Rest tremor in advanced essential tremor: a post-mortem study of nine cases
- Elan D Louis1,2,3,4,
- Nana Asabere1,
- Angus Agnew1,
- Carol B Moskowitz2,
- Arlene Lawton1,3,
- Etty Cortes3,
- Phyllis L Faust5,
- Jean-Paul G Vonsattel3,5
- 1GH Sergievsky Center, College of Physicians and Surgeons, Columbia University, New York, USA
- 2Department of Neurology, College of Physicians and Surgeons, Columbia University, New York, USA
- 3Taub Institute for Research on Alzheimer's Disease and the Ageing Brain, College of Physicians and Surgeons, Columbia University, New York, USA
- 4Department of Epidemiology, Mailman School of Public Health, Columbia University, New York, USA
- 5Department of Pathology and Cell Biology, College of Physicians and Surgeons, Columbia University, New York, USA
- Correspondence to Dr Elan Louis, Unit 198, Neurological Institute, 710 West 168th Street, New York, NY 10032, USA; EDL2{at}columbia.edu
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Contributors The statistical analyses were conducted by EDL.
- Received 20 April 2010
- Revised 3 June 2010
- Accepted 10 June 2010
- Published Online First 27 August 2010
Abstract
Background Rest tremor may occur in as many as 30% of essential tremor (ET) patients. It is not clear whether this tremor is a sentinel marker for brainstem Lewy body pathology. Here we report the clinical and post-mortem findings of nine ET cases with upper-extremity rest tremor in the absence of other parkinsonian features.
Methods All brains had a complete neuropathological assessment. Tissue sections from the brainstem and basal ganglia were immunostained with α-synuclein antibody.
Results All cases had longstanding ET (median duration=42 years) with moderate to severe arm tremor. Rest tremor involved both arms in seven (77.8%) cases and one arm in two cases. The rest tremor score was correlated with the total action tremor score (r=0.69, p=0.04). The number of torpedoes was elevated, and Purkinje cells, reduced. Post-mortem changes in the substantia nigra pars compacta (SNc), caudate, putamen and globus pallidum were minimal, and neither Lewy bodies nor Lewy neurites were evident.
Conclusions In nine ET brains with upper-extremity rest tremor, neither Lewy body-containing neurons nor Lewy neurites were found on α-synuclein immunostained sections, and other pathological changes in the basal ganglia were minimal. These data support the notion that isolated rest tremor in longstanding ET is not the expression of underlying Lewy body pathology in the SNc.
- Essential tremor
- post-mortem
- pathology
- pathophysiology
- rest tremor
- clinical
- movement disorders
- neuropathology
- post-mortem
- tremor
Footnotes
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Funding R01 NS042859 from the National Institutes of Health (Bethesda, MD); the Parkinson's Disease Foundation; the Arlene Bronstein Essential Tremor Research Fund (Columbia University).
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Competing interests None.
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Patient consent Obtained.
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Ethics approval Ethics approval was provided by the Columbia University.
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Provenance and peer review Not commissioned; externally peer reviewed.
