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RFC1 repeat expansions: A recurrent cause of sensory and autonomic neuropathy with cough and ataxia.

Danique BeijerMaike F DohrnJonathan De WinterSarah FazalAndreas CorteseTanya StojkovicGorka Fernández-EulateGauthier RemicheMattia GentileRudy Van CosterClaudia DufkeMatthis SynofzikPeter De JongheStephan ZüchnerJonathan Baets
Published in: European journal of neurology (2022)
We demonstrate that RFC1 is a frequent cause of (WES-negative) HSAN with chronic cough and ataxia. The diagnostic yield of RFC1 repeat-primed PCR was surprisingly high, given that HSAN is genetically poorly understood. This combination of HSAN, ataxia, and chronic cough symptoms represents a new nosological entity within the neuropathy-ataxia spectrum.
Keyphrases
  • early onset
  • heart rate variability
  • heart rate
  • healthcare