• Journal of Korean Neurosurgical Society
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• v.57 no.2
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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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• v.52 no.3
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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.

• The Korean Journal of Nuclear Medicine
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• v.22 no.2
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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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• v.17 no.4
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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.

• The Korean Journal of Zoology
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• v.26 no.2
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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.

• Korean Journal of Veterinary Research
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• v.36 no.1
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• pp.39-49
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• 1996

The present study was performed to investigate the distribution of neuropeptide Y immunoreactivities in the corpus striatum of the Korean squirrels. The animals were perfused with 4%-paraformaldehyde and the brain was cut serially into $40{\mu}m$ thick coronal sections. Sections either were stained with cresyl violet or were stained immunohistochemically. The corpus striatum was divided into the caudate nucleus, putamen and globus pallidus. Anterior part. however, of the striatum was observed as the combined caudate-putamen. NPY immunoreactive (NPY-IR) neurons were medium-sized. The corpus striatum contained a low level of NPY-IR fibers, whose distribution appeared to be related to the immunoreactive perikarya. Large numbers of NPY-IR neurons in the caudate-putamen and caudate nucleus were expressed in medial and ventral parts. In the anterior part of the putamen NPY-IR neurons were scattered throughout the nucleus; in posterior part were found generally in the lateral and ventral parts. The density of NPY-IR fibers of the putamen were low, whose distribution appeared to be related to the perikarya. The globus pallidus contained NPY-IR fibers only in the lowest density. In brief, NPY-immunoreactivities in the corpus striatum are heterogenous in distribution. These findings may reflect innate characteristics of the specific neural circuit in the corpus striatum itself.

• Investigative Magnetic Resonance Imaging
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• v.21 no.3
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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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• v.48 no.2
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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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• v.46 no.4
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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.

• Journal of the Korean Magnetic Resonance Society
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• v.7 no.1
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• pp.1-15
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• 2003

PURPOSE - To investigate neurometabolism from the brain destructive lesions and striatal putamen-pallidus regions to the clinically worst side in patients with Parkinson's disease after stereotactic functional neurosurgery. METHODS - Using proton magnetic resonance spectroscopy ($^1$H MRS), fifteen patients (7 males and 8 females; mean age 56.5 years; age range 43-67 years) with Parkinson's disease (PD) were studied to measure N-acetylaspartate (NAA), creatine (Cr), choline-containing compounds (Cho) and lactate (Lac) levels on the neurosurgical lesions of thalamus, globus pallidus and striatal putamen-pallidus regions in a brain. RESULTS - Brain destructive lesion and striatal putamen-pallidus region in PD compared with controls were highly and significantly related to NAA/Cho ratios reduction, respectively (P =0.002, P =0.04), but showed no difference from the same regions of PD prior to neurosergery (P =0.06, P =0.77). Increased lactate peaks at 1.3 ppm were present in all the cerebral lesions, and these resonances were confirmed at a long TE =136 ms, indicating that these signals distinguished from lipids. CONCLUSIONS - Our results suggest that NAA/Cho ratios may provide as a neurometabolite marker for neurochemical changes in brain surgical lesion, and the ratios might be related to functional change of neuropathophysiological status in the striatal putamen-pallidus region of PD. Increase of lactate signals, being remarkable in surgical lesions, could be consistent with a common consequence of surgical necrosis. Therefore, MR spectroscopy could be a sensitive diagnostic tool in monitoring neurometabolic changes in PD with neurosurgical treatment.