Primary Lateral Sclerosis (PLS) can be diagnosed if certain findings are present. The authors are from multiple institutions from around the world participating in the 2nd International PLS Conference.
Criteria:
(1) age >= 25 years
(2) progressive upper motor neuron dysfunction for >= 2 years
(3) signs of upper motor neuron dysfunction in >=2 of 3 regions (bulbar, upper extremities, lower extremities)
(4) absence of certain findings (below)
Findings that should be absent:
(1) sensory symptoms not explained by a comorbid condition
(2) active lower motor neuron degeneration
(3) alternative cause of upper motor neuron dysfunction that can explain findings
Signs of upper motor neuron pathology:
(1) spasticity and associated weakness
(2) pathological hyperreflexia (Hoffman's sign, bilateral Babinski)
(3) pseudobulbar affect
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Absence of Significant Active Lower Motor Neuron Degeneration from Onset
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Diagnostic Certainty
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> 4 years
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definite
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2 to 4 years
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probable
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