急性間欠性ポルフィリン症のリハビリテーションの経験

概要

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We reported a 25-year-old woman with acute intermittent porphyria(AIP), who developed severe neurological symptomes, but recovered with a rehabilitation program started at an early stage. She showed psychiatric disturbances and symptoms of porphyric neuropathy. She became tetraplegic and respirator-dependent. After starting hypertonic glucose, the psychiatric disturbances improved, but the neuropathy did not. We tried rehabilitation at an early stage of AIP, and started haeme-arginate at 40 days from the onset. The Barthel Index improved from 0 to 100 after 250 days from the onset. This suggests that hypertonic glucose, although regarded as one of the treatments of choice for AIP, may be ineffective in neuropathy, and early rehabilitation and haeme-arginate may be the treatment of choice.
社団法人日本リハビリテーション医学会の論文
2001-12-18