Table 2.

Clinical features

Disorder	Clinical features
MSA	Age at onset >30 years Parkinsonism (hypokinesia, rigidity > tremor) Cerebellar signs: gait ataxia, dysarthria, limb ataxia Ocular motor signs: impaired vestibulo-ocular reflex suppression, gaze-evoked nystagmus, downbeat nystagmus, square wave jerks Pyramidal signs: positive Babinski sign, hyperreflexia > spasticity Autonomic signs: orthostatic hypotension, urinary incontinence/urgency, erectile dysfunction, anhidrosis Dysphagia Dystonia (laryngeal stridor), antecollis (DD: dystonic, due to myopathy) Sleep apnea, rapid eye movement sleep behavior disorder Executive dysfunction, cognitive impairment Depression, anxiety Pain
PSP	Age at onset ≥40 years (carriers of rare MAPT mutations may have an earlier onset) Vertical supranuclear gaze palsy (downward > upward) Decreased velocity of saccades (vertical > horizontal), frequent square wave jerks Impaired postural reflexes with falls (typically backwards) Rigidity (axial > limbs), hypokinesia (symmetric, can be asymmetric in PSP-P) > tremor Dysarthria, dysphagia, pathological crying/laughter Freezing of gait, start hesitation ‘Astonished facies’ (Cowper’s sign), overactivity of the frontalis muscle, ‘apraxia of eyelid opening’, reducing blinking Behavioral changes, executive dysfunction, impaired inhibition, apathy, memory dysfunction, slowed thinking Speech apraxia (effortful slow speech with dysprosody and buccofacial apraxia), non-fluent aphasia (PSP-SL) Alien limb phenomenon, limb rigidity/hypokinesia/apraxia/myoclonus, cortical sensory deficit (PSP-CBS)
CBD	Age at onset ≥50 for probable sporadic CBD, no minimum age for possible CBD Parkinsonism (rigidity and bradykinesia typically starting in one limb > tremor, asymmetric) Dystonia (typically of one limb), contractures Stimulus-sensitive myoclonus Limb and ideomotor apraxia, oro-buccal apraxia Alien limb phenomenon (arm > leg) Cortical sensory deficits Depression, behavioral changes, compulsive behavior Executive dysfunction, speech apraxia, non-fluent aphasia, visuospatial deficits
bvFTD	Behavioral changes (disinhibition, apathy/inertia, loss of empathy, reduced social interest) Perseverative stereotyped movements, including language stereotypes Hyperorality (binge eating, consumption of inedible objects, altered food preferences) Executive dysfunction Parkinsonism (rigidity, bradykinesia, gait impairment, postural tremor)
svPPA	Impaired single-word comprehension, impaired object naming (anomia) Impaired object knowledge Dyslexia, dysgraphia
navFTD	Speech apraxia, agrammatism, effortful speech, impaired articulation and prosody
lvPPA	Impaired single-word retrieval, reduced speech rate, pauses, vague sentences, erroneous repetition due to short-term memory deficits
FTD-MND	FTD associated with signs of motor neuron disease