Rapid progression of gait impairment requiring regular use of wheelchair within 5 years of onset.
Absence of progression of motor symptoms or signs over 5 or more years unless related to treatment.
Early bulbar dysfunction: severe dysphonia or dysarthria or severe dysphagia within first 5 years.
Inspiratory respiratory dysfunction: either diurnal or nocturnal inspiratory stridor or frequent inspiratory sighs.
Severe autonomic failure in the first 5 years of the disease. E.g., (a) severe orthostatic decrease of blood pressure within 3 min of standing by at least 30 mm Hg systolic or 15 mm Hg diastolic or (b) severe urinary retention or urinary incontinence in the first 5 years of the disease.
Recurrent (> 1/year) falls because of impaired balance within 3 years of disease.
Disproportionate anterocollis (dystonic) or contractures of hand or feet within the first 10 years.
Absence of any of the common nonmotor features of disease despite 5 years of the disease duration including sleep disorders, autonomic dysfunction, hyposmia, neuropsychiatric disorders (depression, anxiety, or hallucinations).
Otherwise-unexplained pyramidal tract signs, defined as pyramidal weakness or clear pathologic hyperreflexia.
Bilateral symmetric parkinsonism. The patient or caregiver reports bilateral symptom onset with no side predominance and no side predominance is observed on objective examination.