{"title":"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。","authors":"Reihane Sabermoghadam, Toktam Maleki Shahmahmood, Pooria Sarvghadi, Mehran Beiraghi Toosi","doi":"10.22037/ijcn.v17i1.37156","DOIUrl":null,"url":null,"abstract":"<p><p>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.</p>","PeriodicalId":14537,"journal":{"name":"Iranian Journal of Child Neurology","volume":null,"pages":null},"PeriodicalIF":0.8000,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/5c/94/ijcn-17-149.PMC10114270.pdf","citationCount":"0","resultStr":"{\"title\":\"The Gross, Fine, and Oral Motor Functions in a Patient with Megalencephalic Leukoencephalopathy with Subcortical Cyst: A Case Report.\",\"authors\":\"Reihane Sabermoghadam, Toktam Maleki Shahmahmood, Pooria Sarvghadi, Mehran Beiraghi Toosi\",\"doi\":\"10.22037/ijcn.v17i1.37156\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>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.</p>\",\"PeriodicalId\":14537,\"journal\":{\"name\":\"Iranian Journal of Child Neurology\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":0.8000,\"publicationDate\":\"2023-01-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/5c/94/ijcn-17-149.PMC10114270.pdf\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Iranian Journal of Child Neurology\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.22037/ijcn.v17i1.37156\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q4\",\"JCRName\":\"CLINICAL NEUROLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Iranian Journal of Child Neurology","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.22037/ijcn.v17i1.37156","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"CLINICAL NEUROLOGY","Score":null,"Total":0}
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.