Atypical Bourneville sclerosis without epilepsy and mental retardation: case report and literature review

Vol. 55 No. 2 Suppl., 2014
This supplement was not sponsored by Outside Organizations.


Adela Magdalena Ciobanu, Mihai Gheorghe Lisievici, Teodora Camelia Coman, Jean Ciurea, Mihaela Camelia Popa

Twenty-four-year-old woman without familiar detected signs of Bourneville s disease or tuberous sclerosis complex (TSC) was diagnosed with this disease by casual discovery on cerebral magnetic resonance imaging (MRI) of an intraventricular tumor, after symptoms consist in headache, equilibrium disturbances, and progressive loss of vision. MRI shows an intracranial mass, 33/24/30 mm in size, localized at the level of third ventricle and lateral ventricles, with irregular shape, interesting the foramen of Monroe. There are also nodular areas of calcification and a supratentorial hydrocephalus involving the lateral ventricles and the posterior part of the third ventricle. The patients present facial angiofibromas, but from the classical triad of the disease, the epilepsy and mental retardation were absent, the patient never presented seizures. The total removal of the tumor (peace to peace) was performed surgically, the macroscopic features of resected tumor (20/10/10 mm) was of white-gray color, elastic consistency, localized in the both lateral ventricles (left>right) and into the third ventricle, traversing the foramen Monroe. The histopatological examination associated with specific localization of tumor and the facial angiofibromas are very suggestive for subependimar giant cell astrocytoma (SEGA). We have a rare case of atypical or incomplete TSC in which the epileptic seizures and the mental retardation are absent, the intelligence is normal, but occur some psychical symptoms: anxiety, sleeplessness, and autism or behavior disturbances. The evolution of this case was marked by complications because of postoperative hydrocephalus and multiple shunt insertions and revisions were performed after the tumor resection.

Corresponding author: Adela Magdalena Ciobanu, Lecturer, MD, PhD; e-mail:

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Radu Mircea Neagoe, Daniela Tatiana Sala, Angela Borda, Carmen Aurelia Mogoanta, Gheorghe Muhlfay

In the last decade, the clinical picture of primary hyperparathyroidism has changed, with the majority of patients being diagnosed while asymptomatic and the classical clinical pattern characterized by bone disease, recurrent nephrolithiasis, peptic ulcer disease, neurological or psychiatric disorders being rarely encountered. In this context, most patients have minimal hypercalcemia and small parathyroid adenomas. Not surprisingly, giant parathyroid adenomas have seldom been described in the literature. We herein report three cases of giant parathyroid adenomas weighing more than 30 g and discuss their clinicopathological and therapeutic particularities. We also review the relevant literature, with the principal aim of outlining the rarity of these giant parathyroid adenomas and the issues concerning their diagnosis and treatment.

Corresponding author: Daniela Tatiana Sala, MD; e-mail:

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