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Primary Lateral Sclerosis

Sondra M. M De Antonio, MD, MD and John Francis Bertagnolli, D.O. Reviewed 06/2022
 


BASICS

DESCRIPTION

Primary lateral sclerosis (PLS) is a rare progressive degenerative disease of the corticospinal and corticobulbar tracts (upper motor neurons [UMNs]), which spares the anterior horn ...

DIAGNOSIS

  • PLS is a clinical diagnosis of exclusion.

  • Clinical diagnosis is defined as UMN only involvement for at least 4 years from symptom onset.

  • Multiple diagnostic criteria exist. Consensus diagnosti...

TREATMENT

Treatment is symptom-directed; there is no known cure. 

GENERAL MEASURES

  • Mainly supportive care

  • Nursing home placement or hospice at end stage

  • Discussion of advance directives and completion of ...

ONGOING CARE

PROGNOSIS

  • PLS has not been shown to shorten life expectancy.

  • Median survival after diagnosis is 19 years.

  • An older age at onset is associated with rapid decline in functional status.

COMPLICATIONS

REFERENCES

1
Kosaka  T, Fu  YJ, Shiga  A, et al. Primary lateral sclerosis: upper-motor-predominant amyotrophic lateral sclerosis with frontotemporal lobar degeneration—immunohi...

ADDITIONAL READING

  • Pringle  CE, Hudson  AJ, Munoz  DG, et al. Primary lateral sclerosis. Clinical features, neuropathology and diagnostic criteria. Brain. ...

CODES

ICD10

G12.29 Other motor neuron disease 

SNOMED

81211007 Primary lateral sclerosis 

CLINICAL PEARLS

  • PLS affects only UMNs.

  • Onset is usually asymmetric spasticity in the lower extremities. The patient may present complaining of stiffness, slowness, clumsiness, falls, balance issues, an...

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