title: Decompressive surgery in a patient with hyperostosis corticalis generalisata for relief of cognitive disability and dysaesthesia.
abstract: A 28-year-old man with genetically confirmed hyperostosis corticalis generalisata (Van Buchem disease) suffered from headache and progressive cognitive and sensibility disorders. Bone formation of the skull was ongoing, leading to narrowing of the intracranial space and foramen magnum. A large bilateral frontoparietal craniotomy and decompression of the foramen magnum resulted in almost complete relief of his symptoms. This is the first report on successful decompressive surgery as a treatment of cognitive impairment and dysaesthesia.
authors.affiliation: Department of Neurology, Leiden University Medical Centre, Postbus 9600, 2300 RC, Leiden, The Netherlands, m.datema@lumc.nl.
authors.forename: Mirjam
authors.initials: M
authors.lastname: Datema
authors.forename: Natasha M
authors.initials: NM
authors.lastname: Appelman-Dijkstra
authors.forename: Stefan A
authors.initials: SA
authors.lastname: Hoyng
authors.forename: Marco J T
authors.initials: MJ
authors.lastname: Verstegen
authors.forename: Radboud W
authors.initials: RW
authors.lastname: Koot
mesh_terms: D000328:Adult; D003072:Cognition Disorders / Q000209:etiology / Q000601:surgery*; D019299:Decompression, Surgical / Q000379:methods*; D006801:Humans; D008297:Male; D010009:Osteochondrodysplasias / Q000150:complications / Q000601:surgery*; D010292:Paresthesia / Q000209:etiology / Q000601:surgery*; D012886:Skull / Q000002:abnormalities*