Demographic, Clinical and Etiological Profile of Young Dystonia Less Than Forty Years of Age – A Hospital Based Study

TLDR: Familiarity showed that focal dystonia were more common even in younger age group with Writers cramp most common among all dySTONia followed by cervical dystonic followed by writer’s cramp.

Abstract: Objective: To study the demographic, clinical and etiological profile of dystonia in patients less than forty years of age. Material and Methods: In this study two hundred and nineteen patients with different dystonia were included. Data on demographic profile, clinical and etiological profile was recorded and statistical analysis was done by using descriptive analysis (frequency distribution). Results: Out of two hundred and nineteen patients, one hundred thirteen (51.7%) were <26 years of age of onset and one hundred six (48.4%) had >26 years of age of onset with mean age of onset 22.3 years, one hundred seventy six were males and forty four females. Mean duration of illness was 4.4 years. Frequency of different dystonia was focal dystonia 57.72%, multifocal 1.36%, segmental 10%, generalized 29.09%, hemidystonia 3.63% and paroxysmal kinesigenic dystonia 2.72%. Among focal dystoniaWriter’s cramp was most common (63%) followed by cervical dystonia (17.32%), and blepharospasm(7%). Among generalized dystonia Wilson’s disease was most common etiological factor in 31.25% cases. Primary generalized dystonia was seen in 17.2% cases. MRI was abnormal in all eight cases of hemidystonia. SPECT brain was abnormal in 1 out of four cases of paroxysmal kinesigenic dystonia. DYT1 was positive in four out of eleven patients of primary generalized dystonia. Conclusion: Present study showed that focal dystonia were more common even in younger age group with Writers cramp most common among all dystonia followed by cervical dystonia. In hemidystonia structural lesion should always be ruled out.