Distal acquired demyelinating symmetric neuropathy (DADS) is a variant of chronic inflammatory demyelinating polyneuropathy (CIDP) characterized by symmetrical, distal, sensory or sensorimotor involvement. DADS with M-protein (DADS-M) is less responsive to immunotherapy compared to those without M-protein (DADS-I). We report a case of DADS-I with severe clinical presentation viz. early hand involvement with marked wasting, inexcitable peripheral nerves on neurophysiology and poor response to immunotherapy. Despite the unusual presentation, ancillary tests including cerebrospinal fluid analysis, nerve biopsy and nerve ultrasound were supportive of an inflammatory demyelinating polyneuropathy. This case demonstrated the heterogeneity of the disorder and expands the clinical spectrum of DADS neuropathy.
Peripheral neuropathy is a known manifestation of systemic lupus erythematosus. However, the association of primary autoimmune inflammatory neuropathies such as chronic inflammatory demyelinating polyneuropathy (CIDP) with SLE is uncommon. We report a 26-year-old man who simultaneously presented with severe CIDP and photosensitive rash, but was unresponsive to intravenous immunoglobulin infusion and continued to progress. He was found to have underlying SLE and improved with combined corticosteroid and immunosuppressive therapy with oral cyclophosphamide. CIDP with underlying SLE may be more resistant to conventional therapy with IVIG, requiring the addition of other immunosuppressive agents.