Journal of neurology, neurosurgery, and psychiatry
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J. Neurol. Neurosurg. Psychiatr. · Jun 2019
Electrodiagnostic data-driven clustering identifies a prognostically different subgroup of patients with chronic inflammatory demyelinating polyneuropathy.
This study aimed to explore the correlations between electrodiagnostic (EDX) features in patients with chronic inflammatory demyelinating polyneuropathy (CIDP) and to investigate whether EDX data-driven clustering can identify a distinct subgroup regarding clinical phenotype and treatment response. ⋯ Our results show that EDX data-driven clustering could differentiate a pattern of EDX features with prognostic implication in patients with CIDP. Reduced distally evoked CMAPs may not necessarily predict poor responses to treatment, and active treatment is warranted when prominent slowing of conduction is accompanied in the distal segments.
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J. Neurol. Neurosurg. Psychiatr. · Jun 2019
Early weight loss in amyotrophic lateral sclerosis: outcome relevance and clinical correlates in a population-based cohort.
To assess the role of body mass index (BMI) and of the rate of weight loss as prognostic factors in amyotrophic lateral sclerosis (ALS) and to explore the clinical correlates of weight loss in the early phases of the disease. ⋯ The rate of weight loss from onset to diagnosis was found to be a strong and independent prognostic factor in ALS. Weight loss was mainly due to the reduction of nutritional intake related to dysphagia, but a subgroup of spinal onset patients without dysphagia at diagnosis had a severe weight loss and an outcome similar to bulbar patients. According to our findings, we recommend that in clinical trials patients should be stratified according to the presence of dysphagia at the time of enrolment and not by site of onset of symptoms.
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J. Neurol. Neurosurg. Psychiatr. · Jun 2019
HLA class II allele DRB1*16:02 is associated with anti-NMDAR encephalitis.
Aetiology and pathogenesis of anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis, the most common autoimmune encephalitis, is largely unknown. Since an association of the disease with the human leucocyte antigen (HLA) has not been shown so far, we here investigated whether anti-NMDAR encephalitis is associated with the HLA locus. ⋯ This study for the first time demonstrates an association between specific HLA class II alleles and anti-NMDAR encephalitis, providing novel insights into the pathomechanism of the disease.
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J. Neurol. Neurosurg. Psychiatr. · Jun 2019
Clinical presentation, natural history and outcomes of intramedullary spinal cord cavernous malformations.
There is a paucity of literature investigating the clinical course of patients with spinal intramedullary cavernous malformations (ISCMs). We present a large case series of ISCMs to describe clinical presentation, natural history and outcomes of both surgical and conservative management. ⋯ Large, symptomatic ISCMs appear to be at increased risk for subsequent haemorrhage. Surgery may be considered in such lesions to prevent rebleeding and subsequent neurological worsening.