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ENRAH


Research & Development

AHC is a rare neurological disorder characterized with:

  • An early onset - up to 18 months ;
  • Recurrent episodes of half and full body paralysis;
  • Other paroxysmal events such as dystonic attacks, migraine headaches, nystagmus , autonomic disturbances;
  • permanent neurological abnormalities, including choreothetosis, dystonia, or ataxia, and developmental delay.
Most of the reported cases of AHC are sporadic (no family history of the disease) and several cases, where more than one sibling in the family is affected, have been described. AHC is , yet, symptomatically described and diagnosed and thus, proper and timely diagnosis can be a challenge in the medical practice. At present, the prevalence of AHC is calculated to less than 1 per 1 million. It affects equally both genders and all races.

AHC was first described by Verret and Steele in 1971 as closely related to migraine. Later on, links between AHC and mitochondrial dysfunction, epilepsy, movement disorders, and vascular disorders have been made. Clinical investigations on single or few cases with AHC have not revealed any consistent abnormalities to suggest directions for further research. Thus, the etiology and pathophysiology of AHC remain largely unknown.

Download selected recent publications