Sturge Weber Syndrome
Sturge-Weber Syndrome (or disease) is a congenital vascular malformation
affecting the head, face, and brain. The primary process appears to be
faulty development of the venous drainage for the cerebral capillary bed.
A similar process affects the skin, eye, and the soft-tissues of the head.
Development of the brain usually proceeds to a normal size, but after
birth, there is progressive atrophy of the affected hemisphere(s). The
disease is usually unilateral, but bilateral cases can occur. Typically
the patient presents at birth with a "Port Wine Nevus" - a reddish-brown
or pink discoloration of the face, often following the distribution of
the trigeminal nerve. Intracranially, ipsilateral to the facial nevus,
there is abnormal circulation that leads to cerebral dysfunction, electrical
instability (seizures), and cerebral cortical atrophy. Seizures usually
present within the first two years of life. Typically the occipital lobes
are affected first, and most severely, but the disease may also involve
the parietal and temporal lobes.
Imaging findings include tram-track calcification following the gyri of
the involved portion of the cerebral hemisphere (on plain films), gyral
calcification, and cerebral atrophy. Though gyriform enhancement may be
seen in the region of the pial angioma on CECT, contrast-MR is more sensitive
for their detection and characterization, especially of the meninges and
Deepak Patkar, MRI Centre, Nanavati Hospital, Mumbai