Hereditary Spastic Paraplegia－Clinical Analysis and Experience of Rehabilitation：A Case Report

遺傳性痙攣性下身麻痺，為一不常見之中樞神經退化性疾病，其引起之原因仍不清楚，但可經由隱性或顯性正染色體作為其遺傳之途徑。其臨床特徵為病患均有正常之出生及發展史，但於童年時，逐漸出現緩慢但進行性之雙下肢麻痺、痙攣、反射增強、肌肉張力增加，以致影響其正常之步行及步態。本疾病可分為單純型及複雜型兩類，後者可能同時合併有視力障礙、皮膚及心臟疾病之產生。過往文獻甚少討論復健治療對本病之影響，故本病例報告提出加以討論。本研究病例為一位12歲之男性病患，其家屬發現病人於7歲開始步態異常，跑步時容易跌倒，但經藥物治療後均無效。病患於民國84年7月23日至本院就診，臨床理學檢查發現其雙下肢肌力及感覺正常，但深部肌腱反射增強及痙攣性步態，脊髓攝影、核磁共振檢查均為正常，腦部電腦斷層掃瞄並無異狀。經八週之復健治療訓練後，使用Vicon步態分析儀檢查，發現其單腳及雙腳著力期之分配及關節屈伸變化度均有差異。在此提出此一病例，以作為臨床診斷及治療上之參考。

關鍵字

遺傳性痙攣性下身麻痺；復健；步態

並列摘要

Hereditary spastic paraplegia (HSP) is a rare degenerative disorder of the central nervous system. The mode of inheritance may be autosomal dominant, autosomal recessive, or X-linked. Clinical features include a normal perinatal and birth history, slow progressive spasticity of the bilateral lower limbs, hyperreflexia, increased extensor plantar responses, and positive knee and ankle clonus resulting in walking difficulty and an abnormal gait. Rehabilitation training for hereditary spastic paraplegia has seldom been discussed. We present a case of HSP who received physical and occupational therapies. A 12-year-old male patient was admitted to our hospital on July 23, 1995 due to insidious onset of an abnormal gait. The patient had a 5-year history of running instability. Physical examination revealed that bilateral lower limbs had increased muscle tone, exaggerated muscle stretch reflexes, and a spastic gait with normal muscle power and sensation. The whole spine myelography, magnetic resonance imaging of the spine, and brain computed tomography were all normal. Vicon gait analysis after eight weeks of rehabilitation training revealed improved knee flexion and extension, ankle dorsiflexion and plantar flexion, and a single support interval on the left side. These findings show that rehabilitation training can improve gait performance and that long-term prognosis needs further investigation.

並列關鍵字

hereditary spastic paraplegia ； rehabilitation ； gait

國際替代計量

Hereditary Spastic Paraplegia－Clinical Analysis and Experience of Rehabilitation：A Case Report

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