A case report: application of the upss scoring system in the diagnosis of chronicinflammatory demyelinating polyradiculoneuropathy

Tóm tắt

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is an acquired immune-mediated peripheral neuropathy characterized by progressive motor weakness and sensory deficits affecting both upper and lower limbs. The pathophysiology involves recurrent demyelination and remyelination of peripheral nerves, leading to segmental conduction block and impaired nerve signal transmission. Diagnosis is often challenging due to clinical and electrophysiological overlap with other neuropathies such as Guillain–Barré syndrome (GBS)/Acute Inflammatory Demyelinating Polyneuropathy (AIDP) and Chronic Immune Sensory Polyradiculopathy (CISP). The Ultrasound Pattern Sum Score (UPSS), particularly the UPSS-A component, has been shown to be a useful tool in the differential diagnosis of CIDP.
We report a case of a 20-year-old male with no prior medical history, admitted for progressive numbness in both lower limbs and upper limbs, reduced deep tendon reflexes, and diminished vibration sense. Electromyography (EMG) showed reduced motor and sensory conduction velocities in multiple peripheral nerves bilaterally. Peripheral nerve ultrasound demonstrated hypoechoic, multifocal nerve enlargement involving various fascicles and segments. UPSS scores were as follows: UPSS-A: 14; UPSS-B:1; UPSS-C: 1; total score =16. The final diagnosis was CIDP.