Abstract | December 20, 2022
Atypical Presentation of a Rare Subtype of Chronic Inflammatory Demyelinating Polyneuropathy
Learning Objectives
- Describe the etiology and typical presentation of chronic inflammatory demyelinating polyneuropathy.
- Name several differential diagnoses for chronic inflammatory demyelinating polyneuropathy.
- Describe the treatment options for chronic inflammatory demyelinating polyneuropathy.
Introduction: Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune-mediated progressive disorder that is often difficult to diagnose due to its shared similarities with many other neurologic conditions and variations in presentation owing to its various subtypes. The most common subtype of CIDP is symmetric sensorimotor, which presents with a gradual onset of symmetrical distal and proximal muscle weakness. However, other subtypes present differently, and often patients do not have a typical presentation. CIDP is an important disorder for clinicians to be aware of, as when left untreated, it can lead to multiple relapses and severe disability.
Case Presentation: This case report details the diagnostic work up of one particular patient with both asymmetric peripheral nerve and cranial nerve involvement, both of which are rather uncommon presentations of CIDP according to recent literature. The aim of this case report is not only to bring to light the many ways CIDP may manifest in a given patient (thus adding to a physician’s list of neurologic differentials) but to guide medical decision making in patients with less common conditions.
Final/Working Diagnosis: Approximately 9 years after the patient’s initial presentation with cranial nerve palsy, a diagnosis of CIDP was made.
Management/Outcome: Our patient was responsive to IVIG therapy at presentation of first exacerbation which served both therapeutic and diagnostic purposes. Few patients are responsive to mono-therapy when needed to be treated chronically and additional add-on therapies such as corticosteroids, plasmapheresis and immunosuppressants like mycophenolate, azathioprine and cyclophosphamide. Our patient was discharged with PT orders and it was decided to initiate regular IVIG monthly with the addition of mycophenolate mofetil for immunosuppression. Careful consideration of pain management, physical therapy for ambulatory support, and various other modalities to manage symptoms are also crucial to improve the patient’s quality of life and minimize long term sequelae when a patient reaches remission.
