Cortical and Subcortical Motor Systems and Disorders
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Transcript of Cortical and Subcortical Motor Systems and Disorders
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Cortical and Subcortical Motor Systems Cortical and Subcortical Motor Systems and Disordersand Disorders
Nancy L. Sicotte, M.D.Nancy L. Sicotte, M.D.
Director, Multiple Sclerosis ProgramDirector, Multiple Sclerosis Program
Cedars-Sinai Medical CenterCedars-Sinai Medical Center
Associate Professor in ResidenceAssociate Professor in Residence
Department of Neurology Department of Neurology
David Geffen School of Medicine at UCLADavid Geffen School of Medicine at UCLA
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Overview
• Anatomy - Cortical
• Anatomy – Subcortical
• Upper versus Lower Motor Neuron Changes
• Syndromes
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Cortical Motor Areas
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Major Cortical MotorPathways
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Motor and Sensory TractsThrough the Brainstem
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Basal Ganglia
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Cortical/Subcortical ConnectionsFor Motor control
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Arterial Supplies to the Brain
Four arteries in the neck supply blood to the brain:
• two internal carotid arteries coming off the common carotid arteries.
• two vertebral arteries which also supply blood to the spinal cord.
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Middle Cerebral Artery Territory
The Middle Cerebral Artery (MCA) territory is most frequently involved in strokes of the anterior circulation.
This artery supplies the biggest territory in the brain and some of the most important brain structures reside in this territory.
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Middle Cerebral Artery Territory
Full territory MCA strokes leave a person severely impaired.
Symptoms include paralysis, numbness, and blindness on the opposite side of the stroke. Left MCA strokes often leave the patient without ability to speak or understand language. Right MCA stroke leaves patient with inattention to the left side of the world and inability to recognize their own deficits.
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Left Middle Cerebral Artery Stroke
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Lacunar Syndromes
• Pure Motor Stroke – most common– Localization: posterior limb of internal capsule
• Ataxic Hemiparesis – PLIC, pons, coronal radiata, leg>arm (crural paresis)
• Dysarthria/clumsy hand– Pons (variant of ataxic hemiparesis)
• Pure Sensory Stroke– PLIC plus thalamus
• Mixed Sensorimotor Stroke– Larger PLIC plus thalamus
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Lateral Medullary Syndrome(Wallenberg)
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Spinal connections - peripheral nerve
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Upper Motor Neuron (UMN) vs. Lower Motor Neuron (LMN) Signs
SIGNS UMN Lesions LMN Lesions
Weakness Yes Yes
Atrophy* No Yes
Fasciculations No Yes
Reflexes Increased Decreased
Tone Increased Decreased
* Some atrophy may develop due to disuse
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Basal Ganglia
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Neurochemistry of the Basal Ganglia
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Connectivity diagram showingexcitatory glutamatergic pathwaysas red, inhibitory GABAergic pathways as blue, and modulatory dopaminergic as magenta.
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All Together Now..
Direct pathway lesions=> HypokinesiaIndirect Lesions=> Dyskinesias
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The Cerebellum
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Histology of the Cerebellum
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Single Folia
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Cerebellar Connections
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Motor Disorders
• Amyotrophic Lateral Sclerosis• Multiple Sclerosis • Parkinson Disease• “Parkinson Plus” - PSP, CBD, Shy-Drager• Huntington Chorea• Tardive Dyskinesia• Spinal Cerebellar Atrophy (SCAs)• Benign Essential Tremor
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ALS - “Lou Gehrig’s Disease”
• “Pure” Motor System Neurodegenerative disorder • Affects Upper and Lower Motor Neurons• Onset in late middle age, Men>Women• Familial form (SOD mutation) accounts for 10% of
cases• Median survival = 5 years (3 years for bulbar forms)• Cognitive impairment now recognized to occur • Pathological laughing/crying
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Multiple Sclerosis
• Autoimmune demyelinating disease of the central nervous system
• Age of onset 20-40, affects women: men - 2:1• Wide spread white matter lesions • Gray matter atrophy• Relapsing-remitting course usually followed by progressive
myelopathy• Cognitive impairment/disinhibition common (la belle
indifference)
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Courtesy of Rudick R
MS Lesions: Axonal Changes
A B
A B C
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Basal Ganglia Connections
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Parkinson Disease
• Neurodenerative disease resulting in loss of pigmented neurons in the substantia nigra – mitochondria?
• Occurs in elderly men>women• Environmental factors likely important (pesticides, MPTP)• Difficulties with initiation of movement• Tremor at rest• Depression/cognitive impairment common• Treatment: Dopamine agonists• Deep brain stimulators/stereotaxic surgery
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Parkinson “Plus”
• Share phenotype of tremor, stiffness, impaired initiation but with other features - many with Lewy bodies
• Progressive supranuclear palsy (PSP) - eye movement impairment, axial rigidity, personality changes
• Corticobasal degeneration (CBD) - present with unilateral (usually upper limb) apraxia, progressive motor/cognitive impairment, cortical lewy bodies
• Dementia with Lewy bodies (DLB)- may look like Alzheimer’s, frequent hallucinations, sleep disturbance
• Shy-Drager - like Parkinson disease, but severe autonomic involvement early
• Drug induced Parkinsonism - very common
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Huntington Chorea
• Autosomal dominant disorder, first CAG repeat disorder described
• Symptoms begin in 40-50s.• May present with psychiatric symptoms, depression,
psychosis, mania• Caudate atrophy• Choreathetoid movements develop later• Progressive decline in function over 10-15 years• No effective treatment
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Tardive Dyskinesia
• Develops after long term use of neuroleptics (dopamine antagonists)
• Frequently seen in patients with schizophrenia• More likely with “high potency” forms ie Haldol• Symptoms improve with use of offending agent -
vicious cycle
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Dystonia
• Abnormal, sustained contraction of muscles - (agonists and antagonists)
• Common forms - cervical, hand (writers cramp), can be generalized
• Cervical dystonia (torticollis) - “sensory tricks” sometimes work
• Some rare forms are responsive to dopamine agonists• Cause not well understood ?traumatic, overuse?• Can be caused acutely by certain drugs• Most common treatment now is Botox
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Spinocerebellar Atrophy
• Multiple genetic forms now recognized• Many with triple nucleotide repeats• Variety of phenotypes, many involving cognitive
impairment• Severe Cerebellar Atrophy• Currently no effective treatments
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Structural Imaging
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Water Diffusion - Isotropic and Anisotropic
Beaulieu, C. NMR Biomed 2002;15:435-455
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Diffusion along axons is anisotropic
Beaulieu, C. NMR Biomed 2002;15:435-455
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Diffusion gradients applied in 6 directions
Masutani et al. Eur J Radiology, 46(2003):53-66
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Tensor Displayed as an Ellipsoid
Masutani et al. Eur J Radiology, 46(2003):53-66
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Diffusion Tensor Imaging ofMajor White Matter Tracts
Pajevic and Pierpaoli, Mag Res Med (1999);42:526-540
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Diffusion Tensor Imaging
Myelin Stain - Mid pons
FIBER ORIENTATION:Red = right/leftBlue = dorsal/caudalGreen = anterior/posterior
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Horizontal Gaze Palsy with Progressive Scoliosis (HGGPS)
Crisfield 1974 Dretakis & Kondoyannis 1974Sharpe et al., 1975
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MRI HGPPS Patient
Sicotte et al Neurology, (67):519-521, 2006
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Midpons - DTI
Control HGPPS
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Sagittal View - DTI
Control HGPPS
Sicotte et al Neurology, (67):519-521, 2006
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Coronal View - DTI
Control HGPPS
Sicotte et al Neurology, (67):519-521, 2006
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Tractography
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Tract tracing withDiffusion TensorImaging (DTI)
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Diffusion Tensor Fiber Tracking:Corpus Callosum
Huang et al. Neuroimage 26(2005):195-205
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Diffusion Tensor Imaging Metrics
• Mean Diffusivity (MD) - average of diffusion in all directions
• Axial Diffusion (AD) - 1 first eigenvalue - parallel to tract
• Radial Diffusion (RD) - 2,3 average of 2nd and 3rd eigenvalues - perpendicular to tract -
• Fractional Anisotropy (fA) - dimensionless number ranging from 0 (isotropic) to 1 corresponding to the degree of preferential flow
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Tract Based Spatial Statistics (TBSS) Skeletons
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Group Differences: Fractional Anisotropy in RRMS vs Controls
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fA group/correlation and with composite motor score
Controls vs. RRMS Correlation with motor function
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RD group comparisons andcorrelations with 9HPT
Radial Diffusivity controls vs. RRMSCorrelation with 9HPT
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RD correlations 9HPTAxial View
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Corticospinal tracts
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CSTs Rendered in 3D
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Masutani et al. Eur J Radiology, 46(2003):53-66
DTT in Tumor Mapping
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Presurgical fMRI in a patient with an astrocytoma
What task was the patient performing?
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DTI of Motor Pathways:RRMS vs Controls
Kern et al. NeuroImage, in press
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Radial Diffusivity in Callosal Pathways Predicts Worsening of Hand Motor Function in RRMS
Kern et al. NeuroImage, in press
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Compensation or Disinhibition? fMRI during finger movements
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Resting State fMRI: Default Network
Damoiseaux and Greicius Brain Struct Funct 2009, 213:525-533
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Altered DMN Activity in MS
Bonavita et al Mult Sclerosis epub Jan 2011
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Sensorimotor Network
Human Connectome Project: http://humanconnectome.org
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Conclusions
• Human Motor System is a complex network involving multiple cortical and subcortical areas
• Impairments in motor control are common in many neurodegenerative disorders
• Multimodal imaging approaches can be used to study changes in structural and functional integrity: in particular combined DTI/fMRI
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Thank you for your attention!Thank you for your attention!