Categories
Neurochecklists updates

30 recently revised and updated practical neurology checklists

Neurochecklists is proud of being comprehensive and practical.

But more than that, we take pride in keeping each checklist updated.

To do this, we keep a keen eye on all relevant developments in the literature.

And below are just a few of the checklists that we have recently revised.

They all reflect findings from the latest neurology publications.

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Anti Ma2 syndrome

Cerebral aneurysms rupture risk factors

Cerebral vein thrombosis

CGRP monoclonal antibodies

CJD

Dural AV fistula management

Giant cell arteritis treatment

Gluten sensitivity neurology

IIH clinical features

King-Denborough syndrome

Levetiracetam

Migraine acute treatment

MND c9orf72

Multiple sclerosis clinical features

Narcolepsy

Normal pressure hydrocephalus

Ocular myasthenia gravis

Oculopharyngeal muscular dystrophy

Ocrelizumab

Parkinson’s disease risk factors

Post stroke recrudescence

PSP variants

Psychogenic seizures

Rapid onset dystonia-parkinsonism

Retinal migraine

Seizure manifestations

Stiff person syndrome

Subacute combined degeneration

Suprascapular neuropathy

Thrombolysis

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Categories
Autoimmune

Is rituximab any good for stiff person syndrome?

A double blind, placebo-controlled study of rituximab in patients with stiff person syndrome.

Dalakas MC, Rakocevic G, Dambrosia JM, Alexopoulos H, McElroy B.

Ann Neurol 2017; 82:271-277.

Abstract

Objective

In Stiff-Person Syndrome (SPS), an antibody-mediated impaired GABAergic neurotransmission is believed to cause muscle stiffness and spasms. Patients improve with GABA-enhancing drugs and IVIg, but several respond poorly and remain disabled. The need for more effective therapy prompted a trial with the anti-CD20 monoclonal antibody rituximab.

Methods

This was a placebo-controlled randomized trial of rituximab (two bi-weekly infusions of 1gr each). The primary outcome was a change in stiffness scores at 6 months. Secondary outcomes were changes in heightened-sensitivity and quality of life scores. Enrolling 24 patients was calculated to detect 50% change in stiffness scores.

Results

Randomization was balanced for age, sex, disease duration and GAD autoantibody titers. No significant changes were noted at 6 months after treatment in all outcomes. Specifically, no differences were noted in the stiffness index, the primary outcome, or sensitivity scores, the secondary outcome, at 3 or 6 months. Quality of life scores improved significantly (p<0.01) at 3 months in both groups, but not at 6 months, denoting an early placebo effect. Blinded self-assessment rating of the overall stiffness for individual patients revealed improvement in four patients in each group. At 6 months, improvement persisted in one patient in the placebo group vs. three out of 4 in the rituximab group, where these meaningful improvements were also captured by video recordings.

Interpretation

This is the largest controlled trial conducted in SPS patients demonstrating no statistically significant difference in the efficacy measures between rituximab and placebo. The lack of rituximab’s efficacy could be due to a considerable placebo effect; insensitivity of scales to quantify stiffness especially in the less severely affected patients; or drug effectiveness only in a small patient subset. This article is protected by copyright.

This article is cited in the neurochecklist:

Stiff person syndrome (SPS): management

Abstract link

By Marta Rizzi et al – PLoS One, CC BY 2.5, Link
Categories
Autoimmune

Why should duloxetine be avoided in people with stiff person syndrome?

Serotonin-norepinephrine reuptake inhibitors may exacerbate stiff person syndrome

Benavides DR, Newsome SD.

Neurol Neuroimmunol Neuroinflamm 2016; 3:e281.

Stiff-person syndrome (SPS) is a neuroimmunologic disorder characterized by painful spasms and muscle rigidity attributed to impaired inhibitory γ-aminobutyric acid (GABA) descending pathways.The immunopathogenesis remains unknown and the pathogenic significance of antibodies against glutamic acid decarboxylase (GAD) is controversial.

Herein, we report 4 cases of anti-GAD65-positive patients with SPS  who had significant worsening of symptoms and disability with the serotonin-norepinephrine reuptake inhibitor (SNRI) duloxetine. While causation cannot be proven, these observations suggest the importance of investigating the relationship between SNRI use and SPS clinical exacerbations.

This reference is now included in the neurochecklist:

Stiff person syndrome (SPS): clinical features

By Fuse809 (talk) - Own work, Public Domain, Link
By Fuse809 (talk) – Own work, Public Domain, Link