Neurology Alert
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Auditory Startle Response as a Predictor of Recovery from Coma
Preserved habituation of the auditory startle response, performed at the bedside, indicates intact cortical structures and cortico-cortical white matter tract connections. Preservation of this reaction in patients with unresponsive wakefulness can help distinguish the vegetative state from minimally conscious state and may even predict eventual awakening.
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Oral Calcitonin Gene-Related Peptide Antagonist for Prevention of Migraine
Atogepant, an oral calcitonin gene-related peptide (CGRP) antagonist, was shown to be effective and safe for migraine prevention. This adds an additional CGRP antagonist to the growing arsenal of such medications, both oral and parenteral, for both prevention and treatment of migraine.
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A Novel Therapy for Amyotrophic Lateral Sclerosis Shows Promise
A novel drug combination was taken from an animal model and tested in a Phase II trial of patients with amyotrophic lateral sclerosis. Although there was a trend toward benefit, there were no statistically significant endpoints at the end of the trial. Future trials need to enroll a larger number of patients.
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Transthyretin Amyloidosis and Neuropathy
Wild-type transthyretin amyloidosis, referred to as “senile” amyloidosis previously, usually occurs in the aging population and affects the peripheral nerves and the heart. Usually, neurological symptoms precede cardiac symptoms.
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Nerve Disorders from Mutations in the BSCL2 Gene
The neuromuscular syndromes caused by BSCL2 gene mutations may mimic several other disorders, including Charcot-Marie-Tooth disease and amyotrophic lateral sclerosis.
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Molecular and Cellular Correlates of Human Nerve Regeneration
Specific and objective measures of improvement after carpal tunnel decompression (i.e., electrodiagnostic testing, somatosensory function, and intraepidermal nerve fiber density) also correlated with upregulation of ADCYAP1/PACAP genes. These genes may be potential therapeutic targets in the future.
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Dermatomyositis Sine Dermatitis Is an Important Clinical Entity
Dermatomyositis is an autoimmune disorder of skeletal muscle associated with a variety of auto-antibodies and specific muscle pathology but it may not have a skin rash. Muscle pathology and antibody determinations are important for accurate diagnosis and treatment.
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Randomized Diagnostic Trial of First-Line Genome Sequencing in Pediatric White Matter Disorders
The results of this study indicate that first-line genome sequencing in pediatric patients with suspected genetic white matter disease is more diagnostically efficient, defined as higher diagnostic efficacy and shorter time to diagnosis, than current standard of care approaches.
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Plasma Phospho-Tau217 Is a Promising Alzheimer’s Disease Biomarker
Validation and replication of findings in the use of the blood biomarker phospho-tau 217 for the diagnosis of Alzheimer’s disease holds out the possibility for a new era in the early diagnosis and treatment of this debilitating illness.
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