Major criteria:
(1) onset after age 40
(2) progressive course
(3) supranuclear gaze palsy affecting downward gaze
Additional criteria:
(1) early postural instability with/without falls
(2) bradykinesia
(3) early dysphagia or dysarthria
(4) prominent frontal lobe syndrome (bradyphrenia, apathy, perseveration, grasping)
(5) prominent axial dystonia (neck and/or trunk hyperextended)
Exclusion criteria - all of the following must be absent:
(1) prominent cerebellar signs
(2) unexplained polyneuropathy
(3) unexplained dysautonomic symptoms (early incontinence or symptomatic postural hypotension)
(4) focal sensory deficits, primary or cortical
(5) unilateral limb apraxia or alien-hand syndrome
(6) Parkinsonism with full or persistent response to levodopa
where:
• The alien hand phenomenon is when the hand "behaves" in an uncooperative or foreign fashion.
• Perseveration refers to continuation of an action after the stimulus has stopped.
• Bradyphrenia refers to extreme fatigability of interest or initiative.
|
Major Criteria
|
Additional
|
Exclusion
|
Diagnosis
|
|
all 3
|
>= 3
|
0
|
probable
|
|
all 3
|
2
|
0
|
possible
|
|
all 3
|
1
|
0
|
indeterminate
|
|
any
|
any
|
>= 1
|
excluded
|
