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Differential Diagnosis in
Parkinson’s Disease
Randy M Rosenberg MD FAAN FACP
Assistant Professor of Neurology
Lewis Katz School of Medicine at Temple University
What ISN’T Parkinson’s Disease?
• Parkinson’s does not typically begin with falls
• Parkinson’s is an asymmetrical illness
• Tremor is usually (but not always) a part of the clinical picture
• Look beyond the hands
• Parkinson’s gets better with dopamine replacement
• Parkinson’s get worse over time
• There is essentially no neuroprotection
• Dysautonomia (eg, urinary incontinence, fecal incontinence,
catheterization for urinary retention, persistent erectile failure,
prominent symptomatic orthostatic hypotension)
Differential diagnosis of parkinson's disease
Principles in the Interpretation of a DaT Scan
• In earlyPD there is usually an asymmetrical pattern of reduced
DaT binding starting in the dorsal putamen contralateral to the
clinically most symptomatic body side.
• Activity in the caudate nucleus is relatively preserved in early disease
but will also decline with advancing disease.
• Parkinsonian syndromes (MSA, PSP), there usually will be a
more symmetrical decrease in DaT binding and relatively more
involvement of the caudate nucleus
• In DLB, DaT binding is reduced, typically with bilateral loss of
DaT binding, mainly in the putamen but also in the caudate and
generally with less asymmetry than in PD
DaT Scan in Progressive Parkinson’s Disease
DaT Scan Transport Imaging
Multiple System Atrophy: Clinical Presentation
• Synucleinopathy
• Abnormalities of alpha-synuclein metabolism, resulting in intracellular
deposition
• Highly variable
• These symptoms progress rapidly
• About 1 in 5 MSA patients will suffer a fall in their first year of
disease.
• Patients are disabled by 5 years and die from MSA-related
complications after 9 years of symptom duration on average.
Multiple System Atrophy: Clinical
Presentation
Most common presenting symptoms:
Parkinsonism (MSA-P)
Cerebellar ataxia (MSA-C)
Autonomic failure (previously called Shy
Drager)
Orthostatic hypotension
Urinary incontinence
Impotency
Constipation
Xerostomia
Sudomotor dysfunction
Sleep apnea
MRI in Multiple Sysystem Atrophy
T2:hyperintensities typically present in
the pontocerebellar tracts pons: hot
cross bun sign (MSA-C)
T2: posterior lateral putamenal
hyperintensity
Multiple System Atrophy
Progressive Supranuclear Palsy: Clinical Presentation
The cardinal manifestations of PSP are as follows:
• Supranuclear ophthalmoplegia
• Pseudobulbar palsy
• Prominent neck dystonia (often dystonia in extension)
• Parkinsonism
• Behavioral, cognitive, and gait disturbances that cause
imbalance
• Frequent falls/impaired postural reflexes
Progressive Supranuclear Palsy: Physical
Findings
Findings on physical examination can
include the following:
• Poor postural reflexes,
• Axial rigidity greater than appendicular rigidity
• Dysarthria (monotone with slight hypophonic quality)
• Absence of cogwheeling or tremor
• Widely based and unstable gait
• Bradykinesia with masked facies and a startled
expression
• Retrocollis may be present
PSP: Ocular Motility Disturbance
Slow vertical saccades and square wave jerks on
ocular examination (early signs)
Supranuclear ophthalmoplegia (classic gaze palsy
in PSP)
Downgaze typically involved before upgaze
Eyelid retraction, eyelid opening or closing,
apraxia, blepharospasm, or lid lag
Cognitive Dysfunction in PSP
• Slowed cognitive processing, sequencing and
planning difficulties, mild memory difficulty,
and apathy (generally more prominent in late
disease)
• High apathy scores coupled with low agitation
and anxiety scale scores on Neuropsychiatric
Inventory testing
MRI in Progressive Supranuclear Palsy
Atrophy of the midbrain. “Hummingbird sign”
(Mickey Mouse ears in axial projection)
Progressive Supranuclear Palsy (PSP)
Corticobasilar Degeneration: Motor Findings
Corticoasilar Degeneration: Cortical Findings
Corticobasilar Degeneration
Symptoms of Corticobasilar Degeneration
• Alien Hand
• Sensation of absent control over the movement
of a hand (or occasionally a leg)
• Hand acts independent of conscious voluntary
control
• Complex acts can be performed such as
undoing buttons, removing clothing and
manipulating tools
Alien Hand Syndrome
Your thoughts on this one…?
Differential diagnosis of parkinson's disease

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Differential diagnosis of parkinson's disease

  • 1. Differential Diagnosis in Parkinson’s Disease Randy M Rosenberg MD FAAN FACP Assistant Professor of Neurology Lewis Katz School of Medicine at Temple University
  • 2. What ISN’T Parkinson’s Disease? • Parkinson’s does not typically begin with falls • Parkinson’s is an asymmetrical illness • Tremor is usually (but not always) a part of the clinical picture • Look beyond the hands • Parkinson’s gets better with dopamine replacement • Parkinson’s get worse over time • There is essentially no neuroprotection • Dysautonomia (eg, urinary incontinence, fecal incontinence, catheterization for urinary retention, persistent erectile failure, prominent symptomatic orthostatic hypotension)
  • 4. Principles in the Interpretation of a DaT Scan • In earlyPD there is usually an asymmetrical pattern of reduced DaT binding starting in the dorsal putamen contralateral to the clinically most symptomatic body side. • Activity in the caudate nucleus is relatively preserved in early disease but will also decline with advancing disease. • Parkinsonian syndromes (MSA, PSP), there usually will be a more symmetrical decrease in DaT binding and relatively more involvement of the caudate nucleus • In DLB, DaT binding is reduced, typically with bilateral loss of DaT binding, mainly in the putamen but also in the caudate and generally with less asymmetry than in PD
  • 5. DaT Scan in Progressive Parkinson’s Disease
  • 7. Multiple System Atrophy: Clinical Presentation • Synucleinopathy • Abnormalities of alpha-synuclein metabolism, resulting in intracellular deposition • Highly variable • These symptoms progress rapidly • About 1 in 5 MSA patients will suffer a fall in their first year of disease. • Patients are disabled by 5 years and die from MSA-related complications after 9 years of symptom duration on average.
  • 8. Multiple System Atrophy: Clinical Presentation Most common presenting symptoms: Parkinsonism (MSA-P) Cerebellar ataxia (MSA-C) Autonomic failure (previously called Shy Drager) Orthostatic hypotension Urinary incontinence Impotency Constipation Xerostomia Sudomotor dysfunction Sleep apnea
  • 9. MRI in Multiple Sysystem Atrophy T2:hyperintensities typically present in the pontocerebellar tracts pons: hot cross bun sign (MSA-C) T2: posterior lateral putamenal hyperintensity
  • 11. Progressive Supranuclear Palsy: Clinical Presentation The cardinal manifestations of PSP are as follows: • Supranuclear ophthalmoplegia • Pseudobulbar palsy • Prominent neck dystonia (often dystonia in extension) • Parkinsonism • Behavioral, cognitive, and gait disturbances that cause imbalance • Frequent falls/impaired postural reflexes
  • 12. Progressive Supranuclear Palsy: Physical Findings Findings on physical examination can include the following: • Poor postural reflexes, • Axial rigidity greater than appendicular rigidity • Dysarthria (monotone with slight hypophonic quality) • Absence of cogwheeling or tremor • Widely based and unstable gait • Bradykinesia with masked facies and a startled expression • Retrocollis may be present
  • 13. PSP: Ocular Motility Disturbance Slow vertical saccades and square wave jerks on ocular examination (early signs) Supranuclear ophthalmoplegia (classic gaze palsy in PSP) Downgaze typically involved before upgaze Eyelid retraction, eyelid opening or closing, apraxia, blepharospasm, or lid lag
  • 14. Cognitive Dysfunction in PSP • Slowed cognitive processing, sequencing and planning difficulties, mild memory difficulty, and apathy (generally more prominent in late disease) • High apathy scores coupled with low agitation and anxiety scale scores on Neuropsychiatric Inventory testing
  • 15. MRI in Progressive Supranuclear Palsy Atrophy of the midbrain. “Hummingbird sign” (Mickey Mouse ears in axial projection)
  • 20. Symptoms of Corticobasilar Degeneration • Alien Hand • Sensation of absent control over the movement of a hand (or occasionally a leg) • Hand acts independent of conscious voluntary control • Complex acts can be performed such as undoing buttons, removing clothing and manipulating tools
  • 22. Your thoughts on this one…?