Endocrine Abstracts

Case Report

A 36 year old Asian female presented to the Accident and Emergency with a sudden collapse.On examination, the patient was pyrexial at 38.3 degC and had a Glasgow Coma Scale of 4 associated with nuchal rigidity. The patient was ventilated and transferred to ITU. An MRI head scan revealed a large ring enhancing lesion in the hypothalamus which extended to the third ventricle. There was also diffuse lepto-meningeal enhancement with hydrocephalus. A transfrontal burr hole performed by the neurosurgeons revealed a low opening pressure, a protein of 0.66 grams per litre, a glucose of 3.5 millimoles per litre with a white cell count of 6 per centimeter but without any organisms on staining. On further questioning of the relatives it transpired that the patient had been amenorrhoeic for approximately nine months prior to this collapse and had suffered intermittent headaches. On the basis of the clinical findings, the radiology and the history, the diagnosis was considered to be either tuberculous meningitis or neurosarcoidosis. The patient was treated with anti-tuberculous therapy consisting of rifampicin, isoniazid and pyrazinamide and also received dexamethasone 16 milligrams per day. Subsequent endocrine investigations revealed hypopituitarism with diabetes insipidus. Tuberculosis PCR was negative and the cerebrospinal fluid ACE level was within the normal range. She made no clinical response to therapy and underwent a biopsy of the cranial mass through a right temporal craniotomy. Surprisingly histology revealed a craniopharyngioma. The patient remained in a vegetative state following admission and died 4 months later.

Discussion

Craniopharyngiomas are rarely reported to rupture and present with acute signs and symptoms that may be indistinguishable from a meningitic process. Although ruptured craniopharyngioma was not initially suspected in this case and required histological confirmation, the few published reports indicate that the diagnosis may be aided by demonstrating cholesterol crystals on CSF analysis. This case illustrates how a craniopharyngioma may rarely present as an acute medical emergency