Document Detail

The clinical problem of hyperinsulinemic hypoglycemia and resultant infantile spasms.
MedLine Citation:
PMID:  20956428     Owner:  NLM     Status:  MEDLINE    
Hyperinsulinemic hypoglycemia (HH) is a cause of severe hypoglycemia in the newborn and infancy period and is associated with a high risk of neurologic handicap and epilepsy. Infantile spasms after exposure to HH is rare and has been described in only 1 previous report. We report the clinical, biochemical, and neurodevelopmental characteristics of 5 patients with neonatal-onset HH who subsequently developed infantile spasms. All 5 patients had neonatal-onset HH of varying severity and duration. These patients presented with the characteristic ictal pattern of spasms in clusters at a mean age of 6.6 months. Characteristic hypsarrhythmia was noted in only 3 of 5 patients. Structural abnormality was found in only 1 of 4 patients who underwent MRI of the brain. Infantile spasms responded to medical treatment in 3 patients, spasms in 1 patient were refractory to antiepileptic drugs, and treatment duration was insufficient for us to comment on the response in 1 patient. Developmental delay was evident in all of them. In conclusion neonatal HH of varying severity is associated with later (after a latent period) development of infantile spasms. The latent period before the onset of the spasms can be variable; hence, long-term neurodevelopmental follow-up (until 1 year of age) is necessary.
Anitha Kumaran; Sri Kar; Ritika R Kapoor; Khalid Hussain
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Publication Detail:
Type:  Case Reports; Journal Article     Date:  2010-10-18
Journal Detail:
Title:  Pediatrics     Volume:  126     ISSN:  1098-4275     ISO Abbreviation:  Pediatrics     Publication Date:  2010 Nov 
Date Detail:
Created Date:  2010-11-02     Completed Date:  2010-11-22     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0376422     Medline TA:  Pediatrics     Country:  United States    
Other Details:
Languages:  eng     Pagination:  e1231-6     Citation Subset:  AIM; IM    
Developmental Endocrinology Research Group, Clinical and Molecular Genetics Unit, Institute of Child Health, University College London, 30 Guilford St, London WC1N 1EH, United Kingdom.
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MeSH Terms
Anticonvulsants / therapeutic use
Brain / pathology
Developmental Disabilities / diagnosis,  etiology
Diagnosis, Differential
Electroencephalography / drug effects
Follow-Up Studies
Infant, Newborn
Magnetic Resonance Imaging
Persistent Hyperinsulinemia Hypoglycemia of Infancy / complications*,  diagnosis
Spasms, Infantile / diagnosis,  drug therapy,  etiology*
Reg. No./Substance:

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