Elsa V Arocho-Quinones1, Jennifer Koop2, Sean M Lew3. 1. Department of Neurosurgery, Medical College of Wisconsin, Milwaukee, Wisconsin, USA. 2. Department of Neurosurgery, Medical College of Wisconsin, Milwaukee, Wisconsin, USA; Department of Neurology (Neuropsychology), Medical College of Wisconsin, Wisconsin, USA. 3. Department of Neurosurgery, Medical College of Wisconsin, Milwaukee, Wisconsin, USA; Department of Neurosurgery, Children's Hospital of Wisconsin, Milwaukee, Wisconsin, USA. Electronic address: slew@mcw.edu.
Abstract
BACKGROUND: Hypothalamic hamartomas (HHs) are nonneoplastic congenital malformations associated with refractory epilepsy and behavioral disorders. Improvement in behavioral functioning following resection of HHs has been reported. Stereotactic laser ablation (SLA), a minimally invasive technique, has been used for the treatment of HH-related epilepsy. We report the case of child with an HH, gelastic seizures, and severe psychiatric dysfunction who was successfully treated via SLA therapy. CASE DESCRIPTION: The patient was an 11-year-old female with a history of central hypothyroidism, precocious puberty, and localization-related epilepsy thought to be secondary to an HH. She had a significant psychiatric history including attention deficit hyperactivity disorder, depressed mood, impulsivity, threatening behavior, and suicidal ideation requiring management with dexmethylphenidate, bupropion, and aripiprazole. Seizure onset occurred at age 7, and her semiology included nighttime hypermotor seizures and uncontrollable laughing spells thought to be gelastic seizures. Her hypermotor seizures were successfully managed with oxcabazepine monotherapy, but she continued to have several weekly laughing spells and self-harming behavior. Her HH was successfully treated via SLA. Postoperatively, she remained neurologically intact and was discharged the next day. At her 6-month follow-up, she had a markedly improved affect and general mood. At 3 years postprocedure, she remains seizure free and has been weaned off her antiepileptic and antipsychotic medications. CONCLUSIONS: Severe behavioral dysfunction in the setting of an HH may constitute an indication for surgical intervention. The outcome of this case suggests there may be a role for SLA in the management of HH-related psychiatric dysfunction, even in patients with good seizure control.
BACKGROUND:Hypothalamic hamartomas (HHs) are nonneoplastic congenital malformations associated with refractory epilepsy and behavioral disorders. Improvement in behavioral functioning following resection of HHs has been reported. Stereotactic laser ablation (SLA), a minimally invasive technique, has been used for the treatment of HH-related epilepsy. We report the case of child with an HH, gelastic seizures, and severe psychiatric dysfunction who was successfully treated via SLA therapy. CASE DESCRIPTION: The patient was an 11-year-old female with a history of central hypothyroidism, precocious puberty, and localization-related epilepsy thought to be secondary to an HH. She had a significant psychiatric history including attention deficit hyperactivity disorder, depressed mood, impulsivity, threatening behavior, and suicidal ideation requiring management with dexmethylphenidate, bupropion, and aripiprazole. Seizure onset occurred at age 7, and her semiology included nighttime hypermotor seizures and uncontrollable laughing spells thought to be gelastic seizures. Her hypermotor seizures were successfully managed with oxcabazepine monotherapy, but she continued to have several weekly laughing spells and self-harming behavior. Her HH was successfully treated via SLA. Postoperatively, she remained neurologically intact and was discharged the next day. At her 6-month follow-up, she had a markedly improved affect and general mood. At 3 years postprocedure, she remains seizure free and has been weaned off her antiepileptic and antipsychotic medications. CONCLUSIONS: Severe behavioral dysfunction in the setting of an HH may constitute an indication for surgical intervention. The outcome of this case suggests there may be a role for SLA in the management of HH-related psychiatric dysfunction, even in patients with good seizure control.