A girl with atypical chronic inflammatory demyelinating polyneuropathy

Abstract

Purpose: Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is a chronic, acquired immune and inflammatory disorder that targets the peripheral nerves, with a progressive or a relapsing-remitting course, presenting with predominant weakness. Though pain is not a typical presentation in CIDP, it has been shown that fibromyalgia patient can have CIDP and respond well to IVIG. Our patient demonstrates a close relationship between CIDP and fibromyalgia. Methods: Case report: A 17 year old girl was admitted with increase falling and progressive difficulty in raising arms for six months. The weakness had a waxing and waning course. Five months after onset of symptoms, she noticed shoulder pain, tiredness and diurnal variation of weakness with severe weakness in the morning. Examination showed multiple sites of tenderness, stiffness and fatiguibility. She was initially suspected to have fibromyalgia with a slightly elevated ESR and anti-dsDNA. Subsequent nerve conduction study confirmed acquired demyelinating sensorimotor polyneuropathy with sural sparing. Lumbar puncture showed protein-cytological dissociation. MRI spine demonstrated gadolinium contrast enhancement of nerve roots at the cauda equina. Result: CIDP was diagnosed and she was started on intravenous immunoglobulin with rapid clinical improvement. Conclusion: The presence of shoulder and neck pain, multiple sites of tenderness and chronic fatigue symptom in our patient lead to the initial diagnosis of fibromyalgia. The predominant fibromyalgia-like presentation of our patient highlighting that neuropathic nature of pain and morning stiffness can be the atypical presentation of CIDP at some stage.