Abstract
Back ground and aims: To find out the spectrum of neurodegenerative disorders of childhood, its diagnosis and role of paediatician in the management It isa descriptive study conducted at Department of Neurology Children's Hospital, Lahore from June 1, 2005 to May 31, 2009. Methods: A total of 2273 patients were admitted in the Neurology department with the diagnosis of DBD. Results: Male to female ratio was 14:1 Age range was one to fifteen years Metachromatic leukodystrophy was the predominant type (21%) followed by adrenoleukodystrophy (16%), SSPE (12%), Wilson Disease 10%, Alexander disease 3%, Hellervordenspatz disease2% and canavans leukodystrophy in 15%. Conclusion: Degenerative brain diseases are not uncommon entity in paediatric population. Commonest presentation is regression of mile stones though it may be variable Physicians must look into it when dealing with children having regression of milestones to diagnose them earlier. As in majority of patients there is yet no curative therapy available, so role of palliative care is very important Collaboration should be established between the regional institutes to promote further research in future.
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