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Updated: Feb 3, 2026

A Simple Composite Phenotype Scoring System for Evaluating Mouse Models of Cerebellar Ataxia
Published on: May 21, 2010
Clinico-Pathological Correlation in Progressive Ataxia and Palatal Tremor: A Novel Tauopathy
Zoltan Mari1,2, Andrew J M Halls2, Alexander Vortmeyer3
1Human Motor Control Section Medical Neurology Branch National Institute of Neurological Disorders and Stroke National Institutes of Health Bethesda Maryland USA.
Abstract:
Palatal tremor (PT) is an uncommon movement disorder that has been subdivided into essential and symptomatic forms. A distinct subgroup of the symptomatic form presents with progressive ataxia and PT. The histopathology of progressive ataxia and PT has not been previously determined. This study consisted of a clinical review, histopathology, and electron microscopy of the brain of a man with progressive ataxia and PT. The inferior olivary hypertrophy was symmetrical and homogenous, and no focal pathologic lesions could be identified in the brainstem. Insoluble tau deposits were found in neurons, exclusively infratentorially. We present the clinical and pathological evaluation of a case of progressive ataxia and PT that provide evidence for a unique form of 4R tauopathy.
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