Which conditions are included in the differential diagnoses of Sturge-Weber syndrome (SWS)?

Updated: Dec 26, 2018
  • Author: Masanori Takeoka, MD; Chief Editor: George I Jallo, MD  more...
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Other differentials

Conditions that should also be considered in the differential diagnosis of SWS include the following:

  • Chronic headache

  • Vascular malformations and hematomas of the brain

  • PWS - May be isolated, occurring without SWS

  • Arteriovenous malformation [56] - May calcify

  • Macrocephaly with SWS - Related to hydrocephalus or intracranial hypertension, secondary to the abnormal venous drainage

  • Cyst of the posterior fossa with partial cerebellar agenesis in association with facial angiomas - Angioma flat or tuberous, lies in the territory of the first division of the trigeminal nerve; may be familial

  • Celiac disease - Has been associated with epilepsy with bilateral occipital calcifications [57]

  • Rendu-Osler-Weber syndrome - Hereditary hemorrhagic telangiectasia

  • von Hippel-Lindau syndrome - Cerebellar or spinal hemangioma with retinal angioblastoma, pancreatic cysts, and renal cell carcinoma

  • Wyburn-Mason syndrome - Retinal arteriovenous angioma

  • Shapiro-Shulman syndrome - Bilateral facial nevi and abnormal venous drainage

  • Divry-van Bogaert syndrome - Leptomeningeal angioma (noncalcifying) with diffuse sclerosis, progressive neurologic disorder, and livedo reticularis

  • Bannayan-Zonana syndrome - Macrocephaly, lipomatosis, and cutaneous hemangiomas

  • Cobb syndrome - Cutaneomeningospinal angiomatosis

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