Outcome of Hospitalized Parkinson's Disease Patients with and without COVID-19.

Background: The Parkinson's disease (PD) patient population, with an already reduced life expectancy, is rendered particularly vulnerable by the Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2). Objectives: We determined the risk factors that increase the risk of death in patients with Parkinson's disease who are infected by SARS-CoV-2. Methods: Patients with a diagnosis of PD admitted to Montefiore Hospital (Bronx, New York) and tested for SARS-CoV-2 were identified. Retrospective review of electronic medical records confirmed the diagnosis; patients were classified by severity of PD. PD severity, demographic, socioeconomic factors, and co-morbidities were correlated with mortality rates in patients with SARS-CoV-2. Results: We identified 162 patients meeting criteria; chart review confirmed a diagnosis of PD in 70 patients. Of the 70 patients, 53 were positive for SARS-CoV-2 and 17 were negative. PD patients with SARS-CoV-2 infection had a higher mortality rate (35.8%) compared to PD patients without the infection (5.9%, P = 0.028). PD patients older than 70 years of age, those with advanced Parkinson's disease, those with reductions in their medications, and non-Hispanics (largely comprised of Black/African- Americans) had a statistically significant higher mortality rate, if infected. Conclusions: PD did not increase mortality rates from SARS-CoV-2 infection when age was controlled. However, certain unalterable factors (advanced disease and age greater than 70 years) and alterable ones (reductions in PD medications) placed PD patients at increased risk for mortality. Also several socioeconomic factors contributed to mortality, for example, non-Hispanic patients with SARS-CoV-2 infection fared worse, likely driven by poorer outcomes in the Black/African-American cohort.

Association of Sjögren's syndrome with myotonic dystrophy type 1.

A 47-year-old woman presented with sicca symptoms, polyarthralgias, polymyalgias and dysphagia. She was found to have positive antinuclear, anti-SSA-Ro and anti-SSB-La antibodies. Slit lamp exam confirmed the presence of keratoconjunctivitis sicca, and the patient was diagnosed with Sjögren's syndrome. Three years later, she was referred for evaluation of gait instability associated with recent falls. On physical examination, the patient was found to have bilateral ptosis, percussion myotonia, distal upper and lower extremity weakness, and a steppage gait. Electromyography demonstrated electrical myotonia. Genetic testing revealed expanded CTG repeats (733 and 533) in the myotonic dystrophy type 1 (DM1) protein kinase gene, confirming the diagnosis of DM1. Dysphagia, pain and eye discomfort may occur in both Sjögren's syndrome and DM1, and in this case, may have delayed the diagnosis of muscular dystrophy.

Optochiasmatic and peripheral neuropathy due to ethambutol overtreatment.

Ethambutol is known to cause optic neuropathy and, more rarely, axonal polyneuropathy. We characterize the clinical, neurophysiological, and neuroimaging findings in a 72-year-old man who developed visual loss and paresthesias after 11 weeks of exposure to a supratherapeutic dose of ethambutol. This case demonstrates the selective vulnerability of the anterior visual pathways and peripheral nerves to ethambutol toxicity.

Teaching video neuroimages: orthostatic tremor: the helicopter sign.

A 63-year-old man presented with 4 years of orthostatic tremor while standing, resolving after sitting or leaning against a wall. There was marked subjective unsteadiness, but no falls. Surface EMG in arms and legs demonstrated a 14- to 16-Hz synchronous tremor in antagonist muscles (video on the Neurology Web site at www.neurology.org.).

Rapid-onset dystonia-parkinsonism.

Rapid-onset dystonia-parkinsonism (RDP) is a rare condition with autosomal-dominant inheritance causing dystonia and parkinsonism which develop over a short period of time. It results from abnormalities in the Na(+)/K(+)-ATPase pump due to mutations in the ATP1A3 gene. This chapter reviews the clinical features, genetics, and diagnosis of this disorder.

The diagnosis of dystonia.

Dystonia is a movement disorder with many presentations and diverse causes. A systematic approach to dystonia helps to ensure that patients with this disorder receive optimum care. This Review begins with a summary of the clinical features of dystonia, followed by a discussion of other disorders to be considered and excluded before assigning the diagnosis of dystonia. Next, we emphasise the importance of classifying dystonia along several dimensions, and we explain how doing so aids in narrowing the differential diagnosis. The more common forms of dystonia are discussed in detail. Finally, we describe how to apply the clinical information for selection of appropriate laboratory investigations.

Methyl bromide intoxication causes reversible symmetric brainstem and cerebellar MRI lesions.

Methyl bromide is toxic to the central and peripheral nervous systems. A patient with occupational exposure to this agent is described. MRI showed strikingly symmetric brainstem and cerebellar lesions. The patient's clinical course and the topography and resolution of his MRI abnormalities suggest that this condition is an energy deprivation syndrome.