1043-3155 Pediatr Neurol Briefs pedneurbriefs Pediatr Neurol Briefs Pediatric Neurology Briefs Pediatr Neurol Briefs 2166-6482 1043-3155 2166-3155 Pediatric Neurology Briefs Publishers Chicago, IL, USA PNB-11-10-a 10.15844/pedneurbriefs-11-2-2 Seizure Disorders Neurology Pediatrics Nervous System Diseases Child Development Brain Diseases Neurosurgery Child Infant Hypsarrhythmia Variant Patterns http://orcid.org/0000-0002-0173-7931 Millichap J. Gordon MD 1 2 * Division of Neurology, Children's Memorial Hospital, Chicago, IL Departments of Pediatrics and Neurology, Northwestern University Feinberg School of Medicine, Chicago, IL Correspondence: Dr. J. Gordon Millichap, E-mail: jgmillichap@northwestern.edu 02 1997 01 05 2016 11 2 10 10 Copyright: © 1997 The Author(s) 1997 This work is licensed under the Creative Commons Attribution 4.0 International License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Hypsarrhythmia: Frequency of variant patterns and correlation with etiology and outcome

The frequency of hypsarrhythmia variant or atypical patterns was determined by analysis of pre-ACTH EEG records of 53 consecutive patients with infantile spasms at Children’s Hospital, Harvard Medical School, Boston, MA.

 Hypsarrhythmia Variant Atypical Patterns Cortical Hypometabolism

The frequency of hypsarrhythmia variant or atypical patterns was determined by analysis of pre-ACTH EEG records of 53 consecutive patients with infantile spasms at Children’s Hospital, Harvard Medical School, Boston, MA. Hypsarrhythmia was scored (16 most severe, 0 normal EEG) based on degree of disorganization, diffuse delta, voltage, spikes, burst suppression (BS), etc. Variant patterns occurred in 69% of records. Hemihypsarrhythmia or BS was characteristic of spasms caused by cerebral dysgenesis. Sleep patterns were persistently abnormal in infants with perinatal hypoxic-ischemic encephalopathy. Lower hypsarrhythmia scores (<10), <75% delta activity, and absence of electrodecremental discharges correlated with more favorable outcome, whereas variant patterns had no prognostic significance. [1]

COMMENT. Analysis of pre-ACTH EEGs in patients with West syndrome may reflect the pathophysiology and prognosis. Higher severity scores of hypsarrhythmia correlate with poor outcome. Variant patterns of hypsarrhythmia are frequent and show no correlation with prognosis; they should be included within the definition and indications for treatment with ACTH.

Cortical hypometabolism and delayed myelination were reported in a study of 18 children with West syndrome at Nagoya University School of Medicine, Japan [2]. Hypometabolism on PET scans at the onset of spasms or at 10 months is correlated with MRI evidence of delayed myelination at age 10 months.

Kramer U Sue WC Mikati MA Hypsarrhythmia: Frequency of variant patterns and correlation with etiology and outcome Neurology 1997 Jan 48 1 197 203 10.1212/WNL.48.1.197 9008518 Natsume J Watanabe K Maeda N Kasai K Negoro T Aso K Cortical hypometabolism and delayed myelination in West syndrome Epilepsia 1996 Dec 37 12 1180 1184 10.1111/j.1528-1157.1996.tb01853.x 8956849