Datema Mirjam, Appelman-Dijkstra Natasha M, Hoyng Stefan A, Verstegen Marco J T, Koot Radboud W
Department of Neurology, Leiden University Medical Centre, Postbus 9600, 2300 RC, Leiden, The Netherlands,
Acta Neurochir (Wien). 2015 Jul;157(7):1215-8; discussion 1219. doi: 10.1007/s00701-015-2445-1. Epub 2015 May 15.
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.
