Primary Lateral Sclerosis
Definition[edit | edit source]
Epidemiology[edit | edit source]
Etiology[edit | edit source]
Clinical Presentation[edit | edit source]
Prognosis[edit | edit source]
Interventions[edit | edit source]
Physical Therapy [edit | edit source]
Medical and Surgical[edit | edit source]
Differential Diagnoses[edit | edit source]
(Statland et al, 2015); (Kuipers-Upmeijer et al, 2001); (Strong & Gordon, 2005)
As PLS is an upper motor neuron issue, there are several potential differential diagnosis. The 2 most likely upper motor neuron diseases that is associated with PLS are …
- Amyotrophic lateral sclerosis (Statland et al, 2015); (Strong & Gordon, 2005)
- ALS presents with lower and/or upper motor deficits and has fast(er) progression; PLS is slower progression and typically only affects upper motor neurons.
- ALS is more commonly associated with stiffness then PLS (47% vs 4%)
- Both ALS and PLS can be assoicated with cognitive issues but is not present in its ‘uncomplicated’ forms (Strong & Gordon, 2005).
- Hereditary spastic paraplegias (Strong & Gordon, 2005)
- HSP has spasticity of the lower limbs; PLS is associated with spasticity as well and slight weakness but spasticity can affect speech (spastic dysarthria)
- Complicated cases of HSP can result in dementia & mental retardation which may mimic cognitive issues possibly present in complicated cases of PLS.
Other potential, but less specific, differential considerations include (Statland et al, 2015)…
- Structural lesions, especially related to the spine (eg. cervical spondylmyelopathy)
- Infection (eg. HIV, syphilis)
- Demyelinating disease (eg. mutliple sclerosis)
- Metabolic / toxic (eg. vitamin E deficiency)
- Neurodegenerative (eg. Parkinsons and parkinson's-plus syndromes)
Special care must be taken when diagnosing as PLS can be misdiagnosed as the above examples, and many more (Kuipers-Upmeijer et al, 2001).