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4/2/2021 Parkinson disease | Radiology Reference Article | Radiopaedia.org
Dr Daniel J Bell ◉ and Assoc Prof Frank Gaillard ◉ ◈ et al.

Parkinson disease (PD), also known as idiopathic parkinsonism, is a neurodegenerative disease and movement
disorder characterized by resting tremor, rigidity and hypokinesia due to progressive degeneration of dopaminergic neurons in the
substantia nigra.

On this page:
Article:

Epidemiology
Clinical presentation
Pathology
Radiographic features
Treatment and prognosis
History and etymology
Differential diagnosis
Related articles
References

Images:

Cases and figures

Epidemiology

Parkinson disease is by far the most common cause of the parkinsonian syndrome, accounting for approximately 80% of cases (the
remainder being due to other neurodegenerative diseases, such as Lewy body dementia) 1.

The most common form is encountered in elderly patients and is common, seen in 2-4% of all individuals older than 65 years of age.

A juvenile form of Parkinson disease is also recognized, manifesting between 20-40 years of age 1.

The majority of cases (85-90%) are sporadic. However, 10-15% of patients have a positive family history 1.

Clinical presentation

Parkinson disease is characterized by both motor and non-motor clinical features. The classic cardinal motor features of Parkinson
disease, which are often asymmetric, include:

1. resting tremor
classically 5 Hz 'pill-rolling' resting tremor
most prominent in the distal upper limbs
2. rigidity
often described as 'cog-wheel' in nature in the limbs due to superimposed tremor
also affects the trunk and contributes to the stooped flexed posture that is often seen
3. bradykinesia
typically decrementing in nature and accompanied by hypokinesia
many manifestations:
e.g. in the gait, manifests as a festinating and shuffling short-steppage gait, that characteristically has freezing, turning
en bloc, and reduced arm swing
e.g. in writing, manifests as progressive micrographia
e.g. in the face, manifests as hypomimia with decreased eye blinking
4. postural instability
sometimes added as a fourth cardinal feature 3
is generally a late feature

Non-motor features include:

constipation: often an early symptom pre-dating the cardinal features

anosmia or hyposmia: often an early symptom predating the cardinal features
REM sleep behavioral disorder
autonomic dysfunction
postural hypotension: almost 20% at diagnosis, >1/3 patients by 7 years post diagnosis 17
psychosis, especially visual hallucinations (reported to occur in 6-75% of patients (most reports suggest an incidence of 25-50%),
more frequently in patients treated with dopaminergic medication 9,10)
bradyphrenia and dementia: generally a late feature
in contrast, Lewy body dementia has cognitive impairment either preceding or at most within 12 months of clinical onset of
parkinsonian symptoms 2
fatigue and somnolence

Pathology
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The dopaminergic tract is predominantly affected in Parkinson disease, and histologically, it is characterized by nigrostriatal
dopaminergic degeneration leading to neuronal loss in the substantia nigra pars compacta, most conspicuous in the ventrolateral tier of
neurons 11. A number of other regions including parts of the basal ganglia, brainstem, autonomic nervous system and cerebral cortex 3.

At least eleven genes have been implicated in various forms of Parkinson disease 3. Interestingly depending on which genes are
involved, various clinical features are more or less prominent (e.g. Kufor-Rakeb syndrome).

Even more interestingly not all mutations result in Lewy bodies. For example, juvenile Parkinson disease has been linked to mutations
in the PARK2 gene, which encodes for the enzyme ubiquitin ligase-L3. In normal individuals, ubiquitin ligase-L3 is involved in
ubiquitination of alpha-synuclein (the main component of Lewy bodies) and allows the formation of Lewy bodies. In patients with
juvenile Parkinson disease, its function is impaired, and the formation of Lewy bodies is impossible. This finding suggests that Lewy
bodies cannot be thought of as synonymous with, and causative of Parkinson disease. Perhaps even Lewy bodies play a protective role in
other forms of Parkinson disease, which manifests 20-40 years later 1.

Radiographic features

Initial imaging findings are subtle and only potentially seen on MRI. With advanced disease, non-specific generalized minor cerebral
volume loss can be demonstrated.

MRI

Loss of the normal swallow tail appearance of susceptibility signal pattern in the substantia nigra on axial imaging is perhaps the most
promising diagnostic sign 12. Apart from these changes, the signal intensity in substantia nigra depends on loss of neuromelanin and
iron accumulation. In addition to aiding diagnosis, MRI is also used to identify features which may indicate secondary parkinsonism
rather than primary disease, such as extensive small vessel ischemic change.

Features of Parkinson disease include 1:

T1
may show mild hyperintensity of compact and reticular parts of the substantia nigra and red nuclei (due to iron
accumulation) 1
may show loss of normal slight hyperintensity in substantia nigra due to loss of neuromelanin 13

T2* (GRE/SWI)
absent swallow tail sign
nigrosome-1 is usually SWI hyperintense, but this is lost in Parkinson disease
reported diagnostic accuracy of over 90%, with a 100% sensitivity and negative predictive value, 95% specificity, and
69% positive predictive value being reported in one study 12
may show loss of normal susceptibility signal drop-out of the substantia nigra and red nuclei (due to loss of melanin-
containing neurons)
dot-like areas of hyperintensity in the compact part of the substantia nigra 1
may show a confluence of the normal hypointense regions of substantia nigra (due to iron accumulation) 1

Recent studies with ultra-high-field MRI (7 T) have shown promising results regarding both sensitivity and specificity 4,5.

Nuclear medicine

Both SPECT and PET tracers can be used with high sensitivity for assessment of presynaptic dopaminergic deficits 6,8.

I-123 ioflupane SPECT

I-123 ioflupane (brand name DaTScan) is taken up by presynaptic dopamine transporters that are abundant in areas rich in axonal
synapses form dopaminergic neurons. In the setting of Parkinson disease, the neurons have their cell bodies in the substantia nigra and
project axons into the corpus striatum. Imaging with I-123 ioflupane, therefore, demonstrates a lack of normal uptake in the corpus
striatum in individuals with Parkinson's disease 16.

This appears as a loss of the normal comma-shaped or crescent-shaped tracer uptake in the striatum. Instead, a period-shaped or oval-
shaped uptake is seen within the caudate nucleus head without tracer uptake in the putamen. Eventually, even caudate uptake reduces
16. Quantitative assessment reveals reduced uptake in the putamen compared to norms.

Differentiation between Parkinson disease and atypical parkinsonism is also possible, with different tracers 7,8.

Treatment and prognosis

The mainstay of treatment is medical. In patients with refractory symptoms, deep brain stimulation may be useful 15.

History and etymology

Parkinson disease was first described by British physician James Parkinson (1755-1824) in his 1817 seminal work "An Essay on the
Shaking Palsy", which was reproduced in its entirety in 2002 by The Journal of Neuropsychiatry and Clinical Neurosciences 14. In this
work he describes a 'shaking palsy' as an "involuntary tremulous motion, with lessened muscular power, in parts not in action and even
when supported; with a propensity to bend the trunk forward, and to pass from a walking to a running pace; the senses and intellects
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being uninjured" 14. Remarkably, other than his comment regarding dementia not being a feature of the disease, his original clinical
descriptions are still accurate after more than 200 years.

Differential diagnosis

There is significant overlap between many neurodegenerative diseases, and Parkinson disease is no exception. Clinically the differential
includes 1,3:

dementia with Lewy bodies

dementia is clinically evident before, concurrently or at most within 12 months of onset of parkinsonian symptoms 2
multiple system atrophy (MSA)
progressive supranuclear palsy (PSP)
corticobasal degeneration
cerebrovascular disease
Perry syndrome
postencephalitic parkinsonism
metabolic diseases with parkinsonian signs and symptoms: basal ganglia signal abnormalities are usually more pronounced 1
Wilson disease
manganism
chronic hepatitis

Quiz questions
References
Related Radiopaedia articles

Neurodegenerative diseases

Neurodegenerative diseases are legion and their classification just as protean. A useful approach is to divide them according to
underlying pathological process, although even using this schema, there is much overlap and thus resulting confusion.

neurodegenerative MRI brain (an approach)

measurements and ratios
midbrain to pons area ratio (for PSP)
Magnetic Resonance Parkinsonism Index (MRPI) (for PSP)
frontal horn width to intercaudate distance ratio (FH/CC) (for Huntington disease)
intercaudate distance to inner table width ratio (CC/IT) (for Huntington disease)
signs
hummingbird sign (of PSP)
Mickey Mouse sign (of PSP)
morning glory sign (of PSP)
hot cross bun sign (of MSA-C)
hockey stick sign (of Creutzfeldt-Jakob disease)
pulvinar sign (of Creutzfeldt-Jakob disease)
scoring systems
Fazekas scale for white matter lesions
posterior atrophy score of parietal atrophy (PA/PCA) (Koedam score)
medial temporal lobe atrophy score (MTA score)
global cortical atrophy scale (GCA scale)
neurodegenerative diseases
synucleinopathies
diseases with Lewy bodies
Parkinson disease
Parkinson-plus syndrome
Lewy body disease
multiple systemic atrophy (MSA)
Shy-Drager syndrome
MSA-P (striatonigral degeneration)
putaminal rim sign
olivopontocerebellar atrophy (MSA-C)
tauopathies
Alzheimer disease
typical/classical Alzheimer disease
variant (e.g. posterior cortical atrophy)
chronic traumatic encephalopathy (CTE)
corticobasal degeneration
frontotemporal lobar degeneration (FTLD) (not all are tau)
behavioral variant frontotemporal dementia (bvFTLD)
language variant frontotemporal dementia (lvFTLD) (primary progressive aphasia (PPA))
progressive non-fluent aphasia (PNFA)
semantic dementia
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logopenic dementia
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right temporal variant frontotemporal dementia
Pick disease
progressive supranuclear palsy (PSP)
amyloidoses
cerebral amyloid angiopathy (CAA)
Boston criteria for cerebral amyloid angiopathy
modified Boston criteria for cerebral amyloid angiopathy
Edinburgh criteria for lobar intracerebral hemorrhage associated with cerebral amyloid angiopathy
transthyretine-associated cerebral amyloidosis
neuronal intranuclear hyaline inclusion disease (NIHID)
TDP-43 proteinopathies
amyotrophic lateral sclerosis (ALS)
frontotemporal lobar degeneration (FTLD) (not all are TDP-43)
limbic-predominant age-related TDP-43 encephalopathy
Perry syndrome
spinocerebellar ataxias
Huntington disease
hereditary spastic paraplegia
clinically unclassifiable parkinsonism (CUP)
Unverricht-Lundborg disease
prion diseases (not always included as neurodegenerative)
Creutzfeldt-Jakob disease (sporadic, variant, familial, and iatrogenic)
Brownell-Oppenheimer variant of sporadic Creutzfeldt-Jakob disease
Heidenhain variant of sporadic Creutzfeldt-Jakob disease
fatal familial insomnia
Gerstmann-Straussler-Scheinker disease
kuru
variably protease-sensitive prionopathy

Promoted articles (advertising)

Medicinal Plants and Natural Products as Potential Sources for Antiparkinson Drugs
José-Luis Ríos et al., Planta Medica, 2016

Seeing Parkinson Disease in the Retina

Jonathan B. Lin et al., JAMA Ophthalmology, 2020

Hypothesis of Ascension in Idiopathic Parkinson’s Disease

L. Klingelhoefer et al., Aktuelle Neurologie, 2017

The “Sick-but-not-Dead” Phenomenon Applied to Catecholamine Deficiency in Neurodegenerative Diseases

David S. Goldstein, Seminars in Neurology, 2020

Preliminary Evidence for Neurodegenerative Changes in the Substantia Nigra of Rett Syndrome
C. A. Kitt et al., Neuropediatrics, 1995

Maternal Heart Health Shown to Predict Cardiovascular Disease Onset in Offspring

Cardiology Advisor, 2020

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Article information
rID: 13027
System: Central Nervous System
Tags: rg_40_1_edit, neurodegenerative, neurodegenerative
Synonyms or Alternate Spellings:

Parkinson disease
Parkinson disease (PD)
Parkinson's disease (PD)
Idiopathic parkinsonism

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