Plasma metanephrine, 24-h urine creatinine, total catecholamines, vanillylmandelic acid and metanephrines were all elevated (>two times above the normal values). Full screening of the pituitary axis did not reveal any other abnormality. MRI of the abdomen revealed a small paraganglioma in the left adrenal gland. The tumour was removed surgically, the headaches were resolved, and blood pressure was normalised confirming the diagnosis of headache attributed to phaeochromocytoma. Biopsy revealed non-malignant tissue with zellballen pattern, typical for phaeochromocytoma. Brain T2 lesions were considered as lacunes due to hypertension or non-specific T2 lesions. The patient had another brain MRI after surgery that did not show changes. Aspirin was withdrawn.

Phaeochromocytoma (pheo-chroma means dark colour in Greek) is a rare catecholamine-secreting tumour derived from chromaffin cells. Only 0.2 % of hypertensive individuals have this condition that may be asymptomatic in several cases. Phaeochromocytomas may occur in certain familial syndromes, including multiple endocrine neoplasia (MEN) 2A and 2B, neurofibromatosis and von Hippel-Lindau (VHL) disease. About 10 % of cases are malignant however. Phaeochromocytoma causes headache, epigastric pain, tremor, nausea, weakness, weight loss, palpitations, anxiety and constipation, but explosive headache remains the most often presenting symptom. Delay in diagnosis is common. Subarachnoid haemorrhage and reversible cerebral vasoconstriction syndrome are potential life-threatening complications. Seizures, stroke and delirium may also occur. Arterial hypertension may cause headache, often bilateral and pulsating, usually during an acute rise in systolic (to 180 mmHg) and/or diastolic (to 120 mmHg) blood pressure that is remitting after blood pressure normalisation. Notably, mild (140–159/90–99 mmHg) or moderate (160–179/100–109 mmHg) chronic arterial hypertension does not appear to cause headache. Headache should be developed in temporal relation to the onset of hypertension, worsened in parallel with worsening hypertension and significantly improved with improvement in hypertension. Whether moderate hypertension predisposes to headache at all remains controversial, but there is some evidence that it does. Ambulatory blood pressure monitoring in patients with mild and moderate hypertension has shown no convincing relationship between blood pressure fluctuations over a 24-h period and presence or absence of headache. Hypertensive encephalopathy presents with persistent elevation of blood pressure to 180/120 mmHg and at least two of confusion, reduced level of consciousness, visual disturbances including blindness and seizures. It is thought to occur when compensatory cerebrovascular vasoconstriction can no longer prevent cerebral hyperperfusion as blood pressure rises. As normal cerebral autoregulation of blood flow is overwhelmed, endothelial permeability increases and cerebral oedema occurs. On MRI, this is often most prominent in the parieto-occipital white matter. Headache in hypertensive encephalopathy is a presenting symptom, and it is usually bilateral and pulsating, and it improves after normalisation of blood pressure. Headache, with the same characteristics, also occurs in women during pregnancy or the immediate puerperium with pre-eclampsia or eclampsia. It remits after resolution of the pre-eclampsia or eclampsia. The posterior reversible encephalopathy syndrome (PRES) characterised by headache, visual disorders, seizures, altered mentation, consciousness disturbances and focal neurological signs was initially described in these patients. Whether breakdown or activation of cerebral autoregulatory system results in fluid leakage remains debatable. Headache attributed to phaeochromocytoma occurs as a paroxysmal headache in most (51–80 %) of patients with phaeochromocytoma. It is often severe, frontal or occipital and usually described as either pulsating or constant in quality. Headache is typically short lasting (<15 min in 50 % and < 1 h in 70 % of patients). Associated features include apprehension and/or anxiety, often with a sense of impending death, tremor, visual disturbances, abdominal or chest pain, nausea, vomiting and occasionally paraesthesia. The face can blanch or flush during the attack.