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Tremor Other Hyperkinet Mov (N Y). 2017 Nov 17;7:513. doi: 10.7916/D8W66ZBH. eCollection 2017.

Pathogenesis of Primary Orthostatic Tremor: Current Concepts and Controversies.

Author information

1
Department of Clinical Neurosciences, National Institute of Mental Health and Neurosciences, Bangalore, India.
2
Department of Neurology, National Institute of Mental Health and Neurosciences, Bangalore, India.
3
Department of Neurological Sciences, University of Nebraska Medical Center, Omaha, NE, USA.
4
Division of Movement Disorders, Department of Neurology, Yale School of Medicine, Yale University, New Haven, CT, USA.
5
Department of Chronic Disease Epidemiology, Yale School of Public Health, Yale University, New Haven, CT, USA.
6
Center for Neuroepidemiology and Clinical Neurological Research, Yale School of Medicine, Yale University, New Haven, CT, USA.

Abstract

Background:

Orthostatic tremor (OT), a rare and complex movement disorder, is characterized by rapid tremor of both legs and the trunk while standing. These disappear while the patient is either lying down or walking. OT may be idiopathic/primary or it may coexist with several neurological conditions (secondary OT/OT plus). Primary OT remains an enigmatic movement disorder and its pathogenesis and neural correlates are not fully understood.

Methods:

A PubMed search was conducted in July 2017 to identify articles for this review.

Results:

Structural and functional neuroimaging studies of OT suggest possible alterations in the cerebello-thalamo-cortical network. As with essential tremor, the presence of a central oscillator has been postulated for OT; however, the location of the oscillator within the tremor network remains elusive. Studies have speculated a possible dopaminergic deficit in the pathogenesis of primary OT; however, the evidence in favor of this concept is not particularly robust. There is also limited evidence favoring the concept that primary OT is a neurodegenerative disorder, as a magnetic resonance spectroscopic imaging study revealed significant reduction in cerebral and cerebellar N-acetyl aspartate (NAA) levels, a marker of neuronal compromise or loss.

Discussion:

Based on the above, it is clear that the pathogenesis of primary OT still remains unclear. However, the available evidence most strongly favors the existence of a central oscillatory network, and involvement of the cerebellum and its connections.

KEYWORDS:

Orthostatic tremor; cerebellum; neurodegeneration; pathogenesis

PMID:
29204315
PMCID:
PMC5712672
DOI:
10.7916/D8W66ZBH
[Indexed for MEDLINE]
Free PMC Article

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