Outcome of pallidal stimulation of idiopathic generalized dystonia with predominant mobile truncal dystonia: case report.

BACKGROUND: Further reports are required to describe the outcome of truncal dystonia treated by bilateral pallidal stimulation (globus pallidus interna deep brain stimulation [GPi-DBS]), owing to the small number of reports and clinical variability and complexity of truncal dystonia. Retrospectively, we report our experience of treating three patients with idiopathic generalized dystonia, with predominant mobile truncal dystonia by bilateral GPi-DBS. METHODS: Three patients with idiopathic generalized dystonia underwent bilateral GPi-DBS. One patient had adult-onset dystonia, while two patients had childhood-onset dystonia. All patients had predominant mobile truncal dystonia of mixed abnormal postures (camptocormia and lateral tilt), while one patient had also truncal twist. Patients were assessed pre- and post-GPi-DBS using the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) and Dystonia Disability Scale (DDS). RESULTS: The three patients showed marked improvement of global (94.78%, 92.4% and 80.95%) and truncal BFMDRS (all abnormal postures) (87.5%, 93.75% and 87.5%) and DDS (95.84% and 50%), using high amplitude monopolar settings, with a dramatic improvement of the mobile component. Improvement was persistent for 1.5, 3 and 6 years. CONCLUSION: Bilateral GPi-DBS improves markedly the mobile truncal dystonia and associated abnormal postures in patients with adult and childhood-onset idiopathic generalized dystonia. Improvement was persistent for up to 6 years.

Combined Unilateral Posteroventral Pallidotomy and Ventral Intermediate Nucleus Thalamotomy in Tremor-Dominant Parkinson's Disease versus Posteroventral Pallidotomy Alone: A Prospective Comparative Study.

BACKGROUND: The optimum target in surgery for Parkinson's disease (PD) is still controversial, especially in patients with tremor-dominant PD. We aim to compare results in tremor-dominant patients undergoing pallidotomy vs. those undergoing simultaneous posteroventral pallidotomy (PVP) and ventral intermediate nucleus (VIM) thalamotomy. METHODS: Twenty-four patients with tremor-dominant PD were included in this study. Twelve patients received unilateral PVP contralateral to the most affected side. The other 12 patients received simultaneous unilateral PVP and VIM thalamotomy contralateral to the most affected side. Assessment of results in both groups was achieved using both UPDRS "off" motor scores and UPDRS rest tremor subscores. RESULTS: The mean UPDRS off motor score improved in the pallidotomy group from 61.3 preoperatively to 36.8 at 12 months. In the combined group, it improved from 59.6 to 35.2 at 12 months, with no statistically significant difference between both groups. On the other hand, while the mean tremor subscore in the pallidotomy group improved from a mean of 2.3-0.8, the tremors were abolished in all of the patients in the combined group except for 1 patient who showed slight infrequent tremors at 12 months. CONCLUSION: Patients with tremor-dominant PD achieve more improvement in tremor control after combined PVP and VIM thalamotomy.

Pure spinal epidural cavernous hemangioma: A case series of seven cases.

INTRODUCTION: Pure spinal epidural cavernous hemangiomas (PSECHs) are rare vascular lesions with about 100 cases reported. Herein, we present a case series of 7 PSECHs discussing their clinical presentation, radiological characteristics, surgical technique and intraoperative findings, pathological features, and functional outcome. MATERIALS AND METHODS: We retrieved from the retrolective databases of the senior authors, patients with pathologically confirmed PSECH operated between January 2002 and November 2015. From their medical records, the patients' sociodemographic, clinical, radiological, surgical, and histopathological data were retrieved and analyzed. RESULTS: The mean age of the seven cases was 50.3 years. Four were females. All the five cases (71.4%) in the thoracic spine had myelopathy and the 2 (28.6%) lumbar cases had sciatica. Local pain was present in all the cases. All the lesions were isointense on T1-weighted images, hyperintense on T2-weighted images, and in five cases there was strong homogeneous enhancement. In six cases (85.7%), classical laminectomy was done; lesions resected in one piece in five cases. Total excision was achieved in all the cases. Lesions were thin-walled dilated blood vessels, lined with endothelium, and engorged with blood and with scanty loose fibrous stroma. The median follow-up was 12 months (range: 1-144 months). All patients gradually improved neurologically and achieved a good outcome with no recurrence at the last follow-up. CONCLUSION: PSECH although rare is increasing reported and ought to be included in the differential diagnosis of spinal epidural lesions. Early surgical treatment with total resection is recommended as would result in a good prognosis.