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Primary Lateral Sclerosis

A rare, slowly progressive neurodegenerative disease that affects upper motor neurons.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Nöroloji department. Book Appointment →

What is Primary Lateral Sclerosis?

Primary lateral sclerosis (PLS) is a disease that progresses with degeneration of only upper motor neurons; unlike ALS (involvement of both motor neurons), it is slower-progressing and longer-lasting.

The disease usually begins in the legs and over time progresses to the arms and the muscles controlling swallowing and speech. Spasticity (muscle stiffness), weakness, and balance impairment are the main findings.

There is no lower motor neuron involvement in PLS; muscle wasting and fasciculation are not seen. Long-term clinical observation is needed for definitive diagnosis, because ALS can sometimes initially appear similar to PLS.

Symptoms

Progressive muscle stiffness (spasticity) in the legs
Balance impairment and difficulty walking
Weakness with arm involvement
Speech difficulty (pseudobulbar symptoms)
Emotional lability (causeless laughter or crying)
Difficulty holding urine

Risk Factors

More common in men and between ages 40-60
SOD1 and other gene mutations (in a small number of cases)
Family history of motor neuron disease
Long-term exposure to environmental toxins (under investigation)
Smoking

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Unexplained progressive stiffness or weakness in the legs
  • Marked deterioration in gait
  • If speech and swallowing difficulty are added
  • If ALS or motor neuron disease is suspected

Treatment Methods

01
Baclofen or tizanidine for spasticity
02
Preservation of motor function with physiotherapy
03
Speech therapy (if bulbar involvement is present)
04
Assistive devices and ergonomic adjustments
05
Psychosocial support and family counseling

Which Department to Visit?

You can visit our Nöroloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.