MR spectroscopy - decreased NAA 7
DSA - angiography
In most cases (82%) angiography is abnormal and demonstrates absent superficial cortical veins with abnormal and enlarged deep venous drainage 2.
Treatment and prognosis
Treatment revolves primarily around seizure control, with surgical resection only indicated rarely in refractory cases. Ophthalmological examination is also essential to identify and treat ocular involvement 4.
Etymology
Sturge-Weber syndrome was first described by Sturge in 1879, followed by Weber and Volland in 1912 who described the intracranial calcification. Radiographic identification of cerebral calcification was first described by Dimitri in 1922 2-3.
William Allen Sturge - English physician (1850-1919) 3
Frederick Parkes Weber - English physician (1863-1962) 3
Vincente Dimitri - Austrian dermatologist (1885-1955) 3
Differential diagnosis
The differential is a combination of that for multiple intracranial calcifications, cerebral hemiatrophy and leptomeningeal enhancement, and therefore includes includes:
cerebral arteriovenous malformation (AVM)
infection
TORCH infection
neurocysticercosis
cutaneous haemangioma–vascular complex syndrome 8
healed cortical infarct
radiotherapy
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