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Decompressive surgery in a patient with hyperostosis corticalis generalisata for relief of cognitive disability and
Mirjam Datema1, Natasha M Appelman-Dijkstra, Stefan A Hoyng
1Department of Neurology, Leiden University Medical Centre, Postbus 9600, 2300 RC, Leiden, The Netherlands, m.datema@lumc.nl.
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.
