• Journal of Korean Neurosurgical Society
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• 제57권2호
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• pp.143-146
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• 2015

Chorea-acanthocytosis (ChAc) is a rare hereditary disorder characterized by involuntary choreiform movements and erythrocytic acanthocytosis. Pharmacotherapy for control of involuntary movements has generally been of limited benefit. Deep brain stimulation (DBS) has recently been used for treatment of some refractory cases of ChAc. We report here on the effect of bilateral high-frequency DBS of globus pallidus interna in a patient with ChAc.

• Journal of Korean Neurosurgical Society
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• 제52권3호
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• pp.261-263
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• 2012

The experience of pediatric deep brain stimulation (DBS) of the globus pallidus internus (GPi) in the treatment of early-onset DYT1 generalized dystonia is still limited. Here, we report the surgical experience of bilateral GPi-DBS under general anesthesia by using microelectrode recording in a 7-year-old girl with early-onset DYT1 generalized dystonia. Excellent improvement of her dystonia without neurological complications was achieved. This case report demonstrates that GPi-DBS is an effective and safe method for the treatment of medically refractory early-onset DYT1 generalized dystonia in children.

• 대한핵의학회지
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• 제22권2호
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• pp.163-170
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• 1988

8 patients of delayed CO sequelae were evaluated using Brain CT and $^{99m}Tc-HMPAO$ SPECT. The results were as follows; 1) CT findings of delayed CO sequleae were bilateral low density lesion in globus pallidus (l pt.), diffuse low density in white matter with bilateral low density in white matter (l pt.), diffuse low density in white matter with bilateral low density in globus pallidus (l pt.), diffuse low density in white matter with cortical atrophy (l pt.), bilateral low density in globus pallidus and diffuse low density in white matter with cortical atrophy (l pt.) and normal in 3 pts. 2) $^{99m}Tc-HMPAO$ Brain SPECT findings of delayed CO sequelae were decreased regional cerebral blood flow (rCBF) in frontal (1 among 8 pts.), frontal and basal ganglia (3 among 8 pts.), and diffuse patch decreased rCBF pattern (4 among 8 pts.) 3) $^{99m}Tc-HMPAO$ Brain SPECT study was well correlated with neurologic symptoms and signs in delayed CO sequelae. Our results may suggest that reduced cerebral blood flow contributes to the development of delayed CO sequelae.

• The Korean Journal of Physiology and Pharmacology
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• 제17권4호
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• pp.299-306
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• 2013

Deep brain stimulation (DBS) of the subthalamic nucleus (STN) has been widely used as a treatment for the movement disturbances caused by Parkinson's disease (PD). Despite successful application of DBS, its mechanism of therapeutic effect is not clearly understood. Because PD results from the degeneration of dopamine neurons that affect the basal ganglia (BG) network, investigation of neuronal responses of BG neurons during STN DBS can provide informative insights for the understanding of the mechanism of therapeutic effect. However, it is difficult to observe neuronal activity during DBS because of large stimulation artifacts. Here, we report the observation of neuronal activities of the globus pallidus (GP) in normal and PD model rats during electrical stimulation of the STN. A custom artifact removal technique was devised to enable monitoring of neural activity during stimulation. We investigated how GP neurons responded to STN stimulation at various stimulation frequencies (10, 50, 90 and 130 Hz). It was observed that activities of GP neurons were modulated by stimulation frequency of the STN and significantly inhibited by high frequency stimulation above 50 Hz. These findings suggest that GP neuronal activity is effectively modulated by STN stimulation and strongly dependent on the frequency of stimulation.

• 한국동물학회지
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• 제26권2호
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• pp.107-123
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• 1983

The globus pallidus of normal cats were prepared for electron microscopic study following perfusion with a mixture of 1% paraformaldehyde and 1% glutaraldehyde solution. Neurons of two size categories were identified in 1 $\\mu$m araldite sections and their ultrastructural characteristics were studied in adjacent thin section. 1. Large neurons ($30 \\mum \\times 45 \\mum$ in diameter) had extensive areas of rough surfaced endoplasmic reticulm, abundant perinuclear Golgi complex, numerous mitochondria and lipofusin granule, and had a large spherical nucleus with shallow indentation of nuclear manbrane. Small neurons ($17 \\mum \\times 27 \\mum$ in diameter) had poorly rough surfaced endoplasmic reticulum, moderate number of mitochondria and randomly distributed Golgi complex. The nuclear envelope of this cell frequently showed multiple deep invagination. 2. Three types of axo-somatic synapses were identified on the basis of the size and shape of vesicle in the axon terminal and the symmetrical or asymmetrical thickening at the synaptic site. Type I synaptic terminal shows an even distribution of round and oval synaptic vesicles, and has a symmetrical synaptic thickening. Type II axon terminals reveal mostly round and pleomorphic vesicles and a few vesicles were localized near the presynaptic membrane in pale axoplasm and its synaptic thickening were symmetric. Type III axon terminals contain round vesicles, which were aggregated in the axoplasm, and has a asymmetrical synaptic thickening. 3. The majority of axo-somatic contact with the large and small neurons were type I, and type II and III synapes were rare.

• 대한수의학회지
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• 제36권1호
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• pp.39-49
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• 1996

야생 설치류인 청서 뇌의 줄무늬체에서 neuropeptide Y(NPY) 면역반응신경세포의 분포상태를 관찰하기 위하여 뇌를 4% paraformaldehyde로 관류고정한 전뇌를 $40{\mu}m$의 냉동연속관상절편으로 작성한 후 cresyl violet로 염색하여 전뇌의 각 부분을 구분하고 NPY항체에 대한 면역염색을 실시하였다. 줄무늬체는 속섬유막(internal capsule)보다 앞쪽에서는 꼬리핵(caudate nucleus)과 조가비핵(putamen)이 꼬리-조가 비핵(caudate-putamen)의 한부분으로 구성되어 있는 반면, 속섬유막이 관통하는 부분에서는 꼬리핵, 조가비핵 및 창백핵(globus pallidus)의 세부분으로 구분되었다. 줄무늬체의 NPY면역반응신경세포는 대부분이 중간크기($12-18{\mu}m$)의 세포였다. NPY세포체의 분포는 꼬리-조가비핵과 꼬리핵의 경우에 내측 및 배쪽부분에서 다수 존재하였으며, 조가비핵의 경우는 전체적으로 불규칙하였는데 그중 중심부분이 더욱 적은 수로 관찰되었다. NPY면역반응신경섬유의 밀집도는 전체적으로 낮았으나 반응신경세포체가 많은 부분에서 높은 밀집도를 보였다. 꼬리-조가비핵과 꼬리핵의 경우 내측과 배쪽부분에서 높은 밀집도를 나타내었고 조가비핵의 경우는 불규칙한 밀집도를 보였다. 창백핵에서는 매우 적은 수의 반응섬유들만 관찰되었다. 따라서 청서의 줄무늬체는 꼬리핵, 조가비핵 및 창백핵으로 구분되어 비교적 발달된 구조를 가졌으며, NPY함유 신경세포의 분포형태도 이질성을 나타내는 것으로 보아 줄무늬체의 신경환로가 복잡함을 암시하였다.

• Korean Journal of Radiology
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• 제23권7호
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• pp.742-751
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• 2022

Objective: To assess focal mineral deposition in the globus pallidus (GP) by CT and quantitative susceptibility mapping (QSM) of MRI scans and evaluate its clinical significance, particularly cerebrovascular degeneration. Materials and Methods: This study included 105 patients (66.1 ± 13.7 years; 40 male and 65 female) who underwent both CT and MRI with available QSM data between January 2017 and December 2019. The presence of focal mineral deposition in the GP on QSM (GP_QSM) and CT (GP_CT) was assessed visually using a three-point scale. Cerebrovascular risk factors and small vessel disease (SVD) imaging markers were also assessed. The clinical and radiological findings were compared between the different grades of GP_QSM and GP_CT. The relationship between GP grades and cerebrovascular risk factors and SVD imaging markers was assessed using univariable and multivariable linear regression analyses. Results: GP_CT and GP_QSM were significantly associated (p < 0.001) but were not identical. Higher GP_CT and GP_QSM grades showed smaller gray matter (p = 0.030 and p = 0.025, respectively) and white matter (p = 0.013 and p = 0.019, respectively) volumes, as well as larger GP volumes (p < 0.001 for both). Among SVD markers, white matter hyperintensity was significantly associated with GP_CT (p = 0.006) and brain atrophy was significantly associated with GP_QSM (p = 0.032) in at univariable analysis. In multivariable analysis, the normalized volume of the GP was independently positively associated with GP_CT (p < 0.001) and GP_QSM (p = 0.002), while the normalized volume of the GM was independently negatively associated with GP_CT (p = 0.040) and GP_QSM (p = 0.035). Conclusion: Focal mineral deposition in the GP on CT and QSM might be a potential imaging marker of cerebral vascular degeneration. Both were associated with increased GP volume.

• Investigative Magnetic Resonance Imaging
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• 제21권3호
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• pp.125-130
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• 2017

Purpose: To evaluate the clinical significance of T1 high signal intensity on the globus pallidus as a predictor of severe hepatic encephalopathy in patients with acute-on-chronic liver failure (ACLF), which is a distinct syndrome characterized by multi-organ dysfunction including cerebral failure. Materials and Methods: From January 2002 to April 2014, we retrospectively reviewed the magnetic resonance imaging (MRI) findings and clinical and magnetic resonance (MR) features of 74 consecutive patients (44 men and 30 women; mean age, 59.5 years) with liver cirrhosis. The chronic liver failure-sequential organ failure assessment score was used to diagnose ACLF. The pallidal index (PI), calculated by dividing the mean signal intensity of the globus pallidus by that of the subcortical frontal white matter were compared according to ACLF. The PI was compared with the Model for End-Stage Liver Disease (MELD) score in predicting the development of ACLF. Results: Fifteen patients who were diagnosed with ACLF had higher hepatic encephalopathy grades (initial, P = 0.024; follow-up, P = 0.002), MELD scores (P < 0.001), and PI (P = 0.048). In the ACLF group, the mean PI in patients with cerebral failure was significantly higher than that in the patients without cerebral failure (1.33 vs. 1.20, P = 0.039). In patients with ACLF, the area under the curve (AUC) for PI was 0.680 (95% confidence intervals [CI], 0.52-0.85), which was significantly lower than that for the MELD score (AUC, 0.88; 95% CI, 0.77-0.99) (P = 0.04). Conclusion: The PI can be an ancillary biomarker for predicting the development of ACLF and severe hepatic encephalopathy.

• Journal of Korean Neurosurgical Society
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• 제48권2호
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• pp.134-138
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• 2010

Objective : Meige syndrome is the combination of blepharospasm and oromandibular dystonia. We assessed the surgical results of bilateral globus pallidus internus (GPi) deep brain stimulation (DBS) in patients with medically refractory Meige syndrome. Methods : Eleven patients were retrospectively analyzed with follow-ups of more than 12 months. The mean follow-up period was $23.1{\pm}6.4$ months. The mean age at time of surgery was $58.0{\pm}7.8$ years. The mean duration of symptoms was $8.7 {\pm}7.6$ years. DBS electrodes were placed under local anesthesia using microelectrode recording and stimulation. After $2.4{\pm}1.3$ days of trial tests, the stimulation device was implanted under general anesthesia. Patients were evaluated using the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS). Results : BFMDRS total movement scores improved by 59.8%, 63.5%, 74.1%, 74.5%, and 85.5% during the immediate postoperative period of test stimulation, 3, 6, 12, and 24 months (n = 5) after surgery, respectively. The BFMDRS total movement scores were reduced gradually and the results reached statistical significance in the postoperative period (test period, p < 0.001; 3 months, p < 0.001; 6 months, p = 0.003; 12 months, p < 0.001; 24 months, p = 0.042). There was no statistical difference between 12 months and 24 months. BFM subscores improved by 63.3% for the eyes, 80.9% for the mouth, 68.4% for speech/swallowing, and 87.9% for the neck at 12 months after surgery. The adverse effects were insignificant. Conclusion : The bilateral GPi-DBS can be effective for the treatment of intractable Meige syndrome without significant side effects.

• Journal of Korean Neurosurgical Society
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• 제46권4호
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• pp.346-350
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• 2009

Objective : The purpose of this study was to analyze in detail the relationship between outcome and time course of effect in medically refractory primary cervical dystonia (CD) with phasic type that was treated by bilateral globus pallidus internus (Gpi) deep brain stimulation (DBS). Methods : Six patients underwent bilateral implantation of DBS into the Gpi under the guide of microelectrode recording and were followed for $18.7{\pm}11.1$ months. The mean duration of the CD was $5.8{\pm}3.4$ years. The mean age at time of surgery was $54.2{\pm}10.2$ years. Patients were evaluated with the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) and relief scale using patient self-reporting. Results : The TWSTRS total scores improved by 64.5%, 65.5%, 75.8%, and 76.0% at 3, 6, 12 months, and at the last available follow-up after surgery, respectively. Statistically significant improvements in the TWSTRS scores were observed 3 months after surgery (p=0.028) with gradual improvement up to 12 months after surgery, thereafter, the improvement was sustained. However, there was no statistically significant difference between the scores at 3 and 12 months. Subjective improvement reported averaged $81.7{\pm}6.8%$ at last follow-up. Mild dysarthria, the most frequent adverse event, occurred in 3 patients. Conclusions : Our results show that the bilateral Gpi-DBS can offer a significant therapeutic effect from 3 months postoperatively in patients with primary CD with phasic type, without significant side effects.