Journal of neurology, neurosurgery, and psychiatry
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J. Neurol. Neurosurg. Psychiatr. · Mar 2004
Clinical TrialThe exercise test as a monitor of disease status in hypokalaemic periodic paralysis.
To evaluate exercise test responses in hypokalaemic periodic paralysis (HPP), to determine its value as a diagnostic tool and the factors that could affect the responses. ⋯ The exercise test is a useful diagnostic test for periodic paralysis, but in the absence of recent weakness negative results must be viewed with caution. It has advantages over the DNA test in being a non-invasive functional test that can provide insights into abnormalities of muscle excitability.
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J. Neurol. Neurosurg. Psychiatr. · Mar 2004
Case ReportsSpinocerebellar ataxia type 2 with glial cell cytoplasmic inclusions.
Glial cell cytoplasmic inclusions were identified in a case of spinocerebellar ataxia type 2. These have not been reported before. The inclusions were found in low frequency in the dentate nucleus, cerebellar white matter, pontine transverse fibres, and the inferior olivary nucleus. ⋯ However, their immunohistochemical profile was different as they did not show immunoreactivity for either tau protein or alpha-synuclein. There was no evidence of expanded polyglutamine tracts in these inclusions, which also failed to label with silver stains. As in many other neurodegenerative diseases, in spinocerebellar ataxia type 2 there may be pathogenic contributions of glial cells other than the common astrogliotic response to neuronal damage.
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J. Neurol. Neurosurg. Psychiatr. · Mar 2004
Case ReportsAcute ophthalmoparesis in the anti-GQ1b antibody syndrome: electrophysiological evidence of neuromuscular transmission defect in the orbicularis oculi.
To prospectively study anti-GQ1b antibody positive cases of acute ophthalmoparesis (AO) clinically and electrophysiologically. ⋯ This study demonstrated electrophysiologically the dynamic improvement of neuromuscular transmission of anti-GQ1b antibody positive cases of AO, in tandem with clinical recovery. SFEMG is of value in differentiating weakness due to neuromuscular transmission defect from neuropathy in these clinical situations.
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J. Neurol. Neurosurg. Psychiatr. · Mar 2004
Clinical TrialA short scale for the assessment of motor impairments and disabilities in Parkinson's disease: the SPES/SCOPA.
To evaluate the reliability and validity of the Short Parkinson's Evaluation Scale (SPES)/SCales for Outcomes in Parkinson's disease (SCOPA)-a short scale developed to assess motor function in patients with Parkinson's disease (PD). ⋯ The SPES/SCOPA is a short, reliable, and valid scale that can adequately be used in both research and clinical practice.
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J. Neurol. Neurosurg. Psychiatr. · Mar 2004
Autosomal dominant cerebellar ataxia: SCA2 is the most frequent mutation in eastern India.
Spinocerebellar ataxia type 2 (SCA2) has been reported as the commonest dominant hereditary ataxia in India. However, India is an ethnically and religiously diverse population. Previous studies have not clearly indicated exact ethnic and religious origins, and must therefore be interpreted with caution. The purpose of this study was to determine the prevalence of different SCA mutations in a relatively homogeneous population from eastern India. ⋯ We conclude that (1) although slow ocular saccades are highly suggestive of SCA2, that they are not universal, nor are they exclusive to this disorder and (2) SCA2 is likely to be the commonest dominant ataxia in eastern India, with further evidence for a founder effect.