Disclosures: Weston Rogers: No financial relationships or conflicts of interest

Case Diagnosis: Amyotrophic lateral sclerosis in a patient with history of motor vehicle crash causing residual neurological deficits.

Case Description or Program Description: A 49-year-old male with cervical stenosis after MVC s/p corpectomy with discectomies of C5-C6 three years prior presented with progressive bilateral (R>L) upper extremity weakness. Physical exam showed spasticity, hyporeflexia, and right finger flexor contractures. Recent MRI showed myelomalacia involving C5-C6 attributed to post-surgical changes without indication for intervention per Neurosurgery. Electromyography (EMG) results showed active denervation in cervical region and complex repetitive discharges in right thoracic paraspinals, concerning for polyradiculopathy vs motor neuron disease. Findings were attributed to central cord syndrome as sequelae of the initial insult. Patient was given baclofen and Botox injections for spasticity and grew weaker despite ongoing therapy and prior surgical decompression.

Setting: Outpatient spine clinic.

Assessment/Results: 18 months later, patient presented to the ED with respiratory distress, worsening weakness, and increased dependence for self-care. Emergency intubation was performed for acute respiratory failure. Repeat EMG/NCS showed widespread acute and chronic denervation in multiple segments without evidence of demyelinating polyneuropathy. Amyotrophic Lateral Sclerosis (ALS) was diagnosed and Riluzole therapy was started. Patient was stably discharged after tracheostomy and PEG placement.

Discussion (relevance): ALS typically presents as an insidious, progressive upper and lower motor neuron disease spreading to other body segments. In this case, the presentation of cervical stenosis after MVC led to the presumptive diagnosis of cervical polyradiculopathy. Cervical polyradiculopathy can mimic ALS so evaluating bulbar muscles and thoracic paraspinals with EMG could show benefit. Additionally, the progression of radiculopathy is expected to be stepwise with periodic improvement. Progressive weakness without improvement should increase suspicion for ALS.

Conclusions: This case provides support for repeat diagnostic studies in patients with a history of SCI that continue to grow weaker and show no improvement with therapy. Evaluation includes EMG/NCS and MRI, especially if bulbar symptoms are present.

Level of Evidence: Level V

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PM&R Meeting Abstracts - https://pmrjabstracts.org/abstract/diagnosis-of-amyotrophic-lateral-sclerosis-in-a-patient-with-residual-neurological-deficits-following-motor-vehicle-crash/