- J Korean Neurol Assoc. 2002;20(5):475-478.
- Neurological Manifestations of Acute Intermittent Porphyria
- Jee heun Kim, M.D.
- Department of Neurology, Yonsei University College of Medicine
- 급성 간헐성 포르피리아의 신경학적 증상
- 김지현 , 조정희 ·김승민 ·선우일남
- 연세대학교 의과대학 신경과학교실
- Abstract
- "Background : Acute intermittent porphyria (AIP) is an autosomal dominant disorder characterized by recurrent
abdominal pain with neuropsychiatric symptoms and motor dominant polyneuropathy. The purpose of this study is to
characterize neurological manifestations and clinical course of AIP in Korean. Methods : Medical records from 1984 to
2001 were reviewed retrospectively. There were 23 cases of laboratory confirmed AIP. Results : The clinical character-istics
of AIP are the acute abdominal pain without fever or leukocytosis. Neuropsychiatric symptoms or polyneuropathy
occurred in 13 cases (56.5%). Among the 13 cases, there were 4 cases with neuropsychiatric symptoms only, 3 with
polyneuropathy only and 6 with both of them. Polyneuropathy was usually preceded by neuropsychiatric symptoms.
Severe sensori-motor polyneuropathy with respiratory failure occurred in 4 cases, where one expired. Hyponatremia
was seen in 14 cases (60.8%) and 7 of them had the neuropsychiatric symptoms. Conclusions : The clinical characteris-tics
of AIP include acute abdominal pain without leukocytosis or fever, and hyponateremia. The neuropsychiatric symp-toms
and peripheral neuropathy in AIP may occur by improper treatment for the acute abdominal pain, and can worsen
by wrong treatment without clinical suspicion.Key Words : Acute intermittent porphyria, Abdominal pain, Seizure, Polyneuropathies"
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