Neurology Alert
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Neuropathological Variability of NMDAR-Encephalitis
The neuropathological features of N-methyl-D-aspartate receptor (NMDAR)-encephalitis are described in an autopsy cohort of four patients — two diagnosed in life with comorbid brain disorders, and two diagnosed at autopsy and never treated. The two untreated patients had inflammatory infiltrates composed of perivascular and parenchymal T cells and B cells/plasma cells in the basal ganglia, amygdala, and hippocampus. The two treated patients had variable pathologies that reflected their underlying neurological disorders (lymphoproliferative disease and multiple sclerosis). Overall, the topographic distribution of inflammation in patients with NMDAR-encephalitis reflects the clinical symptoms of movement disorders, abnormal behavior, and memory dysfunction with inflammation predominantly observed in the basal ganglia, amygdala, and hippocampus. Loss of NMDAR-immunoreactivity correlated with disease severity.
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Intravenous Immunoglobulin for Stable CIDP: Stop or Taper?
First-line therapy for chronic inflammatory demyelinating polyneuropathy is intravenous immunoglobulin (IVIG), but the timing and method for withdrawal of this treatment are uncertain. In a retrospective review of stable patients on IVIG, investigators at the National Hospital in London observed that there was no significant difference in the likelihood of deterioration or response to retreatment if IVIG was stopped abruptly or tapered slowly.
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Neuropathological Findings in the Brains of Patients Who Died from COVID-19
In an autopsy study of 41 patients who died from COVID-19 in a single medical center in New York City, most of the brain pathology was the result of hypoxic-ischemic injury, infarction, and hemorrhage, with microglial activation and neuronophagia caused by inflammation. Studies for the presence of viral proteins were negative, and very low levels of viral ribonucleic acid were detected.
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Thalamic Stroke and Sleep Impairment: An Experiment of Nature
In a detailed clinical and electrophysiological study of sleep patterns in 12 patients with thalamic stroke, comparing them with 11 patients who had extrathalamic stroke, the investigators identified a marked decrease in slow wave sleep activity in the group with thalamic stroke. The clinical significance of this finding is uncertain but may have an effect on daytime cognitive performance.
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Eculizumab for Refractory Myasthenia Gravis
Eculizumab, a monoclonal antibody that inhibits the C-5 complement terminal complex formation, is a safe and effective treatment for generalized myasthenia gravis with acetylcholine receptor auto-antibodies, even after failed treatment with other immunosuppressive regimens.
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Infections Before Age 20 Years Increase the Risk of Multiple Sclerosis
The relationship between childhood infections and the risk of multiple sclerosis is supported by increasing evidence. Using the Swedish Total Population Register, researchers found that patients diagnosed with infection in adolescence showed an increased risk of multiple sclerosis, even after exclusion of infectious mononucleosis, pneumonia, and central nervous system infection.
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Gene and Substrate Therapy for Neurogenetic Disease: A Combined Approach to Treat Mitochondrial Myopathy
A combined adenoviral-mediated gene therapy plus substrate therapy delivered to a mouse model of thymidine kinase 2 (TK2) deficiency, manifested most often as a fatal mitochondrial myopathy in infants and children, rescued TK2 activity and prolonged animal lifespan, thus indicating a promising therapeutic approach for affected patients.
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TSPO PET Imaging for Monitoring Natalizumab-Associated Progressive Multifocal Leukoencephalopathy
Authors of a recent study evaluated inflammatory activity in multiple sclerosis patients who developed natalizumab-associated progressive multifocal leukoencephalopathy (PML) using translocator protein positron emission tomography (TSPO PET) for imaging of microglia. They demonstrated that TSPO PET can monitor PML longitudinally.
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Incidence, Prevalence, and Long-Term Consequences of Small Fiber Neuropathy
Small fiber neuropathy (SFN), a subtype of peripheral neuropathy characterized by painful distal neuropathic pain and autonomic symptoms, is increasing in incidence. SFN associated with diabetes and other causes is more likely to progress to large-fiber polyneuropathy and have faster deterioration and higher disability compared to slow progression with idiopathic SFN. Glucose impairment, obesity, and elevated triglycerides are modifiable risk factors of idiopathic SFN. Although major disability and neurologic impairment are uncommon in this slowly progressive condition, higher mortality and cardiovascular events are noted in patients with SFN.
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Electrodiagnostic Findings in Thoracic Outlet Syndrome
The diagnosis of thoracic outlet syndrome may be difficult and relies on specific clinical, imaging, vascular, and electrodiagnostic features. None of the currently recommended treatments have been shown to be effective, but there are few randomized clinical treatment trials.