THYROID/IMMUNE DYSFUNCTION AND FAMILIAL MOVEMENT DISORDER IN CERVICAL DYSTONIA

Duane, M.D., J.L. Case, Ph.D., L.L. LaPointe, Ph.D.

Arizona Dystonia Institute/Arizona State University

Scottsdale/Tempe, Arizona

Thyroid disorder, immunologic abnormality, familial movement disorder are each prevalent in focal dystonia. Their occurrence and inter-relationship was examined in 154 consecutive patients with cervical dystonia (CD): 113FI41M, mean age CD onset 44 years (range 15-73), mean duration nine years (range 1-36). Findings were contrasted with 100 control non-dystonic patients: 61FI39M, mean age 49 years (range 19-73). Compared were: thyroid dysfunction -- definite history thyroid disorder, abnormal thyroid stimulating hormone (TSH), thyroxine (T4), immune dysfunction -- antinuclear antibody (ANA) > 1:80, positive rheumatoid factor, increased thyroid antibodies; family history -- essential tremor (ET), torticollis (ST), scoliosis (scal), facial dyskinesia (FD), Parkinson disease (PD).

Thyroid dysfunction occurred in 21% of CD (28 of 112F, 4 of 41M) versus 13% of controls (11 of 61F, 2 of 39M) p .2 for females and .3 for males. Immunologic abnormality occurred in 36% of those with CD (46 of 1 1 2F, 1 0 of 41 M) versus 24% f or controls (1 8 of 61 F, 2 of 39M) p . 1 f or f emales, .3 f or males. Of the 32 patients with thyroid dysfunction, 56% (16F/2M) also had serologic immune abnormalities. Both thyroid and immune abnormality occurred in 2F controls. In CD, a family history of ET occurred in 40% (49FI13M), scol 13%, ST 6%, FD 5%, PD 4%. For controls, family history of ET was 4%, scol 4%, PD 6%, and the others 0. Thyroid dysfunction and family history of movement disorder (16 ET and 6 other movement disorder) co-occurred in 32F/5M with CD and in 2F controls. All three (thyroid dysfunction, immune abnormality, family history movement disorder -- 11 ET) co-occurred in llFl2M with CD, but in no controls.

Immune mechanisms may influence thyroid function in CD and, although thyroid dysfunction is more frequent among those with CO, that increase is not highly statistically significant. There may be a link, however, between cervical dystania with a family history of essential tremor, immune antibodies and female sex. A shared gene locus or other genetic mechanism may be a factor in that association. The mechanism of gender effects (sex chromosome, sex steroid or other) remains obscure.

D.D. Duane, J.L. Case, L.L. LaPointe. Thyroid/Immune Dysfunction and Familial Movement Disorder in Cervical Dystonia. Neurology 41, [Supplement 1].292, 1991.

Poster presentation, April 25, 19.91, Boston, MA, 43rd Annual Meeting of the AAN.

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