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Primary Lateral Sclerosis

Reviewed 06/2018
 


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 diagnosis of exclusion.

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

  • The most recent set of diagnostic criteria was presented by Singer e...

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—immunohistochemical and biochemical...

ADDITIONAL READING

  • Pringle CE, Hudson AJ, Munoz DG, et al. Primary lateral sclerosis. Clinical features, neuropathology and diagnostic criteria. Brain.  1992;115(Pt 2):495–520.

  • Tartaglia MC, Laluz...

CODES

ICD10

G12.29 Other motor neuron disease 

ICD9

335.24 Primary lateral sclerosis 

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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