Journal of the neurological sciences
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Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired neurological disorder characterized clinically by weakness and impaired sensory function evolving over 2 months or more, loss or significant decrease in deep tendon reflexes, and by electrophysiological evidence of peripheral nerve demyelination. Expeditious diagnosis and treatment of CIDP early in the disease course is critical such that irreversible disability can be avoided. ⋯ The different options for administration of IG treatment create the need for information to assist clinicians and patients in choosing the optimal therapeutic approach. Considerations for pharmacokinetics, administration procedures, adverse events, patient variables, and cost will all be discussed in this article.
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Stiff-person syndrome (SPS), first described in 1956 by Moersch and Woltman, is a progressive autoimmune disorder with core features of chronic fluctuating progressive truncal and limb rigidity and painful muscle spasms leading to gait difficulties, falls and an appearance that resembles tin soldiers. The syndrome is a rare, highly disabling disorder of the central nervous and frequently results in significant disability. Understanding of the etiology, clinical spectrum, diagnostic workup and therapeutic modalities for this painful and disabling disorder has vastly evolved over the past few years with more confidence in classifying and treating the patients. The purpose of this review is to increase the awareness, early detection, and treatment of this disabling disease. ⋯ In this article we will discuss the epidemiology, presentation and classification. Explain the pathophysiology of SPS and the autoimmune mechanisms involved. Discuss the diagnostic approach and treatments available, as well as, the prognosis and outcome.
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Meta Analysis
Nocebo in cerebellar ataxia: A systematic review and meta-analysis of placebo-controlled clinical trials.
Nocebo, the negative counterpart of the placebo phenomenon results in the induction of adverse events (AEs) following the administration of an inert substance. Nocebo has been demonstrated to be associated with low treatment compliance in clinical trials, thus affecting treatment outcomes. This study sought to determine the prevalence of nocebo in cerebellar ataxia. ⋯ Our results demonstrate that the nocebo effect in cerebellar ataxia is amongst the lowest in neurological diseases.
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Letter Review Case Reports
Acute truncal ataxia in a healthy adult with varicella zoster virus cerebellitis: A case report and literature review.
Acute cerebellitis is a well recognized complication of varicella zoster virus (VZV) infection in children. It has been described in adults in the setting of virus reactivation with a preceding herpes zoster rash, but it is exceedingly rare in adults who are not elderly or immunocompromised, particularly in the absence of a rash. ⋯ He had robust improvement with intravenous acyclovir treatment and was free of neurologic disability at two month follow-up. This case highlights the importance of virological evaluation in patients with acute ataxia, even in the absence of typical features of infection.
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Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder of the motor pathways, invariably leading to death within a few years of onset. Most cases of ALS are sporadic, but familial forms of the disease (FALS) constitute 10% of the cases. Since the first identification of a causative gene in the 1990s and with recent advances in genetics, more than twenty genes have now been linked to FALS. This increased number of genes led to a tremendous amount of research, clearly contributed to a better understanding of the pathophysiology of this disorder, and paved the way for the development of new therapeutics and new hope for this fatal disease.