The promise of deep brain stimulation in X-linked dystonia parkinsonism

X-linked dystonia parkinsonism (XDP) is a rapidly progressive and disabling neurodegenerative disease affecting mainly male Filiponos with origins from Panay Island. We reviewed all the past neurosurgical ablative procedures done for XDP patients listed in the Philippine XDP registry. From 1960 to 1982, six patients had undergone bilateral chemopallidotomies or bilateral thalomotomies stage over time. Half of these patients had significant improvement in their symptoms but five of the six patients (83%) developed postoperative morbidities, mainly speech impairment or hemiparesis, All the five reported GPi deep brain stimulation (DBS) cases for XDP were also reviewed, showing consistently immediate improvement of symptoms (61.5%-88.3% decrease in the Burke-Marsden Dystonia Rating Scale) lasting up to a year with effects noted. We also present the first Philippine case of GPi DBS done in the youngest XDP patients to date. This present case showed dramatic improvement(83.3% desrease of the Burke-Marsden_Fahn Dystonia Rating Scale) of his dystonic symptoms, without incurring any persistent adverse effects. The results of these early cases of pallidal DBS for XDP show that DBS is generally a safe and effective procedure for alleviating the disabling symptoms of XDP in contrast to previous ablative surgeries on these patient

Endoscopic third ventriculostomy: An outcome analysis of 30 pediatric cases

OBJECTIVE: Endoscopic third ventriculostomy (ETV) has been shown to be a sufficient alternative in the surgical treatment of hydrocephalus. Our goal in this retrospective study is to analyze our results with the use of ETV in our first 30 cases that it may provide us with selection criteria as to who among our patients will benefit most from this procedure.METHODOLOGY: Thirty ETVs were performed in 30 patients. Their ages ranged from 2-155 months. Hydrocephalus was caused by aqueductal stenosis in 17 patients, tumors in 7, post-infectious in 3, Dandy-Walker malformation in 2 and arachnoid cyst in 1 patient. The outcome of ETV was evaluated in 26 of the cases that were available for follow-up RESULTS: The overall success rate was 69.2 percent. Patients with non-communicating hydrocephalus from post-infectious causes, tumors and aqueductal stenosis had high success rates. Patients less than 6 months of age had a poor outcome. Complications included ventriculitis in 1 patient CONCLUSION: ETV is a viable treatment option for non-communicating hydrocephalus secondary to post-infectious cause, aqueductal stenosis and tumors. A successful outcome is more likely if ETV is done in patients more than 6 months of age Patients who have previously undergone shunting and who have non-communicating hydrocephalus should undergo ETV at the time of shunt failure. These patients showed good outcome.