Electrophysiological features of the peripheral neuropathy in patients with pathologic biallelic RFC1 repeat expansions.
Calezis ClaudiaBonello-Palot NathalieVerschueren AnnieAzulay Jean-PhilippeFortanier EtienneGrapperon Aude-MarieKouton LudivineGallard JulienSalort-Campana EmmanuelleAttarian ShahramEmilien DelmontPublished in: Muscle & nerve (2024)
Less than half of the patients with RFC1 expansions exhibited the full clinical CANVAS spectrum, but nearly all exhibited typical sensory neuronopathy and abnormal H-reflexes. Involvement of small nerve fibers and brainstem neurons was less common.