A Case of Trigeminal Neuralgia in an Adult Patient With Lambdoid Synostosis.

Trigeminal neuralgia (TN) is characterized by sudden, brief intense pain in the distribution of the unilateral trigeminal nerve (TGN). Neurovascular compression (NVC) of the TGN is the most common cause of TN. Recent studies have suggested that a structural anomaly of the posterior cranial fossa might be involved in the development of TN, and several studies have documented the association between NVC-related TN and congenital posterior cranial deformities in adults. We present the case of a 56-year-old woman with NVC-related TN and unilateral lambdoid synostosis (ULS), along with a literature review, to investigate the relationship between TN and structural anomalies of the posterior fossa. This is the first report of TN in an adult with ULS. Mild and asymptomatic cases of lambdoid synostosis might have a higher incidence of NVC-related TN in association with posterior cranial fossa deformities.

Effect of deep learning-based reconstruction on high-resolution three-dimensional T2-weighted fast asymmetric spin-echo imaging in the preoperative evaluation of cerebellopontine angle tumors.

PURPOSE: We aimed to evaluate the effect of deep learning-based reconstruction (DLR) on high-spatial-resolution three-dimensional T2-weighted fast asymmetric spin-echo (HR-3D T2-FASE) imaging in the preoperative evaluation of cerebellopontine angle (CPA) tumors. METHODS: This study included 13 consecutive patients who underwent preoperative HR-3D T2-FASE imaging using a 3 T MRI scanner. The reconstruction voxel size of HR-3D T2-FASE imaging was 0.23 × 0.23 × 0.5 mm. The contrast-to-noise ratios (CNRs) of the structures were compared between HR-3D T2-FASE images with and without DLR. The observers' preferences based on four categories on the tumor side on HR-3D T2-FASE images were evaluated. The facial nerve in relation to the tumor on HR-3D T2-FASE images was assessed with reference to intraoperative findings. RESULTS: The mean CNR between the tumor and trigeminal nerve and between the cerebrospinal fluid and trigeminal nerve was significantly higher for DLR images than non-DLR-based images (14.3 ± 8.9 vs. 12.0 ± 7.6, and 66.4 ± 12.0 vs. 53.9 ± 8.5, P < 0.001, respectively). The observer's preference for the depiction and delineation of the tumor, cranial nerves, vessels, and location relation on DLR HR-3D T2FASE images was superior to that on non-DLR HR-3D T2FASE images in 7 (54%), 6 (46%), 6 (46%), and 6 (46%) of 13 cases, respectively. The facial nerves around the tumor on HR-3D T2-FASE images were visualized accurately in five (38%) cases with DLR and in four (31%) without DLR. CONCLUSION: DLR HR-3D T2-FASE imaging is useful for the preoperative assessment of CPA tumors.

Accelerating TOF-MRA: The impact of the combined use of compressed sensitivity encoding and spiral imaging.

PURPOSE: To evaluate the image quality of the combined technique of compressed sensitivity encoding (CS) and spiral imaging in time-of-flight magnetic resonance angiography (TOF-MRA), which is approximately 2.5 times faster than conventional methods. METHODS: Twenty volunteers underwent four TOF-MRA sequences: sensitivity encoding (SENSE) with acceleration factor of 4 (acquisition time: 4:55 min), CS with acceleration factor of 10.9, and spiral and CS-spiral (both 1:55 min). A quantitative image analysis (signal-to-noise ratio [SNR], contrast, and full width at half maximum [FWHM] edge criterion measurements) was performed on four TOF sequences. For qualitative image analysis, two board-certified radiologists evaluated the overall depiction of the proximal, intermediate, and distal branches in CS, spiral, and CS-spiral images using SENSE as a reference. RESULTS: The SNR of BA in spiral and CS-spiral imaging was significantly lower than that in SENSE (p = 0.009). The contrasts of ACA and BA in CS-spiral were significantly higher and those in spiral were significantly lower than those in SENSE (p < 0.001). The FWHM in the CS image was significantly higher than that of SENSE; however, no significant differences were observed between the spiral or CS-spiral and SENSE. In qualitative analysis, the depiction of proximal vascular branches was significantly impaired in spiral than in others and that of distal vascular branches was significantly impaired in CS than in others (p < 0.001). CONCLUSIONS: In TOF-MRA, which is approximately 2.5 times faster than conventional methods, the combined use of CS and spiral imaging demonstrated an improvement in image quality compared to either CS or spiral imaging alone. SUMMARY STATEMENT: The image quality of Compressed SENSE and spiral imaging is particularly poor in the proximal and distal vascular branches, respectively at an extremely high acceleration factor; however, CS-spiral provided stable image quality in all regions as compared with the SENSE technique.

A Consideration of Optimal Head Position in Transsylvian Selective Amygdalohippocampectomy.

Transsylvian selective amygdalohippocampectomy (TSA) is one of the predominant surgical options for drug-resistant mesial temporal lobe epilepsy. The purpose of this article is to highlight the unique features of TSA and determine the setting to perform safe and secure TSA with special reference to the optimal head position. TSA should be performed via a small surgical corridor in the temporal stem that contains functionally important fiber tracts, including the uncinate fasciculus, the inferior fronto-occipital fasciculus, and the optic radiation. Graphical simulations proposed that low-degree (<30°) head rotation had the advantage of sufficiently opening the surgical field in TSA and may help surgical procedures within the limited exposure of the medial temporal structures. Inspection of the surgical videos implied that the collapse of the inferior horn was prevented in low-degree rotation, probably because the deformation due to the brain shift was minimized in the medial temporal structures. A simulation also implied that chin-up position had the advantage of resecting the tail of the hippocampus in a straightforward manner. We suggest that the setting is optimized in TSA with low-degree rotation and chin-up head position.

Importance of Age and Noncontrast-Enhancing Tumor as Biomarkers for Isocitrate Dehydrogenase-Mutant Glioblastoma: A Multicenter Study.

PURPOSE: This study aimed to investigate the most useful clinical and magnetic resonance imaging (MRI) parameters for differentiating isocitrate dehydrogenase (IDH)-mutant and -wildtype glioblastomas in the 2016 World Health Organization Classification of Tumors of the Central Nervous System. METHODS: This multicenter study included 327 patients with IDH-mutant or IDH-wildtype glioblastoma in the 2016 World Health Organization classification who preoperatively underwent MRI. Isocitrate dehydrogenase mutation status was determined by immunohistochemistry, high-resolution melting analysis, and/or IDH1/2 sequencing. Three radiologists independently reviewed the tumor location, tumor contrast enhancement, noncontrast-enhancing tumor (nCET), and peritumoral edema. Two radiologists independently measured the maximum tumor size and mean and minimum apparent diffusion coefficients of the tumor. Univariate and multivariate logistic regression analyses with an odds ratio (OR) were performed. RESULTS: The tumors were IDH-wildtype glioblastoma in 306 cases and IDH-mutant glioblastoma in 21. Interobserver agreement for both qualitative and quantitative evaluations was moderate to excellent. The univariate analyses revealed a significant difference in age, seizure, tumor contrast enhancement, and nCET ( P < 0.05). The multivariate analysis revealed significant difference in age for all 3 readers (reader 1, odds ratio [OR] = 0.960, P = 0.012; reader 2, OR = 0.966, P = 0.048; reader 3, OR = 0.964, P = 0.026) and nCET for 2 readers (reader 1, OR = 3.082, P = 0.080; reader 2, OR = 4.500, P = 0.003; reader 3, OR = 3.078, P = 0.022). CONCLUSIONS: Age and nCET are the most useful parameters among the clinical and MRI parameters for differentiating IDH-mutant and IDH-wildtype glioblastomas.

An epileptogenic intracranial cystic lesion lined with fallopian tube-type epithelium: illustrative case.

BACKGROUND: Intracranial cystic lesions are often a trigger for epileptic seizures. However, there has never been a report of a cystic lesion lined with fallopian tube-type epithelium. OBSERVATIONS: A 48-year-old female presented with a cystic lesion in the right occipital lobe, which gradually grew over 8 years. Right occipital lobe epilepsy was diagnosed based on visual aura, convulsive seizures, and electroencephalogram findings and the cyst was surgically removed. Further examination revealed the cyst was lined with ciliated cells, which had morphological and immunohistochemical features similar to those of fallopian tube epithelium. LESSONS: The characteristics of the cyst did not conform to any known types of benign cystic lesion. To the authors' knowledge, no such cyst has been reported before. The authors discuss the origins and pathogenesis of this unfamiliar cystic lesion.

Whole Tumor Radiomics Analysis for Risk Factors Associated With Rapid Growth of Vestibular Schwannoma in Contrast-Enhanced T1-Weighted Images.

OBJECTIVE: To investigate the features associated with rapid growth of vestibular schwannoma using radiomics analysis on magnetic resonance imaging (MRI) together with clinical factors. METHODS: From August 2005 to February 2019, 67 patients with vestibular schwannoma underwent contrast-enhanced T1-weighted MRI at least twice as part of their diagnosis. After excluding 3 cases with an extremely short follow-up period of 15 days or less, 64 patients were finally enrolled in this study. Ninety-three texture features were extracted from the tumor image data using 3D Slicer software (http://www.slicer.org/). We determined the texture features that significantly affected maximal tumor diameter growth of more than 2 mm/year using Random Forest and Bounty. We also analyzed age and tumor size as clinical factors. We calculated the areas under the curve (AUCs) using receiver operating characteristic analysis for prediction models using texture, clinical, and mixed factors by Random Forest and 5-fold cross-validation. RESULTS: Two texture features, low minimum signal and high inverse difference moment normalized (Idmn), were significantly associated with rapid growth of vestibular schwannoma. The mixed model of texture features and clinical factors offered the highest AUC (0.69), followed by the pure texture (0.67), and pure clinical (0.63) models. The minimum signal was the most important variable followed by tumor size, Idmn, and age. CONCLUSIONS: Our radiomics analysis found that texture features were significantly associated with the rapid growth of vestibular schwannoma in contrast-enhanced T1-weighted images. The mixed model offered a higher diagnostic performance than the pure texture or clinical models.

Variations in the branching patterns of the anterior choroidal artery: an angiographic study with special reference to temporal lobe epilepsy surgery.

BACKGROUND: The preservation of the anterior choroidal artery (AChA) is essential for avoiding neurological sequelae after mesial temporal lobe epilepsy (mTLE) surgery. The purpose of this study is to reveal the anatomical variation in which the perforating branches arise from the plexal segment of the AChA by using a modern neuroimaging modality. METHODS: This study analyzed 3D rotational angiography (3DRA) images from 56 subjects. The AChA and perforating branches were visualized using slab MIP. We analyzed branching patterns, courses of the perforating arteries arising from the plexal segment of the AChA, and the anastomosis of the AChA with other cerebral arteries. RESULTS: The slab MIP applied to 3DRA visualized one or more perforating branches from the AChA in 92.9% of cases. The presence of perforating branches arising from the AChA plexal segment was 17.3%. Most of the branching points of plexal perforators were likely located in the operative field during hippocampal resection. The course of the AChA plexal perforators included the posterior limb of the internal capsule. Anastomosis with other cerebral arteries was visualized in 25% of the AChA with plexal perforators. CONCLUSIONS: 3DRA slab MIP was useful for visualizing the perforating branches of the AChA. Our results showed the possibility that surgical manipulation of the choroid plexus may cause infarction in the AChA territory. We suggest that the existence of the AChA plexal perforators should be recognized to further enhance the safety of hippocampal resection for mTLE.

Macrophage/microglia-derived IL-1ß induces glioblastoma growth via the STAT3/NF-κB pathway.

Glioblastoma is a glioma characterized by highly malignant features. Numerous studies conducted on the relationship between glioblastoma and the microenvironment have indicated the significance of tumor-associated macrophages/microglia (TAMs) in glioblastoma progression. Since interleukin (IL)-1ß secreted by TAMs has been suggested to promote glioblastoma growth, we attempted to elucidate the detailed mechanisms of IL-1ß in glioblastoma growth in this study. A phospho-receptor tyrosine kinase array and RNA-sequencing studies indicated that IL-1ß induced the activation of signal transducer and activator of transcription-3 and nuclear factor-kappa B signaling. Glioblastoma cells stimulated by IL-1ß induced the production of IL-6 and CXCL8, which synergistically promoted glioblastoma growth via signal transducer and activator of transcription-3 and nuclear factor-kappa B signaling. By immunohistochemistry, IL-1ß expression was seen on TAMs, especially in perinecrotic areas. These results suggest that IL-1ß might be a useful target molecule for anti-glioblastoma therapy.

Hemichorea induced by a sphenoid ridge meningioma.

BACKGROUND: Movement disorders are rare in brain tumors. We describe a 45-year-old woman with hemichorea, a concomitant contralateral sphenoid ridge meningioma. CASE DESCRIPTION: The meningioma enlarged as her hemichorea worsened, and after meningioma resection, the hemichorea gradually subsided. N-isopropyl-p-[123I]-iodoamphetamine single-photon emission computed tomography performed preoperatively showed decreased regional cerebral blood flow (CBF) to the basal ganglia circuit ipsilateral to the tumor and, when repeated postoperatively, confirmed improved regional CBF. CONCLUSION: We propose that the enlarging sphenoid ridge meningioma had a remote effect on regional CBF and the thalamocortical motor center and that complex changes in the basal ganglia output may have caused the hemichorea.

Sympathetic hyperactivity, hypertension, and tachycardia induced by stimulation of the ponto-medullary junction in humans.

OBJECTIVE: The purpose of this study is to investigate changes in autonomic activities and systemic circulation generated by surgical manipulation or electrical stimulation to the human brain stem. METHODS: We constructed a system that simultaneously recorded microsurgical field videos and heart rate variability (HRV) that represent autonomic activities. In 20 brain stem surgeries recorded, HRV features and sites of surgical manipulation were analyzed in 19 hypertensive epochs, defined as the periods with transient increases in the blood pressure. We analyzed the period during electrical stimulation to the ponto-medullary junction, performed for the purpose of monitoring a cranial nerve function. RESULTS: In the hypertensive epoch, HRV analysis showed that sympathetic activity predominated over the parasympathetic activity. The hypertensive epoch was more associated with surgical manipulation of the area in the caudal pons or the rostral medulla oblongata compared to controls. During the period of electrical stimulation, there were significant increases in blood pressures and heart rates, accompanied by sympathetic overdrive. CONCLUSIONS: Our results provide physiological evidence that there is an important autonomic center located adjacent to the ponto-medullary junction. SIGNIFICANCE: A large study would reveal a candidate target of neuromodulation for disorders with autonomic imbalances such as drug-resistant hypertension.

A preliminary study of deep learning-based reconstruction specialized for denoising in high-frequency domain: usefulness in high-resolution three-dimensional magnetic resonance cisternography of the cerebellopontine angle.

PURPOSE: Deep learning-based reconstruction (DLR) has been developed to reduce image noise and increase the signal-to-noise ratio (SNR). We aimed to evaluate the efficacy of DLR for high spatial resolution (HR)-MR cisternography. METHODS: This retrospective study included 35 patients who underwent HR-MR cisternography. The images were reconstructed with or without DLR. The SNRs of the CSF and pons, contrast of the CSF and pons, and sharpness of the normal-side trigeminal nerve using full width at half maximum (FWHM) were compared between the two image types. Noise quality, sharpness, artifacts, and overall image quality of these two types of images were qualitatively scored. RESULTS: The SNRs of the CSF and pons were significantly higher with DLR than without DLR (CSF 21.81 ± 7.60 vs. 15.33 ± 4.03, p < 0.001; pons 5.96 ± 1.38 vs. 3.99 ± 0.48, p < 0.001). There were no significant differences in the contrast of the CSF and pons (p = 0.225) and sharpness of the normal-side trigeminal nerve using FWHM (p = 0.185) without and with DLR, respectively. Noise quality and the overall image quality were significantly higher with DLR than without DLR (noise quality 3.95 ± 0.19 vs. 2.53 ± 0.44, p < 0.001; overall image quality 3.97 ± 0.17 vs. 2.97 ± 0.12, p < 0.001). There were no significant differences in sharpness (p = 0.371) and artifacts (p = 1) without and with DLR. CONCLUSION: DLR can improve the image quality of HR-MR cisternography by reducing image noise without sacrificing contrast or sharpness.

The Stabilization of Central Sympathetic Nerve Activation by Renal Denervation Prevents Cerebral Vasospasm after Subarachnoid Hemorrhage in Rats.

The poor prognosis of subarachnoid hemorrhage (SAH) might be associated with sympathetic nerve activation (catecholamine surge) initiated by hypothalamic injury. As renal denervation (RD) has been shown to exert protective effects on cardiovascular dysfunction by suppressing increased central sympathetic nerve activation, we examined whether RD improved the experimental SAH prognosis in this study. Two hundred thirty-eight male Sprague-Dawley rats were divided into sham-operated and SAH-operated groups, and then each rat was further separated into Sham-operated and RD-operated groups. Bilateral RD was performed approximately 45 min after SAH induction. We examined the effect of RD on early brain injury (EBI) and delayed cerebral ischemia (DCI) as a primary endpoint, and also explored the effect on cerebral vasospasm (CVS) as a secondary endpoint. Although RD did not exert significant effects on primary endpoint, RD significantly prevented CVS and reduced SAH-induced increases in the number of phosphorylated extracellular signal-regulated kinase (ERK)-positive endothelial cells, cyclooxygenase-2 expression, and macrophage infiltration in major cerebral arteries. Moreover, RD significantly decreased the areas displaying dopamine ß-hydroxylase and glial fibrillary acidic protein immunopositivity in the paraventricular nucleus of the hypothalamus and serum angiotensin II levels, all of which were increased by SAH. Although RD decreased systolic blood pressure, significant changes in cerebral blood flow were not observed compared with SAH + Sham group. Based on the findings, RD improved CVS by reducing endothelial cell damage and the effects were associated with the stabilization of central sympathetic nerve activation in a SAH model.

Parallel Emergence of a Compartmentalized Striatum with the Phylogenetic Development of the Cerebral Cortex.

The intricate neuronal architecture of the striatum plays a pivotal role in the functioning of the basal ganglia circuits involved in the control of various aspects of motor, cognitive, and emotional functions. Unlike the cerebral cortex, which has a laminar structure, the striatum is primarily composed of two functional subdivisions (i.e., the striosome and matrix compartments) arranged in a mosaic fashion. This review addresses whether striatal compartmentalization is present in non-mammalian vertebrates, in which simple cognitive and behavioral functions are executed by primitive sensori-motor systems. Studies show that neuronal subpopulations that share neurochemical and connective properties with striosomal and matrix neurons are present in the striata of not only anamniotes (fishes and amphibians), but also amniotes (reptiles and birds). However, these neurons do not form clearly segregated compartments in these vertebrates, suggesting that such compartmentalization is unique to mammals. In the ontogeny of the mammalian forebrain, the later-born matrix neurons disperse the early-born striosome neurons into clusters to form the compartments in tandem with the development of striatal afferents from the cortex. We propose that striatal compartmentalization in mammals emerged in parallel with the evolution of the cortex and possibly enhanced complex processing of sensory information and behavioral flexibility phylogenetically.

Is hemifacial spasm affected by changes in the heart rate? A study using heart rate variability analysis.

OBJECTIVE: Hemifacial spasm (HFS) is caused by arterial conflict at the root exit zone of the facial nerve. As the offending artery is pulsatile in nature, this study investigated the association of heart rate fluctuation with HFS. METHODS: Twenty-four preoperative patients underwent simultaneous recordings of facial electromyogram and electrocardiogram overnight. Series of R-wave to R-wave intervals (RRIs) in the electrocardiogram were analyzed across subjects in relation to HFS. The degree of heart rate fluctuation was quantified by analyzing the heart rate variability (HRV). The sleep stage was evaluated during the period of HFS. RESULTS: A 0.1 Hz fluctuation in RRIs by 5% compared to the baseline preceded a few seconds the onset of the HFS, indicating that a significant increase in the heart rate coincided with HFS. HRV analysis demonstrated that fluctuations in the heart rate were significantly enhanced during HFS. Wake or light sleep stages were more often accompanied by HFS, suggesting an association with autonomic activities. CONCLUSION: Our findings suggest that the etiology of HFS is more than just a mechanical compression of the facial nerve and may involve changes in pulsatile frequency in offending arteries. SIGNIFICANCE: We propose the etiology of HFS from a unique standpoint.

Improvement of visual field defects after focal resection for occipital lobe epilepsy: case report.

Improvement of visual field defects after surgical treatment for occipital lobe epilepsy is rare. Here, the authors report on a 24-year-old man with a 15-year history of refractory epilepsy that developed after he had undergone an occipital craniotomy to remove a cerebellar astrocytoma at the age of 4. His seizures started with an elementary visual aura, followed by secondary generalized tonic-clonic convulsion. Perimetry revealed left-sided incomplete hemianopia, and MRI showed an old contusion in the right occipital lobe. After evaluation with ictal video-electroencephalography, electrocorticography, and mapping of the visual cortex with subdural electrodes, the patient underwent resection of the scarred tissue, including the epileptic focus at the occipital lobe. After surgery, he became seizure free and his visual field defect improved gradually. In addition, postoperative 123I-iomazenil (IMZ) SPECT showed partly normalized IMZ uptake in the visual cortex. This case is a practical example suggesting that neurological deficits attributable to the functional deficit zone can be remedied by successful focal resection.

Pregabalin as a salvage preoperative treatment for refractory trigeminal neuralgia.

Only few studies have investigated the effectiveness of pregabalin (PGB) treatment for trigeminal neuralgia (TN). We aimed to retrospectively analyze the effectiveness of PGB treatment in refractory TN as a salvage treatment preceding surgery. Of 61 patients with TN refractory to prior treatment with carbamazepine (CBZ), we enrolled 33 patients in our study who agreed to receive PGB before they underwent surgery. The patients were divided into effective and ineffective groups depending on the patient-reported outcome. Correlations between effectiveness and clinical characteristics such as the age, sex, disease duration, initial CBZ responsiveness, the number of patients who underwent polytherapy with PGB and CBZ, final doses of CBZ and PGB at the time of evaluation, and the etiology of the neurovascular compression were statistically analyzed. Furthermore, a linear discriminant analysis was performed to predict effectiveness. TN was improved in 16 patients (48.5%) in the PGB-treatment group but none in patients without PGB-treatment. The final dose of PGB was 166.7 mg at the mean follow-up period of 5.5 months. Our results showed that age was the only factor that significantly differed between PGB-effective and ineffective groups. A logistic regression analysis also demonstrated that among all the clinical variables considered, only older age was significantly associated with effectiveness of PGB treatment. Effectiveness was correctly predicted at a threshold value of 62.7-years-old with 69.7% reliability. We suggest that PGB is useful, even at the low-dose, as a salvage preoperative treatment for patients with refractory TN, particularly for elderly patients.

Flatness of the infratentorial space associated with hemifacial spasm.

BACKGROUND: Whether a difference in morphology of the infratentorial space is associated with hemifacial spasm is not well understood. The aim of this study was to analyze the three-dimensional conformation of the infratentorial space and evaluate any possible contribution of morphological characteristics to the development of neurovascular compression leading to hemifacial spasm. METHODS: We enrolled 25 patients with hemifacial spasm and matched them by age and sex to controls. The extent of the three-dimensional axes and the volume of the infratentorial space were measured using image analysis software for three-dimensional MRI. We evaluated the correlation between a morphological difference in the infratentorial space and changes in vascular configuration in the brain stem. RESULTS: We found no statistical difference in volumetric analyses. The mean aspect ratio on the coronal plane (the ratio of the Z to X extent) of the infratentorial space in patients with hemifacial spasm was significantly lower (p < 0.01) than that in controls, as was the mean aspect ratio on the sagittal plane (the ratio of Z to Y extent, p < 0.01). A smaller sagittal aspect ratio was correlated (p < 0.05) with greater lateral deviation of the basilar artery. CONCLUSIONS: Our results suggest that flatness of the superior-inferior dimension of the infratentorial space is an anatomical feature that characterizes patients with hemifacial spasm. We hypothesize that this unique structural variation may exaggerate the lateral deviation of the vertebrobasilar arteries due to arteriosclerosis and exacerbate the space competition among vessels and cranial nerves.

Surgical management of Eagle's syndrome: an approach to shooting craniofacial pain.

Eagle's syndrome (ES) and glossopharyngeal neuralgia (GPN) display very similar symptoms preoperatively. The objective of this study is to determine the surgical outcome of intraoral resection of the styloid process (IRSP) for ES, and to observe preoperative findings and treatment outcome of our cases presenting shooting craniofacial pain. In total, 14 symptomatic patients who presented with typical shooting craniofacial pain, had a styloid process longer than 25 mm, and underwent surgical intervention or medication alone from 2011 to 2015 were involved. They were divided into two groups: Group I included eight patients who underwent surgery following 3 months of medication failure, and Group II included six patients who received medication alone. Preoperative physical, radiographic findings and surgical outcomes were examined. In Group I patients, six cases received IRSP and five of those six cases experienced complete relief from symptoms and were confirmed as ES. Two other cases in Group I received microvascular decompression. One showed complete relief from symptoms, and was confirmed as GPN. The other case showed recurrence 1 year postoperatively, received IRSP with complete relief from symptoms, and was confirmed as ES. In Group II, three cases experienced complete relief from symptoms with 3 months of medication alone. IRSP is an effective treatment for ES. There was no clear difference in the preoperative findings for ES and GPN, suggesting the difficulty in making a preoperative differential diagnosis between the two conditions. Close cooperation between ENT and neurosurgery surgeons is needed.

Pallidal stimulation for medically intractable blepharospasm.

Deep brain stimulation (DBS) of the globus pallidus internus (GPi) is an established procedure to improve generalised, segmental craniofacial dystonia. However, no studies have addressed the applicability of DBS for blepharospasm as a focal craniofacial dystonia. A 52-year-old man developed medically intractable involuntary eye closure. Because the abnormal movement was observed exclusively in the eyelids, he was diagnosed not with Meige's syndrome but with blepharospasm as a focal craniofacial dystonia. He underwent stereotactic surgery under general anaesthesia for bilateral GPi-DBS. Continuous GPi stimulation almost completely abolished the blepharospasm. 15 months after the operation, his preoperative scores on the Burke-Fahn-Marsden Dystonia Rating Scale (=8 points) decreased to 1 (87.5% improvement). The present study demonstrates the applicability of GPi-DBS for treating blepharospasm presenting as focal dystonia. Further studies with accumulated case series are needed to confirm the effect of DBS on blepharospasm and other focal craniofacial dystonias.