Focused Ultrasound Thalamotomy for Tremor-dominant Parkinson's Disease: A Prospective 1-year Follow-up Study.

Transcranial magnetic resonance (MR)-guided focused ultrasound (FUS) therapy is an emerging and minimally invasive treatment for movement disorders. There are limited reports on its long-term outcomes for tremor-dominant Parkinson's disease (TDPD). We aimed to investigate the 1-year outcomes of ventralis intermedius (VIM) thalamotomy with FUS in patients with TDPD. Patients with medication-refractory TDPD were enrolled and underwent unilateral VIM-FUS thalamotomy. Neurologists specializing in movement disorders evaluated the tremor symptoms and disability using Parts A, B, and C of the Clinical Rating Scale for Tremor (CRST) at baseline and at 1, 3, and 12 months. In all, 11 patients (mean age: 71.6 years) were included in the analysis. Of these, five were men. The median (interquartile range) improvement from baseline in hand tremor score, the total score, and functional disability score were 87.9% (70.5-100.0), 65.3% (55.7-87.7), and 66.7% (15.5-85.1), respectively, at 12 months postoperatively. This prospective study demonstrated an improvement in the tremor and disability of patients at 12 months after unilateral VIM-FUS thalamotomy for TDPD. In addition, there were no serious persistent adverse events. Our results indicate that VIM-FUS thalamotomy can be safely and effectively used to treat patients with TDPD. A randomized controlled trial with a larger cohort and long blinded period would help investigate the recurrence, adverse effects, placebo effects, and longer efficacy of this technique.

Two-year Follow-up Results of Magnetic Resonance Imaging-guided Focused Ultrasound Unilateral Thalamotomy for Medication-refractory Essential Tremor.

Objective To investigate the long-term efficacy and safety of magnetic resonance imaging-guided focused ultrasound (MRgFUS) unilateral ventral intermediate nucleus (Vim) thalamotomy for medication-refractory essential tremor (ET). Methods We performed MRgFUS left-sided Vim thalamotomy for 10 medication-refractory ET patients (8 men and 2 women, aged 67.1±17.5 years, all right-handed). We followed them for 2 years using the clinical rating scale for tremor (CRST) and the quality of life in essential tremor questionnaire (QUEST). Results Right-handed tremor improved immediately after the left Vim thalamotomy in all patients. The tremor became re-exacerbated in 2 patients by 6 months after treatment; however, an approximately 60% decrease in the average CRST score of the right hand persisted until 2 years. On the other hand, the average CRST score of the left hand and the average QUEST score showed no improvement. Headache was the most common adverse event during the sonication (8 patients), followed by a floating sensation (4 patients). On the other hand, sensory disturbances (4 patients) and gait instability (4 patients) were observed after the treatment, but most of them were mild and transient. There were no delayed adverse events. Conclusion MRgFUS unilateral Vim thalamotomy could be adopted as one of the therapeutic options for intractable ET. Further improvement of tremor in the targeted hand or contralateral Vim thalamotomy may be necessary to improve the quality of life.

Magnetic Resonance Imaging-guided Focused Ultrasound Thalamotomy for Parkinson's Disease.

Thalamotomy is effective in treating refractory tremor in Parkinson's disease (PD). We herein report a PD patient who underwent left ventral intermediate nucleus and ventro oralis posterior nucleus thalamotomy using magnetic resonance imaging-guided focused ultrasound (MRgFUS). Right-side resting tremor and rigidity were abolished immediately following the ultrasound energy delivery. In addition, left-side resting tremor and rigidity also improved. No adverse events occurred during the procedure. We observed the exacerbation of bradykinesia, which might have been caused by edema around the target. This is the first report of thalamotomy using MRgFUS for PD patient from Japan. Further investigations concerning the efficacy and safety of this procedure are necessary.

An Autopsy Case of Amyotrophic Lateral Sclerosis with Diaphragm Pacing.

Respiratory insufficiency is a critical problem in amyotrophic lateral sclerosis (ALS) patients. We herein present the case of an autopsied patient with sporadic ALS who underwent diaphragm pacing (DP). The pathology showed several localized adhesions with a markedly atrophied diaphragm. A marked loss of motor neurons with Bunina bodies and phosphorylated TDP-43 positive inclusions was found in the spinal cord and primary motor cortex. Mild hyalinization and a few multinucleated giant cells were present around the electrode tracks in the diaphragm. However, no infiltration of inflammatory cells was detected. Our findings suggest that full-time DP might not cause severe damage to adjacent diaphragm tissue.