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458 - Progressive Ascending Weakness in the Inpatient Rehabilitation Setting: A Textbook Presentation?

Thursday, February 27, 2025

5:00 PM - 6:30 PM MST

Presenting Author(s)

RM

Ryan Mortman, MD

Resident
UPMC
Pittsburgh, Pennsylvania, United States

Co-Author(s)

AG

Amanda Garver, DO

Spinal Cord Injury Fellow
UPMC
Pittsburgh, Pennsylvania, United States
CL

Catherine Lawton, BS

Medical Student
Jacobs School of Medicine and Biomedical Sciences
Buffalo, New York, United States
Amanda L. Harrington, MD (she/her/hers)

Director of Spinal Cord Injury Services, APD PM&R Residency, PD SCIM Fellowship
UPMC, University of Pittsburgh
Pittsburgh, Pennsylvania, United States

Case Diagnosis: Undiagnosed progressive ascending weakness

Case Description: 66-year-old male presented with one month of progressive bilateral lower extremity weakness and bowel/urinary incontinence two months following a respiratory infection. He was empirically treated for Acute Inflammatory Demyelinating Polyneuropathy (AIDP) with no response to plasmapheresis, IV immunoglobulin, or steroids. His weakness progressed to bilateral upper extremities. Multiple neurodiagnostic studies demonstrated length-dependent large fiber sensorimotor polyneuropathy/polyradiculopathy with axonal degeneration. Neuroaxis imaging was negative for cord involvement, tumor, or arteriovenous fistula. A lumbar puncture (LP) noted elevated CD5+ presumptive T-cells. He completed one round of rituximab for possible intravascular lymphoma (IVL) without response. Lastly, he underwent multiple biopsies of the sural nerve, gastrocnemius, and brain dura, all negative for vasculitis or IVL. He was discharged from acute rehabilitation with deteriorating function and a lack of diagnosis. Although neurodiagnostic studies were not consistent with amyotrophic lateral sclerosis (ALS), he was referred to ALS clinic for ongoing evaluation given his progressive symptoms.

Discussions: The differential diagnosis of ascending weakness is expansive1, but this patient’s workup was centered on AIDP, ALS, and IVL. While AIDP similarly presents with ascending weakness, our patient did not respond to treatment, nor did he have electrodiagnostic (absent or prolonged H-reflexes and/or F-wave latencies) or LP findings (albuminocytologic dissociation) to support this diagnosis2. Common electrodiagnostic findings in patients with ALS are fibrillation potentials and spontaneous denervation discharges3, which our patient did not have on four separate studies reviewed by neuromuscular specialists. Lastly, a solid organ biopsy is required for IVL diagnosis4, and our patient had three negative biopsies.

Conclusions: This was a unique case of undiagnosed acute progressive ascending weakness despite multiple diagnostic tests and treatments. It presented an opportunity for the inpatient rehabilitation team to collaborate with multiple specialties to optimize care while navigating his ongoing deterioration in the rehabilitation setting.