Journal of neurology, neurosurgery, and psychiatry
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J. Neurol. Neurosurg. Psychiatr. · Oct 2013
ReviewTranscranial magnetic stimulation and amyotrophic lateral sclerosis: pathophysiological insights.
Amyotrophic lateral sclerosis (ALS) is a rapidly progressive neurodegenerative disorder of the motor neurons in the motor cortex, brainstem and spinal cord. A combination of upper and lower motor neuron dysfunction comprises the clinical ALS phenotype. Although the ALS phenotype was first observed by Charcot over 100 years ago, the site of ALS onset and the pathophysiological mechanisms underlying the development of motor neuron degeneration remain to be elucidated. ⋯ Taken together, a central origin of ALS is supported by TMS studies, with an anterograde transsynaptic mechanism implicated in ALS pathogenesis. Of further relevance, TMS techniques reliably distinguish ALS from mimic disorders, despite a compatible peripheral disease burden, thereby suggesting a potential diagnostic utility of TMS in ALS. This review will focus on the mechanisms underlying the generation of TMS measures used in assessment of cortical excitability, the contribution of TMS in enhancing the understanding of ALS pathophysiology and the potential diagnostic utility of TMS techniques in ALS.
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J. Neurol. Neurosurg. Psychiatr. · Oct 2013
ReviewPrimary progressive multiple sclerosis: progress and challenges.
Primary progressive multiple sclerosis (MS) has long been recognised as presenting great difficulties to our management of what is increasingly a treatable neurological disease. Here we review some basic and clinical aspects of primary progressive MS, and describe how the disorder in fact offers powerful insights and opportunities for better understanding multiple sclerosis, and from a practical perspective an invaluable clinical substrate for studying and treating progressive disability in MS. Difficult hurdles remain, however, and these too are reviewed.
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J. Neurol. Neurosurg. Psychiatr. · Oct 2013
ReviewHIV, dementia and antiretroviral drugs: 30 years of an epidemic.
Neurological complications due to the HIV itself became apparent early on in the course of the AIDS epidemic. The most feared were the cognitive and motor complications termed AIDS dementia complex or HIV-associated dementia. With the introduction of combination antiretroviral therapy, the incidence of HIV-associated dementia has been dramatically reduced. ⋯ There is controversy about whether some patients may continue with progressive cognitive decline despite adequate suppression of the HIV. The salient issues are those of cerebrospinal fluid (CSF) drug penetration, drug neurotoxicity and persistent immune activation and inflammation. This review will also discuss other newly encountered complications, including the compartmentalisation (or CSF escape) and immune reconstitution inflammatory syndromes.
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J. Neurol. Neurosurg. Psychiatr. · Oct 2013
Comparative StudyInsight into the frequent occurrence of dura mater graft-associated Creutzfeldt-Jakob disease in Japan.
More than 60% of patients worldwide with Creutzfeldt-Jakob disease (CJD) associated with dura mater graft (dCJD) have been diagnosed in Japan. The remarkable frequency of dura mater grafts in Japan may possibly contribute to the elevated incidence of dCJD, but reasons for the disproportionate use of this procedure in Japan remain unclear. We investigated differences between dCJD patients in Japan and those elsewhere to help explain the more frequent use of cadaveric dura mater and the high incidence of dCJD in Japan. ⋯ Differences in the medical conditions precipitating dura mater graft may contribute to the frequent use of cadaveric dura mater and the higher incidence of dCJD in Japan.
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J. Neurol. Neurosurg. Psychiatr. · Oct 2013
Long term (13 years) prognosis after primary intracerebral haemorrhage: a prospective population based study of long term mortality, prognostic factors and causes of death.
Many studies have focused on short term mortality after primary intracerebral haemorrhage (ICH) whereas long term prognosis and causes of death have been less studied. We therefore examined these issues in a population based cohort of 1 year ICH survivors. ⋯ One year survivors after ICH had a substantial and persisting excess mortality compared with the general population. Major causes of death were stroke and ischaemic heart disease.