摘要:

IntroductionCerebrospinal fluid (CSF) may provide markers of severity and outcome of optic neuritis.Material and methodsWe examined the CSF from 29 patients with acute monosymptomatic optic neuritis (AMON) for content of myelin basic protein‐like material (MBPLM) and correlated the levels with results of cranial magnetic resonance imaging. The length of the time from onset of AMON to the time of CSF collection did not exceed four weeks.ResultsOnly two patients (7%), one of whom developed an acute myelopathy one month after AMON, showed an elevated value of CSF MBPLM. No MBPLM was detected in 6 patients (21%), and other 21 (72%) had detectable levels of MBPLM but below the upper limit of normal of 0.1 ng/ml. The value of MBPLM was not significantly correlated with the interval to CSF sampling from onset of AMON or with severity of decreased visual acuity. The highest values of CSF MBPLM were observed among patients with severely decreased visual acuity and among patients with an abnormal MRI (13 of 27 i.e. 48%).ConclusionsCSF MBPLM was rarely abnormal in AMON. However, CSF MBPLM may have potential value in reflecting disease activity, as the highest values were obtained among patients with CSF sampled soon after the maximum visual dysfunction was reached, with severe visual impairment, and with an abnormal MR

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1996.tb07070.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

Acute mental disorder in early Multiple Sclerosis (MS) is rare and little is known about the structural and metabolic changes in this relation. We present an MS patient with discrete motor and sensory deficits, who developed severe behavioral changes over a period of nine months during the initial course of the disease. The cerebral metabolic rate of glucose (CMRglc) was measured using positron emission tomography (PET), and the patient underwent MRI as well as a comprehensive battery of neuropsychological tests. Significantly reduced values of CMRglc were found bilaterally in the frontal and temporal cortex, the putamen, the thalamus and the hippocampus. The MRI revealed progression of MS lesions in the frontal lobes during the development of mental symptoms. Neuropsychological examination showed wide spread cognitive dysfunction, and a pronounced frontal lobe syndrome. The study demonstrates the remote metabolic effects of lesions affecting subcortical neural connections in an MS patient with severe cognitive dysfunction.

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1996.tb07071.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

Socio‐demographic data, initial clinical manifestations, outcome and family aggregation of febrile convulsion (FC) were compared between 85 population and 364 multicenter patients. More patients presented with clustering of seizures and had recurrence of FC in multicenter group than in population group. Multicenter patients had more fever episodes per year more day‐care attendance and a higher incidence of FC history in their parents. The odds ratio of FC in the siblings was 6:1 in population group and 12:1 in multicenter group. Lower socioeconomic status influenced the presence of FC in the sibling of population group. Instead, FC histories in the father and paternal cousins of probands had influence on the presence of FC in the siblings of multicenter group. We conclude that the population FC sample rather than the hospital sample is the more representative. And there are differences not only in the clinical manifestations but also in the effect of environmental and genetic influences on the family aggregation in population and hospital patie

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1996.tb07072.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

ObjectivesA retrospective study was conducted to evaluate clinical usefulness of video EEG monitoring in patients with suspected epileptic seizures.Material and methodsA total of 444 patients who had diagnostic video EEG from January 1989 to December 1992 were studied after excluding those with known medically refractory focal epilepsy undergoing presurgical evaluation. Most were outpatients and had video EEG monitoring during normal working hours over 1–5 days.ResultsThe procedure had a success rate of 73%; characteristic events were captured and categorized in 53% of the patients. Patients with an event frequency of at least one per week or those who had events characterized by motor manifestations showed a higher yield. Thirty‐two percent of the patients had psychogenic seizures. Thirty‐four percent of the patients had either epileptic seizures recorded during the study or showed clear‐cut interictal epileptiform abnormalities providing strong evidence of underlying epileptic process.ConclusionIn patients with frequent paroxysmal events, a video EEG study provides diagnostically critical information. It can be performed usually as an outpatient invest

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1996.tb07073.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

We investigated the genetic association between intronic polymorphism in Presenilin‐1 (PS‐1) gene and patients with various types of dementia such as Alzheimer's disease (AD), vascular dementia (VD) and alcohol associated dementia (ALD), in Japanese population. Homozygosity for allele 1 of the PS‐1 polymorphism was significantly increased in late‐onset sporadic AD, but not in early‐onset sporadic AD, familial AD, VD and ALD. When late‐onset sporadic AD patients were divided on the basis of apolipoprotein E (APOE) genotype, homozygosity for the allele 1 of the PS‐1 polymorphism was significantly increased in patients with late‐onset sporadic AD without APOE εe 4 allele, but not in those with APOE εe 4 allele. Intronic mutation in PS‐1 gene may be specific and one of the genetic risk factor for lat

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1996.tb07074.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

The occurrence of psychosis is frequent during the evolution of Parkinson's disease. The reduction of therapeutics or the use of classical neuroleptics may improve the symptoms, but usually worsens parkinsonism. Clozapine is an atypical neuroleptic with only few extrapyramidal effects, which has been proposed at low dose in this indication since 1985. A review of the literature, about more than 200 patients shows good results in approximately 90% without worsening of extrapyramidal symptoms. Some patients even noted an improvement of their motor state while treated by clozapine alone or as dopatherapy was secondarily increased. More controversial results were obtained in demented or depressed patients. Sedation is one of the most frequently encountered side‐effect but rarely necessitates the withdrawal of clozapine. Even if the risk of agranulocytosis is slight, regular blood cell counts must be don

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1996.tb07075.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

ObjectivesBrainstem infarctions frequently cause disturbances of cardiovascular and other autonomic functions, but the pathophysiologic mechanisms of these prognostically unfavourable complications are not well‐known.Material&methodsIn order to evaluate the effects of ischemic brainstem infarction on autonomic cardiac regulation, we analyzed the power spectrum of heart rate variability in 15 consecutive patients with brainstem infarction and in 15 age‐ and sex‐matched healthy control subjects. The components of the power spectrum which reflect quantitatively both sympathetic and parasympathetic cardiovascular regulatory functions were measured from 24‐hour electrocardiogram in the acute phase and at 1 month and 6 months after the infarction.ResultsAll the measured components of heart rate variability, i.e. total power (p<0.01), very‐low‐frequency power (p<0.001), low‐frequency power (p<0.01), and high‐frequency power (p<0.05), were significantly lower in the patients with medullary brainstem infarction than in the control subjects in the acute phase of the infarction. By 6 months, these abnormalities had been reversed. On the contrary, heart rate variability in pontine brainstem infarct patients did not differ significantly from that in the control subjects.ConclusionsThese results suggest that brainstem infarction located in the medulla oblongata causes cardiovascular autonomic dysregulation manifesting as impaired heart rate variability. Medullary brainstem infarction seems to cause both sympathetic and parasympathetic dysfunction, which may contribute to the occurrence of cardiac complica

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1996.tb07076.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

In this study 37 CTS hands underwent pre‐ and post‐operative (15 days, 2 and 6 months) evaluation of median nerve distal motor latency (DML) and sensory nerve conduction velocities (SNCV: I digit and III digit‐wrist). Pre‐operatively, CTS hands were classified as mild (decreased SNCV, normal DML), moderate (decreased SNCV, increased DML) or severe (absent sensory nerve action potentials, increased DML). Post‐operatively, all hands presented clinical and neurophysiological improvement. The three groups of patient showed different clinical and neurophysiological responses to nerve decompression: we observed a rapidrestitutio ad integrumin mild group, an improvement with normalisation in about 50% of the hands in moderate group, and a high percentage of restore of the sensory responses with no normalisation in severe group. A marked improvement of sensory symptoms was observed in all cases, but some degree of motor and/or sensory deficit was still present six months after surgery in more advanced cases. Preoperative electrophysiological assessment of median nerve function in CTS hands have an important role in predicting the outcome of surgical deco

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1996.tb07077.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

Periodic leg movements (PLM) in sleep are supposed to constitute a frequent cause of insomnia. There is some controversy whether PLM are the cause of insomnia by provoking microarousals or whether they are simply an epiphenomenon not causally related to the insomnia. We examined four patients with PLM by overnight polysomnography and concomitant transcranial Doppler sonography (TCD) monitoring, 13 patients with suspected normal pressure hydrocephalus by concomitant overnight polysomnography and intracranial pressure recording, and 10 healthy volunteers by concomitant overnight polysomnography and TCD monitoring. EEG arousals occurred more frequently before than after the PLM. PLM were associated with increase in heart rate, breathing amplitude, and cerebral blood flow velocity assessed by TCD. PLM occurred with a mean wavelength of 40.5 s. This mean wavelength corresponded to similar values calculated for intracranial pressure B‐waves (43.3 s) in 13 patients with suspected normal‐pressure hydrocephalus, TCD B‐wave equivalents (42.2 s) in 10 healthy young adults and the frequency of the cyclic alternating pattern in EEg recordings (40 s). Our data suggest that these cyclic variations with a wavelength of about 40 s are part of a common endogenous rhythm. PLM seem to be an epiphenomenon of this rhythm and not the cause of ins

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1996.tb07078.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

We present an 18‐year‐old patient with Degos' disease who manifested the sudden onset of paraplegia. Neurological examination revealed a rthemianopsia, paraparesis (with a sensory level at Th12), and a neurogenic bladder. Brain MRI showed multiple cerebral infarctions accompanied by small hemorrhagic areas and GdDTPA enhancement of the dura. A cerebral angiogram displayed stenosis, ectasia, and aneurysms involving the peripheral branch of arteries. Stenoses also were observed in the celiac artery and small arteries in the lt‐kidney. Skin biopsy exhibited hyperkeratosis, atrophy of the epidermis, and necrobiosis of the collagen layer. Laboratory examinations disclosed persistent elevations of the thrombin‐antithrombin III complex (TAT), plasmin‐α2 plasmin inhibitor complex (PIC), and cytotoxic T cell subset (CD8 + CD11‐), illustrating the coagulative, fibrinolytic, and immunological implications of

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1996.tb07079.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY