Dietary Treatment of Seizures from a Hypothalamic Hamartoma

Vijay Maggio, James Wheless

Research output: Chapter in Book/Report/Conference proceedingChapter

1 Citation (Scopus)

Abstract

This chapter highlights the case of a patient suffering from refractory seizures with hypothalamic hamartoma. The 16-year-old male had a history of seizure onset at the age of 2 years. Initially, the seizures were brief and consisted of staring spells, occurring mainly at night during the transition from waking to sleep. They were refractory to medical treatment and increased in frequency with time. Neuroimaging studies revealed a hypothalamic hamartoma. Medications tried alone or in combination included acetazolamide, carbamazepine, valproate, felbamate, gabapentin, phenytoin, phenobarbital, and lamotrigine. He had been treated with methylphenidate, amoxapine, and thioridazine for behavioral modification. Magnetic resonance imaging of the brain revealed a hypothalamic mass 10-12 mm in diameter from the center of the hypothalamus, consistent with a hypothalamic hamartoma. The patient was initiated on the ketogenic diet and within days showed remarkable improvement. Within 1 year his antiepileptic drugs and the drugs used for behavioral therapy were withdrawn. He tolerates the ketogenic diet wonderfully and has had no complications. His lipid profile is normal. His dysarthria has showed significant improvement. Hypothalamic hamartomas are congenital malformations that consist of masses of neuronal tissue in ectopic locations. They typically originate from one of the mammillary bodies and extend into the interpeduncular cistern.

Original languageEnglish (US)
Title of host publicationPuzzling Cases of Epilepsy
PublisherElsevier Inc.
Pages350-353
Number of pages4
ISBN (Print)9780123740052
DOIs
StatePublished - Dec 1 2008

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Seizures
Ketogenic Diet
felbamate
Amoxapine
Mammillary Bodies
Thioridazine
Choristoma
Dysarthria
Methylphenidate
Acetazolamide
Carbamazepine
Valproic Acid
Phenytoin
Therapeutics
Phenobarbital
Age of Onset
Neuroimaging
Anticonvulsants
Hypothalamus
Sleep

All Science Journal Classification (ASJC) codes

  • Neuroscience(all)

Cite this

Dietary Treatment of Seizures from a Hypothalamic Hamartoma. / Maggio, Vijay; Wheless, James.

Puzzling Cases of Epilepsy. Elsevier Inc., 2008. p. 350-353.

Research output: Chapter in Book/Report/Conference proceedingChapter

Maggio, Vijay ; Wheless, James. / Dietary Treatment of Seizures from a Hypothalamic Hamartoma. Puzzling Cases of Epilepsy. Elsevier Inc., 2008. pp. 350-353
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