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抗N-甲基-D-天冬氨酸受体脑炎的长期康复管理与结局:病例报告

Long term rehabilitation management and outcome of anti-NMDA receptor encephalitis: case reports.

作者信息

Bach Laura J

出版信息

NeuroRehabilitation. 2014;35(4):863-75. doi: 10.3233/NRE-141176.

DOI:10.3233/NRE-141176
PMID:25323083
Abstract

BACKGROUND

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an auto immune-disorder. It is a life threatening condition that typically presents with viral illness, headaches, severe psychiatric symptoms, seizures, behavioural changes, decreasing levels of unconsciousness and progressive unresponsiveness, cognitive impairment, abnormal movements (e.g., dyskinesia), ataxia and hypoventilation.

OBJECTIVE

This paper describes the long term outcome and rehabilitation management of patients with NMDAR encephalitis and highlights the diverse outcome of this condition and the unique and individual long term management needs associated with this disorder.

METHODS

This is a case report study of three different patients with NMDAR encephalitis. All three cases are young women, two of whom presented with ovarian teratoma. Patient KH is the most impaired and was resident in a slow stream rehabilitation care home and presented with challenging behaviour. Patients RM and OA both lived in the community and presented with similar anxieties but diverse levels of cognition and motivation. A review of the literature is provided summarizing the disorder, interventions, management and challenges of this varied and complex condition. Standard neuropsychological tests and questionnaires to assess community integration (BICRO-39), quality of life (QOLIBRI-OS) and mood (HADS) were administered.

RESULTS

Positive outcomes were achieved for all three patients using a variety of interventions which included behavioural management, family psycho-education and an integrated holistic multi-disciplinary team community approach. Memory and executive deficits were persistent in the long term and severity of impairments showed wide variability between patients. Emotional distress and behavioural difficulties were prominent and persistent and had a pronounced impact on rehabilitation. Continence issues were also a major factor impacting on the rehabilitation.

CONCLUSIONS

Long term integrated and multi-disciplinary input by a variety of therapies and health disciplines is required in order to improve the long term outcome and quality of life for NMDAR patients and their families, and ultimately leads to improved positive outcomes. Each of these cases had markedly differing cognitive profiles suggesting that in the context of long term rehabilitation outcome, cognition may have less valence than emotional and behavioural factors. Guidelines and standardised procedures for ethical issues and counselling for iatrogenic infertility should be developed and integrated into long term programmes of rehabilitation care.

摘要

背景

抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎是一种自身免疫性疾病。它是一种危及生命的病症,通常表现为病毒感染、头痛、严重精神症状、癫痫发作、行为改变、意识水平下降和进行性无反应、认知障碍、异常运动(如运动障碍)、共济失调和通气不足。

目的

本文描述了NMDAR脑炎患者的长期预后及康复管理,强调了该病症的多样预后以及与该疾病相关的独特且个性化的长期管理需求。

方法

这是一项对三名不同的NMDAR脑炎患者的病例报告研究。所有三例均为年轻女性,其中两例患有卵巢畸胎瘤。患者KH受损最严重,住在一家慢流康复护理院,存在具有挑战性的行为。患者RM和OA都生活在社区,表现出类似的焦虑,但认知和动机水平各不相同。提供了一篇文献综述,总结了这种多样且复杂病症的疾病情况、干预措施、管理及挑战。进行了标准神经心理学测试以及用于评估社区融入(BICRO - 39)、生活质量(QOLIBRI - OS)和情绪(HADS)的问卷调查。

结果

通过包括行为管理、家庭心理教育和综合整体多学科团队社区方法在内的多种干预措施,三名患者均取得了积极的预后。记忆和执行功能缺陷长期存在,患者之间损伤的严重程度差异很大。情绪困扰和行为困难突出且持续存在,对康复有显著影响。大小便失禁问题也是影响康复的一个主要因素。

结论

需要多种疗法和健康学科进行长期的综合多学科投入,以改善NMDAR患者及其家庭的长期预后和生活质量,并最终带来更好的积极预后。这些病例中的每一例都有明显不同的认知特征,这表明在长期康复预后方面,认知的重要性可能低于情绪和行为因素。应制定关于医源性不育伦理问题和咨询的指南及标准化程序,并将其纳入长期康复护理计划。

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