Combined Central and Peripheral Demyelination in Pregnancy: A Case of Dual Neurofascin-140 and Sulfatide Antibody Positivity
Ilaiyabharathi Thulasimani1, Nirumal Khumar2, Aishwarya J3, Sathish Mallikarjuna2, Nooka Monika Reddy2, Neelakandan Sivathanu1, Sai Venkata Manoj Kotharu4
1Madras Medical College, 2Neurology, Saveetha Medical College and Hospital, 3Saveetha Medical College and Hospital, 4Osmania Medical College
Background:
Combined central and peripheral demyelination lies at the interface between Multiple Sclerosis and Chronic Inflammatory Demyelinating Polyneuropathy (CIDP), but remains poorly defined without standardized diagnostic criteria. Its presentation during pregnancy is infrequent, warranting special consideration given the naturally altered immune function and therapeutic constraints.
Design/Methods:
Single-case report.
Results:
We report a 25-year-old woman, six weeks pregnant, who presented to the clinic with a 3-day history of paresthesia involving both limbs, which progressed to difficulty walking and left-leg buckling. She had experienced similar episodes in the past, including transient diplopia. Examination revealed a predominantly proximal weakness of the left leg with an extensor plantar response, indicating upper motor neuron involvement. Nerve conduction studies indicated peripheral demyelination with severely reduced conduction velocities, absent SNAPs, and prolonged F waves. MRI of the brain showed several focal T2/FLAIR hyperintense lesions. The cervical and thoracic spines also showed multiple short-segment T2-hyperintense lesions suggestive of central demyelination. Antibody testing was negative for aquaporin-4, anti-MOG, and oligoclonal bands, but competitive ELISA were positive for Neurofascin-140 (303ng/dl) and sulfatide IgM antibodies (326ng/dl). These findings pointed to an immune process targeting the nodo-paranodal region. She was diagnosed with combined central and peripheral demyelination and received pulse corticosteroids. She showed full clinical recovery and is under follow-up in the clinic, with rituximab planned as maintenance therapy postpartum.
Conclusions:
This antenatal case of CCPD with dual NF140 and sulfatide antibody positivity further expands the immunological spectrum of the disorder. It highlights the utility of nodo-paranodal antibody testing when standard screening is negative in suspected peripheral demyelinating disorders. It calls attention to the challenges encountered in treating these disorders in pregnancy - the need for balancing maternal recovery against fetal well-being. Systematic reporting and registry collaboration are necessary to determine prognosis and establish safe, evidence-based therapeutic guidelines for this clinically relevant subgroup.
Generative AI Usage
No, did not use generative AI in the drafting or editing in this abstract.
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