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Neurological Rehabilitation

Frail leg syndrome: A rare variant of ALS

(Kiosk 3) Frail Leg Syndrome: A Rare Variant of ALS

Thursday, November 16, 2023
5:00 PM – 7:00 PM CT

    Primary Author(s)

  • Ricardo J. Fuentes-Saavedra, MD

    Resident Physician
    Montefiore Medical Center/Albert Einstein College of Medicine PM&R Program
    New York, New York

    • Co-Author(s)

  • Eric Martinez, DO

    Resident Physician
    Montefiore Medical Center/Albert Einstein College of Medicine PM&R Program
    New York, New York

  • GS

    Geum Yeon Sim, MD

    Resident Physician
    Montefiore Medical Center/Albert Einstein College of Medicine PM&R Program
    New York, New York

Case Diagnosis: Freil Leg syndrome

Case Description or Program Description: Case of a 44-year-old male without past medical history presents with right foot drop, which started 9 months ago. This began after a fall with minor trauma, not requiring medical attention. Insidious progression to bilateral foot drop pronounced on the right side. Patient reported transient right foot numbness that resolved spontaneously. Patient reported a normal brain MRI which was requested by Neurology.

Physical exam relevant for distal more than proximal weakness predominantly on dorsiflexors, without upper motor neuron signs on September/22. EMG exam on November/22 revealed progressive weakness now with pelvic-perineal pattern with preserved sensation and markedly right atrophy of quadriceps. Noted poor trunk control and preserved reflexes.

Setting: Outpatient Electrodiagnostic lab

Assessment/Results: The electrodiagnostic evaluation showed normal sensory conduction, and reduced CMAP amplitude, without evidence of demyelination or conduction block. Electromyography showed denervating changes including thoracolumbar paraspinals, fibrillation potentials, positive sharp waves, and long-duration polyphasic motor units.

Discussion (relevance): A progressive onset of distal weakness in the absence of sensory loss or upper motor neuron signs in a middle-aged male draws attention toward Leg Amyotrophic Diplegia, also known as a pseudopolyneuritic variant of Amyotrophic Lateral Sclerosis. Much less common than its equivalent Brachial Amyotrophic Diplegia, this variant represents 2.6%-6.3% of motor neuron diseases, has a presentation within 12-24 months, and has a much better prognosis than ALS with a survival rate of 63.9–76.9%.

Conclusions: Progressive lumbosacral motor disease with spared sensation and no signs of upper motor neuron is a rare presentation of ALS in middle-aged males. Early diagnosis and intervention make a difference in the quality of life in patients with higher survival rates compared to ALS.