Abstract
Peripheral neuropathies with demyelinating features, whether acquired or hereditary, are being increasingly identified in Vietnam owing to greater accessibility to electrophysiological studies, along with the development of the Vietnam Association of Neuromuscular Medicine and Electrodiagnosis (VANEM). Among these, chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a condition of particular interest due to its diverse clinical presentation and its treatability, especially within the group of "CIDP variants." The distal acquired demyelinating symmetric neuropathy variant (DADS, also known as distal CIDP)—characterized by distally predominant, symmetric clinical symptoms with sensory predominance and a slowly progressive course—requires a different diagnostic approach compared to "typical CIDP," since approximately two-thirds of patients in this group have a distinct immune-mediated mechanism that is not classified as CIDP and does not respond to first-line CIDP treatments such as intravenous immunoglobulin or corticosteroids. In this presentation, I report a case of a patient with distal demyelinating sensorimotor polyneuropathy with a chronic clinical course, meeting the electrophysiological diagnostic criteria for "distal CIDP" according to the 2021 EFNS criteria, but with several notable features on electrophysiological examination. I discuss the clinical approach for performing appropriate paraclinical work-up and antibody testing for this group of diseases, in order to arrive at the best possible diagnosis, and I discuss the follow-up plan as well as the subsequent treatment strategy for the patient.
