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Cerebral gigantism-jaw cysts syndrome

Synonym(s) Cramer-Niederdellmann syndrome
Prevalence <1 / 1 000 000
Inheritance Unknown
Age of onset -
  • Q04.8
MeSH -
MedDRA -


Disease definition

Cerebral gigantism-jaw cysts syndrome is characterised by cerebral gigantism associated with a jaw cyst basal cell naevoid syndrome.


It has been reported in less than ten patients from two families.

Clinical description

Neurological manifestations include hydrocephalus, ventricular malformation, a cerebellar syndrome, intracranial calcification, oculomotor disturbances and, in some cases, mild peripheral nervous disorders.

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