A 7-year old boy presents with a right-sided hemangioma and left-sided focal seizures. The most likely diagnosis is;
Ans. d. Sturge-Weber syndrome (Ref: Bailey 26/e p599; Nelson 19/e p2051)A 7-year old boy presents with a right-sided hemangioma and left-sided focal seizures. The most likely diagnosis is Sturge-Weber syndrome.Sturge-Weber syndrome/Encephalotrigemmal syndromeUsually sporadic, characterized by:Large unilateral cutaneous angiomaQ (port-wine stain)Angiomas in brain involving ipsilateral cerebral hemisphere and meninges Focal seizuresQ typically occurs opposite to the side of lesionQAdrenal pheochromocytomaCerebral angiomas lead to cortical atrophyQAngiomas are visible radiologically as Tram-track or rail track calcification mainly in occipital regionQ
Neurofibromatosis
Incontinetia pigmenti
Hypermelanosis of Ito
Sturge-Weber disease
Central Nervous System Tumors