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小儿神经型类鼻疽:一例康复病例报告。

Paediatric neurological melioidosis: a rehabilitation case report.

作者信息

White Meagan E, Hunt Jacqueline, Connell Cheraine, Langdon Katherine

机构信息

Princess Margaret Hospital for Children, Subiaco, Perth, Western Australia, Australia.

出版信息

Rural Remote Health. 2016 Jan-Mar;16(1):3702. Epub 2016 Feb 21.

Abstract

CONTEXT

Melioidosis is a rare condition, endemic to northern Australia and south-east Asia, caused by an infection from the bacteria . The largest epidemiological review to date describes 540 cases of melioidosis seen at Darwin Hospital, in northern Australia, over a 20-year period. Of these, 14 (less than 3%) presented with neurological manifestation, with three deaths. Reports of paediatric cases of melioidosis are rarer. In a review of paediatric cases in northern Australia only eight cases were identified in 10 years. Three of these patients presented with neurological melioidosis, of whom two died in hospital.

ISSUES

Whilst the literature refers to prolonged periods of hospitalisation for survivors, the trajectory of functional recovery and process of rehabilitation has not been described. This is a case report describing a 14-year-old boy who presented to a remote medical post with acute neurological symptoms (vomiting, severe headache, ataxia, cranial nerve VI and VII palsy) and was referred to the tertiary paediatric hospital in Perth, Western Australia. Cranial magnetic resonance imaging showed an extensive infiltrative lesion in the posterior fossa and hydrocephalus. Diagnosis of neurological melioidosis required isolation of the pathogen by brain biopsy through sub-occipital craniotomy. Medical treatment included surgical management of hydrocephalus, parenteral antibiotic treatment with meropenem and then a prolonged course of oral co-trimoxazole, enteral feeding and tonal management with levodopa-carbidopa and botulinum toxin A injections. Associated neurological signs and symptoms (bradykinesia, tremor, dysphagia, aphasia, hypertonia, exotropia) required intensive rehabilitation to address functional deficits and to promote independence. The purpose of this case report is to document the functional recovery and rehabilitation process of a paediatric case of neurological melioidosis. Knowledge of the recovery pathway is important to add to the understanding of natural history and treatment of this rare disease.

LESSONS LEARNED

Occasions of service and functional assessments were recorded prospectively. Inpatient therapy (932 hours, with 934 occasions of service) was delivered across physiotherapy, occupational therapy and speech pathology over 9 months of an inpatient admission. Initial paediatric functional independence measure (WeeFIM) was 18/126, indicating complete dependence in all physical and cognitive domains. Following 9 months of intensive rehabilitation the WeeFIM was 53/126, indicating significant residual disability. This proved to be a challenge for discharge planning back to a remote region of Western Australia. Paediatric neurological melioidosis can lead to significant disability and long-term dependence, despite the provision of lengthy intensive rehabilitation. This case report highlights the challenges and complexity of the rehabilitation services required to optimise outcomes for this patient and achieve a safe discharge to a remote community where limited support services are available.

摘要

背景

类鼻疽是一种罕见疾病,在澳大利亚北部和东南亚为地方病,由细菌感染引起。迄今为止最大规模的流行病学综述描述了澳大利亚北部达尔文医院在20年期间收治的540例类鼻疽病例。其中,14例(不到3%)出现神经症状,3例死亡。儿童类鼻疽病例的报告更为罕见。在对澳大利亚北部儿童病例的综述中,10年间仅发现8例。这些患者中有3例出现神经类鼻疽,其中2例在医院死亡。

问题

虽然文献提到幸存者住院时间较长,但功能恢复轨迹和康复过程尚未得到描述。本病例报告描述了一名14岁男孩,他因急性神经症状(呕吐、严重头痛、共济失调、第六和第七颅神经麻痹)到偏远医疗站就诊,随后被转诊至西澳大利亚州珀斯的三级儿童医院。头颅磁共振成像显示后颅窝有广泛浸润性病变和脑积水。神经类鼻疽的诊断需要通过枕下开颅术进行脑活检以分离病原体。治疗包括脑积水的手术治疗、美罗培南静脉抗生素治疗,然后是长期口服复方新诺明、肠内营养以及左旋多巴 - 卡比多巴和肉毒杆菌毒素A注射的音调管理。相关的神经体征和症状(运动迟缓、震颤、吞咽困难、失语、张力亢进、外斜视)需要强化康复以解决功能缺陷并促进独立。本病例报告的目的是记录一例儿童神经类鼻疽病例的功能恢复和康复过程。了解恢复途径对于增进对这种罕见疾病的自然史和治疗的理解很重要。

经验教训

前瞻性记录了服务和功能评估情况。在住院9个月期间,通过物理治疗、职业治疗和言语病理学提供了住院治疗(932小时,934次服务)。初始儿童功能独立性测量(小儿功能独立性测量量表)为18/126,表明在所有身体和认知领域完全依赖。经过9个月的强化康复,小儿功能独立性测量量表为53/126,表明仍有明显残疾。这对转回西澳大利亚偏远地区的出院计划构成了挑战。尽管提供了长时间的强化康复,儿童神经类鼻疽仍可导致严重残疾和长期依赖。本病例报告突出了为该患者优化治疗结果并安全出院至支持服务有限的偏远社区所需康复服务的挑战和复杂性。

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