Diencephalic syndrome with GH deficiency, hyperprolactinemia and neurovegetative and behavioral disturbances, a case report

Abstract

We report the clinical history of a 13 year old girl with no familial pathology. The onset took place shortly after a non complicated small-pox vaccination (18 mo.). At completion it combined : 1 - An endocrine dysfunction : +5 SD obesity and polyphagia a complete GH deficiency treated on from age 7, a breast hypertrophy with normal estrogens but important hyperprolactinemia, easily blocked by bromocriptine. Other hypophyseal stimulines (ACTH, FSH, LH, TSH, ADH) were normal. 2 - Behaviour disturbances : retirement, multiple obsessions and fears being part of a marked psychotic behaviour causing a secondary deterioration. 3 - Signs of neurovegetative dysautonomia : absent tears, bouts of hyper -or- hypothermia (40° to 33° C), cyclic vomiting divergent squint, acrocyanosis… This manifold semiology points out to a diencephalic localization. All neuroradiologic investigations failed to find any diencephalic tumour. Further investigations showed an abnormal EEG, diminished REM-sleep, lowered CSF-5HT turn-over under benemide but did not give a satisfactory pathogenic explanation for the child's disease.