Which conditions are included in the differential diagnoses of primary lateral sclerosis (PLS)?

Updated: Sep 06, 2019
  • Author: Carmel Armon, MD, MSc, MHS; Chief Editor: Nicholas Lorenzo, MD, MHCM, CPE, FAAPL  more...
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The most common causes of myelopathy in the age group in which PLS most often occurs are cervical spondylosis and chronic progressive multiple sclerosis (MS). Both are more common than PLS.

Some conditions may present initially with pure upper motor neuron dysfunction, such as progressive multifocal leukoencephalopathy (rarely without the characteristic MRI findings) or spongiform encephalopathy, but they have a rapid course that would preclude consideration of PLS.

If appropriate, also consider and exclude myelopathy associated with human immunodeficiency virus (HIV) infection.

Other disorders to consider in the differential diagnosis of PLS, aside from those in the next section, include the following conditions:

  • Hereditary spastic paraparesis (HSP)

  • Konzo

  • Neurolathyrism

  • Spinocerebellar ataxias

  • Tumors of the spinal cord

  • Low-grade astrocytoma

  • Meningioma

  • Multiple system atrophy

  • Neurosyphilis

  • Oligodendroglioma

  • Olivopontocerebellar atrophy

  • Parkinson-plus syndromes

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