Transcallosal resection of hypothalamic hamartoma for gelastic epilepsy. 2008

M Andrew, and J R Parr, and R Stacey, and J V Rosenfeld, and Y Hart, and P Pretorius, and S Nijhawan, and Z Zaiwalla, and M A McShane
Department of Paediatric Neurology, Oxford Radcliffe Hospitals NHS Trust, Oxford, UK. moragandrew@hotmail.com

BACKGROUND Hypothalamic hamartomas (HHs) are commonly associated with severe epilepsy resistant to anticonvulsant therapy. Historically, surgical resection of HHs resulted in considerable morbidity. CONCLUSIONS Two series of patients who successfully underwent resection using a transcallosal approach have now been published; we report the first UK experience of this technique in a series of five patients with HHs and gelastic epilepsy resistant to anticonvulsant therapy. Patients were assessed pre- and postoperatively for seizure activity, endocrine function, ophthalmology, and neurocognitive function. Two patients had precocious puberty and all had evidence of developmental delay and behavioral problems. Postoperatively, all children experienced at least a 50% reduction in seizure frequency with abolition of major seizure types; one child remains seizure-free. One child developed a mild postoperative right hemiparesis and one developed transient diabetes insipidus. CONCLUSIONS There were no adverse developmental effects of surgery. Transcallosal resection of HHs ameliorates resistant epilepsy syndromes associated with HH.

UI MeSH Term Description Entries
D007027 Hypothalamic Diseases Neoplastic, inflammatory, infectious, and other diseases of the hypothalamus. Clinical manifestations include appetite disorders; AUTONOMIC NERVOUS SYSTEM DISEASES; SLEEP DISORDERS; behavioral symptoms related to dysfunction of the LIMBIC SYSTEM; and neuroendocrine disorders. Froehlich's Syndrome,Hypothalamic-Neurohypophyseal Disorders,Pituitary Diencephalic Syndrome,Hypothalamic Dysfunction Syndromes,Hypothalamic Dysinhibition Syndrome,Hypothalamic Overactivity Syndrome,Hypothalamic Pseudopuberty,Hypothalamic-Adenohypophyseal Disorders,Diencephalic Syndrome, Pituitary,Diencephalic Syndromes, Pituitary,Disease, Hypothalamic,Diseases, Hypothalamic,Disorder, Hypothalamic-Adenohypophyseal,Disorder, Hypothalamic-Neurohypophyseal,Disorders, Hypothalamic-Adenohypophyseal,Disorders, Hypothalamic-Neurohypophyseal,Dysfunction Syndrome, Hypothalamic,Dysfunction Syndromes, Hypothalamic,Dysinhibition Syndrome, Hypothalamic,Dysinhibition Syndromes, Hypothalamic,Froehlich Syndrome,Froehlichs Syndrome,Hypothalamic Adenohypophyseal Disorders,Hypothalamic Disease,Hypothalamic Dysfunction Syndrome,Hypothalamic Dysinhibition Syndromes,Hypothalamic Neurohypophyseal Disorders,Hypothalamic Overactivity Syndromes,Hypothalamic Pseudopuberties,Hypothalamic-Adenohypophyseal Disorder,Hypothalamic-Neurohypophyseal Disorder,Overactivity Syndrome, Hypothalamic,Overactivity Syndromes, Hypothalamic,Pituitary Diencephalic Syndromes,Pseudopuberties, Hypothalamic,Pseudopuberty, Hypothalamic,Syndrome, Froehlich's,Syndrome, Hypothalamic Dysfunction,Syndrome, Hypothalamic Dysinhibition,Syndrome, Hypothalamic Overactivity,Syndromes, Hypothalamic Dysfunction,Syndromes, Hypothalamic Dysinhibition,Syndromes, Hypothalamic Overactivity,Syndromes, Pituitary Diencephalic
D007223 Infant A child between 1 and 23 months of age. Infants
D008297 Male Males
D011183 Postoperative Complications Pathologic processes that affect patients after a surgical procedure. They may or may not be related to the disease for which the surgery was done, and they may or may not be direct results of the surgery. Complication, Postoperative,Complications, Postoperative,Postoperative Complication
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D003337 Corpus Callosum Broad plate of dense myelinated fibers that reciprocally interconnect regions of the cortex in all lobes with corresponding regions of the opposite hemisphere. The corpus callosum is located deep in the longitudinal fissure. Interhemispheric Commissure,Neocortical Commissure,Callosum, Corpus,Callosums, Corpus,Commissure, Interhemispheric,Commissure, Neocortical,Commissures, Interhemispheric,Commissures, Neocortical,Corpus Callosums,Interhemispheric Commissures,Neocortical Commissures
D004828 Epilepsies, Partial Conditions characterized by recurrent paroxysmal neuronal discharges which arise from a focal region of the brain. Partial seizures are divided into simple and complex, depending on whether consciousness is unaltered (simple partial seizure) or disturbed (complex partial seizure). Both types may feature a wide variety of motor, sensory, and autonomic symptoms. Partial seizures may be classified by associated clinical features or anatomic location of the seizure focus. A secondary generalized seizure refers to a partial seizure that spreads to involve the brain diffusely. (From Adams et al., Principles of Neurology, 6th ed, pp317) Abdominal Epilepsy,Digestive Epilepsy,Epilepsy, Focal,Epilepsy, Simple Partial,Focal Seizure Disorder,Gelastic Epilepsy,Partial Epilepsy,Partial Seizure Disorder,Seizure Disorder, Partial,Simple Partial Seizures,Amygdalo-Hippocampal Epilepsy,Benign Focal Epilepsy, Childhood,Benign Occipital Epilepsy,Benign Occipital Epilepsy, Childhood,Childhood Benign Focal Epilepsy,Childhood Benign Occipital Epilepsy,Epilepsy, Benign Occipital,Epilepsy, Localization-Related,Epilepsy, Partial,Occipital Lobe Epilepsy,Panayiotopoulos Syndrome,Partial Seizures, Simple, Consciousness Preserved,Rhinencephalic Epilepsy,Seizure Disorder, Focal,Subclinical Seizure,Uncinate Seizures,Abdominal Epilepsies,Amygdalo-Hippocampal Epilepsies,Benign Occipital Epilepsies,Digestive Epilepsies,Disorders, Focal Seizure,Disorders, Partial Seizure,Epilepsies, Abdominal,Epilepsies, Amygdalo-Hippocampal,Epilepsies, Benign Occipital,Epilepsies, Digestive,Epilepsies, Focal,Epilepsies, Gelastic,Epilepsies, Localization-Related,Epilepsies, Occipital Lobe,Epilepsies, Rhinencephalic,Epilepsies, Simple Partial,Epilepsy, Abdominal,Focal Epilepsies,Focal Epilepsy,Focal Seizure Disorders,Gelastic Epilepsies,Lobe Epilepsy, Occipital,Localization-Related Epilepsies,Localization-Related Epilepsy,Occipital Epilepsies, Benign,Occipital Epilepsy, Benign,Occipital Lobe Epilepsies,Partial Epilepsies,Partial Epilepsies, Simple,Partial Seizure Disorders,Partial Seizures, Simple,Rhinencephalic Epilepsies,Seizure Disorders, Focal,Seizure Disorders, Partial,Seizure, Subclinical,Seizure, Uncinate,Seizures, Simple Partial,Seizures, Subclinical,Seizures, Uncinate,Simple Partial Epilepsies,Subclinical Seizures,Uncinate Seizure
D006222 Hamartoma A focal malformation resembling a neoplasm, composed of an overgrowth of mature cells and tissues that normally occur in the affected area. Hamartomas
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man

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