Bilateral pallidal stimulation in parkinsonism predominant XDP

ABSTRACT

@#X linked Dystonia Parkinsonism (XDP) is a progressive movement disorder among male inhabitants of the island of Panay in the Philippinesi, it would start as a focal dystonia then become generalised. The disease is believed to be due to a founder mutation of some 50 meiotic generations ago;;_ Although initially seen in male population, current data shows otherwise. Reports of about 11 female cases are detected and listed in the registry. Male more than female predilection is seen with a ratio of 751 iii_ XDP is a very disabling degenerative disease causing involuntary torsion of the body, torticolis, blepharospasm and eventually parkinsonian featuresiv. There is no known treatment, and alleviation of symptoms is difficult to achieve, some patients even die due to infection and self harm. This report will present a case of XDP with predominating parkinsonian feature, who has undergone bilateral Globus Pallidus lnterna(GPi} Deep brain Stimulation (DBS}. After the operation, return to functionality and improvements in activities in daily living is seen. The UPDRS and FMDRS scores show improvement noted on regular follow up. However adverse effects such as slurring of speech and minimal blepharospasm are still observed.