• 한국응용약물학회:학술대회논문집
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• 한국응용약물학회 1993년도 제2회 신약개발 연구발표회 초록집
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• pp.165-165
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• 1993

목적:지금까지 Risperidone의 치료 효능과 안전성에 대한 연구들이 모두 만성 정신분열병 환자들만을 대상으로 이루어졌고, 효율적인 약물 요법의 중요한 지침이 되는 유효치료용량(effective therapeutic dosage)에 대한 연구가 이루어지지 않고 있는 바, 본 연구에서는 초발인 급성 정신분열병 환자들을 대상으로 첫째, Risperidone의 항정신병 효과(antipsychotic effect)와 추체외로계증상(extrapyramidal symptoms)을 평가하고 둘째, Risperidone의 항정신병 효과와 혈장 Risperidone 및 9-hydroxyrisperidone 농도와의 상관관계를 분석하여 유효혈장농도(therapeutic dose range)와 유효치료용량(effective therapeutic dosage)의 존재 여부를 검토코자 한다. 방법: DSM-III-R 진단기준(APA 1987)예 의해 정신분열병형 장애로 진단된 환자 11명(남자 5, 여자 6)을 대상으로 Risperidone을 이중맹검법(double blind design)으로 6주간 투약하였다. 정신병리의 평가는 PANSS(Kay 1988)를 이용하였고 추체외로계 부작용의 평가는 ESRS(Chouinard 1980)를 이용하였다. 혈장 Risperidone 농도와 혈장 9-hydroxyrisperidone 농도는 Jansen사의 Radioimmunoassay Kit를 이용하여 3회 측정하였다. 결과: PANSS 점수는 Risperidone 투여 후 1주째부터 통계적으로 유의하게 감소하였다. Parkinsonism과 dyskinesa에 대한 physician's examination 점수는 전체 연구 기간 동안 유의한 변화를 보이지 않았다. Dystonia에 대한 physician's examination 점수는 Risperidone 투여 후 1주째에는 평균 5.96점으로 높았으나 2주째부터 통계적으로 유의하게 감소되어 6주째에는 유지되었다. 혈장 Risperidone 농도와 혈장 9-hydroxyrisperidone 농도는 PANSS 점수와 유의한 상관관계를 보이지 않았다.

• 한국전문물리치료학회지
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• 제9권4호
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• pp.13-35
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• 2002

Lesch-Nyhan 증후근(LNS)은 hypoxanthine guanine phosphoribosyle transferase(HGPRT) 효소를 암호화 하는 X 염색체가 불완전해서 일어나는 유전적인 추제외로계(또는 기저핵)의 드문 병변이다. 출생시 LNS 유아는 정상적인 운동발달이 관찰되어진다. LNS에게서 현저하게 진단적인 특징으로 보여지는 운동심리적 행동인 self-mutilating 행위는 4살 이후에나 나타난다. LNS 아이들은 오히려 초기에 Rett's 증후근, 뇌성마비, 자폐, 다운증후근과 유사한 운동행위를 보인다. 그래서 LNS 아이들은 앞에 기술한 신경학적 장애로 오진을 받을 수가 있다. 오진으로 인해 초기에 적절한 치료를 받지 못한다면 LNS는 결과적으로 합병증(신장부전)과 self-mutilating 행위로 인하여 치명적일 수가 있다. 그러므로, 이 연구의 목적은 LNS 평가 동안 더 나은 진단을 하도록 하기 위하여 LNS와 관련된 기능부전에 대한 지식을 임상가들에게 제공하고자 함이었다. 연구 대상자는 10살인 2명의 쌍둥이 남아이었으며 실험은 뻗기 과제 수행(reaching task)시 움직임 특성을 보기 위하여 운동형상학적과 비디오 분석을 사용하였다. 기술통계로 분석 결과 움직임 시간과 단위가 증가됨을 보였고 사지의 분절적 움직임이 협응되지 않음을 보였다. ballistic과 jerky 움직임 양상은 dysmetric과 비긴장성 운동 행위에서 우세하였다. LNS은 추체로계 운동 장애 (과근긴장도나 저긴장도) 와 추체외로계의 운동 장애(dystonia와 choreoathetosis)의 혼합된 형태를 보였다. 결론으로 이 연구는 운동발달 장애를 가진 아이들을 치료하고자 할 때 임상가들한테 LNS 아이들의 움직임 장애의 다른 진단적 특징을 알아야 한다는 것을 제시하고자 한다.

• 생물정신의학
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• 제3권1호
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• pp.30-36
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• 1996

Tardive dyskinesia is a syndrome of involuntary hyperkinetic abnormal movements that occurs during or shortly after the cessation of neuroleptic drug treatment. Typically, the movements are choreoatheoid. Other movements such as tics and dystonia may be present. Nonetheless, any dyskinesia seen in a neuroleptic-treated patient is not always neuroleptic-induced tardive dyskinesia. The prevalence of tardive dyskinesia varies widely, which reflects many methodological problems, such as differential diagnosis. symptom fluctuation, masking effect of neuroleptics, validated diagnostic criteria. Of suggested risk factors, only old age has been consistently found to be associated with an increased frequency of tardive dyskinesia. Many hypotheses about the pathophysiolgy of tardive kinesia are proposeed, but time-honored ones are not present. No consistently safe and effective treatments are found. Various treatment modalities signifies the general ineffectiveness of these agents for most patients. In general, reduction or cessation of neuroleptics, if possible, is recommended. Remission or improvemets of tardive dyskinesia after neuroleptics withdrawal usually occurs among most patients within three months.

• 대한후두음성언어의학회지
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• 제32권1호
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• pp.9-14
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• 2021

Spasmodic dysphonia, essential tremor, and vocal tremor related with Parkinson's disease are different disorders showing fairly similar symptoms such as difficulty in the speech onset, and tremble in the voice. However, the cause and the resulting treatment of these diseases are different. Spasmodic dysphonia is a vocal disorder characterized by spasms of the laryngeal muscles during a speech, invoking broken, tense, forced, and strangled voice patterns. Such difficult-to-treat dysphonia disease is classified as central-origin-focal dystonia, of a yet unknown etiology. Its symptoms arise because of intermittent and involuntary muscle contractions during speech. Essential tremor, on the other hand, is characterized by a rhythmic laryngeal movement, resulting in alterations of rhythmic pitch and loudness during speech or even at rest. Severe cases of tremor may cause speech breaks like those of adductor spasmodic dysphonia. In the case of hyper-functional tension of vocal folds and accompanying tremors, it is necessary to distinguish these disorders from muscular dysfunction. A diversified assessment through the performance of specific speech tasks and a thorough understanding for the identification of the disorder is necessary for accurate diagnosis and effective treatment of patients with vocal tremors.

• Journal of Korean Neurosurgical Society
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• 제66권2호
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• pp.121-132
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• 2023

Objective : The parafascicular nucleus (PF) plays important roles in controlling the basal ganglia. It is not well known whether the PF affects the development of abnormal involuntary movements (AIMs). This study was aimed to find a role of the PF in development of AIMs using optogenetic methods in an animal model. Methods : Fourteen rats were underwent stereotactic operation, in which they were injected with an adeno-associated virus with channelrhodopsin (AAV2-hSyn-ChR2-mCherry) to the lateral one third of the PF. Behavior test was performed with and without optical stimulation 14 days after the injection of the virus. AIM of rat was examined using AIM score. After the behavior test, rat's brain was carefully extracted and the section was examined using a fluorescence microscope to confirm transfection of the PF. Results : Of the 14 rats, seven rats displayed evident involuntary abnormal movements. AIM scores were increased significantly after the stimulation compared to those at baseline. In rats with AIMs, mCherry expression was prominent in the PF, while the rats without AIM lacked with the mCherry expression. Conclusion : AIMs could be reversibly induced by stimulating the PF through an optogenetic method.

• 대한후두음성언어의학회지
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• 제23권2호
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• pp.111-118
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• 2012

Botulinum toxin is a potent neurotoxin that is produced by the bacterium Clostridium botulinum. The agent causes muscle paralysis by preventing the release of acetylcholine at the neuromuscular junction of striated muscle. Botulinum toxin A (Botox, AllerganInc., Irvine, California) is the most potent of seven distinct toxin subtypes that are produced by the bacterium. The toxin was initially used clinically in the treatment of strabismus caused by hypertonicity of the extraocular muscles and was sub-sequently described in the treatment of multiple disorders of muscular spasticity and dystonia. In treating patients with Botox for blepharospasm, Carruthers and Carruthers [5] noticed an improvement in glabellar rhytids. This ultimately led to the introduction and development of Botox as a mainstay in the treatment of hyperfunctional facial lines in the upper face. Since its approval by the U.S. Food and Drug Administration for the treatment of facial rhytids (2002), botulinum toxin A has expanded into wide-spread clinical use. Forehead, glabellar, and periocular rhytids are the most frequently treated facial regions. Indications for alternative uses for Botox in facial plastic and reconstructive surgery are expanding. These include a variety of well-established procedures that use Botox as an adjunctive agent to enhance results. In addition, Botox injection is finding increased usefulness as an independent modality for facial rejuvenation and rehabilitation. The agent is used beyond its role in facial rhytids as an effective agent in the management of dynamic disorders of the face and neck. Botox injection allows the physician to precisely manipulate the balance between complex and conflicting muscular interactions, thus resetting their equilibrium state and exerting a clinical effect. This article will address some of the new and unique indications on Botox injection in the face (the lower face and neck, combination with fillers). Important points in terms of its clinical relevance will be stressed, such as an understanding of functional facial anatomy, the importance of precise injections, and correct dosing all are critical to obtaining natural outcomes.

• Journal of Korean Neurosurgical Society
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• 제38권5호
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• pp.359-365
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• 2005

Objective : The study investigates the extent of brain shift and its effect on the accuracy of the stereotaxic procedure. Methods : Thirty-five patients underwent 40stereotactic procedures between June 2002 and March 2004. There were 26 males, mean age 59years old. There were 34procedures for Parkinson's disease, 2 for essential tremor, 3 for cerebral palsy, 1 for dystonia. Patients were divided in four groups based on postoperative pneumocephalus : under 5cc [9 procedures], between $5{\sim}10cc$ [13procedures], between $10{\sim}15cc$ [11 procedures] and more than 15cc [7procedures]. The coordinates of the anterior commissure[AC], posterior commissure[PC], and target were defined in pre-and intraoperative magnetic resonance image scans and the amount of air volume was measured with @Target (BrainLab, Heimstetten, Germany]. Results : The mean AC-PC was 26.5mm for patients with less than 5cc, 26.9mm for $5{\sim}10cc$, 25.8mm for $10{\sim}15cc$ and 26.2mm for more than 15cc. The length of AC-PC line and coordinates of AC, PC was also not statistically different, Euclidean distance as well as ${\Delta}x$, ${\Delta}y$, ${\Delta}z$ of AC, PC, and target were also not statistically different among the groups [p>,1]. There was a variance in target of $0.7{\sim}7.6mm$, Euclidean distance of 2.5mm, related to electrophysiology but not to brain-shift. Conclusion : The amount of air accumulated in the intracranial space and compressing the cortical surface has no effect on the localization of subcortical stereotactic target and landmarks.

• Journal of the Korean Academy of Child and Adolescent Psychiatry
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• 제24권2호
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• pp.90-95
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• 2013

Botulinum toxin, a neurotoxin, is known to be an inhibitor of cholinergic neuromuscular transmission. Recently, it was reported that the administration of botulinum toxin is effective for the treatment of focal neurological motor disorders such as cervical dystonia, blepharospasm, hemifacial spasm, spasmodic dysphonia, and writer's cramp. Several case studies reported that the botulinum toxin was administered for the treatment of motor tic or vocal tic. It was found that this toxin reduces the frequency and severity of the tic as well as the premonitory urge and symptoms. In our case study, a noticeable decrease of motor tic symptom was observed after an intramuscular injection of 300mg of botulinum toxin in an 18-year-old patient with Tourette's disorder who showed only a little improvement of motor tic and vocal tic symptoms after treatment with antipsychotic drugs for several years. This case is reported in our study and literature survey was undertaken for reviewing similar cases. In our study, an 18-year-old boy diagnosed with Tourette's disorder based on Diagnostic and Statistical Manual of Mental Disorders, fourth edition presented with the following scores : the Clinical Global Impression scale, Yale Global Tic Severity Scale (motor/vocal/severity), Premonitory Urge Score, Korean Attention-Deficit Hyperactivity Disorder Rating scale, and Kovac Depression scale which were performed prior to the treatment were 5, 21/5/50, 100, 17, and 18 points, respectively. Two weeks after the injection of botulinum toxin, the scores were 4, 17/5/40, 50, 16, and 19 points, respectively. Eight weeks after the injection of botulinum toxin, they had become 3, 15/5/30, 25, 16, and 20 points, respectively, which clearly indicates a noticeable decrease of motor tic symptom.

• Journal of Yeungnam Medical Science
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• 제30권1호
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• pp.25-30
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• 2013

Hyponatremia, the most common electrolyte disorder, has been rarely reported as causing rhabdomyolysis. Osmotic demyelination syndrome (ODS), a demyelinating disease of the central pons and/or other areas of the brain, is infrequently reported as associated with rapid correction of hyponatremia. This paper reports a case of ODS after correction of severe hyponatremia complicated by rhabdomyolysis. A 47-year-old female with a history of chronic alcoholism presented herself at the hospital with altered consciousness after three days of nausea and vomiting. She was on a thiazide diuretic for essential hypertension. Her blood tests upon her hospital admission showed hyponatremia ($Na^+$ 98 mEq/L), hypokalemia ($K^+$ 3.0 mEq/L), and elevation of her serum creatine phosphokinase (3,370 IU/L) with an increase in her serum myoglobin level 11,267 ng/mL). She was treated with intravenous fluid therapy that included isotonic and hypertonic salines along with potassium chloride. She became more alert, and her neurological condition gradually improved after the first five days of her therapy. On the ninth day after her admission, she developed progressive quadiaresis associated with dysarthria, dysphagia, and dystonia despite the resolution of her hyponatremia. Magnetic resonance imaging of her brain on 16th day revealed symmetrical areas of signal hyperintensity in her central pons, basal ganglia, and precentral gyrus in T2-weighted images, which are consistent with ODS. Her neurological symptoms steadily improved after six weeks with only supportive treatment and rehabilitation.

• 대한유전성대사질환학회지
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• 제15권3호
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• pp.118-126
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• 2015

Background and objectives: A deficiency of BH4 (tetrahydrobiopterin) not only causes the classical phenylketonuric phenotype, but also is the source of neurological signs and symptoms due to impaired syntheses of L-Dopa and serotonin. The treatment of BH4 deficiency usually consists of replacement with BH4 and the neurotransmitters. We performed this study to finding out long-term follow-up clinical symptoms and prognosis of BH4 deficiency. Methods: Clinical and biochemical, genetic analysis were done retrospectively from January 1999 to July 2015 in Soonchunhyang University Hospital. Results: In our study, total 207 patients were confirmed to hyperphenylalaninemia. Among them, 10 patients were BH4 deficiency. 9 patients were 6-pyruvoyl-tetrahydropterin (PTPS) deficiency and one patient was dihydropteridine reductase (DHPR) deficiency. The patients who received delayed treatment, most of our patients suffered from severe psychomotor retardation, hypotonia and seizure. c.259C>T mutation was identified most commonly in PTPS gene analysis. A patient with DHPR deficiency had a mental retardation, dystonia, seizure. His seizure semiology was dialeptic feature. His EEG showed generalized spike wave patterns. All patients had treated with tolerate L-Dopa, BH4 and 5-hydroxytryptophan. Most of the early treated patients have a good tolerance for drugs well. But some patients had neurologic symptoms, despite early detection and treatment. Conclusion: BH4 deficiency patients who had delayed treatment tend to have severe psychomotor problem and neurologic deficits.