465 - Severe Manifestation of CIDP During Pregnancy: A Case Report

Case Diagnosis: Chronic inflammatory demyelinating polyneuropathy (CIDP) is rare during pregnancy.

Case Description:

A 34-year-old woman developed progressive muscle weakness and hypoesthesia in her second trimester, leading to loss of ambulation and impaired fine motor functions. Hospitalized in the third trimester with a suspected Guillain-Barré syndrome diagnosis, electrodiagnostic studies (EDX) showed absent motor and sensory responses but could not differentiate between axonal and demyelinating involvement. Treatment included plasmapheresis and immunoglobulin.

One year later, she presented with generalized weakness, hypoesthesia, atrophy, areflexia, claw hands, inability to stand, and feet fixed in plantar flexion. Similar but milder symptoms had occurred during her first pregnancy.

Follow-up EDX showed continued absence of motor and sensory responses. A trigeminal facial reflex test indicated bilaterally prolonged R1 responses and no R2 responses, suggestive of demyelination. Neuromuscular ultrasound (USNM) revealed increased cross-sectional area (CSA) in the median, ulnar, tibial, and peroneal nerves, scoring 14 on the Ultrasonographic Pattern Score A (UPS-A). CIDP was diagnosed.



Discussions:

Diagnosis of CIDP relies on clinical presentation and EDX. Absence of motor/sensory responses complicates distinguishing between demyelinating and axonal lesions, making the trigeminal facial reflex test valuable. Demyelinating conditions show prolonged R1, R2i, and R2c latencies, whereas axonal neuropathies exhibit reduced amplitudes. USNM CSA assessment and UPS-A scale are also useful in diagnosing CIDP.

Demyelinating lesions may cause irreversible secondary axonal damage, which negatively impacts prognosis. In this case, the absence of responses indicated severe secondary axonal damage and severe functional dependence, worsening rehabilitation prospects.

Based on the patient's prognosis, a comprehensive rehabilitation plan was ordered to optimize positioning and mobility, promote functional motor patterns, and prevent immobility-related complications. And the patient was referred for interdisciplinary management by seating and neuromuscular boards.

Conclusions:

CIDP during pregnancy is rare. EDX and peripheral nerve USNM are essential for diagnosing CIDP and assessing its severity and rehabilitation outlook.