Journal of neurology, neurosurgery, and psychiatry
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J. Neurol. Neurosurg. Psychiatr. · Jun 2015
Serum high mobility group box 1 is upregulated in myasthenia gravis.
High mobility group box 1 (HMGB1) functions as an inflammatory mediator and is implicated in the pathogenesis of various autoimmune diseases. Our primary aim is to determine whether HMGB1 is involved in the pathogenesis of myasthenia gravis (MG). ⋯ Serum HMGB1 is elevated in patients with MG and may play a key role in the inflammation of the neuromuscular junction.
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J. Neurol. Neurosurg. Psychiatr. · Jun 2015
Defining disability: development and validation of a mobility-Disability Severity Index (mDSI) in Charcot-Marie-tooth disease.
To develop and validate a reliable patient-reported scale that grades the severity of disability in Charcot-Marie-tooth disease (CMT), from an in-depth analysis of patient and healthcare provider perspectives on what mobility changes constitutes mild, moderate and severe disability. ⋯ The mobility-Disability Severity Index is a unique instrument, categorising disability from the patient's perspective, and will undergo further cross-validation studies in CMT.
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J. Neurol. Neurosurg. Psychiatr. · Jun 2015
Neuronal antibodies in patients with suspected or confirmed sporadic Creutzfeldt-Jakob disease.
There have been reports of patients with antibodies to neuronal antigens misdiagnosed as sporadic Creutzfeldt-Jakob disease (sCJD). Conversely, low levels of antibodies to neuronal proteins have been reported in patients with sCJD. However, the frequency of misdiagnoses, or of antibodies in patients with subsequently confirmed sCJD, is not clear. ⋯ This study indicates that <5% patients with sCJD develop serum antibodies to these neuronal antigens and, when positive, only at low titres. By contrast, three patients referred with possible prion disease had a clinical picture in keeping with autoimmune encephalitis and very high VGKC-complex/LGI1 antibodies. Low titres of neuronal antibodies occur only rarely in suspected patients with sCJD and when present should be interpreted with caution.
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J. Neurol. Neurosurg. Psychiatr. · Jun 2015
Comparative StudyThe neural correlates of road sign knowledge and route learning in semantic dementia and Alzheimer's disease.
Although there is a growing body of research on driving and Alzheimer's disease (AD), focal dementias have been understudied. Moreover, driving has never been explored in semantic dementia (SD). ⋯ For the first time, driving skills were explored in SD, and it is showed a differential profile from the one detected in AD. We demonstrate that the left anterior temporal cortex is implicated in road sign knowledge, while a distributed cortical network, including the frontal cortex, is likely to process route learning.