Neurology
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American Academy of Neurology (AAN) practice parameters for amyotrophic lateral sclerosis (ALS) include noninvasive positive pressure ventilation (NIV) for a forced vital capacity (FVC) ≤50%predicted. Despite the limited ability of NIV systems to deliver adequate ventilation synchronous with patient demand, nocturnal patient-ventilator asynchrony has not been systematically studied in patients with ALS prescribed such NIV. ⋯ These findings document frequent nocturnal patient-ventilator asynchrony in patients with ALS consistently using nNIV prescribed as per current AAN practice parameters, and suggest that use of nNIV per these parameters is unlikely to provide patients with ALS optimal nocturnal ventilatory support.
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Stimulation-induced hypokinetic gait disorders with freezing of gait (FOG) have been reported only recently as adverse effects of deep brain stimulation (DBS) of the globus pallidus internus (GPi) in patients with dystonia. The aim of this work was to determine the frequency and the nature of this GPi-DBS-induced phenomenon. ⋯ A hypokinetic gait disorder with FOG can be a complication of GPi-DBS.
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Case Reports
Progressive encephalomyelitis with rigidity and myoclonus: glycine and NMDA receptor antibodies.
The syndrome of progressive encephalopathy with limb rigidity has been historically termed progressive encephalomyelitis with rigidity and myoclonus (PERM) or stiff-person syndrome plus. ⋯ This case displayed not only the clinical features of PERM, previously associated with GlyR antibodies, but also some of the features associated with NMDAR antibodies. This unusual combination of antibodies may be responsible for the particularly progressive course and sudden death.