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What is It? Difficult to Pigeon Hole Tremor: a Clinical–Pathological Study of a Man with Jaw Tremor

Elan D. Louis; Peter G. Bain; Mark Hallett; Joseph Jankovic; Jean Paul Vonsattel

Title:
What is It? Difficult to Pigeon Hole Tremor: a Clinical–Pathological Study of a Man with Jaw Tremor
Author(s):
Louis, Elan D.
Bain, Peter G.
Hallett, Mark
Jankovic, Joseph
Vonsattel, Jean Paul
Date:
Type:
Reports
Department(s):
Center for Parkinson's Disease and Other Movement Disorders
Neurology
Taub Institute
Sergievsky Center
Pathology and Cell Biology
Volume:
2013
Persistent URL:
Book/Journal Title:
Tremor and Other Hyperkinetic Movements
Abstract:
Background: The phenomenology of tremor is broad and its classification is complicated. Furthermore, the full range of tremor phenomenology with respect to specific neurological and neurodegenerative diseases has not been fully elaborated. Case Report: This right‐handed man had a chief complaint of jaw tremor, which began approximately 20 years prior to death at age 101 years. He had been diagnosed with essential tremor (ET) by a local doctor. His examination at age 100 years was notable for marked jaw tremor at rest in the absence of other clear features of parkinsonism, mild kinetic tremor of the hands and, in the last year of life, a score of 22/41 on a cognitive screen. A senior movement disorder neurologist raised doubt about the “ET” diagnosis. The history and videotaped examination were reviewed by three additional senior tremor experts, who raised a number of diagnostic possibilities. A complete postmortem examination was performed by a senior neuropathologist, and was notable for the presence of tufted astrocytes, AT8‐labeled glial cytoplasmic inclusions, and globose neuronal tangles. These changes were widespread and definitive. A neuropathological diagnosis of progressive supranuclear palsy was assigned. Discussion: This case presents with mixed and difficult to clinically classify tremor phenomenology and other neurological findings. The postmortem diagnosis was not predicted based on the clinical features, and it is possible that it does not account for all of the features. The case raises many interesting issues and provides a window into the complexity of the interpretation, nosology, and classification of tremor phenomenology.
Subject(s):
Neurosciences
Kinesiology
Medicine
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Suggested Citation:
Elan D. Louis, Peter G. Bain, Mark Hallett, Joseph Jankovic, Jean Paul Vonsattel, , What is It? Difficult to Pigeon Hole Tremor: a Clinical–Pathological Study of a Man with Jaw Tremor, Columbia University Academic Commons, .

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