• 구강회복응용과학지
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• 제39권4호
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• pp.237-249
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• 2023

구강안면 근긴장이상(Orofacial dystonia)은 자신의 의지와 상관없이 불수의적으로 얼굴, 입술, 혀 및 턱 주변을 불규칙적이거나 반복적으로 움직이는 신경운동학적 장애로 틱장애로 불리기도 한다. 이러한 증상을 갖는 무치악 환자는 총의치 사용의 어려움과 더불어 언어 장애 및 연하곤란, 구강안면의 통증까지 기능적이고 심미적인 문제점들을 겪게 된다. 본 증례에서는 비교적 젊은 나이에 완전 무치악 상태를 경험하게 된 구강안면 근긴장이상 환자를 위해, 양측 구치부 지르코니아 교합면을 갖는 상악 총의치와 하악 임플란트 지지 고정성 보철물의 수복치료를 시행하고 지속적인 미소훈련을 시행하여 안면근육의 심미적인 개선을 도모하고자 하였다. 치료의 결과, 환자는 저작기능과 심미성이 향상된 것 뿐만 아니라, 심리적 안정감을 회복하여 정상적인 일상생활이 가능해진 것에 대해 매우 만족하였기에 이를 보고하고자 한다.

• 대한후두음성언어의학회지
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• 제25권2호
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• pp.79-81
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• 2014

Spasmodic dysphonia (SD) is a chronic, focal, speech-induced, action-specific dystonia, resulting strained voice. Muscle tension dysphonia (MTD) may also result in a strangled, strained voice quality, usually as a result of compensation for underlying laryngeal disease such as glottal insufficiency. Patients with SD and MTD were suffered from the severely limiting people's communication, especially via telephone and in noisy backgrounds. SD is usually of the adductor type characterized by glottic contractions causing tightness and voice breaks, which is difficult to distinguish from MTD. In this review article, we present the characteritics and management of SD and MTD.

• 대한신경과학회지
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• 제36권4호
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• pp.322-324
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• 2018

Oculogyric crisis is an acute dystonia involving ocular muscles characterized by sustained conjugate upward or lateral deviation of the eyes. Metronidazole is a commonly used antimicrobial agent in treatment of anaerobic infections. However, its long-term use can cause toxic encephalopathy particularly in patients with hepatic dysfunction. Here, we describe a case of oculogyric crisis as a presenting manifestation of metronidazole-induced encephalopathy.

• 대한기관식도과학회:학술대회논문집
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• 대한기관식도과학회 2005년도 제44차 추계학술대회
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• pp.20-20
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• 2005

• 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.

• Molecules and Cells
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• 제27권1호
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• pp.89-97
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• 2009

We investigate the molecular and cellular etiologies that underlie the deletion of the six amino acid residues (${\Delta}F323-Y328$; ${\Delta}FY$) in human torsin A (HtorA). The most common and severe mutation involved with early-onset torsion dystonia is a glutamic acid deletion (${\Delta}E$ 302/303; ${\Delta}E$) in HtorA which induces protein aggregates in neurons and cells. Even though ${\Delta}FY$ HtorA forms no protein clusters, flies expressing ${\Delta}FY$ HtorA in neurons or muscles manifested a similar but delayed onset of adult locomotor disability compared with flies expressing ${\Delta}E$ in HtorA. In addition, flies expressing ${\Delta}FY$ HtorA had fewer aberrant ultrastructures at synapses compared with flies expressing ${\Delta}E$ HtorA. Taken together, the ${\Delta}FY$ mutation in HtorA may be responsible for behavioral and anatomical aberrations in Drosophila.

• 동의신경정신과학회지
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• 제19권2호
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• pp.289-297
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• 2008

Paroxysmal Kinesigenic Dyskinesia ( PKD ) is an unusual dyskinesia often precipitated by voluntary movements and characterized by brief episodes of choreic/ dystonic movements. Little is known of the pathophysiology of PKD, although secondary etiologies such as multiple sclerosis, ischemic stroke, birth injury , head trauma, drug abuse, etc. are known to induce the disease. We report a case of a male patient suffering from both legs dystonia for 10 years. He had a past history of drug abuse, and was diagnosed as PKD by a neurologist. We treated him with acupucture and herbal medicine and was able to observe the improvement in certain symtoms and general condition.

• 구강회복응용과학지
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• 제28권2호
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• pp.171-178
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• 2012

보툴리눔 독소 주사는 이마, 눈가 주름치료 등 미용성형분야 뿐만 아니라 구강 악안면 분야에서는 만성 편두통(chronic migraine), 근육긴장이상(dystonia), 경직(spasticity), 측두하악장애(temporomandibular disorders, TMD)의 치료 등에 사용되어 왔다. 최근 보툴리눔 독소 주사가 운동신경에서 마비효과 뿐만 아니라 감각신경에서 말초감작과 신경원성염증과 관련되는 substance P, CGRP, glutamate 등 비콜린성 신경전달물질의 유리를 차단하여 통증신호를 차단하는 역할을 한다는 가설이 제기되고 있다. 따라서 본 연구는 쥐, 토끼 등 동물 신경 손상 모델에 대한 실험과 치과 치료 후 발생한 신경 손상 환자에서 보툴리눔 독소의 진통 효과 및 작용 기전을 살펴보고자 한다.

• 대한치과마취과학회지
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• 제7권2호
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• pp.135-138
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• 2007

Hallervorden-Spatz disease (HSD) is a rare autosomal recessive disorder associated with excessive iron deposition in the basal ganglia. In general, HSD is characterized by onset in first two decade of life and by the presence of extra-pyramidal dysfunction including dystonia, rigidity, choreoathetosis. Other associated features include gait and posture disturbance, intellectual decline, seizure, tremor, dysarthria. These signs and symptoms are progressive. MRI is often demonstrated hypodensity in the basal ganglia which is probably suggestive of accumulation of iron. There is no specific treatment for HSD and 45% of patients die before reaching the age of 20 years. The managements directed at specific symptoms are often helpful. Especially, some surgical procedures like pallidotomy and gastrostomy are performed under general anesthesia. There is special need for careful management because of numerous anesthetic challenges like difficulty in cooperation, life-threatening airway obstruction and possibility of aspiration. We report a successful anesthetic management in a patient with HSD for dental procedures.

• Clinical and Experimental Pediatrics
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• 제45권10호
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• pp.1278-1282
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• 2002

저자들은 경련 발작후 퇴행성 발달 지연과 무도증 양상의 운동을 보이는 13개월 여아를 두부 자기공명 검사와 소변 유기산 검사를 이용하여 국내 최초로 glutaric aciduria type 1 1례로 진단하였기에 보고하는 바이다.