Stereotactic and functional neurosurgery (SFN) is one of the oldest subspecialties of neurosurgery. In Japan, functional epilepsy surgery was performed in the Meiji era, and general surgeons operated ...on patients with intractable cancer pain with open myelotomy and cordotomy even before World War Ⅱ. The knowledge gained from such old procedures contributed to the understanding of neurophysiology. Therefore, functional neurosurgery was known as “applied neurophysiology”. Human stereotactic surgery started in 1947, and many Japanese neurosurgeons, particularly Hirotaro Narabayashi, Keiji Sano, and Chihiro Ohye, have contributed to the development of this field. We also have to remember that common procedures currently used in neurosurgery, such as neuroendoscopy, navigation surgery, intraoperative monitoring, and the concept of exo-scope, are taken from SFN, which pursue less invasive and accurate surgery. Research on the Forel H field in the 1960s in Japan is now being revived for the treatment of epilepsy, Parkinson's disease, and dystonia. Young doctors should learn from the history and understand where we come from, where we are now, and where we are going. This is very important for Japan's contribution to many untreated patients.
Gamma aminobutyric acid (GABA) is an inhibitor neurotransmitter that plays many important roles in the central nervous system. Because the half-life time of GABA is very short in vivo, GABA itself is ...not used for clinical practice. An analogue of GABA, baclofen, is an agonist of GABA-B receptor, and has very strong antispastic effect by acting to the posterior horn of the spinal cord. However, baclofen poorly crosses through the blood brain barrier, and the antispastic effect is modest when administered orally. Therefore, direct continuous infusion of small doses of baclofen into the cerebrospinal fluid (intrathecal baclofen therapy, ITB) has become an established treatment for control of otherwise intractable severe spasticity. Spasticity is clinically defined as hypertonic state of the muscles with increased tendon reflexes, muscles spasm, spasm pain, abnormal posture, and limitation of involuntary movements. Spasticity is a common symptom after damage mainly to the pyramidal tract system in the brain or the spinal cord. Such damage is caused by traumatic brain injury, stroke, spinal cord injury, multiple sclerosis, and so on. Patients in persistent vegetative state (PVS) usually have diffuse and widespread damage to the brain, spasticity is generally seen in such patients. Control of spasticity may become important in the management of PVS patients in terms of nursing care, pain relief, and hygiene, and ITB may be indicated. Among PVS patients who had ITB to control spasticity, sporadic cases of dramatic recovery from PVS after ITB have been reported worldwide. The mechanism of such recovery of consciousness is poorly understood, and it may simply be a coincidence. On the other hand, electrical spinal cord stimulation (SCS) has been tried for many years in many patients in PVS, and some positive effects on recovery of consciousness have been reported. SCS is usually indicated for control of neuropathic pain, but it has also antispastic effect. The mechanism of SCS on pain is known to be mediated through the spinal GABA neuronal system. Thus, ITB and SCS have a common background, spinal GABA neuronal mechanism. The effect of GABA agonists on recovery of consciousness is not yet established, but review of such case studies becomes a clue to solve problems in PVS, and there may be hidden serendipity.
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.
Uncontrolled pilot studies have suggested the efficacy of focused ultrasound thalamotomy with magnetic resonance imaging (MRI) guidance for the treatment of essential tremor.
We enrolled patients ...with moderate-to-severe essential tremor that had not responded to at least two trials of medical therapy and randomly assigned them in a 3:1 ratio to undergo unilateral focused ultrasound thalamotomy or a sham procedure. The Clinical Rating Scale for Tremor and the Quality of Life in Essential Tremor Questionnaire were administered at baseline and at 1, 3, 6, and 12 months. Tremor assessments were videotaped and rated by an independent group of neurologists who were unaware of the treatment assignments. The primary outcome was the between-group difference in the change from baseline to 3 months in hand tremor, rated on a 32-point scale (with higher scores indicating more severe tremor). After 3 months, patients in the sham-procedure group could cross over to active treatment (the open-label extension cohort).
Seventy-six patients were included in the analysis. Hand-tremor scores improved more after focused ultrasound thalamotomy (from 18.1 points at baseline to 9.6 at 3 months) than after the sham procedure (from 16.0 to 15.8 points); the between-group difference in the mean change was 8.3 points (95% confidence interval CI, 5.9 to 10.7; P<0.001). The improvement in the thalamotomy group was maintained at 12 months (change from baseline, 7.2 points; 95% CI, 6.1 to 8.3). Secondary outcome measures assessing disability and quality of life also improved with active treatment (the blinded thalamotomy cohort)as compared with the sham procedure (P<0.001 for both comparisons). Adverse events in the thalamotomy group included gait disturbance in 36% of patients and paresthesias or numbness in 38%; these adverse events persisted at 12 months in 9% and 14% of patients, respectively.
MRI-guided focused ultrasound thalamotomy reduced hand tremor in patients with essential tremor. Side effects included sensory and gait disturbances. (Funded by InSightec and others; ClinicalTrials.gov number, NCT01827904.).
There have been no previous reports of chronic encapsulated expanding hematoma after Gamma Knife thalamotomy. The present case underwent Gamma Knife thalamotomy for essential tremor at the age of 78 ...years. Three- and 12-month posttreatment magnetic resonance imaging (MRI) showed small T2 high-intensity lesions on the target and along with the internal capsule. Hemiparesis developed 17 months after the treatment. Twenty months post treatment, T2-MRI showed a hypointense mass across the target and internal capsule. Gradual expansion of the mass was confirmed on MRI at 26–38 months. A 54-month posttreatment MRI showed marked expansion of the mass with multiple cysts surrounded by a T2-hypointense rim. Gadolinium-enhanced T1-MRI showed partial enhancement of the mass. MRI findings suggested a radiation-induced cavernoma. Hemiparesis, dysesthesia, and pain on the right side of the body persisted even after steroid therapy for several months. Long-term careful observation is necessary after Gamma Knife thalamotomy.
This study evaluated changes of fractional anisotropy (FA) in the ventral intermediate nucleus (VIM) of the thalamus after transcranial MR-guided focused ultrasound (TcMRgFUS) thalamotomy and their ...associations with clinical outcome.
Clinical and radiological data of 12 patients with medically refractory essential tremor (mean age 76.5 years) who underwent TcMRgFUS thalamotomy with VIM targeting were analyzed retrospectively. The Clinical Rating Scale for Tremor (CRST) score was calculated before and at 1 year after treatment. Measurements of the relative FA (rFA) values, defined as ratio of the FA value in the targeted VIM to the FA value in the contralateral VIM, were performed before thalamotomy, and 1 day and 1 year thereafter.
TcMRgFUS thalamotomy was well tolerated and no long-term complications were noted. At 1-year follow-up, 8 patients demonstrated relief of tremor (improvement group), whereas in 4 others persistent tremor was noted (recurrence group). In the entire cohort, mean rFA values in the targeted VIM before treatment, and at 1 day and 1 year after treatment, were 1.12 ± 0.15, 0.44 ± 0.13, and 0.82 ± 0.22, respectively (p < 0.001). rFA values were consistently higher in the recurrence group compared with the improvement group, and the difference reached statistical significance at 1 day (p < 0.05) and 1 year (p < 0.01) after treatment. There was a statistically significant (p < 0.01) positive correlation between rFA values in the targeted VIM at 1 day after thalamotomy and CRST score at 1 year after treatment. Receiver operating characteristic curve analysis revealed that the optimal cutoff value of rFA at 1 day after thalamotomy for prediction of symptomatic improvement at 1-year follow-up is 0.54.
TcMRgFUS thalamotomy results in significant decrease of rFA in the targeted VIM, at both 1 day and 1 year after treatment. Relative FA values at 1 day after treatment showed significant correlation with CRST score at 1-year follow-up. Therefore, FA may be considered a possible imaging biomarker for early prediction of clinical outcome after TcMRgFUS thalamotomy for essential tremor.
We report the case of a patient with hypothalamic hamartoma (HH) who was successfully treated with magnetic resonance–guided focused ultrasound (MRgFUS) for ablation as a disconnection surgery. A ...26-year-old man with gelastic epilepsy had been diagnosed with HH at 3 years of age, and antiepileptic drugs were administered due to worsening episodes. Magnetic resonance imaging showed a sessile parahypothalamic hamartoma and MRgFUS ablation was performed, creating an oval-shaped lesion at the boundary area of the HH. Dramatic improvements in seizure symptoms were noted, and he was seizure-free on decreased antiepileptic drugs without any adverse events over the 1-year follow-up period.
Objective
Musician's dystonia is a task‐specific movement disorder that causes twisting or repetitive abnormal finger postures and movements, which tend to occur only while playing musical ...instruments. Such a movement disorder will probably lead to termination of the careers of affected professional musicians. Most of the currently available treatments have yet to provide consistent and satisfactory results. We present the long‐term follow‐up results of ventro‐oral thalamotomy for 15 patients with musician's dystonia.
Methods
Between October 2003 and September 2010, 15 patients with medically intractable task‐specific focal hand dystonia that occurred only while playing musical instruments underwent ventro‐oral thalamotomy. We used Tubiana's musician's dystonia scale to evaluate the patients' pre‐ and postoperative neurological conditions.
Results
All patients except 1 (93%) experienced dramatic improvement of dystonic symptoms immediately after ventro‐oral thalamotomy. The mean follow‐up period was 30.8 months (range = 4–108 months). None of the patients experienced recurrence or deterioration of symptoms during the follow‐up periods.
Interpretation
Ventro‐oral thalamotomy remarkably improved musician's dystonia, and the effect persisted for a long duration. Ann Neurol 2013;74:648–654
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