M. Oechsner et al., ACUTE REVERSIBLE ENCEPHALOPATHY WITH BRAI N EDEMA AND SERIES OF SEIZURES IN PSEUDOHYPOPARATHYROIDISM, Nervenarzt, 67(10), 1996, pp. 875-879
A 16 year old patient with the typical clinical signs of Albright's he
reditary dystrophia developed series of epileptic seizures with loss o
f consciousness, tonic muscle contractions and bite of the tongue, Aft
er termination of the seizures there was coma without focal neurologic
al signs, CT scan revealed diffuse brain edema, Electroencephalographi
c studies showed generalized slowing. In laboratory tests the only abn
ormalities were marked hypocalcemia (1.15 mmol/l) and hyperphosphatemi
a, Blood parathyroid hormone (PTH) was elevated, PTH-Test confirmed th
e diagnosis of pseudohypoparathyroidism, The patient was treated with
calcium and 1,25-dihydroxy-cholecalciferol. After few days the severe
encephalopathy CT and electroencephalographic changes were completely
reversible, Hereditary disturbances of the parathyroid hormone metabol
ism are rare diseases. Hypocalcemia must be included into the differen
tial diagnosis of seizures and brain edema to avoid invasive diagnosti
c and irrational treatment.