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Head tremor in dentatorubral‐pallidoluysian atrophy
Author(s) -
Ohizumi H.,
Okuma Y.,
Fukae J.,
Fujishima K.,
Goto K.,
Mizuno Y.
Publication year - 2002
Publication title -
acta neurologica scandinavica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.967
H-Index - 95
eISSN - 1600-0404
pISSN - 0001-6314
DOI - 10.1034/j.1600-0404.2002.02061.x
Subject(s) - myoclonus , dystonia , medicine , atrophy , ataxia , dementia , neurological disorder , cerebellar ataxia , psychology , central nervous system disease , neuroscience , pathology , disease
Dentatorubral‐pallidoluysian atrophy (DRPLA) is a rare autosomal‐dominant neurodegenerative disorder characterized by variable combination of clinical manifestations including ataxia, myoclonus, seizures, dementia, and choreic movements. Head tremor has been rarely reported. We report a 66‐year‐old‐woman with genetically determined DRPLA who presented with head tremor. A “no–no” type head tremor was the initial and the most prominent symptom, and mild cerebellar signs and choreic movements were also observed later. Neither hand tremor nor dystonia was noted. The patient did not show dementia, myoclonus, or seizures. Surface electromyogram (EMG) revealed 3.5–4 Hz rhythmic EMG bursts in both sternocleidomastoid muscles. DNA analysis disclosed expanded trinucleotide repeats ( n = 54) in the DRPLA gene. We suggest that isolated head tremor can be a clinical manifestation of DRPLA.