The occurrence of hand tremors in patients with spasmodic torticollis (ST) was investigated in 55 patients by clinical and quantitative measurements. Ten patients had first-order or second-order relatives affected with postural tremor. Although 40% of the patients had a medical history and clinical findings for mild postural and action tremor of the hands, only four had moderate tremor amplitudes. One patient had a severe tremor, including resting tremor, and two had mild voice tremor. A positive correlation was found between hand and head tremor. Patients with hand tremor were younger at the onset of ST than were those without. The mean amplitudes of postural tremor were only slightly higher than for the controls and much smaller than those found in classic essential tremor. The tremor caused only mild disabilities. The tremor frequencies were indistinguishable from physiologic tremor. Further analysis of the tremor records showed evidence for physiologic tremor mechanisms only. We conclude that slightly enhanced postural hand tremors are common in ST but have a low amplitude and are only rarely clinically relevant for ST patients. Although the present data support the notion of an enhanced risk of postural tremor in families of patients affected with ST, none of the criteria allowed the separation of the hand tremor of ST from other postural/action tremors and especially from enhanced physiologic tremor. Thus, the present data do not support the classification of hand tremor in ST as either "dystonic" or essential tremor.
Using high-field MRI, we investigated possible morphologic changes in the basal ganglia of 22 patients with clinically diagnosed idiopathic spasmodic torticollis (iTs) compared with 28 age-matched normal controls. Two patients were found to have distinct basal ganglia lesions and were excluded from further analysis. The frequency of gross morphologic changes (atrophy, enlarged Virchow-Robin spaces) in patients was not significantly higher than in controls. However, T2 values calculated for the putamen and pallidum on both sides were significantly higher in the lentiform nucleus of the patients compared with the controls. In contrast, other well-defined subcortical regions did not exhibit a similar abnormality, nor did the optical or quantified signal analysis of various regions of interest show any differences. This finding suggests a morphologic abnormality in iTs that is not associated with a gross structural lesion. It could reflect focal gliosis and might correspond to earlier sporadic pathoanatomic descriptions of gliosis in idiopathic dystonia.
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