Document Detail

Hypothalamic hamartoma and infantile spasms.
MedLine Citation:
PMID:  17241213     Owner:  NLM     Status:  MEDLINE    
PURPOSE: A small number of patients with hypothalamic hamartoma (HH) present with infantile spasms (IS) as an initial or early seizure type. We describe the clinical features of IS associated with HH. METHODS: Our series of patients with HH and epilepsy was reviewed to identify cases with a history of IS. The clinical features and neuroradiological findings in this study group were compared to a control group of patients with HH and refractory epilepsy, but without a history of IS. RESULTS: We identified six patients with HH and a history of IS in this series (n = 122, 4.9%). Five of the six are male. Four of the six patients (67%) developed IS as their first seizure type. The mean age for onset of IS was 6.2 months (range 4-9 months). Results of electroencephalographic (EEG) study at the time of IS diagnosis showed hypsarrhythmia in two (33%). Five patients were treated with adrenocorticotropic hormone (ACTH), and four of the five (80%) responded with control of IS. However, these patients developed other seizure types, and were ultimately refractory to medical management. Aside from the IS, no significant differences in clinical and imaging features were determined between the study group and the control group. CONCLUSIONS: HH should be included in the differential diagnosis for infants presenting with IS. These patients may have hypsarrhythmia on initial EEG, and may respond to ACTH treatment with improvement of IS. However, all became refractory with other seizure types, more commonly seen in HH patients. Focal pathologies associated with IS may be subcortical, as well as cortical, in nature. We have not identified any predictive features for the occurrence of IS in the HH population.
John F Kerrigan; Yu-tze Ng; Erin Prenger; Kalpathy S Krishnamoorthy; Norman C Wang; Harold L Rekate
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Publication Detail:
Type:  Case Reports; Comparative Study; Journal Article    
Journal Detail:
Title:  Epilepsia     Volume:  48     ISSN:  0013-9580     ISO Abbreviation:  Epilepsia     Publication Date:  2007 Jan 
Date Detail:
Created Date:  2007-01-23     Completed Date:  2007-03-27     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  2983306R     Medline TA:  Epilepsia     Country:  United States    
Other Details:
Languages:  eng     Pagination:  89-95     Citation Subset:  IM    
Epilepsy Center and Division of Pediatric Neurology, Barrow Neurological Institute and Children's Health Center, St Joseph's Hospital and Medical Center, Phoenix, Arizona, USA.
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MeSH Terms
Adrenocorticotropic Hormone / therapeutic use
Age of Onset
Child, Preschool
Corticotropin-Releasing Hormone / physiology
Diagnosis, Differential
Electroencephalography / statistics & numerical data
Epilepsy / diagnosis
Hamartoma / diagnosis*,  epidemiology
Hormones / therapeutic use
Hypothalamic Neoplasms / diagnosis*,  epidemiology
Magnetic Resonance Imaging
Models, Neurological
Sex Factors
Spasms, Infantile / diagnosis*,  drug therapy,  physiopathology
Treatment Outcome
Reg. No./Substance:
0/Hormones; 9002-60-2/Adrenocorticotropic Hormone; 9015-71-8/Corticotropin-Releasing Hormone

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