Journal of neurology, neurosurgery, and psychiatry
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J. Neurol. Neurosurg. Psychiatr. · Oct 2015
Natural history and clinical features of sporadic amyotrophic lateral sclerosis in China.
To describe the natural history and clinical features of sporadic amyotrophic lateral sclerosis (ALS) in Chinese patients, and to report data on the prognostic factors for survival. ⋯ The clinical characteristics and outcomes of Chinese patients with sporadic ALS were different compared with patients from other countries. Compared with other studies, the age at onset of Chinese patients was earlier, the percentage of bulbar-onset ALS was lower and the prognosis was better. This study substantially advances the understanding of the clinical features and epidemiology of this rare disease.
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J. Neurol. Neurosurg. Psychiatr. · Oct 2015
Modelling the natural history of Huntington's disease progression.
The lack of reliable biomarkers to track disease progression is a major problem in clinical research of chronic neurological disorders. Using Huntington's disease (HD) as an example, we describe a novel approach to model HD and show that the progression of a neurological disorder can be predicted for individual patients. ⋯ This new model therefore has important implications for HD research, most obviously in the development of potential disease-modifying therapies. We believe that a similar approach can also be adapted to model disease progression in other chronic neurological disorders.
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J. Neurol. Neurosurg. Psychiatr. · Oct 2015
Different electrophysiological profiles and treatment response in 'typical' and 'atypical' chronic inflammatory demyelinating polyneuropathy.
Chronic inflammatory demyelinating polyneuropathy (CIDP) is currently classified into 'typical' CIDP and 'atypical' subtypes such as multifocal acquired demyelinating sensory and motor neuropathy (MADSAM). ⋯ Among the CIDP spectrum, typical CIDP and MADSAM are the major subtypes, and their pathophysiology appears to be distinct. In typical CIDP, the distal nerve terminals and possibly the nerve roots, where the blood-nerve barrier is anatomically deficient, are preferentially affected, raising the possibility of antibody-mediated demyelination, whereas cellular immunity with breakdown of the barrier may be important in MADSAM neuropathy.
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J. Neurol. Neurosurg. Psychiatr. · Oct 2015
Physiotherapy for functional motor disorders: a consensus recommendation.
Patients with functional motor disorder (FMD) including weakness and paralysis are commonly referred to physiotherapists. There is growing evidence that physiotherapy is an effective treatment, but the existing literature has limited explanations of what physiotherapy should consist of and there are insufficient data to produce evidence-based guidelines. We aim to address this issue by presenting recommendations for physiotherapy treatment. ⋯ Physiotherapy has a key role in the multidisciplinary management of patients with FMD. There appear to be specific physiotherapy techniques which are useful in FMD and which are amenable to and require prospective evaluation. The processes involved in referral, treatment and discharge from physiotherapy should be considered carefully as a part of a treatment package.
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J. Neurol. Neurosurg. Psychiatr. · Oct 2015
Resting cortical PET metabolic changes in psychogenic non-epileptic seizures (PNES).
The pathophysiology of psychogenic non-epileptic seizures (PNES) is poorly understood. Functional neuroimaging data in various functional neurological disorders increasingly support specific neurobiological dysfunction. However, to date, no studies have been reported of positron emission tomography (PET) in patients presenting with PNES. ⋯ To the best of our knowledge, this is the first study describing FDG-PET alterations in patients with PNES. Although we cannot exclude that our data reflect changes due to comorbidities, they may indicate a dysfunction of neural systems in patients with PNES. Hypometabolism regions might relate to two of the pathophysiological mechanisms that may be involved in PNES, that is, emotional dysregulation (anterior cingulate hypometabolism) and dysfunctional processes underlying the consciousness of the self and the environment (right parietal hypometabolism).