Acquired encephaloceles and epilepsy in osteopetrosis.
Osteopetrosis is a condition in which there is a defect in bone resorption by osteoclasts. With thickening of the skull and skull base, the cranial capacity becomes compromised and skull foramina gradually occlude, resulting in a wide range of neurological symptoms and signs. We present a case of autosomal dominant osteopetrosis with temporal lobe epilepsy and nasal obstruction due to acquired bifrontal encephaloceles associated with a decreased intracranial capacity. Neurosurgical reconstruction of the frontal skull base alleviated the symptoms of epilepsy and nasal obstruction.
Mandl
E S
ES
Department of Neurosurgery, VU University Medical Center, Amsterdam, The Netherlands. es.mandl@vumc.nl
Buis
D R
DR
Heimans
J J
JJ
Peerdeman
S M
SM
D000328:Adult; D004677:Encephalocele / Q000209:etiology*; D004833:Epilepsy, Temporal Lobe / Q000209:etiology*; D005260:Female; D006801:Humans; D010022:Osteopetrosis / Q000150:complications* / Q000235:genetics / Q000601:surgery; D019291:Skull Base / Q000601:surgery