6.3.5 Headache attributed to encephalotrigeminal or leptomeningeal angiomatosis (Sturge Weber syndrome)

Identification: Female patient, 12 years old, with a known history of Sturge-Weber syndrome (diagnosed at 6 months), presenting with a port-wine stain in the right trigeminal territory (V1-V2), on valproic acid for epileptic seizures.

Current Illness History: Reports recurrent right-sided hemicranial headache for 3 months.

Characteristics: Fixed right frontotemporal location. Pulsatile character (intensity 6-8/10). Duration of 2-4 hours when untreated. Partial improvement with rest in the dark.

Associated Symptoms: Ipsilateral photophobia to the pain. Nausea (without vomiting). Episodes of transient ipsilateral "visual darkening."

Triggering Factors: Physical exertion. Sleep deprivation.

Previous History: Focal seizures with secondary generalization (last seizure 4 months ago). Mild contralateral hemiparesis. Mild cognitive delay (estimated IQ 68).

Neurological Examination:
Skin: Facial angioma in right V1-V2 territory.
Visual: Left homonymous hemianopia.
Motor: Left brachiocrural hemiparesis (4+/5).
Fundoscopy: Choroidal angiomas on the right.
No meningeal signs.

Complementary Exams:
Brain MRI with contrast: Right parietal-occipital leptomeningeal angiomatosis. Progressive cortical atrophy in the affected territory. Typical "railroad track" pial enhancement. "Railroad track" cortical calcifications.

MR Angiography: Ipsilateral deep cortical vein hypoplasia. Absence of normal superficial venous drainage.

EEG: Epileptiform activity in the right occipital region. Continuous delta slow waves.