Answer:
Sturge Weber Syndrome
Findings:
Prominent leptomeningeal enhancement, enlarged ipsilateral choroid plexus, abnormal deep venous drainage seen as flow voids, cortical atrophy.
Discussion:
The diagnosis is usually obvious on account of a congenital facial cutaneous haemangioma (also known as port wine stain or facial naevus flammeus). This feature is almost always present and usually involves the ophthalmic division (V1) of the trigeminal nerve. Only rarely (~ 5%) is intracranial involvement present without associated cutaneous naevus. In the majority of cases (72%) the naevus is unilateral and ipsilateral to the intracranial abnormality. The most common clinical manifestation is with childhood seizures, present in 71 - 89% of cases 2 that are often refractory to medical therapy. These usually begin in the first few years of life and are often associated with developmental delay and hemispheric symptoms including hemiplegia / hemiparesis and / or hemianopsia. Approximately a third of patients have choroidal or scleral angiomatous involvement, which may be complicated with retinal detachment, buphthalmos or glaucoma
Contributed By: Dr. Arun Chelladurai MD, Dr. Karunakaran M. Kalathi MD
Consultant Radiologists, Aarthi Scans, Thirunelveli, Tamilnadu, India
Sturge Weber Syndrome
Findings:
Prominent leptomeningeal enhancement, enlarged ipsilateral choroid plexus, abnormal deep venous drainage seen as flow voids, cortical atrophy.
Discussion:
The diagnosis is usually obvious on account of a congenital facial cutaneous haemangioma (also known as port wine stain or facial naevus flammeus). This feature is almost always present and usually involves the ophthalmic division (V1) of the trigeminal nerve. Only rarely (~ 5%) is intracranial involvement present without associated cutaneous naevus. In the majority of cases (72%) the naevus is unilateral and ipsilateral to the intracranial abnormality. The most common clinical manifestation is with childhood seizures, present in 71 - 89% of cases 2 that are often refractory to medical therapy. These usually begin in the first few years of life and are often associated with developmental delay and hemispheric symptoms including hemiplegia / hemiparesis and / or hemianopsia. Approximately a third of patients have choroidal or scleral angiomatous involvement, which may be complicated with retinal detachment, buphthalmos or glaucoma
Contributed By: Dr. Arun Chelladurai MD, Dr. Karunakaran M. Kalathi MD
Consultant Radiologists, Aarthi Scans, Thirunelveli, Tamilnadu, India