MRI in in Parkinsonism and Extrapyramidal Disorder MRI in in Parkinsonism and Extrapyramidal Disorder MRI in in Parkinsonism and Extrapyramidal Disorder

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Prof. Dr KRAINIK Alexandre1,2, Dr HOANG Duc Ha3
1Department of Neuroradiology and MRI – University Hospital Grenoble / University Grenoble Alpes
2UMS IRMaGe / Grenoble Institut of Neurosciences
3Department of Radiology, Hai Phong International Hospital
No conflict of Interest
MRI in in Parkinsonism and Extrapyramidal Disorders
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Learning Objectives
• To know imaging diagnosis approach
• To know advantages of MRI
• To recognize semiological spectrum of neurodegenerative
diseases (Park+)

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Diagnoses & Etiologies
Etiology inquiry
• Vascular
– Art, Cap, Veins
– Malformations
– Hematology
• Infection
– Pyogenes
– Virus
– Fungus
– Prions,…
• Inflammatory
– Granulomatosis
– Auto-immune,…
• Trauma
– Accident
– Iatrogenic
Diagnostic goals
• Positive
• Differential
• Severity
• Etiology (causal)
• Risk factors
• Associated diseases
• Pre/Per/Post Care
• Toxic
– Internal
• Endocrine
• Metabolic
– External
• Poison, AODs
• Iatrogenic
• Tumor
– Primitive
– Secondary
– Lymphoma,…
– Paraneo
• Degenerative
• Developmental
• Variants
• « Idiopathic »
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Parkinson’s Disease
• Account for 60-75 % of parkinsonian syndromes
• Sporadic neurodegenerative disease (1-2% > 65 yo)
• Additional hereditary and toxic causes
• Tremor, akinesia, hypertonia
• Asymmetry common
• Dysautonomic disorders
• Cognitive disorders
• L-DOPA responsive

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Anatomo-patho-physiological model
GPe
MidB/SC
STRIATUM
D2 D1
GPi / SNr
PPN
PM M1
THALAMUS
SNc
STN
• D1 (direct path): movement facilitation
• D2 (indirect path): movement inhibition
Dopaminergic neuronal loss of
SNc (striatal projection)
Iron burden in SNc
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BG anatomy

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In PD, conventional MRI used to be … normal

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• Neuromelanin (NM) :
– SNc & locus
coerulus Decrease
in PD
– Paramagnetic =
hyperT1 / hypoT2*
 In PD :
 Loss of hyper T1 of
SNc
Controls Early PD
Advanced MRI in PD
Late PD
3T, FSE T1WI,
600/14/90°nex=8; th=2,5
duration = 12 mn
Ohtsuka, Neurosci let 2013

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The ‘Swallow Tail’ Appearance of the normal nigrosome 1
– A New Accurate Test of Parkinson’s Disease: Schwarz et al.2014
Hypersignal of nigrosome 1 using SWI
SN
N1
PD: loss of nigrosome 1 on SWI

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3T , Philips HR-SWI
3D gradient echo
(FEEPI, TR/TE 60/30, echo train
length 5, Flip angle 19°, number of slices:
70, voxel size0.55 x 0.55 x 0.7 mm, TA : 4
minutes 26 seconds, only magnitude image
used).
PD
PD Control
Control
PD: loss of nigrosome 1 on SWI
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Swan
3 T, GE
Comparison of 3T and 7T susceptibility-weighted angiography of the substantia nigra in diagnosing Parkinson disease.
Cosottini M, AJNR Am J Neuroradiol. 2015
7T 3T
SNr : reticulata
SNcv : compacta ventralis
SNcd : compacta dorsalis
PD: loss of nigrosome 1 on SWI

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Parkinson « + »
• Progressive supranuclear palsy (PSP)
• Multisystem atrophy (MSA)
• Corticobasal degeneration (CBD)
• Lewy bodies disease (LBD)

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Progressive supranuclear palsy (PSP)
• Neurodegenerative disease, tauopathy
• 6th decade
• Prevalence : 1.4/100 000
• Parkinsonian syndrome
• Symmetrical
• Rigidity, gait disorder, no tremor
• Frontal syndrome
• Oculomotor impairment: vertical gaze
• Poorly L-dopa responsive

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Progressive supranuclear palsy (PSP)
• MRI: mesencephalic atrophy
– Posterior tegmental atrophy: Morning glory sign
– Hollow superior aspect of the mesencephalon: Hummingbird sign
– V3 enlargement, interpedoncular enlargement Oba Neurology 2005;64:2050–2055
Adachi, Magn Res Med Sci 2004

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A sporadic, progressive, adult (>30 y) onset disease characterized by:
1.Autonomic failure involving urinary incontinence (inability to control the release
of urine from the bladder, with erectile dysfunction in males) or an orthostatic
decrease of blood pressure within 3 min of standing by at least 30 mm Hg systolic
or 15 mm Hg diastolic and
2.Poorly levodopa-responsive parkinsonism (bradykinesia with rigidity, tremor,
or postural instability) [MSAp] or
3.A cerebellar syndrome (gait ataxia with cerebellar dysarthria, limb ataxia, or
cerebellar oculomotor dysfunction) [MSAc]
Second consensus statement on the
diagnosis of multiple system atrophy, Neurology 2008
Multisystem Atrophy (MSA)

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T2* T1 IR T2
• MRI: putaminal lesions
– Lateral putaminal HyperT2/HypoT1 Savoiardo, Neurology 1997
– Posterior putaminal hypointensity T2* (< 60ans) (1.5T !) Atsuhiko, J Neurol Sci 2015
Multisystem Atrophy-putaminal (MSAp)

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Multisystem Atrophy-putaminal (MSAp)

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• MRI: « hot cross bun sign »
– Hyper T2 of pontine transversal fibers, medial portion of middle cerebellar peduncles
– Hypo T2 of cortico-spinal tracts and superior cerebellar peduncles Savoiardo, Neurology 1997
– 80 % MSAc, non-specific (spinocerebellar ataxia)
Multisystem Atrophy-cerebellar (MSAc)
Best seen on T2* !
Deguchi J neurol 2015

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• MRI: olivo-ponto-cerebellar atrophy
– Olivar abnormalities (rare): atrophy, hypersignal T2
– Pontine and peducular abnormalities: sensitive but poorly specific Schrag, JNNP 1998
– Often associated with MSAp
Multisystem Atrophy-cerebellar (MSAc)

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Mes. Pont Mes./Pont
PD PSP MSA
• MRI: brain stem profile Oba, Neurology 2005
– Volumetric approach provides similar results Paviour, Mov Dis 2006
Differential diagnosis PD/PSP/MSAc
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Dorsolateral Nigral Hyperintensity on 3.0T Susceptibility-Weighted
Imaging in Neurodegenerative Parkinsonism, Mov Disorders, 2015
Nigrosome 1 loss
In PD, MSA, PSP

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• Sporadic neurodegenerative disease, 6th decade
• Subcortical regions: SN pars compacta
• Cortical regions: pericentral
• Akinesia/rigidity: asymmetrical with apraxy
• Epicritical sensitivity disorder
• No cognitive disorder on onset
• Poorly levodopa sensitive
• Asymmetrical pericentral atrophy
• Ipsilateral atrophy of the cerebral peduncle
• WMH (neuronal loss)
• No BG abnormality,…
Corticobasal degeneration

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Corticobasal degeneration

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• Neurodegenerative disease
• 2nd cause of dementia in elderly
• Cholinergic dysfunction
• Lewy Bodies = eosinophilic neuronal inclusion
• Brainstem, BG, limbic lobe, visual cortex
• Progressive cognitive disorder (variable memory impairment (≠ AD)),
sleep and attentional disorders, hallucinations (visual) often before
• Extrapyramidal syndrome (70-90 %), poorly levodopa sensitive
• Fluctuations
• TTT: ACE inhibitors
Lewy Bodies Disease

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Lewy Bodies Disease

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Ballmaier, Neuroimage 2004
Whitwell, Brain 2007
Lewy Bodies Diseases

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Lewy Bodies Disease
Focal atrophy in dementia with Lewy bodies on MRI: a distinct pattern from Alzheimer's disease
Jennifer L. Whitwell, Brain, 2007

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DCL
ALZ
 DATscan®, PIB1,…
Lewy Bodies Diseases

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LBD atrophy > Control
LBD atrophy > PD dementia
Beyer, Neurology 2007
Lewy Bodies Disease
 Operculo-insular atrophy with relatively presenved hippocampus
in severely disabled patients

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Lewy Bodies Diseases
Mak et al. Neuroimaging characteristics of dementia with
Lewy bodies Alzheimer's Research & Therapy 2014, 6:18
Morphologie et signal DAT scan
MA DCL
DCL
MA

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Lewy Bodies Disease
Shams, AJNR 2017

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Some differential diagnoses…

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• Clinical
– Cognitive, walk, and urinary dysfunctions
– Communicating hydrocephalus
• No intracranial hypertension  No CI to CSF
substraction
Chronic Hydrocephalus

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Coulthard, BrJ Radiol 1999
CJD nv
Sporadic CJD
Nougaret, J Neuroradiol 2007
Creutzfeld-Jacob Disease

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Creutzfeld-Jacob Disease
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Serra-Tosio, Feuil Radiol 2006
T2* T2*
Diffuse Axonal Injuries

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Serra-Tosio, Feuil Radiol 2006
Wilson’s Disease

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Gayet-Wernicke
Encephalopathy
Chronic
Encephalopathy
Alcohol abuse

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PKAN
(Hallervorden-Spatz)
Mascalchi, Radiology 2004
Huntington
Genetic Diseases

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Take Home Message
• Clinical data incl. L-Dopa resp.
• Now MRI shows abnormalities in PD
• Nigrosome 1 on SWI in Parkinsonism and Dementia
• Consider DDx & Parkinson+ when L-Dopa non-responsive
– PSP
– MSAp/MSAc
– Lewy bodies diseases
– CBD,…
• Discuss nuclear medicine, esp. Dat-Scan® when unclear

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Thank you for your attention