfusion injury results in an inflammatory response, we tested the hypothesis that the diabetic state increases the...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2006.03.011
更新日期：2006-08-15 00:00:00
abstract：:To distinguish the different origins of cervical N13 potentials in median nerve somatosensory evoked potentials (SSEPs), cervical N13 potentials were recorded by two different montages. The abnormal patterns of the SSEPs were compared to the abnormal evoked spinal cord responses (ESCPs) recorded from posterior epidura...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/s0022-510x(01)00509-3
更新日期：2001-06-15 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
pub_type： 杂志文章
doi：10.1016/0022-510x(87)90005-0
更新日期：1987-12-01 00:00:00
abstract：:We report two cases of adult-onset leukodystrophy with axonal spheroids in a brother and sister, one with an autopsy, the other with a cerebral biopsy. Symptoms developed at 24 and 33years. The sister died within four years, her brother in 2 and a half. Her brain showed a large lesion in the frontal centrum semiovale ...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2010.06.027
更新日期：2010-10-15 00:00:00
abstract：:The recent history of clinical trials in ALS is marked by a great diversity in trial design. This has limited the comparability of the trial results, and has resulted in intense discussions about which parameters should or should not be evaluated when testing a new drug for ALS. This article discusses, without any cla...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/s0022-510x(97)00240-2
更新日期：1997-10-01 00:00:00
abstract：:We conducted an extended clinical evaluation of localized proton magnetic resonance spectroscopy (MRS) of the brain, performed on various brain diseases using short stimulated echo times. Pathologies studied were mainly multiple sclerosis, stroke, leukoaraiosis, AIDS-related leukoencephalopathies and glial tumors. Oth...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(93)90101-4
更新日期：1993-09-01 00:00:00
abstract：BACKGROUND:Glial cell activation and endothelial dysfunction are thought to contribute to the pathophysiology of cerebral small vessel disease (SVD). The purpose of the present study was to determine if levels of S100B, a protein highly expressed in glial cells, and asymmetric dimethylarginine (ADMA), which promotes en...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2015.04.031
更新日期：2015-07-15 00:00:00
abstract：:Anti-beta 2-GPI antibodies (a beta 2-GPI) were found in serum from patients with anti-phospholipid syndrome (APS) and/or systemic lupus erythematosus (SLE). Since a beta 2-GPI are often found in patients with anti-cardiolipin antibodies (aCL), their role in thrombosis as well as other central nervous system (CNS) mani...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/s0022-510x(98)00027-6
更新日期：1998-04-01 00:00:00
abstract：:In view of the hypothesis that a biochemical abnormality in the childhood forms of neuronal ceroid-lipofuscinosis may lie in the utilization of dolichols in glycoprotein synthesis, we analyzed the oligosaccharide structures of brain glycoproteins in infantile neuronal ceroid-lipofuscinosis (INCL). Lectin affinity chro...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(86)90030-4
更新日期：1986-01-01 00:00:00
abstract：BACKGROUND:The aims of this paper are: 1) to present the data of systemic thrombolysis for ischemic stroke in five Croatian centers from July 2008 till January 2010; 2) to compare the results between centers and; 3) to compare data with previously published results from 2006 to 2008 period from our center, and with the...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2011.10.008
更新日期：2012-03-15 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
pub_type： 杂志文章
doi：10.1016/0022-510x(87)90130-4
更新日期：1987-02-01 00:00:00
abstract：:This paper describes 2 brothers with increasingly severe exercise-induced muscle pain and stiffness, beginning in adolescence. Histochemical studies showed that myoadenylate deaminase activity was absent in the propositus, but present in his younger brother. Biochemical examination of muscle homogenates confirmed thes...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(82)90086-7
更新日期：1982-01-01 00:00:00
abstract：:This is the first report of a patient presenting with a combination of Wallenberg and posterior spinal artery syndromes. The patient developed right hemiplegia and sensory disturbances on the right side of the face and over the whole body. MRI showed infarcts of the cerebellum, medulla oblongata, and upper cervical co...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/s0022-510x(00)00318-x
更新日期：2000-06-15 00:00:00
abstract：:N-methyl-d-aspartate receptor (NMDAR) antagonists, including ketamine and nitrous oxide, are currently intensely studied as rapid-acting antidepressant agents. Interestingly, both of these compounds are also drugs of abuse. Intravenous ketamine, a dissociative anesthetic that induces complex downstream effects via NMD...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/j.jns.2020.116778
更新日期：2020-05-15 00:00:00
