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Morgagni-Stewart-Morel syndrome

Disease definition

A rare cranial malformation characterized by hyperostosis frontalis interna, variably associated with metabolic and endocrine disorders (such as obesity, diabetes mellitus, and hirsutism, among others). Compression by calvarial thickening may lead to cerebral atrophy and present with cognitive impairment, neuropsychiatric symptoms, headaches, and epilepsy. The condition predominantly affects women.

ORPHA:77296

Classification level: Disorder
  • Synonym(s):
    • Hyperostosis frontalis interna
  • Prevalence: Unknown
  • Inheritance: Autosomal dominant or X-linked recessive 
  • Age of onset: Adult
  • ICD-10: M85.2
  • OMIM: 144800
  • UMLS: C0020494
  • MeSH: -
  • GARD: 8593
  • MedDRA: -
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