Chronic demyelinating polyneuropathy in systemic lupus erythematosus

Area of Science:

• Neurology
• Rheumatology
• Immunology

Background:

• Systemic lupus erythematosus (SLE) is known to cause neurological complications, including mononeuritis multiplex and acute demyelinating polyneuropathy (Guillain-Barré syndrome).
• Chronic acquired demyelinating polyneuropathy is an infrequently reported neurological manifestation in SLE patients.

Observation:

• Two young female patients presented with progressive weakness and areflexia.
• Cerebrospinal fluid (CSF) analysis revealed elevated protein levels.
• Nerve conduction studies showed significantly slowed velocities.

Findings:

• Sural nerve biopsy in one patient indicated mild loss of myelinated fibers and an increase in thinly myelinated fibers.
• Both patients experienced clinical improvement following steroid therapy.
• The neurological presentation preceded other characteristic systemic features of SLE by several months.

Implications:

• Chronic demyelinating polyneuropathy can be an unusual initial presentation of systemic lupus erythematosus.
• Early recognition of this neurological pattern may aid in the diagnosis of SLE.
• Steroid therapy appears effective in managing this specific SLE-related neuropathy.