THE GREAT PRETENDER: COULD A PHEOCHROMOCYTOMA MANIFEST ITSELF AS A SEIZURE?

Abstract

Objective: Seizures are defined as transient occurrence of signs or symptoms due to the abnormally excessive or synchronous neuronal activity in the cerebral cortex. Pheochromocytomas and paragangliomas (PPGL) are chromaffin cell tumors arising from the adrenal medulla in 80–85% of patients and from extra-adrenal sympathetic tissue in 10–20% of patients. PPGL has been referred to as ‘‘the great mimic’’ disease. We present a patient with pheochromocytoma possibly disguised as seizures. Design and method: A 44 years old woman was referred to University Clinic of Endocrinology by radiologist. Two years earlier she had syncopal crises . She complained of headache, heart palpitation and malaise, heart palpitations and fatigue during exertion and sense of panic. The maximal self-measured blood pressure was 160/100 mmHg, the pulse is 90-100 beats per min. Abdominal US: a hyperechoic change, 76x65mm was seen, then described in the right lobe of the liver. The following year she had two commotional crises of consciousness and spasms of the extremities . She had a non-specific slowing of FC on EEG on the right for 1-2 seconds. MSCT abdomenal : in the right adrenal gland, a mass 144 x 90mm, with clear borders, pressing into the liver and pushing the right kidney,. The 24h urine was collected, which confirmed a norepinephrine secreting pheochromocytoma. We administered long acting alpha blocking agent preoperatively. The complete surgical excision of the right adrenal mass was done, pathohistological diagnosis: Pheochromocytoma. The last EEG after sleep deprivation performed 2 months postoperatively was described as normal. The patient had no headache or seizure. Results: PPGLs have a notable ability of boosting NE levels systemically and hypertension, the most common sign of PPGL could facilitate the permeability of blood brain barrier to NE. Studies showed NE could play its anticonvulsant property at an appropriate concentration but has a proconvulsant effect in either too high or too low concentrations . Conclusions: The definite pathogenetic mechanisms explaining how PPGL can cause seizures are far from being clearly understood, though “the great pretender” should be considered as a possible etiological factor of seizures.