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罕见病例中的罕见情况:一名患有僵人综合征的年轻女性的精神症状

A Rarity Among the Rare: Psychiatric Manifestations in a Young Woman With Stiff-Person Syndrome.

作者信息

Sanak Derek, Marticorena-Martinez Roberto, Acosta Erick

机构信息

College of Osteopathic Medicine, Nova Southeastern University, Fort Lauderdale, USA.

Psychiatry, Mount Sinai Medical Center, Miami Beach, USA.

出版信息

Cureus. 2022 Jul 11;14(7):e26745. doi: 10.7759/cureus.26745. eCollection 2022 Jul.

Abstract

Stiff-person syndrome (SPS) is a rare progressive neurologic disease associated with autoantibodies against glutamic acid decarboxylase, an intracellular enzyme involved in the production of gamma-aminobutyric acid.  We present a case involving a 35-year-old Haitian female who was placed under the Baker Act in the emergency department for suicidal behavior and acute psychosis. She has a history of SPS with a positive glutamic acid decarboxylase (GAD) antibody, a condition most commonly found in females between 20 and 50 years of age. The condition was managed by an outpatient neurologist using diazepam, baclofen, and monthly intravenous immunoglobulin treatments. She also has an extensive history of organic neurological conditions, including traumatic brain injury at 18 years old and COVID-19-related anoxic encephalitis that occurred in December 2020. Both psychiatric and neurological physical exams were completed. They revealed a cerebellar tremor, bilateral ptosis, poor eye contact, decreased concentration, poor insight, depressed mood, flat affect, poor judgement, delusional thoughts and a disorganized thought process with tangential speech. CT and MRI imaging of the brain showed no acute intracranial abnormalities. A quantitative titer of the GAD antibody was completed and shown to be elevated >250IU/mL. Depakote 500mg twice daily and risperidone 3mg twice daily were prescribed. The patient had progressive improvement of psychosis including delusional thoughts over the following five days and was able to be discharged with instructions to follow-up with outpatient neurology.

摘要

僵人综合征(SPS)是一种罕见的进行性神经系统疾病,与针对谷氨酸脱羧酶的自身抗体有关,谷氨酸脱羧酶是一种参与γ-氨基丁酸产生的细胞内酶。我们报告一例病例,患者为一名35岁的海地女性,因自杀行为和急性精神病在急诊科被依据《贝克法案》进行强制住院治疗。她有僵人综合征病史,谷氨酸脱羧酶(GAD)抗体呈阳性,这种病症最常见于20至50岁的女性。该病症由一名门诊神经科医生使用地西泮、巴氯芬和每月一次的静脉注射免疫球蛋白进行治疗。她还有广泛的器质性神经疾病史,包括18岁时的创伤性脑损伤以及2020年12月发生的与新冠病毒相关的缺氧性脑病。完成了精神科和神经科的体格检查。检查发现有小脑震颤、双侧上睑下垂、眼神接触不良、注意力不集中、洞察力差、情绪低落、情感平淡、判断力差、妄想思维以及伴有离题言语的思维紊乱。脑部CT和MRI成像显示无急性颅内异常。完成了GAD抗体的定量检测,结果显示其水平升高>250IU/mL。开具了每日两次、每次500mg的丙戊酸和每日两次、每次3mg的利培酮的处方。在接下来的五天里,患者的精神病症状包括妄想思维逐渐改善,之后得以出院,并被指示到门诊神经科进行随访。

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