| PATTERNS
OF DYSTONIA, TREMOR, VOICE AND LATERALIZATION IN CERVICAL DYSTONIA
D.D.
Duane, M.D., J.L. Case, Ph.D., L.L. LaPointe, Ph.D.
Arizona Dystonia
lnstitute/Arizona State University
Scottsdale/Tempe,
Arizona
Prior chart
review series suggest cervical dystonia (CD) not uncommonly is associated
with other dystonic sites. This study reports 154 consecutive CD
patients, Mean (Mn) age onset CD 44y (15-73), Mn duration 9y (1-36).
All were assessed by the same examiner; 121 (78%) were seen two
or more times; Mn evaluation length 5y (range 1-20 years).
101 (66%) had
isolated CD (80F/21M). Of the 53 (33FI20M) with extranuchal dystonia,
10 (8F/2M) had upper facial involvement including 2F with blepharospasm,
one of whom also had spastic dysphonia (SpD). Oromandibular dystonia
(OMD) was observed in 31 (21FI10M), 15 of whom had other dystonic
sites as well. neck movements were phasic in 80% of OMD vs 56% in
the other 123 patients. The 8 lingual dystonia and 5 dysarthria
cases included at least OMD. In 2 of the 6 CD with SpD, the larynx
was the only extranuchal site. All 11 patients with vocal tremor
had accelerometer documented head tremor vs 67% for all patients.
One woman had a multifocal disorder including leg dystonia. 54%
of the 154 patients rotated the chin to the left without gender
bias. 24 (14FI10M) demonstrated hand and/or arm dystonia. 7 had
CD with only an additional writer's cramp (WrC): All 7 were right-handed;
WrC preceded CD in 4; 5 had family hx of essential tremor (ET),
4 of these 5 also had +ANA serology > 1:80. 6 of the 15 CD with
brachial dystonia (BD) also had WrC, Mn age onset CD 27y, 3 before
age 21. Of the 15 BD: 27% were lefthanded vs 4.3% had perinatal
stress; 20% adult trauma; 60% family hx of ET; the 5 +ANA co-occurred
with family hx ET.
Isolated CD
may be particularly prevalent in females. Cervical-brachial dystonia
may result from genetic or acquired factors, but as in CD generally,
ET and immune abnormality may be linked (D. Duane, et al. Neurology,
41, Supplement 1:292, 1991). lpsilateral chin-arm lateralization
may reflect a central neuroanatomic locus.
Poster,presentation,
American, Neurological Association, September 30, l991, Seattle,
WA.
D.D.
Duane, J.L. Case, L.L. LaPointe. Patterns of Dystonia, Tremor, Voice
ond Lateralization in Cervical Dystonia. Annals of Neurology, 39.259,
1991.
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