摘要:

The use of plasmapheresis in Guillain‐Barré syndrome is reviewed. The clinical data, based on open‐label studies, suggest that PP – if instituted early – prevents further deterioration and accelerates the recovery, e.g. the time until the patient becomes ambulatory. The data do not suggest any effect of PP to patients with axonal damage to those with preceding gastroenteritis orCampylobacter jejuniinfections has not been tested. The use of PP should be weighed against the potential complications, especially in units inexperienced with this p

ISSN:1351-5101    

DOI:10.1111/j.1468-1331.1996.tb00242.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

Migraine associated with head trauma i.e. onset of migraine in close temporal relation to a head trauma was analyzed in a large population based survey of 4000 forty‐year‐old males and females. Of the 29 people with migraine associated with head trauma, 20 had migraine without aura associated with head trauma, six had migrainous disorder without aura associated with head trauma and three had migraine with aura associated with head trauma. Eleven had a significant head trauma (loss of consciousness or posttraumatic amnesia lasting more than 10 minutes) and 18 had a minor head trauma. The overall lifetime prevalence of migraine associated with head trauma was 1.4% with a male‐female ratio of 1:2.4. The symptomatology of migraine associated and unassociated with head trauma was not different. The first degree relatives of probands with migraine associated with head trauma were blindly interviewed by a physician. The first degree relatives of probands with migraine without aura associated with head trauma had a significantly lower risk of migraine without aura than first degree relatives of probands with migraine without aura unassociated with head trauma. We conclude that migraine without aura associated and unassociated with head trauma have a different aetiology. Thus, it is very likely that a head trauma may cause migraine without aura. The mechanisms involved in trauma induced migraine without aura are un

ISSN:1351-5101    

DOI:10.1111/j.1468-1331.1996.tb00243.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

Cerebral atrophy was evaluated in 31 patients with Parkinson's disease (PD) and 24 patients with clinically probable multiple system atrophy (MSA). Measurements of ventricular and brainstem areas, obtained from axial and sagittal MRI sections, were performed using a computer digitalizing system. Cortical and cerebellar atrophies were subjectively assessed. All measures were scored by one observer who was blind to the diagnosis (BD). Age, sex, levodopa dosage, baseline motor Parkinsonian disability (i.e. without levodopa treatment) and neuropsychological impairment were not significantly different between the two groups of patients. The total brainstem area (p<0.001), the mesencephalon (p<0.05) and the pons (p= 0.05) areas were significantly smaller in MSA as were the lobar (p<0.05) and the vermian (p<0.01) parts of the cerebellum. In the MSA group, significant correlations were observed between the brainstem area and items from the motor part of the Unified Parkinson's Disease Rating Scale (dysarthria, posture, arising from a chair), and between the cerebellar atrophy and the gait disturbance. A progressive discriminant analysis using radiologic variables correctly classified 79% of patients in their own diagnosis group with a significant difference (p= 0.01). From these results, it appears that MRI could help to differentiate MSA from PD, especially when MRI detects a severe infratentorial atrophy suggestive of MSA. However, brainstem and cerebellum atrophies are not sufficient criteria to differentiate MSA from PD because they lack both specificity and sensitivity.

ISSN:1351-5101    

DOI:10.1111/j.1468-1331.1996.tb00244.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

Three cases with peduncular hallucinosis are described. The occurrence of complex visual hallucinations in each case were abrupt at the onset of the stroke. Two patients were free of hallucinations after a few days, while in the other they persisted. Magnetic resonance imaging (MRI) or computerized tomographic (CT) brain scan findings revealed thalamic involvement in these three patients.

ISSN:1351-5101    

DOI:10.1111/j.1468-1331.1996.tb00245.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

Recent genetic research revealed that hereditary periodic paralysis is an ion‐channel disorder. Genetic linkage analysis has mapped the autosomal dominant hypokalemic periodic paralysis (HypoPP) locus to chromosome 1q31–32, where the dihydropyridine sensitive calcium channel α1subunit (CACNL1A3) is located. Subsequently, two predominant missense point mutations were found in the CACNL1A3 gene. Both mutations substitute arginine to histidine (Arg528Ilis and Arg1239His). The Arg528His mutation is characterized by incomplete penetrance in females, whereas Arg1239His is not. We analyzed Japanese hypokalemic periodic paralysis patients (familial, sporadic and thyrotoxic), and detected the Arg528His mutation in one HypoPP family. This family shows more severe symptoms in successive generations and the severity of the symptoms is higher in males than in females within the same fa

ISSN:1351-5101    

DOI:10.1111/j.1468-1331.1996.tb00246.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

Recently, regional cerebral blood flow (rCBF) was found to increase slightly in the thalamus after a single administration of thyrotropin‐releasing hormone (TRH) to healthy adults. Regarding the effect of prolonged TRH administration on rCBF, some scintigraphic improvements have been observed recently. However, no study has investigated the effect quantitatively, except for a preliminary study by positron emission tomography. Therefore, we examined the effect of repeated administration of TRH on rCBF in humans quantitatively. Eight patients with spinocerebellar degeneration (SCD) were given TRH intravenously at a daily dose of 2 mg for 14 days and rCBF was measured by the133Xe intravenous injection method before and after repeated TRH administration. TRH caused a significant (p<0.01) increase of 12% in the gray matter flow (fast flow, F|), especially at the parietal and occipital lobes, and also caused a significant (p<0.05) increase of 8% in the initial slope index (ISI), especially at the parietal lobe. Among seven patients who improved clinically after TRH administration, F1values were increased in all of them and ISI values were increased in six. We conclude that repeated TRH administration increases rCBF in humans. These results might warrant clinical investigation of a possible therapeutic role of TRH in patients with cerebral ischemi

ISSN:1351-5101    

DOI:10.1111/j.1468-1331.1996.tb00247.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

The presence of argyrophilic oligodendroglial cytoplasmic inclusions (GCIs) is a pathognomonic feature of multiple system atrophy (MSA), which has established MSA as a nosological entity, and serves as a diagnostic criterion. As a neurodegenerative disease, MSA exhibits neuronal degeneration and loss from several regions of the central nervous system. Recent mapping studies of the distribution of GCIs have shown their presence in regions of the brain previously thought not to be affected in MSA, for example the cerebral cortex. This study has used stereological techniques to establish whether neuronal loss is related to the presence of GCIs in three regions of the cerebral cortex. In the prefrontal cortex, in which GCIs are sparse, there is no significant difference in either neuronal or glial cell numerical density in MSA cases compared to controls. However in two cortical regions which are both GCI rich, the anterior central gyrus and the supplementary motor cortex, there is a marked reduction in neuronal density of 18.7% and 21.4% respectively, which is statistically significant only in the supplementary motor cortex. In both of these regions there is a concomitant increase in glial cell numerical density which results in a significant change in the ratio of neurons to glial cells. These results indicate that there is a regional reduction in the neuronal density in the cortex in multiple system atrophy which is associated with the presence of GCIs.

ISSN:1351-5101    

DOI:10.1111/j.1468-1331.1996.tb00248.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

We describe a patient with high titers of anti‐GM1 antibody but not anti‐GQ1b antibody who had recurrent asymmetric limb weakness with little sensory deficit and developed left abducens palsy. Nerve conduction study showed multifocal conduction block or dispersion of motor and sensory axons in the same mixed nerve. Pulse steroid followed by oral predonisolone treatment rapidly resolved his sympt

ISSN:1351-5101    

DOI:10.1111/j.1468-1331.1996.tb00249.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

Seventy‐seven consecutive male patients with clinically definite or laboratory supported definite multiple sclerosis were submitted to a questionnaire and a clinical interview in order to assess the frequency, the nature of sexual dysfunction and its relationship with clinical findings. Twenty‐six patients (35%) complained of erectile dysfunction: the defect was partial and inconstant in 11 patients (14%), complete and fixed in 15 (21%). Six patients complained of premature ejaculation, in three of whom the symptom was already present before the onset of the disease. Taking the series as a whole, sexual life was poor or not satisfying at all in 34 patients. Erectile dysfunction was associated with the presence and severity of bladder dysfunction and with disability. The relationship with sensory impairment of legs was slight, no relationship was found with motor involvement of legs, type of course, age and disease durat

ISSN:1351-5101    

DOI:10.1111/j.1468-1331.1996.tb00250.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

Activity of glutamic acid decarboxylase (GAD) was determined in extracts from cultured human skin fibroblasts of patients with Huntington's disease (HD) and compared to an age‐ and sex‐matched control group. The fibroblasts were cultured up to eight passages. In patients suffering from HD a significant increased activity of GAD (∼ 3–5 fold compared to age‐ and sex‐matched controls) was found in passages 4–8. This may indicate a defect in the genetic expression of GAD concentration or regulation and/or may indicate that increased levels of glutamate may also be responsible for neuronal degen

ISSN:1351-5101    

DOI:10.1111/j.1468-1331.1996.tb00251.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY