Endocrine Abstracts

Background: Haemorrhagic stroke below age 45 is often associated with hypertension and warrants additional investigations. We describe a young patient presenting with acute intracerebral haemorrhage, and how further investigations led to the underlying endocrine diagnosis.

Case: A 43-year-old female patient with no past medical history presented with facial weakness, speech difficulties and systolic blood pressure above 200mmHg. Brain CT and MRI scans revealed acute intracerebral haemorrhage. Additionally, there was evidence of old intracerebral haemorrhages. She required glyceryl trinitrate infusion. Subsequently three different antihypertensive agents failed to achieve control. HbA1c was elevated at 79 mmol/mol (diabetes>48), and metformin started. Young stroke investigations were unremarkable. She was referred to Endocrinology for further assessments. On examination the patient had proximal myopathy, purple striae, central adiposity, interscapular fat pad, plethoric round facies, hirsutism, skin thinning and spontaneous bruising suggesting Cushing’s syndrome. Overnight 1 mg dexamethasone suppression test failed to suppress cortisol to <50 (690nmo/l), leading to 48hr Low dose (LDDST) and 48hr high dose dexamethasone suppression tests (HDDST). Post-LDDST cortisol was unsuppressed at 390 nmol/l; pre-test ACTH of 82.1ng/l (normal<46.0) reduced to 65.4ng/l confirming hypercortisolism. 24-hour urinary free cortisol was elevated at 330nmol/24h (normal<140). Post-HDDST cortisol was 94 nmol/l; ACTH suppressed from 98.9 to 24.0 pointing to pituitary Cushing’s disease. Other endocrine tests were normal. MRI pituitary demonstrated a 2.5mm microadenoma on the right of the gland. Inferior petrosal sinus sampling to confirm source (due to corticotrophin releasing hormone shortage) and transsphenoidal surgery is planned.

Discussion: This patient’s presentation and the sequence of events serve as a gentle reminder to consider endocrine hypertension in young stroke patients. Our patient had the classical phenotype of Cushing’s syndrome, including refractory hypertension and diabetes. A high index of suspicion is essential for prompt diagnosis in such situations where patients present with complications of severe hypercortisolism to the acute medical take.