Combined central and peripheral demyelination: a case report resembling encephalomyeloradiculoneuropathy

Area of Science:

• Neurology
• Immunology
• Infectious Diseases

Background:

• Combined central and peripheral demyelination (CCPD) is an exceptionally rare autoimmune disorder affecting both the central and peripheral nervous systems.
• The etiology and optimal treatment strategies for CCPD remain poorly understood due to its rarity and heterogeneous presentation.

Observation:

• A 14-year-old patient presented with acute myelitis (AM), later developing symptoms indicative of Guillain-Barré syndrome (GBS).
• Nerve conduction studies (NCV) and cerebrospinal fluid (CSF) analysis confirmed the diagnosis of GBS.
• The patient exhibited abdominal pain and abnormal liver function, identified as hepatitis E virus (HEV) infection, potentially triggering the neurological symptoms.

Findings:

• The patient received a combination of corticosteroids, intravenous immunoglobulin, and Rituximab.
• Therapeutic intervention resulted in notable symptom relief and clinical recovery during follow-up.
• This case suggests that CCPD, despite its severity, may be responsive to immunotherapy and potentially reversible.

Implications:

• This report underscores the importance of considering HEV infection as a potential trigger for GBS and CCPD.
• The successful treatment outcome highlights the potential efficacy of immunomodulatory therapies in managing CCPD.
• There is a critical need for standardized diagnostic criteria and evidence-based treatment guidelines for this rare condition.