• Title/Summary/Keyword: dystonia

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Dental Treatment of a Patient with Hallervorden-Spatz Disease under General Anesthesia -A Case Report- (Hallervorden-Spatz 병 환자의 전신마취 하 치과치료 -증례보고-)

  • Lee, Yong-Ki;Seo, Kwang-Suk;Kim, Hyun-Jeong;Yum, Kwang-Won;Ahn, Byung-Deok
    • Journal of The Korean Dental Society of Anesthesiology
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    • v.7 no.2
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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.

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A Case of Glutaric Aciduria Type 1 (Glutaric Aciduria Type 1 1례)

  • Song, Joon Young;Kim, Cheol Min;Shin, Young Lim;Yoo, Han Wook
    • Clinical and Experimental Pediatrics
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    • v.45 no.10
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    • pp.1278-1282
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    • 2002
  • Glutaric aciduria(type 1) is characterized clinically by progressive dystonia and dyskinesia in childhood, pathologically by degeneration of caudate and putamen, biochemically by tissue deficiency of glutaryl-CoA dehydrogenase(GCDH), and is transmitted as an autosomal recessive traits. Mutations of the GCDH gene on chromosome 19 have been implicated in the causation of glutaric aciduria(type 1). Macrocephaly in infancy and crossing of percentiles for head circumference are real clues to early diagnosis. Acute neuroregression of dystonia following an initial phase of normal or almost normal development is a common mode of presentation, at times preceded by seizures. We experienced a case of glutaric aciduria(type 1) in a 13-month old girl. She was admitted due to development delay and choreoasthetoid movememt that developed after generalized tonic-clonic type seizures. She was diagnosed as having glutaric aciduria(type 1) based on brain MRI and urine organic acid analysis finding.

Treatment of Snoring and Sleep Apnea with Botulinum Toxin (보툴리눔 독소를 이용한 코골이 및 수면무호흡 치료)

  • Jang, Jae-Young;Chung, A-Young;Kim, Seong-Taek
    • Journal of Dental Rehabilitation and Applied Science
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    • v.29 no.4
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    • pp.391-398
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    • 2013
  • Botulinum toxin has been used for treating strabismus, blepharospasm, cerebral palsy, cervical dystonia, hyperhydrosis, facial wrinkle and chronic migraine under US Food and Drug administration approval. Also it has been tried spasticity-induced pain, post-herpetic neuralgia, myofascial pain and aphthous ulcer as off-label use. In this study, we reviewed recent studies that suggested effects of botulinum toxin on snoring and sleep apnea.

Selective Peripheral Denervation for the Treatment of Spasmodic Torticollis

  • Jang, Kyung-Sool;Park, Hea-Kwan;Joo, Won-Il;Ji, Chul;Lee, Kyung-Jin;Choi, Chang-Rak
    • Journal of Korean Neurosurgical Society
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    • v.37 no.5
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    • pp.350-353
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    • 2005
  • Objective: Various methods of treatment for idiopathic cervical dystonia have been tried in the past with unsatisfactory results. The authors report cases of five patients who underwent selective peripheral denervation for spasmodic torticollis. Methods: Between July 2002 and December 2003, 5 patients underwent surgery at St. Mary's Hospital for spasmodic torticollis. Age of the patient at the onset of symptoms ranged from 29 to 56years (mean 43.75years). Selective peripheral denervation(SPD) was performed at 7 to 11 months after the onset of symptoms (mean 8.75 months). A patient was considered to be the candidate for surgery if conservative methods were unsuccessful and symptoms persisted for longer than 7months. In addition, 2patients who refused treatment with botulinum toxin were also enrolled in this study. Results: Although one patient underwent reoperation, all of the five patients' symptoms were improved after the operation. Clinically, patients with retrocollis showed better improvement than laterocollis patients. Conclusion: Although injection of botulinum toxin is the first-choice in treatment modality, when surgery is required, selective peripheral denervation provides good results with minimum side effects.

Preliminary Report of Gamma Knife Radiosurgery for the Movement Disorders (운동성 장애에 대한 감마나이프 시술의 초기경험)

  • Hong, Joon Ki;Kim, Moo Seong;Lee, Sun Il;Jung, Yong Tae;Kim, Soo Chun;Sim, Jae Hong
    • Journal of Korean Neurosurgical Society
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    • v.29 no.8
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    • pp.1024-1029
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    • 2000
  • Objectives : With recent improvements in neuroimaging and the development of third and fourth-generation radiosurgical dose-planning soft ware, came a renewed interest in using radiosurgery for the treatment of movement disorders. Radiosurgery involves no opening of the cranium and no incisions, eliminating both the risk of hemorrhage from passing an electrode to the depths of meningitis from operative infection. It is for these reasons stereotactic radiosurgical treatment of movement disorders has value in a small subgroup of patients. The authors report four cases of Parkinson's disease and one case of dystonia that were treated by Gamma knife. Methods : Radiosurgical nucleus ventralis intermedius thalamotomy using the gamma knife unit was performed to make lesion in two Parkinson's disease patients. A radiation dose of 120Gy was delivered to nucleus using a single 4-mm collimator plug pattern following classic anatomical landmarks. Patients were followed for a median of 10.5 months(range 9-12 months). An independent neurological evaluation of tremor, based on the change in the United Parkinson's Disease Rating Scale tremor score(UPDRS), was correlated with a subjective evaluation. Gamma knife ventrolateral(V.O.P) thalamotomy was performed in one case of dystonia. A central dose of 150Gy was delivered and the patient was followed for 18 months. Gamma knife globus pallidus interna pallidotomy was performed in two Parkinson's disease patients. A radiation dose of 130Gy(range 120-140Gy) was delivered. Patients were followed for a median of 13 months(range 9-14 months). Result : Ventrolateral thalamotomy in dystonia produced regained left hand usage in order to be able to use the telephone. Ventralis intermedius thalamotomy produced an excellent improvement of the tremor in one case, mild improvement of the tremor in the other case of Parkinson's disease. A globus pallidus internalis(GPi) pallidotomy produced improvement of rigidity and dyskinesia : one other showed no change. There were no neurological complications. Conclusion : Gamma Knife thalamotomy considered a safe and effective technique for the treatment of tremor in Parkinson's disease. Although the results from Longer follow-up is not available yet, the short-term results seem to be encouraging.

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Botulinum Toxin A Treatment for Cervical Dystonia Resulting in Endoscopic Thyroidectomy: A Case Report (내시경 갑상샘 절제술 후 발생한 경부 근긴장이상증의 보툴리늄 A 독소를 이용한 치료: 증례보고)

  • Oh, Hwa-Young;Choi, Hwan-Jun;Nam, Doo-Hyun;Kim, Jun-Hyuk;Lee, Young-Man
    • Archives of Plastic Surgery
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    • v.38 no.2
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    • pp.207-211
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    • 2011
  • Purpose: Surgery for thyroid gland requires skin incisions that can result in postsurgical neck scar. To overcome this, many surgeons performed a endoscopic thyroidectomy. But, this approach had a some problems. One of postoperative problems, iatrogenic cervical dystonia (CD) may occur. At common, CD is defined as a syndrome characterized by prolonged muscle contraction causing twisting, repetitive movements or abnormal posture. Botulinum toxin A (BTA, Botox$^{(R)}$, Allergan, Irvine, CA, USA) is well known treatment agent in the treatment of CD. So, the authors applied BTA injection in rare case with iatrogenic CD resulting in endoscopic thyroidectomy. Methods: A 43-year-old female had endoscopic subtotal thyroidectomy operation 3 years ago. She had symptoms such as progressive cervical pain, abnormal neck posture, depression, and sleep difficulty. About 1 year later, the patient who had previous myomectomy of the clavicular head of sternocleidomastoid muscle, however, symptoms were not improved. And then the patient received BTA therapy in our department. The 2 units per 0.1 mL solution was administered in a 1 mL tuberculin syringe. Results: The dose of BTA used in the patient was 36 units for vertical platysmal bands, superficially, and 10 units for ipsilateral sternocleidomastoid muscle, intramuscularly. After 2 weeks, additional the dose of BTA used in the patient was 5 points for remained scar bands, superficially. Complications related to injection such as significant swallowing difficulties, neck muscle weakness, or sensory change were not observed. In 9 months follow-up, the patient maintained a good result from the method of BTA injection alone. Conclusion: The basic concept is selective denervation for the hyperactive individual muscles and scar bands. We conclude that BTA is an effective and safe treatment for CD despite the iatrogenic and complex presentation of this complication.

Clinical Study on 1 Case of Cervical Dystonia Treated by Korean Medicine and Transfer Energy Capacitive and Resistive (TECAR) Therapy (Transfer Energy Capacitive and Resistive (TECAR) Therapy와 한방치료를 병행한 경부 근 긴장성 사경 환자 증례 보고 1예)

  • Lee, Won-Jun;Yoon, Young-Suk;Kim, Jong-Ho;Lee, Keun-Jae;Kim, Mi-Hye;Ryu, Ho-Sun;Han, Su-Bin;Park, Byung-Hak;Son, Jae-Min;Lee, Nam-Woo;Han, Jeong-Hun;Seo, Hye-Jin;Kim, Ji-Hoon
    • Journal of Korean Medicine Rehabilitation
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    • v.29 no.4
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    • pp.109-115
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    • 2019
  • Cervical dystonia (CD) is a disorder characterized by sustained or intermittent, involuntary muscle contractions which cause twisting, repetitive movements and abnormal postures. In this case report, a CD patients was treated with transfer energy capacitive and resistive (TECAR) therapy and conventional Korean medicine, which consists of acupuncture, Chuna manual medicine, pharmacopuncture and herbal medicine. For outcome measures, this study evaluated Cobb's angle, EuroQol five dimension scale (EQ-5D), numerical rating scale (NRS), neck disabillity index (NDI) and cervical range of motion. As a result, improvements were found in Cobb's angle ($18.65^{\circ}$ to $15.90^{\circ}$), EQ-5D score (0.808 to 0.862), NRS (5 to 3), NDI (16 to 8) and angle of cervical lateral bending ($15^{\circ}$ to $25^{\circ}$). In conclusion, this study shows that combined therapy of conventional Korean medicine treatment and TECAR therapy can be effective for CD patients.

Spasmodic Dysphonia Induced by Valproic Acid (Valproic Acid 복용에 의해 유발된 연축성 발성장애 1예)

  • 오지영;조현지;박기덕;정성민
    • Journal of the Korean Society of Laryngology, Phoniatrics and Logopedics
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    • v.14 no.1
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    • pp.54-56
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    • 2003
  • Spasmodic dysphonia is a task-specific dystonia affecting the laryngeal muscles, resulting forced, strained voice. The pathophysiologic mechanism is not fully understood. We experienced a patient with epilepsy developed transient spasmodic dysphonia during valproic acid monotherapy. The spasmodic dysphonia resolved with dose reduction of valproic acid. Change of neurotransmitters, such as GABA in basal ganglia or blockade of sodium channel is possible mechanism in our case of drug-related spasmodic dysphonia.

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Clinical study of acupuncture treatment 1 Case of pruritus ani (항문소양증환자(肛門搔痒症患者) 1례(例)에 대한 임상적(臨床的) 고찰(考察))

  • Kang, Mi-Suk;Byun, Im-Jeoung;Song, Ho-Sueb;Lim, Jeung-Eun;Kwon, Soon-Jung;Lee, Seong-No;Hwang, Hyeun-Seo;Kim, Kee-Hyun
    • Journal of Acupuncture Research
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    • v.18 no.4
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    • pp.201-204
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    • 2001
  • Objective : This study was to report a clinical trial in which acupuncture treatmnt on 會陰(Huiyin, CV1) was effectively applied to a patient who had suffered from teasing pruritus ani for 38 years. pruritus ani was thought to be initiated by dystonia. Methods : The acupoint, 會陰(Huiyin, CV1) was selected, because needling on perineal muscles was mainly used to alleviate pelvic pain or pruritus ani in the light of MPS(Myofascial Pain Syndrome) theory, on which 會陰(Huiyin, CV1) was located and also had pelvic pain and pruritus ani in its indications. On the acupoint, the following maneuver was used. (1) Make a patient stand and bend forward (2) Insert a needle on 會陰(Huiyin, CV1) (3) Repeat lifting and pushing (4) Draw a needle immediately without retaining needle on the acupoint Results : The patient with Pruritus ani recovered after above treatment two times and had no recurrence up to Feb. 2001. Conclusion : In this study, Needling on 會陰(Huiyin, CV1) proved to be effective in the aspect of MPS and acupuncture treatment as well, but more clinical trials are expected to follow this study.

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Literature Review on Alternative Traditional Treatment of Spasmodic Torticollis - focusing on Chinese Traditional Medicine's Journals - (연축성 사경 치료에 관한 문헌적 고찰 - 중국 논문 중심으로 -)

  • Lee, Won Chul;Sun, Seung Ho
    • Journal of Physiology & Pathology in Korean Medicine
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    • v.27 no.1
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    • pp.34-42
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    • 2013
  • Our study is to review Chinese traditional medicine's journals about the diagnosis and treatment of spasmodic torticollis such as category, syndrome differentiation, acupoints of treatment, and herbal medicine. The journal search was performed using the search engine of China Academic Journal (CAJ) and China Doctor/Master's Dissertation (CDMD) in China National Knowledge Infrastructure (CNKI) from January 1982 to October 2012. Searching key words were the diverse combination of "spasmodic torticollis", "cervical dystonia", "Chinese traditonal medicine", "herbal medicine", "acupuncture", and "syndrome differentiation". The inclusion criteria was all kinds of journals including Chinese traditional medicine approach except for experiment study. The category, syndrome differentiation, acupoints of treatment, and herbal medicine from finally selected journals were extracted and summarized. The fourty-seven Chinese journals were selected finally. The category was divided into wind syndrome, trembling syndrome, convulsive syndrome, and convulsions. The syndrome differentiation was classified as internal stirring of liver wind, yin-blood depletion, invasion of external contraction, uncontrol of governor vessel, internal obstruction of phlegm turbidity, dual deficiency of qi and blood, and blood stasis due to qi stagnation. The combination of acupoints to unblock the meridian and dissipate binds and to tonify governor vessel and repel tremor was mainly used in acupuncture treatment. Galgun-tang or galgun-tanggami was primarily used and the others were the prescriptions to tonify liver and kidney, to calm convulsion, and to dispeling wind-phlegm. We suggests that spasmodic torticollis could be treated using Korean medicine's approach in Korea.