Neurodegenerative

Question 1

Progressive Supranuclear Palsy

Answer 1

Most common Parkinson -Plus syndrome
Progressive degeneration of brain structures superior to occulomotor nuclei , SNr affected

Limitation of vertical gaze, especially downwards

Rapid progression, 5-6yr survival
Onset 50-60yo
Early falls
Truncal rigidity>limb rigidity
No tremor

Gait: axial rigidity, early falls, early freezing

Question 2

Multiple Systems Atrophy

Answer 2

Parkinson-Plus syndrome
Corticospinal signs (Babinski & hyperreflexia)
Orthodtatic hypotension, autonomic dysfunction, neuropathy
Can have Cb signs, gaze evoked nystagmus

Early urinate dysfx
Cog dysfx
Prognosis 9-10 yrs
Onset 54.3 hrs

Gait: wide based ataxic gait, impaired stability and early falls

Question 3

Corticobasalganglionic degeneration

Answer 3

Parkinson -Plus syndrome
Atrophy of frontal and parietal lobes, loss of DA in SNr
Initially one side then progresses- limb apraxia, usually 1 arm (alien limb)
Progressive dementia

Visual or spatial neglect
Impaired eye movements, usually NOT downgaze

Severe disability and death 6-8 yrs
Onset 60-80y (mean 63)

Gait: freezing of gait, falls, alien limb, apraxia

Question 4

Parkinson- Plus syndromes

Answer 4

Less responsive to Levodopa
Early onset dementia or instability
More symmetrical onset
Trunk sx>limb sx
Motor apraxia
Ocular signs
Hypotension and incontinence early in disease

Question 5

Left Body Dementia

Answer 5

Parkinson-Plus syndrome
Early cog sx, visual hallucinations 
More rigidity than PD but less tremor
Fluctuations of cog, attention, alertness
Lack of motor asymmetry vs PD

Median survival 8 yrs
Slow onset (50-85 yrs)

Gait: less lateralization than PD, narrow gait, early falls