摘要:

Controversy regarding the nosological position of the syndrome of ophthalmoplegia, ataxia and areflexia (Miller Fisher syndrome) exists. The oculomotor dysfunction was presumed to represent an unusually symmetrical peripheral cranial nerve dysfunction. To investigate the neuro‐ophthalmic manifestations in this rare syndrome we reviewed 109 reports describing 243 cases. The ophthalmoplegia was remarkable in its constant association with a cerebellar type ataxia. It was described to be remarkably symmetrical at all stages of development and recovery. From the early description of the syndrome by Fisher the ophthalmoplegia was observed to evolve as a symmetrical failure of upgaze followed by loss of lateral gaze and last by downgaze, recovery develops in the opposite pattern. Despite the severe nature of the ophthalmoplegia, 58 patients were reported to have sparing of downgaze and 192 (79%) had relative sparing of the eye lids. Active lid retraction and preserved Bell's phenomenon, despite upgaze paralysis, were described in 22 and 15 patients respectively. Upper lid jerks were described in 2, Parinaud's syndrome in 2, convergence spasm in 6, internuclear ophthalmoplegia in 15 and horizontal dissociated nystagmus in 11. Interestingly 23 were reported to present with paralysis of abduction progressing to lateral gaze paralysis and 5 had paralysis of abduction and contralateral gaze paralysis. Four had defective vestibulo‐ocular reflex despite recovery of upgaze, 10 had central type nystagmus including rotatory, retractory and rebound nystagmus. Relative preservation of optokinetic nystagmus and preservation of vestibulo‐ocular reflex despite an otherwise complete ophthalmoplegia were reported in 6 and 2 patients repectively. Dissociated pupillary response was reported in 4. We thus feel that central neuro‐ophthalmic signs occur so regularly in cases of Fisher's syndrome that their presence can not be explained by chance alone. We contend that Fisher's syndrome represents one of the brainstem syndromes rather than being a simple unusual variant of the Guillain‐Barr

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1994.tb01654.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

This case‐control study, performed in a mixed rural and urban province, of 74 patients with Parkinson's disease (PD) and 148 unselected age and sex‐matched controls, attempted to look possible risk factors for PD. Rural living, well‐water drinking, positive family history for PD and postural tremor, were associated to an increased risk for PD, with results regarding exposure to pesticides near to statistical significance. Alcohol‐drinking habit in males were associated to a decreased risk for PD, with results regarding cigarette‐smoking habit in males near to statistical significance. We did not find association between the risk for PD and the following variables: 1) exposure to industrial toxins; 2) agricultural work; 3) cranial trauma; 4) previous common illnesses including some infections, arterial hypertension, diabetes mellitus, coronary heart disease and thyroid disease; 5) coffee and tea drinki

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1994.tb01655.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

Arylsulphatase A (ASA) activity was evaluated in 47 patients with a diagnosis of parkinsonism or essential tremor. Mean ASA activity was significantly reduced compared with both a healthy control group of 71 individuals (p<0.01) and with a group of 44 neurological patients without movement disorders (p<0.02). Using definite clinical criteria the patients were classified as typical or atypical with respect to Parkinson's disease (PD) or essential tremor (ET). A normal ASA level was found in all the cases showing typical clinical features (PD and ET), while ASA activity was significantly lowered (p<0.01) in 55.6% of the atypical cases (Parkinsonian syndrome or symptomatic ET). Our data support the hypothesis of a non‐casual association between low ASA level and the clinical features of parkinsonism or symptomatic E

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1994.tb01656.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

Chédiak‐Higashi syndrome (CHS) in children can be a fatal disease. We describe the oldest known CHS patient first seen with a neurologic disorder in early adult life. From the age of 22, this 39‐year‐old woman developed mental deterioration, parkinsonism including resting tremor at the tongue, mandible, and hands, oculogyric crisis, muscular atrophy of limbs, and loss of tendon reflexes. MRI showed marked temporal dominant brain atrophy and diffuse spinal cord atrophy. Partial albinism, panleukocytic giant granules, and profoundly decreased NK‐cell activity were compatible with childhood CHS, but apparently normal neutrophil function prolonged her survival. Stimulated proliferation of lymphocytes was less than 40% that of normal

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1994.tb01657.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

Genetic factors influence the susceptibility to multiple sclerosis (MS). This disease is accompanied by augmented T cell responses to CNS myelin components such as myelin basic protein. To evaluate the familial occurrence of such T cell autoreactivity, we have studied 12 MS families including 37 healthy first‐degree relatives for occurrence of numbers of interferon‐gamma (IFN‐γ) secreting cells among blood mononuclear after culture in presence of myelin basic protein (MBP), eight synthetic MBP peptides and the control antigen acetylcholine receptor (AChR). There were no differences between MS patients and healthy family members regarding frequencies of autoreactive T cells recognizing MBP, the eight different MBP peptides or AChR. None of the MBP peptides predominated as T cell antigen among the MS patients or their unaffected family members. In some families the highest number of MBP peptide reactive T cells were found among unaffected family members. No correlation was observed between numbers of MBP or MBP peptide reactive T cells in various subjects and their HLA‐DR‐DQ phenotypes. In conclusion, this study has revealed the presence of MBP and MBP peptide reactive T cells of similar frequencies in MS patients and their healthy fami

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1994.tb01658.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

The monthly distribution of births of people who were later diagnosed with multiple sclerosis (MS) did not differ significantly from that of the general population in British Columbia, Canada. This is in contrast to a recent report on the Danish population.

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1994.tb01659.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

Alzheimer's disease (AD) and vascular dementia (VD) are the two most common causes of dementia. As yet, no definitive biological antemortem marker has been established for differential diagnosis of AD or VD. In this study, proteins of cerebrospinal fluid (CSF) from AD, VD and control patients were analyzed by two‐dimensional (2‐D) electrophoresis with immobilized pH gradients in the first dimension. No specific changes for AD or VD could be detected in the 2‐D CSF patterns. However, a spot of haptoglobin alpha‐1 chains (13.5 kDa; approximate pI 4.6) was found to be present in the majority of 2‐D CSF maps from the dementia cases, suggesting a high‐molecular‐weight transudate type of alteration in the blood‐brain barrier with considerable

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1994.tb01660.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

Dementia of frontal type (DFT) is a fairly common degenerative disease distinct from Alzheimer's disease (AD), whose reportedly distinctive instrumental feature is frontal lobe hypoperfusion on SPECT. We evaluated the cortical dopaminergic system in 6 AD, 5 DFT, and 6 control subjects with SPECT and both [99Tc]‐HM‐PAO, a perfusion tracer, and [123I]‐IBZM, a D2 postsynaptic ligand. Both in AD and DFT patients, [99Tc]‐HM‐PAO SPECT showed a relative frontal hypoperfusion. On the contrary, [123I]‐IBZM SPECT showed significantly reduced ligand uptake in superior frontal regions of DFT (0.89 ± 0.08 relative to control subjects) as compared to AD patients (0.97 ± 0.02; difference of means: 0.08, 95% Confidence Interval 0.004 to 0.156; p = 0.041). Results suggest more marked involvement of the frontal cortical dopaminergic system in DFT than i

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1994.tb01661.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

The effect of ischemia‐induced hippocampal neuronal damage on acquisition and performance in the Morris water maze task was investigated in male Wistar rats, subjected to 8 min of transient forebrain ischemia, induced by the 4‐vessel occlusion (4‐VO) method. After a morphological scoring of the neuronal damage within the CA1, CA2, and CA3 subfields of the anterior‐dorsal part of hippocampus we found that rats with a total neuronal cell loss of the anterior‐dorsal CA1 region showed memory performance impairments in the acquisition trials, in a probe trial, and in a reversal experiment. However, rats with only partial damage to the CA1 region did not exhibit significant impairments during the acquisition trials of the water maze test or in the probe trial and the reversal experiment. In conclusion, these results suggest that it is possible to relate the histological damage score of CA1 in the anterior‐dorsal hippocampus to impaired memory performance in the present water

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1994.tb01662.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

Atrial septal aneurysms (ASA) are frequent findings on transesophageal echocardiographies. Whether they are more frequent in patients with cerebral ischemia of unknown cause remains unclear. We investigated this question in 154 consecutive patients with an ischemic stroke or transient ischemic attack. The 16 patients with ASA were younger, less likely to have arterial hypertension and more likely to have a patent foramen ovale (PFO) or an unknown cause of stroke, but they did not differ for number and side of infarcts, other demographic data and vascular risk factors. Patients with ASA unassociated to PFO were also more likely to have an unknown cause of stroke. These findings lead to the hypothesis that ASA might be sources of cerebral emboli.

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1994.tb01663.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY