Hisashi Ito

Magnetic Resonance Imaging-guided Focused Ultrasound Thalamotomy for Parkinson's Disease with Cardiac Pacemaker: A Case Report

Surgical intervention for the ventral intermediate nucleus (Vim) became recognized as a therapeutic option for medication-refractory tremor, and transcranial MRI-guided focused ultrasound (MRgFUS) is a new neurosurgical tool for several neurological disorders. We report the case of a 75-year-old, right-handed man with a 15-year history of tremor-dominant Parkinson’s disease (PD). He suffered from refractory tremor of the right hand despite 300 mg of levodopa with benserazide plus 6 mg of trihexyphenidyl. The Unified Parkinson’s Disease Rating Scale (UPDRS) score off medication was 15 and 17 (part 2 and 3, respectively). Patient suffered from sick sinus syndrome since 69 years old and had been implanted with a MR-conditional cardiac pacemaker system (Advisa DR MRI SureScan and Capsure Z Novus 5054/5554, Medtronic Japan), programed in DDD mode, with 60/130 bpm. Patient refused to continue exploring different drug schedules as well as deep brain stimulation as a possible treatment. Following institutional review board approval, MRgFUS thalamotomy was proposed. The patient provided written informed consent in accordance with the Declaration of Helsinki before treatment. Using 1.5-Tesla MRI (Signa HDx, GE Healthcare) and a focused ultrasound system (Exablate 4000, Insightec), we employed a similar methodology to that in MRgFUS thalamotomy. Before the procedure, we programed the pacemaker in DOO mode with 100 bpm and prepared resuscitation equipment, including an automated external defibrillator. Intraprocedurally, we monitored electrocardiographically and a cardiologist stood by. The shortest distance from the hemisphere with ultrasound transducers to the pacemaker was 20.5 cm. We chose the target in the left Vim at a point 6.8 mm anterior to the posterior commissure, 18.6 mm lateral from the midline (12.3 mm from the ventricle), and 1.5 mm above the anterior commissure–posterior commissure line. Gradually we increased the sonication energy and the resting tremor in the right hand disappeared. The total sonication time was 133 seconds in 8 sonications (mean: 16.6 5.2 seconds, range: 10–25 seconds). The mean energy was 10,181.9 7,662.6 J (range: 1,510–22,050 J) with a maximum temperature of 56°C. Mild headache and a floating sensation occurred during sonications; however, heat sensation, burn, and pacemaker malfunctioning were not observed. Bradykinesia and mild right-sided weakness resulting from the surrounding edema after thalamotomy were improved by prednisolone. The UPDRS score off medication was 5 and 13 (part 2 and 3) at 3 weeks (Fig. 1). MRgFUS is characterized by minimum invasiveness, no exposure to radiation, and an immediate therapeutic effect. However, to date it has not been performed for patients with cardiac pacemakers. Despite the lack of evidence, some pacemakers have no limitation regarding the MR imaging time and intervals; also, therapeutic ultrasound is considered acceptable with precaution. PD is a common neurological disorder and over 700,000 new cardiac pacemakers are implanted per year. However, this is the first report of MRgFUS thalamotomy for a patient with a cardiac pacemaker. Further investigations concerning the safety of MRgFUS for patients with cardiac pacemakers are necessary.

Facial Nerve Palsy after Botulinum Toxin Therapy for Hemifacial Spasm: A Case Report

We herein report a case of left hemifacial spasm (HFS) developed ipsilateral facial nerve palsy 5 days after botulinum toxin type A (BTX-A) injection. Multiple facial muscles presented weakness regardless of BTX-A injection and electrophysiological study revealed left facial nerve palsy. Facial nerve palsy improved completely after 24 weeks. Facial nerve palsy is a common disease and could occur as a coincidence. On the other hand, BTX-A might cause neuropathy in some previously reported cases and our case. Although the pathogenesis of neuropathy following BTX-A injection has not been elucidated, this is the first case of ipsilateral facial nerve palsy following BTXA injection for HFS. Thorough neurological examination and electrophysiological study were useful for distinguishing facial nerve palsy from facial muscles weakness related with botulinum toxin.

2 Cases of Acute Confusional State with Autonomic Symptoms after Designer Tryptamine Abuse

We herein report 2 cases of acute tryptamine abuse. Both cases developed confusional state within 1 hour of 4- acetoxy-N-methyl-N-isopropyl-tryptamine ingestion. On admission, they presented confusion with marked mydriasis. In addition, one case showed hypertension, low grade fever, and hyperhidrosis, and the other showed the contraction of pilomotor muscles. Confusion and other autonomic symptoms improved gradually with intravenous drip infusion of saline. Tryptamine abuse should be considered as differential diagnosis in cases of acute confusional state with various autonomic symptoms.

Diaphragm Pacing Could Have Efficacy for Sleep Condition in Patients with Amyotrophic Lateral Sclerosis

Background: To investigate the efficacy of electrical diaphragm pacing (DP) for sleep condition in patients with amyotrophic lateral sclerosis (ALS). Method: We investigated 5 Japanese ALS patients without positive-pressure mechanical ventilation (3 men and 2 women, aged 59.6 ± 9.6 years). All of them were implanted NeuRx RA/4 Diaphragm Pacing System R (NeuRx) in the diaphragm laparoscopically. We assessed physical status and polysomnographic recordings before implantation and after 6 months of conditioning with DP turned on. Results: 4 patients completed the evaluation. Sleep condition showed the tendency to improve far from getting worse despite of deterioration of physical status and respiratory function. Conclusion: DP with NeuRx might assist the sleep condition in ALS patients at least in 6 months of follow up.

Clinical Efficacy of Istradefylline for Depression in Parkinsonâs Disease

Background: Parkinson’s disease (PD) is a common movement disorder with a wide range of non-motor symptoms. Depression is one of these symptoms; however, its pathomechanism and management remains to be elucidated. We evaluated of istradefylline (ISD), a first selective adenosine A2A receptor antagonist, for the treatment of depression in PD. Method: This was an open-labeled, prospective study that enrolled 15 PD patients (Men 8, Women 7) with motor fluctuations who fully filled UK PD society brain bank clinical diagnostic criteria. We added ISD 20 mg/day for 4 weeks followed with 40 mg/day for next 4 weeks on the preceding anti-parkinsonian medications. We evaluated Patient Health Questionnaire (PHQ-9) and Unified PD Rating Scale (UPDRS) part III (on state) at baseline and 8 weeks follow-up. Results: 14 patients completed the evaluations. PHQ-9 scores improved in 5 patients (responder). PHQ-9 scores of responders at baseline were higher than those of nonresponders; however, there was no significant difference. Furthermore, there were no significant differences in UPDRS part III, age, onset, duration, daily levodopa dose, and levodopa equivalent dose at baseline among both groups. UPDRS part III scores improved in both groups; however, there was also no significant difference between them. Conclusion: ISD could have efficacy to depression in some PD patients.

Magnetic Resonance Imaging-guided Focused Ultrasound Thalamotomy for Parkinson's Disease: A Case Report

Thalamotomy is effective in treating refractory tremor in Parkinsons 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.

PO6.6 Phrenic Nerve Function in Patients with Bulbospinal Muscular Atrophy

Background: The purpose of this study was to retrospectively evaluate the electrophysiological characteristics (both of needle EMG and nerve conduction studies) of hirayama disease. Methods: Electrophysiological records of 77 patients who were diagnosed as hirayama disease were reviewed. We analyzed the amplitude of compound muscle action potential (CMAPs) and sensory nerve action potentials (SNAPs) as well as the F wave frequency of median and ulnar nerve. We also evaluated the changes of needle electromyography. Results: Of the 77 patients, 74 were male and 3 female. Mean age of onset was 17.7±2.6 years and mean disease duration 28.2±25.7 months. 57 (74%) of the 77 patients showed clinical presentation of unilateral involvement (37 were in the right upper limb and 20 in the left) and the other 20 showed bilateral symptoms. Reduced ulnar CMAP amplitude was found in 90.2% and reduced median CMAP amplitude was found in 45.1% patients. Abnormal F wave was found in 91.2% of the patients in both median and ulnar nerves, and there was also correlation between F wave frequency and amplitude of CMAPs. Sensory conduction studies were normal. In the needle EMG, neurogenic changes were found in both upper limbs in 67 cases (87%), in the remaining 10 patients, 6 involved the right side and 4 involved the left. Denervation was found in the C7 T1 myotomes in all of the 77 patients. Among them, 23 (29.9%) also showed C5, C6 involvement with different extent. Conclusions: In this study, the most obvious electrophysiological abnormality was reduced F wave frequency in both median and ulnar nerve (although abnormal median CMAP amplitude was less frequent than the ulnar nerve) followed by reduced ulnar CMAP amplitude. Although most patients showed unilateral involvement clinically, needle EMG found 87% had bilateral neurogenic changes. Although C7, C8,T1 myotomes were mainly damaged, C5 C6 was also involved in around 1/3 of the patients.