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2016 ; 6
(ä): 17-24
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Orthostatic tremor: current challenges and future prospects
#MMPMID30050365
Adebayo PB
Degener Neurol Neuromuscul Dis
2016[]; 6
(ä): 17-24
PMID30050365
show ga
This review provides an outlook of orthostatic tremor (OT), a rare adult-onset
tremor characterized by subjective unsteadiness during standing that is relieved
by sitting or walking. Recent case series with a long-time follow-up have shown
that the disease is slowly progressive, spatially spreads to the upper limbs, and
other neurological disorders may develop in about one-third of the patients. The
diagnosis of OT hinges on the typical history of unsteadiness during standing,
which is confirmed by electromyographic findings of a 13-18 Hz tremor that is
typically absent during tonic activation while the patient is sitting and lying.
Although the tremor is generated by a central oscillator, cerebellar and/or basal
ganglia dysfunction are needed for its manifestation (double lesion hypothesis).
However, functional neuroimaging findings have not consistently implicated the
dopaminergic system in its pathogenesis. Drug treatments have been largely
disappointing with no sustained benefits, although thalamic deep brain
stimulation has helped some patients. Large-scale follow-up studies, more drug
trials, and novel therapies are urgently needed.