J Korean Neurol Assoc.  1999 May;17(3):431-434.

A Case of Benign Intracranial Hypertension in Pseudopseudohypoparathyroidism

• Jung WY
• Kang SJ
• Bae HY

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 might have a precipitating effect.

Keyword

Pseudopseudohypoparathyroidism; Benign intracranial hypertension; Albright's hereditary osteodystrophy

MeSH Terms

Abducens Nerve Diseases
Adolescent
Brain
Calcium
Craniocerebral Trauma
Diplopia
Headache
Humans
Magnetic Resonance Imaging
Male
Metacarpal Bones
Neurologic Examination
Papilledema
Parathyroid Hormone
Phosphorus
Pseudopseudohypoparathyroidism*
Pseudotumor Cerebri*
Vomiting
Calcium
Parathyroid Hormone
Phosphorus