Secondary dystonia following parenchymal brain tumors.

BACKGROUND: Secondary dystonia has been associated with diverse etiologies. Dystonia associated with brain tumors has not been well characterized. OBJECTIVES: To characterize dystonia and relationship with parenchymal brain tumors. METHODS: We present six patients (1.03%) with dystonia related to parenchymal brain tumors, among 580 screened cases. RESULTS: Contralateral hemidystonia was observed in four cases, followed by focal limb (n = 1) and cervical dystonia (n = 1). Dystonia presented during the phase of tumor growth in four cases, and following tumor treatment in two, one case had re-emergent dystonia. Tumors were low-grade (WHO I or II) and located in the basal ganglia (n = 3), cortical areas (n = 2), thalamus (n = 1) and cerebral peduncle (n = 1). CONCLUSIONS: Secondary dystonia may be caused by brain tumors in diverse locations including basal ganglia, cortex and thalamus. It may be the presenting symptom of brain tumor or follow surgical resection combined with ancillary therapy.

Intestinal Decontamination Therapy for Dyskinesia and Motor Fluctuations in Parkinson's Disease.

Parkinson's disease is neurodegenerative disorder with an initial robust response to levodopa. As the disease progresses, patients frequently develop dyskinesia and motor fluctuations, which are sometimes resistant to pharmacological therapy. In recent years, abnormalities in gut microbiota have been identified in these patients with a possible role in motor manifestations. Dysbiosis may reduce levodopa absorption leading to delayed "On" or "no-On" states. Among 84 consecutive patients with PD, we selected 14 with levodopa-induced dyskinesia and motor fluctuations with a Movement Disorders Society Unified Parkinson's Disease Rating Scale (MDS-UPDRS) part IV ≥ 8 points following a trial of pharmacological adjustment 2-3 months prior to study enrollment or adjustments in deep brain stimulation therapy. Patients received treatment with sodium phosphate enema followed by oral rifaximin and polyethylene glycol for 7 and 10 days, respectively. Evaluations between 14 to 21 days after starting treatment showed improvement in MDS-UPDRS-IV (P = 0.001), including duration (P = 0.001) and severity of dyskinesia (P = 0.003); duration of medication "Off"-state (P = 0.004); functional impact of motor fluctuations (P = 0.047) and complexity of motor fluctuations (P = 0.031); no statistical improvement was observed in "Off" dystonia (P = 0.109) and total motor scores (P = 0.430). Marked to moderate improvement in dyskinesia was observed in 57% of cases with blinded evaluation of videos. About 80% of patients perceived moderate to robust improvement at follow-up. A therapeutic strategy aimed at decontamination of intestines showed benefit in motor fluctuations and dyskinesia. Further studies should confirm and clarify the mechanism of improvement observed in these patients.

The safety and efficacy of thalamic deep brain stimulation in essential tremor: 10 years and beyond.

BACKGROUND: Deep brain stimulation (DBS) has proven to be a safe and effective therapy for refractory essential tremor, but information regarding long-term outcomes is lacking. OBJECTIVES: We aimed to assess the long-term safety and efficacy of DBS in patients with essential tremor. METHODS: Patients treated with DBS for essential tremor for at least 8 years were evaluated in the 'on' and 'off' state using the Fahn-Tolosa-Marin tremor rating scale, and their medical records were reviewed to assess complications related to this therapy. RESULTS: We studied 13 patients (7 men): median age at evaluation 79 years (range 47-88), median age at electrode implantation 68 years (range 37-78) and mean time since electrode implantation 132.54±15.3 months (range 114-164). The difference between the 'off' and 'on' state on the motor items of the tremor rating scale was 41.9% (58.62 vs. 34.08, p<0.001) in the non-blinded and 37.2% (56.07 vs. 35.23, p<0.001) in the blinded rating. DBS provided a functional improvement of 31.7% in the 'on' state (15.07 vs. 22.07, p<0.001). A total non-blinded improvement in the tremor rating scale of 39% was observed in the 'on' state (49.15 vs. 80.69, p<0.001). Dysarthria and disequilibrium were common in patients with bilateral stimulation. A DBS-related surgery (electrode revision or internal pulse generator exchange) was necessary on average every 47.9 months to continue with the DBS therapy. CONCLUSIONS: Thalamic DBS is a safe and effective therapy in patients with essential tremor followed for up to 13 years.