• Journal of the Korean Academy of Child and Adolescent Psychiatry
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• 제30권3호
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• pp.127-131
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• 2019

Many neurologic disorders manifest as psychiatric symptoms. Anti-N-Methyl-D-Aspartate (NMDA) receptor encephalitis is an autoimmune disease of the brain characterized by numerous neurological and psychiatric features. Despite being rare, its prevalence is rapidly increasing and early management is critical in ensuring successful and sustainable recovery. Therefore, the illness should be considered as a differential diagnosis when clinically assessing patients. This report presents a case of a female child who was hospitalized for acute psychiatric manifestations, which was later confirmed as anti-NMDA receptor encephalitis. She recovered relatively successfully after combined neurological and psychiatric treatment. This report provides information on the clinical course of early onset anti-NMDA receptor encephalitis, including treatment strategy and prognosis.

• 대한한방내과학회지
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• 제44권6호
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• pp.1337-1345
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• 2023

Objectives: This study reports on the improvement of anti-N-methyl-D-aspartate (NMDA) receptor encephalitis with ovarian teratoma after Korean medicine treatment. Methods: A patient was treated with Korean medicine treatments, such as acupuncture and herbal medications (Gami-ondam-tang and Samulanshin-tang-gamibang). The patient's improvement was evaluated using manual muscle testing (MMT), the Mini-Mental State Exam-Korea (MMSE-K), the modified Barthel index (MBI), and the Clinical Assessment Scale in Autoimmune Encephalitis (CASE). Results: After using Korean medicine treatments, the patient's MMT, MMSE-K, and MBI scores increased, and the CASE score decreased. Conclusion: This case suggests that Korean medicine can be effective in treating the symptoms of anti-NMDA receptor encephalitis.

• 대한영상의학회지
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• 제83권4호
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• pp.945-950
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• 2022

항-N-메틸 D-아스파르테이트 수용체(anti-N-methyl-D-aspartate receptor; 이하 항-NMDAR) 뇌염은 중증이지만 치료가 가능한 흔한 자가면역뇌염이다. 항-NMDAR 뇌염 환자는 종종 망상, 환각 및 편집증과 같은 정신병적 증상뿐만 아니라 기억력 손상 및 지속적인 주의력 상실과 같은 증상을 호소한다. 항-NMDAR 뇌염 환자의 자기공명영상 소견은 대부분의 경우에서 이상 소견을 보이지 않으나, 이상 소견이 보이는 경우에는 뇌 실질의 T2 고강도 병변과 이와 인접한 연수막에 조영증강이 있는 것으로 알려져 있다. 그러나 항-NMDAR 뇌염에서 단독 연수막 조영증강은 드물게 보고되어 있다. 우리는 항-NMDAR 뇌염에서 단독 연수막 조영증강을 보인 환자의 증례 보고와 함께 조영증강 유체감쇠반전회복기법 영상의 진단 가치를 보여주고자 한다.

• 정신신체의학
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• 제29권2호
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• pp.207-212
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• 2021

항-N-methyl-D-aspartate 수용체 뇌염(Anti-NMDAR encephalitis)은 NMDA 수용체에 대한 자가항체로 매개되는 신경 염증성 질환으로 초기에 뚜렷한 신경학 증상 없이 망상, 지각이상, 와해된 행동, 심한 불안, 인지기능저하 등의 정신증상이 두드러질 수 있다. 면역치료 혹은 종양제거와 같은 조기치료가 좋은 예후 인자이므로 질병초기에 정신질환과 구분하여 항-NMDAR 뇌염을 진단하는 것이 중요하다. 본 증례에서는 간질과 정신병적 증상을 보이는 26세 여성 A씨를 조기에 항-NMDAR 뇌염으로 확진한 뒤 양성 및 음성증상 척도(Positive and Negative Syndrome Scale, PANSS)를 사용하여 평가하였다. A씨의 항-NMDAR 뇌염 초기의 정신증상으로 PANSS에서 양성하위척도 보다 음성하위척도 점수가 더 높았다. 정신장애와 비교하여 항-NMDAR 뇌염 초기에 음성증상과 인지장애가 더욱 두드러질 가능성이 있다. A씨의 치료로는 rituximab과 난소 기형종의 제거가 효과적이었고 항정신병제로는 quetiapine을 사용하였다. 특히 젊은 여성에서 망상, 행동장애와 함께 음성증상, 인지장애, 긴장증, 의식수준의 변화, 운동이상증상 등이 관찰될 때 항-NMDAR 뇌염에 대한 평가를 고려해야 한다.

• Clinical and Experimental Pediatrics
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• 제59권sup1호
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• pp.133-138
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• 2016

Anti-N-methyl D-aspartate receptor (anti-NMDAR) encephalitis, recently recognized as a form of paraneoplastic encephalitis, is characterized by a prodromal phase of unspecific illness with fever that resembles a viral disease. The prodromal phase is followed by seizures, disturbed consciousness, psychiatric features, prominent abnormal movements, and autonomic imbalance. Here, we report a case of anti-NMDAR encephalitis with initial symptoms of epilepsia partialis continua in the absence of tumor. Briefly, a 3-year-old girl was admitted to the hospital due to right-sided, complex partial seizures without preceding febrile illness. The seizures evolved into epilepsia partialis continua and were accompanied by epileptiform discharges from the left frontal area. Three weeks after admission, the patient's seizures were reduced with antiepileptic drugs; however, she developed sleep disturbances, cognitive decline, noticeable oro-lingual-facial dyskinesia, and choreoathetoid movements. Anti-NMDAR encephalitis was confirmed by positive detection of NMDAR antibodies in the patient's serum and cerebrospinal fluid, and her condition slowly improved with immunoglobulin, methylprednisolone, and rituximab. At present, the patient is no longer taking multiple antiepileptic or antihypertensive drugs. Moreover, the patient showed gradual improvement of motor and cognitive function. This case serves as an example that a diagnosis of anti-NMDAR encephalitis should be considered when children with uncontrolled seizures develop dyskinesias without evidence of malignant tumor. In these cases, aggressive immunotherapies are needed to improve the outcome of anti-NMDAR encephalitis.

• Clinical and Experimental Pediatrics
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• 제57권1호
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• pp.1-18
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• 2014

Channelopathies are a heterogeneous group of disorders resulting from the dysfunction of ion channels located in the membranes of all cells and many cellular organelles. These include diseases of the nervous system (e.g., generalized epilepsy with febrile seizures plus, familial hemiplegic migraine, episodic ataxia, and hyperkalemic and hypokalemic periodic paralysis), the cardiovascular system (e.g., long QT syndrome, short QT syndrome, Brugada syndrome, and catecholaminergic polymorphic ventricular tachycardia), the respiratory system (e.g., cystic fibrosis), the endocrine system (e.g., neonatal diabetes mellitus, familial hyperinsulinemic hypoglycemia, thyrotoxic hypokalemic periodic paralysis, and familial hyperaldosteronism), the urinary system (e.g., Bartter syndrome, nephrogenic diabetes insipidus, autosomal-dominant polycystic kidney disease, and hypomagnesemia with secondary hypocalcemia), and the immune system (e.g., myasthenia gravis, neuromyelitis optica, Isaac syndrome, and anti-NMDA [N-methyl-D-aspartate] receptor encephalitis). The field of channelopathies is expanding rapidly, as is the utility of molecular-genetic and electrophysiological studies. This review provides a brief overview and update of channelopathies, with a focus on recent advances in the pathophysiological mechanisms that may help clinicians better understand, diagnose, and develop treatments for these diseases.