Consultant Neurosurgeon P D HINDUJA National Hospital, Mumbai. Mumbai, India
Introduction: Surgical intervention for secondary generalized dystonia is less effective than in cases of primary dystonia. We did retrospective analysis over the past 18 years [2004 - 2022] of patients having undergone pallidal surgery for secondary generalized dystonia at our centre.
Methods: The study includes 18 cases of secondary generalized dystonia; 5 cases of Kernicterus, 5 of Pantothenate Kinase Associated Neurodegeneration(PKAN), 3 of birth asphyxia, 2 with unknown etiology, 1 case each of post-traumatic, Lesch-Nyhan Syndrome(LNS) and Wilson’s disease. All the patients were evaluated using Burke Fahn Marsden Dystonia Rating Scale (BFMDRS), before surgery(0 months) and at 6-12-24 months post-surgery. On repeated measures ANOVA analysis, there was significant difference at different time points (0-6-12-24 months) p-value=0.0345.
Results: The results were analysed and percentage changes at 24 months follow-up post-surgery were 101.18%, 52.87%, 51.71%, 43.14%, 8.93%, 8.00%, 4.79% and 4.51% in post-traumatic, unknown etiology, late-onset PKAN, Wilson’s disease, LNS, birth asphyxia, early-onset PKAN and Kernicterus respectively.
Conclusion : We conclude that in cases of secondary generalised dystonia due to certain causes, pallidal surgery may show benefits.
