PGY3 University of New Mexico School of Medicine PM&R Program Albuquerque, New Mexico
Case Diagnosis: Autoimmune nodopathy.
Case Description: 45 year-old male with a history of alcohol-use disorder and treated syphilis who presented to the ED with a 6 month history of progressive sensorimotor deficits. On initial presentation, symptoms reported to be greater distally vs proximally, and lower extremities more affected than upper extremities. The patient was started on IVIG treatment for CIPD, but continued to experience progressive loss of sensorimotor function (lower>upper) with ultimately flaccid lower extremities. During a subsequent hospitalization, the patient was started on biweekly plasma exchange. Following this, the patient had improvement in his upper extremity motor function and coordination. However, there was no noticeable improvement in his lower extremity symptoms.
Discussion: Recent literature has identified Autoimmune nodopathy (AN) as an emerging subset of chronic inflammatory demyelinating polyneuropathy (CIPD). Despite sharing similar immunopathology and clinical features, AN is distinguished from typical CIPD by disease-specific antibodies that primarily target the Nodes of Ranvier. Additionally, recent research suggests nodopathies have differing treatment responses from those of typical CIDP.
Setting: Inpatient rehabillitaiton
Assessment/Results: EMG findings were suggestive of severe, chronic, active, diffuse, symmetric sensorimotor demyelinating peripheral polyneuropathy, consistent with CIDP. Since that time, labs have been collected and sent to Washington University for suspected nodopathy.
Conclusion: This case highlights the potential benefits of exploring different treatment options for patients with suspected CIPD even prior to obtaining a definitive diagnosis. Further, this case illustrates that delays in treatment initiation may result in significant functional loses.
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