November 10, 2015: Primary Lateral Sclerosis
November 10, 2015
Primary Lateral Sclerosis
In 1874, Jean-Martin Charcot first described PLS as a "primary sclerosis of the lateral columns." It is currently recognized as a rare form of motor neuron disease affecting only upper motor neurons and exhibiting a slowly progressive course. Unlike ALS, survival potential is up to a few decades.
Typical PLS begins with unilateral lower extremity involvement characterized by spasticity more pronounced than weakness, and with progression to the trunks, arms, and bulbar muscles. When bulbar signs occur, they classically evolve from dysarthria to dysphagia and pseudobulbar affect, and even anarthria.
- Barohn RJ. Clinical Spectrum of Motor Neuron Disorders. Continuum Lifelong Learning Neurol 2009; 15(1).
- Singer MA, Stagland JM, Wolfe GI, Barohn RJ. Primary lateral sclerosis. Muscle Nerve 2007; 35: 291–302.
Submitted by Sarah Wesley, MD, Department of Neurology, Mount Sinai Beth Israel Medical Center.
Disclosures: Dr. Wesley is a member of the Resident and Fellow Section of Neurology.