Clinical spectrum of dystonia in a tertiary care movement disorders clinic in India

Abstract

CONTEXT: Scant data exist regarding the overall distribution of dystonia phenotypes in individuals presenting with abnormal posturing to a movement disorder center in India. AIM: To identify the proportion of various types of dystonia presenting to a tertiary care, academic movement disorder center in India. SETTINGS AND DESIGN: We conducted a retrospective chart review of consecutive patients presenting to the movement disorder clinic of our tertiary care, university hospital. SUBJECTS AND METHODS: We included subjects evaluated at least once by a movement disorder specialist and documented to have dystonia (n = 170). Dystonia was classified according to the consensus update on phenomenology and classification of dystonia (Axes 1 and 2). We calculated the proportion of patients classified into one of the defined dystonia syndromes: early-onset isolated generalized dystonia, focal or segmental isolated dystonia with onset in adulthood, combined dystonia, and dystonia associated with other neurological or systemic manifestations. RESULTS: Focal or segmental isolated dystonia with onset in adulthood was the most common phenotype. Among focal dystonias, majority were brachial (65.8%), followed by cranial (27.1%) and cervical (15.7%). Task specificity was documented in 51.2% of focal dystonias, all brachial dystonias. Tremor was present in 70.3%. Etiologically (Axis 2), evidence of neurodegeneration was present in 10.0% and structural lesion in 5.9%. CONCLUSION: Writer’s cramp was the most common isolated dystonia identified in this hospital-based series.

Keywords

• combined dystonia
• isolated dystonia
• tremor
• writer’s cramp