title: Cognitive and neurological sequelae after stereoendoscopic disconnection of a hypothalamic hamartoma. A case study.
abstract: Hypothalamic hamartomas (HH) are congenital malformations of the hypothalamus, often generating medically refractory gelastic seizures. There is great risk of progression to various complex partial and generalized seizures and of cognitive and behavioral deterioration. Hence, various surgical approaches have been introduced to resect or disconnect the HH from surrounding tissue, and stereoendoscopic disconnection has been advocated as one of the most lenient approaches to sessile HH embedded in the third ventricle. In fact, no long-term neurological or cognitive impairments have hitherto been reported after this procedure. Yet, unforeseen complications may arise in any surgical intervention on this region. We found serious deterioration of memory and reading skills by comprehensive neuropsychological assessments pre- and postoperatively in a child who, before surgery, was age-appropriate with respect to cognitive, emotional, and behavioral development. The child also contracted a permanent oculomotor paresis. Our results are discussed in light of previous relevant findings.
authors.affiliation: Department of Refractory Epilepsy, Division of Surgery and Clinical Neuroscience, Oslo University Hospital, Norway. kirsten.engberg.stabell@ous-hf.no
authors.forename: Kirsten E
authors.initials: KE
authors.lastname: Stabell
authors.forename: Søren J
authors.initials: SJ
authors.lastname: Bakke
authors.forename: Arild
authors.initials: A
authors.lastname: Egge
mesh_terms: D000293:Adolescent; D001288:Attention / Q000502:physiology; D003072:Cognition Disorders / Q000209:etiology* / Q000523:psychology; D004410:Dyslexia / Q000209:etiology / Q000523:psychology; D004724:Endoscopy / Q000009:adverse effects*; D004828:Epilepsies, Partial / Q000209:etiology; D056344:Executive Function / Q000502:physiology; D005260:Female; D006222:Hamartoma / Q000150:complications / Q000473:pathology / Q000601:surgery*; D006801:Humans; D007027:Hypothalamic Diseases / Q000150:complications / Q000473:pathology / Q000601:surgery*; D008279:Magnetic Resonance Imaging; D008569:Memory Disorders / Q000209:etiology / Q000523:psychology; D009422:Nervous System Diseases / Q000209:etiology* / Q000473:pathology*; D009483:Neuropsychological Tests; D019635:Neurosurgical Procedures / Q000009:adverse effects*; D011183:Postoperative Complications / Q000473:pathology* / Q000523:psychology*; D011597:Psychomotor Performance / Q000502:physiology; D012640:Seizures / Q000503:physiopathology; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome