Table 2.

Diagnostic Criteria for Alternating Hemiplegia of Childhood Proposed by Rissardo et al.

Type of Criteria Recommendations
Clinical criteria (Aicardi criteria)
  • Onset before 18 months of age
  • Episodes of hemiplegia involving alternating body sides
  • Bilateral hemiplegia or quadriplegia as a separate attack or as a generalization of a hemiplegic event
  • Other paroxysmal movements (dystonic spells, abnormal oculomotor symptoms, or autonomic phenomenon) either concurrent with hemiplegic attacks or independently
  • Disappearance of symptoms while sleeping, although attacks may resume soon after awakening
  • Developmental delay or permanent neurological findings such as dystonia, ataxia, and choreoathetosis
Laboratory criterion ATP1A3 mutations, mainly at the p.Asp801Asn, p.Glu815Lys, or p.Gly947Arg protein level
Supporting criteria
  • Recurrent attacks of monocular nystagmus
  • Neuroimaging within normal limits, despite the presence of permanent neurological findings
  • Electroencephalography without abnormal electrical activity
  • Stepwise milestone deterioration in the setting of paroxysmal attacks
  • New permanent neurological deficit with a rostrocaudal gradient distribution
  • Family history negative for hemiplegic symptoms or presence of dominant inheritance
Atypical features
  • Onset after 18 months
  • Neuroimaging showing structural abnormalities at presentation

Diagnostic criteria are based on Rosewich et al. (2017),42 Viollet et al. (2015),23 Panagiotakaki et al. (2010),43 Sweney et al. (2009),5 and Bourgeois et al. (1993).7

RMMJ Rambam Maimonides Medical Journal Rambam Health Care Campus 2024; 15(3): e0015. ISSN: 2076-9172
Published online 2024 July 30. doi: 10.5041/RMMJ.10529
Copyright © 2024 Rissardo et al