A Case of Benign Intracranial Hypertension
in Pseudopseudohypoparathyroidism

J Korean Neurol Assoc > Volume 17(3); 1999 > Article

Journal of the Korean Neurological Association 1999;17(3): 431-434.

양성 두개내 고혈압을 동반한 가성가성부갑상선기능저하증 1례

강상진, 정원영, 배학연

조선대학교 의과대학 신경과학교실, 내과학교실

A Case of Benign Intracranial Hypertension in Pseudopseudohypoparathyroidism

Sang Jinn Kang, M.D., Won Young Jung, M.D, Hak Yeon Bae, M.D.

Department of Neurology, Chosun University Medical College Seosuk 588, Dong-gu, Kwangju, 501-140, Korea

Abstract

We describe a rare case of pseudopseudohypoparathyroidism (PPHP) with benign intracranial hypertension (BIH). A 16 year-old male presented with headache, vomiting, ocular pain, blurred vision, and diplopia following minor head trauma. He showed developmental delay, short stature and short metacarpals and phalanges, which suggested Albright’s hereditary osteodystrophy. Neurologic examination revealed bilateral sixth cranial nerve palsy and bilateral papilledema. Lumbar puncure disclosed an elevated opening CSF pressure with normal biochemical and celluar find-ings. However, the levels of serum calcium, phosphorus and parathyroid hormone were within the normal limits and chromosomal analyses were normal. Brain MRI revealed normal ventricular size and no structural abnormality. We concluded that PPHP seemed to have a key role for the genesis of BIH in this case, although previous mild head trauma 양성 두개내 고혈압을 동반한 가성가성부갑상선기능저하증 1예 might have a precipitating effect. Won Young Jung, M.D. J Kor Neurol Ass 17(3):431 ~ 434, 1999 Key Words : Pseudopseudohypoparathyroidism, Benign intracranial hypertension, Albright’s hereditary osteodystrophy