巨脑白质脑病伴皮质下囊肿患者的大体、精细和口腔运动功能:1例报告。

IF 0.8 Q4 CLINICAL NEUROLOGY Iranian Journal of Child Neurology Pub Date : 2023-01-01 DOI:10.22037/ijcn.v17i1.37156
Reihane Sabermoghadam, Toktam Maleki Shahmahmood, Pooria Sarvghadi, Mehran Beiraghi Toosi
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引用次数: 0

摘要

巨脑白质脑病伴皮质下囊肿(MLC)是一种罕见的常染色体隐性神经退行性疾病。由于运动缺陷是MLC的核心特征,本研究报告了MLC1患者的大体、精细和口腔运动功能。我们的患者表现为大头畸形,运动功能恶化伴共济失调,痉挛和智力残疾。除医疗干预外,患者还接受了职业治疗和语言治疗的康复干预。2岁时和5岁干预后,用磁共振成像(MRI)分析大脑结构,用大运动功能测量(GMFM)、普渡钉板测试(PPT)和口腔运动评估量表(OMAS)分析大体、精细和口腔运动功能。结果显示,虽然运动功能确实因干预而改善,但与同龄人相比,患者在粗大,精细和口腔运动功能方面仍然存在弱点。这些发现强调了早期转诊运动功能康复的重要性,以提高他们的独立性,参与日常任务和生活质量。
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The Gross, Fine, and Oral Motor Functions in a Patient with Megalencephalic Leukoencephalopathy with Subcortical Cyst: A Case Report.

Megalencephalic Leukoencephalopathy with Subcortical Cysts (MLC) is a rare autosomal recessive neurodegenerative disorder. As motor deficits are a core feature of MLC, the present study reported on an MLC1 patient's gross, fine, and oral motor functions. Our patient demonstrated macrocephaly, deterioration of motor functions with ataxia, spasticity, and intellectual disability. In addition to medical interventions, the patient received rehabilitation interventions of occupational therapy and speech therapy. Brain structures were analyzed with magnetic resonance imaging (MRI), and gross, fine, and oral motor functions were analyzed with Gross Motor Function Measurement (GMFM), Purdue Pegboard Test (PPT), and Oral Motor Assessment Scale (OMAS) at age two and after interventions at age five. The results showed that although the motor functions did improve due to the interventions, the patient still had weaknesses in gross, fine, and oral motor functions when compared to his peers. These findings emphasized the importance of early referral for rehabilitation of motor function in order to increase their independence, participation in daily tasks, and quality of life.

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发文量
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