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頁籤選單縮合
題名 | Prognostic Factors for Mental Retardation in Patients with Tuberous Sclerosis Complex=結節性硬化症病人心智障礙之預後因子 |
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作者姓名(中文) | 周秉箴; 張寓智; | 書刊名 | Acta Neurologica Taiwanica |
卷期 | 13:1 2004.03[民93.03] |
頁次 | 頁10-13 |
分類號 | 415.827 |
關鍵詞 | 結節性硬化症; 心智障礙; Tuberous sclerosis complex; Mental retardation; Prognostic factors; |
語文 | 英文(English) |
英文摘要 | Purpose: To analyze the prognostic factors for mental retardation in patients with tuberous sclerosis complex (TSC). Methods: We retrospectively reviewed 35 in-patients with TSC in a medical center and analyzed the clinical features. 19 of 32 patients (59.4%) fulfilled the classical diagnosis of tuberous sclerosis. According to the diagnostic criteria of Roach et al., 30 patients had definite TSC and 5 patients possible TSC. Results: Among the 35 patients, the mean age at diagnosis was 15.9±12.5 years, and the male-to-female ratio was 14:21.11 of 30 patients (36.6%) had a family history of TSC. The most frequent CNS manifestations were seizures (32/35, 91.4%) and mental abnormality (12/32, 62.5%). The most common cutaneous manifestation was facial angiofibroma (27/35, 77.1%) and the most common seizure pattern was generalized tonic clonic seizures (22/32, 62.9%). Poor control of seizures (p=0.006) and the presence of cortical tubercles in imaging studies (p=0.03) were correlated statistically with mental abnormality. Poor control of seizures (15/32, 46%) was more common in generalized tonic clonic seizures than other seizure types (p=0.04l). Twenty-six of 28 patients (92.8%) displayed the typical findings of cortical tubers and subependymal nodules on the brain CT or MRI. Conclusion: Mental retardation in TSC was correlated with poor control of seizures and the presence of CNS lesions. |
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