Bannayan-Zonana syndrome (BZS)

Evidence-based neurology checklist on bannayan-zonana syndrome (bzs): Genetics The transmission is autosomal dominant There is a male predominance Key features Dysmorphic features Neurological features Neoplastic features Mesodermal hamartomas Skeletal features

Genetics

  • The transmission is autosomal dominant
  • There is a male predominance

Key features

Dysmorphic features

Neurological features

Neoplastic features

Mesodermal hamartomas

Skeletal features

References

  1. Miles JH, Zonana J, Mcfarlane J, Aleck KA, Bawle E. Macrocephaly with hamartomas: Bannayan-Zonana syndrome. Am J Med Genet 1984; 19:225-234.
  2. Gujrati M, Thomas C, Zelby A, Jensen E, Lee JM. Bannayan-Zonana syndrome: a rare autosomal dominant syndrome with multiple lipomas and hemangiomas: a case report and review of literature. Surg Neurol 1998; 50:164-168.
  3. Naidich JJ, Rofsky NM, Rosen R, Karp N. Arteriovenous malformation in a patient with Bannayan-Zonana syndrome. Clin Imaging 2001; 25:130-132.
  4. Higginbottom MC, Schultz P. The Bannayan syndrome: an autosomal dominant disorder consisting of macrocephaly, lipomas, hemangiomas, and risk for intracranial tumors. Pediatrics 1982; 69:632-634.
  5. Khadir K, Eddaoui A, Chiheb S, et al. Fatal progression of Bannayan-Zonana syndrome. Arch Pediatr 2009; 16:364-367. 
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