Pallidal deep brain stimulation for patients with myoclonus-dystonia without SGCE mutations.

BACKGROUND: Pallidal deep brain stimulation (GPi-DBS) is effective for treating myoclonus and dystonia caused by SGCE mutations (DYT-SGCE, DYT11). However, it is unknown whether GPi-DBS is effective for the treatment of myoclonus-dystonia which is not associated with the SGCE gene mutations. In this study, we investigated the efficacy of GPi-DBS in treating myoclonus-dystonia in SGCE mutation-negative cases. METHODS: Three patients with myoclonus-dystonia without SGCE mutations who underwent GPi-DBS were evaluated preoperatively and 6 months postoperatively using the Unified Myoclonus Rating Scale (UMRS) and Fahn-Marsden Dystonia Rating Scale (FMDRS) for myoclonus and dystonia, respectively. In two of the three patients, myoclonus was more evident during action. Myoclonus was predominant at rest in the other patient, and he was unaware of his dystonia symptoms. The results were compared with those of the four DYT-SGCE cases. RESULTS: The mean UMRS score in patients with myoclonus-dystonia without SGCE mutations improved from 61.7 to 33.7 pre- and postoperatively, respectively, and the mean FMDRS score improved from 7.2 to 4.5. However, the degree of improvement in myoclonus-dystonia in patients without SGCE mutations was inferior to that in patients with DYT-SGCE (the UMRS score improved by 45% and 69%, respectively). CONCLUSIONS: GPi-DBS is effective for treating myoclonus-dystonia in patients with and without SGCE mutations. GPi-DBS should be considered as a treatment option for myoclonus-dystonia without SGCE mutations.

Deep brain stimulation for refractory obsessive-compulsive disorder (OCD): emerging or established therapy?

A consensus has yet to emerge whether deep brain stimulation (DBS) for treatment-refractory obsessive-compulsive disorder (OCD) can be considered an established therapy. In 2014, the World Society for Stereotactic and Functional Neurosurgery (WSSFN) published consensus guidelines stating that a therapy becomes established when "at least two blinded randomized controlled clinical trials from two different groups of researchers are published, both reporting an acceptable risk-benefit ratio, at least comparable with other existing therapies. The clinical trials should be on the same brain area for the same psychiatric indication." The authors have now compiled the available evidence to make a clear statement on whether DBS for OCD is established therapy. Two blinded randomized controlled trials have been published, one with level I evidence (Yale-Brown Obsessive Compulsive Scale (Y-BOCS) score improved 37% during stimulation on), the other with level II evidence (25% improvement). A clinical cohort study (N = 70) showed 40% Y-BOCS score improvement during DBS, and a prospective international multi-center study 42% improvement (N = 30). The WSSFN states that electrical stimulation for otherwise treatment refractory OCD using a multipolar electrode implanted in the ventral anterior capsule region (including bed nucleus of stria terminalis and nucleus accumbens) remains investigational. It represents an emerging, but not yet established therapy. A multidisciplinary team involving psychiatrists and neurosurgeons is a prerequisite for such therapy, and the future of surgical treatment of psychiatric patients remains in the realm of the psychiatrist.

Tau progression in single severe frontal traumatic brain injury in human brains.

The neuropathological features of chronic traumatic encephalopathy (CTE), caused by repeated traumatic brain injury (TBI), include abnormal accumulations of hyper-phosphorylated tau (p-tau) protein in neurons, neurites and astrocytes, considered to progress via neuronal circuits in brains. Some previous reports suggest that a single severe TBI (sTBI) can also induce CTE and p-tau accumulation, but it is not clear whether the pathology is the same as that of repetitive TBI (rTBI). Since prefrontal leucotomy could be regarded as a model of sTBI, in this study we evaluated two autopsied schizophrenia with this procedure. Histopathologically, gliosis and neuronal loss were found not only in the primary ablated prefrontal region, but also in secondary affected areas, i.e., cingulate gyrus, medial nucleus of the thalamus, and nucleus accumbens, which are connected to prefrontal areas. Accumulation of p-tau was mostly seen in neurons, neurites and glias around small blood vessels in the leucotomized prefrontal region. In addition, secondary regions showed some p-tau-positive neurons/glias, as well as many axonal spheroids. Regions of neuronal/glial p-tau pathology showed immunoreactivity to both 3R/4R tau antibodies. Immunoblot analyses of sarkosyl-insoluble tau from frozen brains showed an AD-type tau banding pattern with strong immunoreactivities. sTBI patients showed limited comorbidities, such as TDP-43, alpha-synuclein or AD pathology, whereas rTBI patients have high frequencies of them. The findings suggest that p-tau in the primary affected lesion might progress to connected regions via neuronal circuits over time, and a single severe axonal injury might lead to CTE pathology different from that caused by rTBI.

The outcome of a surgical protocol based on ischemia overprotection in large and giant aneurysms of the anterior cerebral circulation.

Aiming to define the optimal treatment of large and giant aneurysms (LGAs) in the anterior circulation, we present our surgical protocol and patient outcome. A series of 42 patients with intracavernous LGAs (n = 16), paraclinoid (C2) LGAs (n = 17), and peripheral (middle cerebral artery-MCA or anterior cerebral artery-ACA) LGAs (n = 9) were treated after bypass under motor evoked potential (MEP) monitoring. Preoperatively, three categories of ischemic tolerance during internal carotid artery (ICA) occlusion were defined on conventional angiography: optimal, suboptimal, and insufficient collaterals. Accordingly, three types of bypass: low flow (LFB), middle flow (MFB) and high flow (HFB) were applied for the cases with optimal, suboptimal, and insufficient collaterals, respectively. Outcome was evaluated by the Glasgow Outcome Scale (GOS). All patients had excellent GOS score except one, who suffered a major ischemic stroke immediately after surgery for a paraclinoid lesion. Forty-one patients were followed up for 87.1 ± 40.1 months (range 13-144 months). Intracavernous LGAs were all treated by proximal occlusion with bypass surgery. Of paraclinoid LGA patients, 15 patients had direct clipping under suction decompression and other 2 patients with recurrent aneurysms had ICA (C2) proximal clipping with HFB. MEP monitoring guided for temporary clipping time and clip repositioning, observing significant MEP changes for up to 6 min duration. Of 9 peripheral LGAs patients 7 MCA LGAs had reconstructive clipping (n = 4) or trapping (n = 3) with bypass including LFB in 3 cases, MFB in 1 and HFB in 1. Two ACA LGAs had clipping (n = 1) or trapping (n = 1) with A3-A3 bypass. The applied protocol provided excellent results in intracavernous, paraclinoid, and peripheral thrombosed LGAs of the anterior circulation.

Vim thalamotomy in a patient with Holmes' tremor and palatal tremor - Pathophysiological considerations.

BACKGROUND: We peformed a ventral intermediate nucleus (Vim) thalamotomy in a patient with Holmes' tremor and palatal tremor. The frequencies of these movement disorders were 4 Hz and 3 Hz, respectively. Vim thalamotomy stopped the Holmes' tremor but not the palatal tremor. Our observations suggest different mechanisms for these two involuntary movements. CASE PRESENTATION: A 57-arm 11 months after a pontine hemorrhage. Transoral carotid ultrasonography revealed periodic motion of her posterior pharyngeal wall with a frequency of 3 Hz. Recording of neuronal activities in the thalamus revealed a 4Hz rhythmic discharge time that was associated with her tremor in the contralateral arm. A left Vim thalamotomy was performed. The resting tremor of the upper limb stopped, but the kinetic tremor recurred 6 months after the thalamotomy. No effect was observed on her palatal tremor. CONCLUSIONS: The different effects of Vim thalamotomy on the Holmes' tremor and palatal tremor suggest different oscillation sources for these two involuntary movements.

[DBS therapy for Parkinson's disease - our approach].

It is controversial whether the STN or the GPi, the main targets of DBS therapy in patients with Parkinson's disease, is the appropriate target. We select GPi-DBS in patients judged by our cognitive function test battery to be at high-risk for cognitive decline after STN-DBS. While DBS surgery is usually performed under local anesthesia for the precise placement of DBS electrodes, general anesthesia might be useful in patients intolerant of long-lasting surgical stress. Our monkey experiments revealed that the most medial part of the STN receives direct input from the limbic cortex, suggesting that the spread of stimulation to these limbic territories may elicit adverse emotional effects. Other monkey experiments on the physiological mechanism of DBS suggest that high-frequency GPi stimulation disrupts information flow through the GPi.

Presynaptic actions of D2-like receptors in the rat cortico-striato-globus pallidus disynaptic connection in vitro.

The cerebral cortex, the neostriatum (Str), and the external segment of the globus pallidus (GPe) form a cortico-Str-GPe disynaptic connection, which is one of the major connections in the basal ganglia circuitries and a target of dopamine modulation. The aim of this study was to examine the actions of D2-like dopamine receptors (D2LRs) in this connection using rat brain slice preparations. Electrical stimulation of the frontal cortex evoked disynaptic inhibitory postsynaptic currents (IPSCs) in cesium-filled GPe neurons voltage-clamped at 0 mV. The IPSCs evoked by threshold stimulation were small, <10 pA. Bath or local applications of the D2LR agonist quinpirole to Str decreased the amplitude of the cortical stimulation-induced IPSCs. Electrical stimulation of Str evoked monosynaptic IPSCs in GPe neurons. Local application of quinpirole to GPe decreased the Str stimulation-induced IPSCs. Bath application of quinpirole decreased the frequency of large miniature IPSCs (mIPSCs) that were considered to be evoked by local collateral axons of GPe neurons. These results suggested that activation of D2LRs decrease the gain of the cortico-Str-GPe disynaptic connection, with the decrease attributed to activation of D2LRs in Str and GPe, and that both Str-GPe and GPe-GPe GABAergic inhibitions are under the control of presynaptic D2LRs.

Effect of ventralis intermedius thalamotomy on the area in the sensorimotor cortex activated by passive hand movements: fMR imaging study.

Stereotactic ventralis intermedius (Vim) thalamotomy is effective for essential tremor (ET) of the limb, but the effect on the activity of the sensorimotor cortex is still unclear. The functional changes in this cortical area of patients with ET after Vim thalamotomy were investigated using functional magnetic resonance (fMR) imaging. Six patients underwent Vim thalamotomy for medically intractable ET, predominantly in the right hand. 1.5-tesla fMR imaging was performed using the blood oxygenation level-dependent sequence, before and after Vim thalamotomy, during passive movements with right wrist flexion and extension. Before and after images were analyzed using SPM99 software. Activation in the sensorimotor cortex and supplementary motor area evoked by wrist passive movement was observed both before and after surgery. Group analysis of changes in the blood oxygenation level-dependent response revealed a significantly smaller activated area postoperatively. Activation at the fundus of the central sulcus was characteristically decreased. All patients showed marked improvement in tremor after Vim thalamotomy. No patient experienced neurological deficits. fMR imaging showed that activation at the fundus of the central sulcus evoked by passive wrist movement was suppressed after Vim thalamotomy in ET patients, probably due to disruption of the thalamocortical pathway. The fundus of the central sulcus (Brodmann area 3a) is likely to be one of the key relays in the tremor circuit.

Transcranial electrical stimulation through screw electrodes for intraoperative monitoring of motor evoked potentials. Technical note.

The feasibility of high-frequency transcranial electrical stimulation (TES) through screw electrodes placed in the skull was investigated for use in intraoperative monitoring of the motor pathways in patients who are in a state of general anesthesia during cerebral and spinal operations. Motor evoked potentials (MEPs) were elicited by TES with a train of five square-wave pulses (duration 400 microsec, intensity < or = 200 mA, frequency 500 Hz) delivered through metal screw electrodes placed in the outer table of the skull over the primary motor cortex in 42 patients. Myogenic MEPs to anodal stimulation were recorded from the abductor pollicis brevis (APB) and tibialis anterior (TA) muscles. The mean threshold stimulation intensity was 48 +/- 17 mA for the APB muscles, and 112 +/- 35 mA for the TA muscles. The electrodes were firmly fixed at the site and were not dislodged by surgical manipulation throughout the operation. No adverse reactions attributable to the TES were observed. Passing current through the screw electrodes stimulates the motor cortex more effectively than conventional methods of TES. The method is safe and inexpensive, and it is convenient for intraoperative monitoring of motor pathways.

Reliable and convenient method for the fixation of recording electrodes on nonshaved scalp for intraoperative electrophysiological monitoring: technical note.

BACKGROUND: Difficulties with the intraoperative monitoring of evoked responses sometimes occur because of displacement or dislodgment of the recording electrodes during surgery, especially if placed on nonshaved scalp. METHODS: An alternative fixation method of the recording electrode on the nonshaved scalp was developed. The sterile subdermal needle electrode was attached on the scalp by application of a transparent dressing (Tegaderm) over the electrode followed by fixing with a skin stapler. RESULTS: The needle electrodes were easily, quickly, and firmly positioned even on the nonshaved scalp. The responses of the intraoperative evoked potentials were highly stable and reproducible throughout the operation. CONCLUSION: This fixation method for subdermal needle electrodes on the scalp is safe, reliable, and convenient for intraoperative electrophysiological monitoring of evoked potentials, even if the scalp is not shaved.

Analysis of taste disturbance before and after surgery in patients with vestibular schwannoma.

OBJECT: The frequency, nature, and history of subjective taste disturbance before and after vestibular schwannoma (VS) surgery was investigated. METHODS: Personal interviews were conducted in 108 patients with unilateral VS. Abnormalities in taste perception, either a significant reduction or a change in character, were experienced by 31 patients (28.7%) before surgery and by 37 (34.3%) after tumor removal. Preoperative taste disturbance worsened after surgery in five (16.1%) of the 31 patients, remained unchanged in eight (25.8%), improved in two (6.5%), and became normal in 16 (51.6%). Taste disturbance occurred postoperatively in 22 (28.6%) of 77 patients who had experienced no preoperative taste disturbance. The mean onset of the abnormality after resection was 1.1 +/- 1.7 months. Postoperative taste disturbance resolved in 24 of the 37 patients (64.9%) within 1 year after onset. CONCLUSIONS: Subjective taste disturbance was common before and after VS removal, and the natural history of this condition was very variable in the pre- and postoperative periods. All patients who undergo surgery for VS should receive appropriate counseling about the likelihood and course of postoperative complications, including dysfunction of the sensory component of the facial nerve.