Abstract：
:We performed post-mortem examinations of three patients with progressive neurogenic amyotrophy of long duration. One patient had been clinically diagnosed as having sporadic amyotrophic lateral sclerosis (ALS) and two had been diagnosed with progressive spinal muscular atrophy (PSMA). The disease durations were 10, 17 and 20 years, respectively, and all of the patients died of respiratory failure with no artificial respiratory support. In all of the patients, both the upper and lower motor neuron systems were affected; degeneration of the former was definite, but was milder than that usually encountered in sporadic ALS patients, and the histopathology of the latter was identical to that of sporadic ALS. Light microscopy revealed Bunina bodies, which are characteristic of sporadic ALS, in the remaining anterior horn cells of each patient. In addition, ubiquitin-positive skein-like inclusions were also identified, immunohistochemically, in the remaining anterior horn cells of each patient. Neuron counts indicated that the number of neurons was preserved in Clarke's column in these patients, but was significantly reduced in the intermediolateral nucleus, compared with control subjects. Based on these findings, we think that these three patients, with long disease durations, were affected by essentially the same underlying disease process as that of sporadic, classical ALS. Moreover, we question the neuropathological occurrence of sporadic ALS without involvement of the upper motor neuron system, namely, pure PSMA or lower motor neuron disease.
journal_name
J Neurol Scijournal_title
Journal of the neurological sciencesauthors
Iwanaga K,Hayashi S,Oyake M,Horikawa Y,Hayashi T,Wakabayashi M,Kondo H,Tsuji S,Takahashi Hdoi
10.1016/s0022-510x(96)00297-3subject
Has Abstractpub_date
1997-03-10 00:00:00pages
139-43issue
2eissn
0022-510Xissn
1878-5883pii
S0022-510X(96)00297-3journal_volume
146pub_type
杂志文章abstract：:The mirror system includes a group of neurons in the monkey cortex that discharge both when a movement is executed and when that same movement is observed. An equivalent system in humans has been proposed to mediate action and emotion understanding, being therefore related to theory of mind. Theory of mind (TOM) is th...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/j.jns.2011.07.030
更新日期：2011-11-15 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
pub_type： 杂志文章
doi：10.1016/0022-510x(89)90160-3
更新日期：1989-10-01 00:00:00
abstract：:We assessed the relationship between key trace elements and neurocognitive and motor impairments observed in konzo, a motor neuron disease associated with cassava cyanogenic exposure in nutritionally challenged African children. Serum concentrations of iron, copper, zinc, selenium, and neurotoxic lead, mercury, mangan...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2015.01.007
更新日期：2015-02-15 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：:Hyperthermic isolated limb perfusion (HILP) with tumor necrosis factor alpha (TNFalpha) is effective for advanced melanoma and sarcoma of the limbs. Ten patients undergoing HILP with TNFalpha were evaluated by neurological examinations, nerve conduction studies (NCS), sympathetic skin responses (SSR) and conventional ...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/s0022-510x(98)00098-7
更新日期：1998-06-11 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
pub_type： 杂志文章
doi：10.1016/0022-510x(88)90251-1
更新日期：1988-11-01 00:00:00
abstract：:We ascertained two families in Eastern Canada segregating a form of ataxia consistent with a recessive mode of inheritance. We performed a whole genome scan using dense SNP genotyping, and despite an absence of shared homozygosity in the families we defined linkage to a small region on chromosome 13. Direct DNA resequ...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2009.09.034
更新日期：2010-01-15 00:00:00
abstract：:Depression is frequently associated with Parkinson disease (PD) but neural basis is still unclear. In previous studies white matter changes present as signal hyperintesities on T2-wighted MRI studies (WMHs) commonly observed in older adults have been associated with depressive symptomatology. In this study we investig...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2012.07.021
更新日期：2012-11-15 00:00:00
abstract：BACKGROUND:Contactin-associated protein 2 (Caspr2) antibody is a neuronal surface antibody (NSAb) capable of causing disorders involving central and peripheral nervous systems (PNS). Thymoma can be found in patients with Caspr2 antibodies and is most frequently associated with PNS symptoms. Myasthenia gravis can be fou...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2014.03.043
更新日期：2014-06-15 00:00:00
abstract：:The lead content of cerebrospinal fluid (CSF) was found to be significantly elevated in 12 patients with amyotrophic lateral sclerosis, when compared to 28 control subjects having non-degenerative neurological disorders. The difference could not be explained as being merely secondary to blood-CSF barrier damage. A hyp...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(76)90175-1
更新日期：1976-10-01 00:00:00
abstract：:A 55-year-old woman developed an intractable right orbitofrontal headache. The symptoms subsided spontaneously 2 months after onset, but diplopia due to right abducens nerve palsy had occurred, and gradually worsened. Orbito-ocular signs were never observed throughout the clinical course. Brain MRI and MR angiography ...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2005.03.037
更新日期：2005-09-15 00:00:00
abstract：:Two siblings who exhibited hereditary parkinsonism with pyramidal signs and cerebellar ataxia are reported. Anticholinergics had a dramatic beneficial effect in both cases, but levodopa did not. This responsiveness, which is similar to that reported in patients with Joseph's disease, suggests dysfunction of an "indire...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(94)90028-0
更新日期：1994-09-01 00:00:00
abstract：:Although control of hypertension is established as an important factor in the primary and secondary prevention of stroke, management of blood pressure in the setting of acute ischemic stroke remains controversial. Given limited data, the general consensus is that there is no proven benefit to lowering blood pressure i...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/j.jns.2007.04.033
更新日期：2007-10-15 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：:Neuropsychological impairment is a common manifestation in multiple sclerosis (MS) and is found in 40-60% of patients. The pattern of cognitive impairment in MS is characterized by difficulties in recent memory, sustained attention, executive functions and information processing speed. These cognitive deficits have a ...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/j.jns.2005.07.021
更新日期：2006-06-15 00:00:00
abstract：:We describe here a 10-year-old patient with high levels of serum IgE and inflammatory myopathy whose muscle fibers exhibit excessive autophagy. Previous studies have demonstrated surface expression of class I MHC antigens on muscle fibers from patients with inflammatory myopathy. The muscle fibers of this patients sho...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(91)90195-d
更新日期：1991-11-01 00:00:00
abstract：:Recent evidence has suggested that Alzheimer's disease may result from an underlying defect of protein catabolism. In an attempt to identify such a defect, we have determined the levels of Ca(2+)-activated proteinase (principally calpain II) and endogenous inhibitor (calpastatin) activity in normal and Alzheimer's dis...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(91)90072-f
更新日期：1991-04-01 00:00:00
abstract：:The pathogenic mechanism underlying the vascular changes in Binswanger's encephalopathy (BE) is unknown. To test whether alterations of the humoral immunity may lead to endothelium damage, we analyzed serum levels of anti-brain endothelium antibodies (ABEA) (IgG and IgM) in 16 BE patients, 19 subjects with ischemic va...
journal_title：Journal of the neurological sciences
pub_type： 临床试验,杂志文章
doi：10.1016/0022-510x(94)00215-a
更新日期：1995-01-01 00:00:00
abstract：:We studied a 21-year-old patient with clinical, biochemical and histochemical evidence of myophosphorylase deficiency and unusual repetitive episodes of pigmenturia. His muscle biopsy also revealed morphological signs of mitochondrial proliferation and a defect of complex I of the respiratory chain. His mother had exe...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/s0022-510x(98)00263-9
更新日期：1998-12-11 00:00:00
abstract：:To determine the prevalence and subtypes of dementia in southern Taiwan, a two-phase study consisting of a phase I screening survey using the Mini-Mental Status Examination (MMSE) and a phase II diagnostic examination using the CERAD neuropsychological battery and the neurobehavioral examination was conducted. Accordi...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/s0022-510x(98)00225-1
更新日期：1998-09-18 00:00:00
abstract：INTRODUCTION:Although cerebral small vessel disease is a significant contributor to the development of imbalance and falls in the elderly, whether it causes dizziness is not known. METHODS:A retrospective case analysis was conducted for 122 dizzy patients referred to two neuro-otology tertiary centres in London and Pi...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,多中心研究
doi：10.1016/j.jns.2015.09.006
更新日期：2015-11-15 00:00:00
abstract：OBJECTIVE:Aneurysm growth is a risk factor for rupture, however the detailed mechanism remains unclear. The present study was performed to identify whether hemodynamic insult could prompt small unruptured aneurysms to grow. METHODS:Six pairs of unruptured small (<5 mm) cerebral aneurysms from patients followed with lo...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2020.116798
更新日期：2020-06-15 00:00:00
abstract：:To elucidate a possible role of vitamin A in the pathogenesis of Parkinson's disease (PD) we compared serum levels of retinol (vitamin A), measured by HPLC, and the vitamin A/retinol binding protein (RBP) ratio of 42 PD patients (22 males and 20 females, mean age 67.3 +/- 1.34 years) and their respective spouses as co...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(92)90114-z
更新日期：1992-08-01 00:00:00
abstract：:Laryngeal stridor is recognized as a characteristic clinical manifestation in patients with multiple system atrophy (MSA). However, the pathogenic mechanisms underlying this symptom are controversial. Neurogenic atrophy of the posterior cricoarytenoid muscle has been identified in cases of MSA, suggesting that larynge...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/j.jns.2016.01.007
更新日期：2016-02-15 00:00:00
abstract：:Corticosteroids (Cs) are widely used for treatment of multiple sclerosis (MS) acute relapses because of the potent immunosuppressive and anti-inflammatory properties. As for patients with relapsing-remitting (RR) MS, short-term administrations of Cs markedly less severity of symptoms and promote faster recovery of cli...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/j.jns.2004.04.019
更新日期：2004-08-15 00:00:00
abstract：BACKGROUND:CYP2D6 polymorphisms have been reported to be associated with Parkinson's disease (PD) susceptibility, but the results of these previous studies were inconsistent. OBJECTIVES:To explore whether PD patients with CYP2D6 gene variation have different risk to PD to those with normal function of CYP2D6. METHODS...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,meta分析
doi：10.1016/j.jns.2013.10.030
更新日期：2014-01-15 00:00:00
abstract：:Since the original description of side effects of neuroleptics, different terminologies and definitions for tardive dyskinesia (TD) and tardive syndrome (TS) have been used by different authors, and often these two terms have been used interchangeably. This paper proposes a nosology designed to define and clarify vari...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2018.02.008
更新日期：2018-06-15 00:00:00
abstract：:The brain constitutes an environment that is specifically designed to accommodate, regulate and shape immune responses. On one hand, the central nervous system (CNS) has traditionally been regarded as an immunologically privileged organ, owing to local tissue barrier and immunosuppressive microenvironment. On the othe...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/s0022-510x(98)00049-5
更新日期：1998-04-15 00:00:00
abstract：:Zika virus infection represents a new neuropathological agent with association to a wide spectrum of neurological complications: a) Congenital Zika Syndrome by affecting the neural stem cells of the human fetal brain; b) Guillain-Barré Syndrome by an autoimmune response against peripheral myelin and/or axonal componen...
journal_title：Journal of the neurological sciences
pub_type： 社论
doi：10.1016/j.jns.2017.10.046
更新日期：2017-12-15 00:00:00
abstract：:The 3243A>G mutation of mtDNA usually is associated with MELAS syndrome. Here we report a patient with the 3243A>G mutation presenting only recurrent muscle fatigue and elevated levels of serum creatine kinase (CK). The mother of the proband was referred to us for type 2 diabetes mellitus, muscle pain and sensorineura...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2014.01.010
更新日期：2014-03-15 00:00:00
