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    Basal

    Ganglia

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    Muhammad Ali in Alanta Olympic

    Parkinsons Disease

    Disease of mesostriatal

    dopaminergic system

    PD

    normal

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    Substantia Nigra,Pars Compacta (SNc)

    DOPAminergic Neuron

    Slowness of Movement- Difficulty in Initiation and Cessationof Movement

    Clinical Feature (1)

    Parkinsons Disease - Paralysis Agitans

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    Substantia Nigra,Pars Compacta (SNc)

    DOPAminergic Neuron

    Slowness of Movement- Difficulty in Initiation and Cessationof Movement

    Clinical Feature (1)

    Parkinsons Disease - Paralysis Agitans

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    Clinical Feature (2)

    Resting TremorParkinsonian PostureRigidity-Cogwheel Rigidity

    Parkinsons Disease

    Paralysis Agitans

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    Clinical Feature

    Principal Pathologic Lesion:Corpus Striatum (esp. caudate nucleus)

    and Cerebral Cortex

    - Predominantly autosomal dominantlyinherited chronic fatal disease(Gene: chromosome 4)

    - Insidious onset: Usually 40-50

    - Choreic movements in onset- Frequently associated withemotional disturbances

    - Ultimately, grotesque gait and severdysarthria, progressive dementiaensues.

    HUNTINGTONS CHOREA

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    HEMIBALLISM

    - Usually results from CVA(Cerebrovascular Accident)involving subthalamic nucleus

    - sudden onset-Violent, writhing, involuntarymovements of wide excursionconfined to one half of the body

    - The movements are continuousand often exhausting but ceaseduring sleep

    - Sometimes fatal due to exhaustion- Could be controlled byphenothiazines and stereotaxicsurgery

    Clinical Feature

    Lesion:Subthalamic Nucleus

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    SYDENHAMS CHOREA

    - Complication ofRheumatic Fever

    - Fine, disorganized , andrandom movements of

    extremities, face andtongue

    - Accompanied byMuscular Hypotonia

    - Typical exaggeration ofassociated movements

    during voluntary activity- Usually recoversspontaneouslyin 1 to 4 months

    Clinical Feature

    Principal Pathologic Lesion:Corpus Striatum

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    Traditional Concepts of Basal Ganglia

    Corpus StriatumCaudate Nucleus

    Lenticular Nucleus Putamen

    Globus Pallidus Paleostriatum Pallidum

    Corpus Amygdaloideum Archistriatum

    Neostriatum Striatum

    Basal Ganglia Introduction

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    1. Putamen

    2. Tail of caudatenucleus

    3. Caudatolenticulargray bridge

    4. Amygdaloid body

    5. thalamus

    Lateral surface

    of basal ganglia

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    1. head of

    caudate nucelus

    2. body of

    caudate nucelus

    3. caudatolenticular

    gray bridge

    4. putamen

    5. tail of

    caudate nucleus

    6. external segment ofglobus pallidus

    7. internal segment ofglobus pallidus

    8. amygdaloid body

    9. nucleus accumbenssepti

    Medial surface of basal ganglia

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    Components of Basal Ganglia

    Corpus Striatum

    Striatum ----- Caudate Nucleus & Putamen

    Pallidum ----- Globus Pallidus (GP)

    Substantia NigraPars Compacta (SNc)

    Pars Reticulata (SNr)

    Subthalamic Nucleus (STN)

    Ventral Striatum and Ventral Pallidum

    Nucleus Accumbens SeptiNon cholinergic portion of Substantia Innominata

    Basal Ganglia Components

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    STRIATUM

    Caudate Nucleus

    Head, (Corpus), Tail

    caudatolenticular gray bridge

    Putamen

    Ventral Striatum: Nucleus Accumbens (Septi)

    Basal Ganglia Components

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    Striatal Compartments

    (Mosaic or Modular Organization)

    1. Striosome (Patches)

    - 10-20% of total striatal mass

    - low acetylcholinesterase (AchE) activity

    - high substance P (SP), neurotensin (NT), tyrosine hydroxylase- high expression of D1 dopamine receptor

    - high opiate receptor

    2. Matrix

    - high acetylcholinesterase (AchE) activity

    - high somatostatin (SRIF) activity

    - high D2 dopamine receptor

    Basal Ganglia Components

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    Spiny I

    neuron

    Spiny II

    neuron

    Aspiny I

    neuron

    Aspiny II

    neuron

    Aspiny III

    neuron

    Neurogliformcell

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    Basal Ganglia Components

    Striosome andMatrix compartment

    AchE

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    Basal Ganglia Components

    Inputs to the

    spiny striatal neurons

    distal extrinsic

    proximal - intrinsic

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    PALLIDUM

    Globus Pallidus (Pallidum)

    Internal or Medial Segment (GPi)

    internal medullary lamina

    External or Lateral Segment (GPe)

    Ventral Pallidum

    Non-cholinergic portions

    of substantia innominata

    Basal Ganglia Components

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    Substantia Nigra

    Pars Compacta (SNc)

    Dopaminergic Cell Group (A9)

    cf. Ventral Tegmental Area (VTA, A10)

    Retrorubral Area (A8)

    Pars Reticulata (SNr)

    Non-dopaminergic Portion

    GABAergic Neuron

    Basal Ganglia Components

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    Subthalamus

    Subthalamic Nucleus (STN)

    zona incertaField of Forel

    H ansa lenticularis

    H1 thalamic fasciculus

    H2 lenticular fasciculus

    Basal Ganglia Components

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    Putamen

    Globus pallidus

    external segment

    internal segment

    Subthalamic Nucleus

    Substantia nigra

    Internal capsule

    Components of

    Basal Ganglia

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    Basal Ganglia Components

    Subthalamic Nucleus1. epithalamus2. dorsal thalamus3. subthalamus

    3-1. subthalamic

    nucleus3-2. zona incerta3-3. globus pallidus

    4. hypothalamus5. putamen

    arrow: internal capsule

    Origin of subthalamic nucleus and putamen (Kuhlenbeck)

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    Input Portion

    STRIATUM

    (Caudate Nucleus and Putamen)

    Output Portion

    1. PALLIDUM (Globus Pallidus)

    2. SNr (Substantia Nigra, Pars Reticulata)

    Basal Ganglia Connections

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    STRIATUMAfferentsfrom1. Cerebral Cortex [Corticostriatal Projection]2. Thalamus (CM-PF complex) [Thalamostriatal Projection]3. SNc (Substantia Nigra, pars compacta),

    Ventral Tegmental Area (VTA, A10)and A8(Retrorubral Area) [Nigrostriatal Projection]4. Dorsal Raphe Nucleus (B6, B8) [Raphestriatal Projection]5. Amygdaloid Nuclear Complex [Amygdalostriatal Projection]

    Efferentsto

    1. Pallidum (GPi & GPe) [Striopallidal Projection]2. SNr [Strionigral Projection]3. SNc from striosome

    Basal Ganglia Connections

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    Afferent Connections of Basal Ganglia

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    Input-OutputRelationshipof Basal Ganglia

    1. striopallidal fiber2. strionigral fiber3. strionigral fiber

    from striosometo SNc

    4. ventrostrio-ventro-pallidal fiber

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    PALLIDUM (Globus Pallidus)

    Afferentsfrom1. Striatum [Striatopallidal Projection]

    2. STN [Subthalamopallidal Projection]

    Efferentsto

    1. Thalamus (VLo, VApc, CM) [Pallidothalamic Projection]

    2. STN [Pallidosubthalamic Projection]

    SNr (Substantia Nigra, Pars Reticulata)

    Afferents from Striatum [Striatonigral Projection]

    Efferents to

    1. Thalamus (VLm, VAmc, MD) [Nigrothalamic Projection]

    2. Brain Stem Nucleus: Tectum (Superior Colliculus) &

    Pedunculopontine Nucleus (PPN)

    Basal Ganglia Connections

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    Efferent Connections of Basal Ganglia

    Basal Ganglia Connections

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    Pallidothalamic Fibers

    from GPi to VLo, VApc, CMAnsa Lenticularis

    from outer (lateral) portion of GPifibers sweep ventromedially and rostrally around

    the posterior limb of the internal capsule

    enters Forels field H

    Lenticular Fasciculusfrom inner (medial) portion of GPiperforate internal capsule

    enters Forels field H2

    Thalamic fasciculusansa lenticularis, lenticular fasciculus

    and cerebellothalamic fibers

    Forels field H1

    Basal Ganglia Connections

    C ti f th B l G li

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    habenularnucleus

    tectum(superior colliculus)

    PPN(pedunculopontine nucleus)

    amygdaloid body

    raphe

    CerebralCortex

    STN

    Pallidum

    SNr

    STRIATUM

    Connections of the Basal Ganglia

    SNc

    Thalamus

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    Basal Ganglia (Main Motor Circuit) Connections

    SupplementaryMotor Area

    (SMA)

    PrimaryMotor Area

    (M I)

    THALAMUS

    (VLo, VApc, CM)

    STRIATUM(Putamen)

    PALLIDUM

    (GPi)

    pyramidaltract

    LMN

    ansalenticularis

    lenticularfasciculus

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    Basal Ganglia (Prefronatal Complex Loop) Connections

    PrefrontalAssociation

    Cortex

    PrimaryMotor Area

    (M I)

    THALAMUS

    (VLm, VAmc, MD)

    STRIATUM(CaudateNucleus)

    SNr

    (Substantia Nigra,pars reticulata)

    pyramidaltract

    LMN

    Basal Ganglia (Microcircuitary) Connections

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    GP, SNr

    StriatumMotorCortex

    VA-VL

    complex

    - GABA

    -

    GABA

    +glutamate ?

    glutamate

    +

    glutamate

    +

    Basal Ganglia (Microcircuitary) Connections

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    Basal Ganglia (Limbic Loop) Connections

    Orbitofrontal CortexAnterior Cingulate GyrusHippocampal Formation

    THALAMUS

    (VAmc, MD)

    Ventral StriatumCaudate Nucleus

    Ventral Pallidum

    GPi, SNr

    Basal Ganglia (Oculomotor Loop) Connections

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    Basal Ganglia (Oculomotor Loop) Connections

    FrontalEye Field(area 8)

    PrimaryMotor Area

    (M I)

    THALAMUS(VLm, VAmc, MD)

    STRIATUM(CaudateNucleus)

    SNr(Substantia Nigra,

    pars reticulata)

    pyramidaltract

    LMN Tectum

    B l G li

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    Basal Ganglia(SNc and CM-PF nuclear complex) Connections

    Pallidum

    Striatum

    THALAMUS

    (CM-PF)

    Pallidum

    Striatum

    SNc

    B l G li (B i St Eff t ) Ph l

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    Basal Ganglia (Brain Stem Efferents) Phylogeny

    GPi

    SNr

    Tectum(superiorcolliculus)

    SpLnDCP

    DIP (thalamus)Neostriatum

    Intermediale(motor cortex)

    aves (birds)

    GPi

    SNrTectum(superiorcolliculus)

    nPC

    VA-VL complex(thalamus)

    motorcortex

    mammals

    B l G li (STN) C i

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    Basal Ganglia (STN) Connections

    SNrGPi

    STN

    GPe

    subthalamic

    fasciculus

    OutputPortionofBasalGanglia

    Cerebral Coretx

    Thalamus (CM-PF)

    PPN

    Subthalamic

    Nucleus

    B l G li (V t l St i t d V t l P llid ) C ti

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    Basal Ganglia (Ventral Striatum and Ventral Pallidum) Connections

    VentralPallidum

    VentralStriatum

    Hippocampal

    Formation

    BasolateralAmygdala

    Temporal &Frontal Cortex

    SNc, VTA &Dorsal Raphe

    Thalamus(MD)

    BasolateralAmygdala

    PPN & VTA

    Thalamus(MD)

    Basal Ganglia (Brain Stem Efferents) Connections

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    Basal Ganglia (Brain Stem Efferents) Connections

    PPN(midbrain

    locomotorregion)

    Tectum(superiorcolliculus)

    GPi, SNr

    Striatum(caudate nucleus)

    LowerMotor

    Neuron

    viareticularformation

    tectospinaltract

    Basal Ganglia

    Brain Stem & Spinal Cord

    Basal Ganglia and Pyramidal Tract

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    SMA

    (supplementary motor area)

    Basal Ganglia and Pyramidal Tract

    upper motor neuron

    UMN

    lower motor neuron

    LMN

    pyramidaltract

    BASALGANGLIA

    CIRCUIT

    Basal Forebrain Area Components

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    BASAL FORBRAIN AREA (Heimer)

    Ventral Striatum, Part ofVentral PallidumBasal Nucleus of Meynert (Ch4)Extended Amygdala

    cf. Cholinergic Cell Groups (Mesulam)

    (Magnocellular Basomedial Telencephalic Complex)

    1. Ch1 (Medial Septal Nucleus)

    2. Ch2 (Nucleus of Diagonal Band of Broca)

    3. Ch3 (Nucleus of Diagonal Band of Broca)4. Ch4(Basal Nucleus of Meynert)

    5. Ch5 (Pedunculopontine Tegmental Nucleus)

    Basal Ganglia Functional Consideration

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    Basal Ganglia Functional Consideration

    Functional Consideration

    1. Selection of Preprogramed (learned) motor plans

    Basal Gangla Circuit ----Selection Mechanism

    Selection Inability -------- Akinesia and Hypokinesia

    Faulty Selection ----------- Hyperkinesia

    2. Generation (learning) of motor programs

    Programming of several motor fragments into

    complex motor routinesCerebral Palsy------------- Disordered motor program

    SYDENHAMS CHOREA

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    SYDENHAM S CHOREA

    - Complication ofRheumatic Fever

    - Fine, disorganized , andrandom movements of

    extremities, face andtongue- Accompanied byMuscular Hypotonia

    - Typical exaggeration ofassociated movementsduring voluntary activity

    - Usually recoversspontaneouslyin 1 to 4 months

    Clinical Feature

    Principal Pathologic Lesion:Corpus Striatum

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    Clinical Feature

    Principal Pathologic Lesion:Corpus Striatum (esp. caudate nucleus)

    and Cerebral Cortex

    - Predominantly autosomal dominantlyinherited chronic fatal disease(Gene: chromosome 4)

    - Insidious onset: Usually 40-50- Choreic movements in onset

    - Frequently associated withemotional disturbances

    - Ultimately, grotesque gait and severdysarthria, progressive dementiaensues.

    HUNTINGTONS CHOREA

    HEMIBALLISM

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    HEMIBALLISM

    - Usually results from CVA(Cerebrovascular Accident)involving subthalamic nucleus

    - sudden onset

    -Violent, writhing, involuntarymovements of wide excursionconfined to one half of the body

    - The movements are continuousand often exhausting but ceaseduring sleep

    - Sometimes fatal due to exhaustion- Could be controlled byphenothiazines and stereotaxicsurgery

    Clinical Feature

    Lesion:Subthalamic Nucleus

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    Muhammad Ali in Alanta Olympic

    Parkinsons Disease

    Disease of mesostriatal

    dopaminergic system

    PD

    normal

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    Substantia Nigra,Pars Compacta (SNc)

    DOPAminergic Neuron

    Slowness of Movement

    - Difficulty in Initiation and Cessationof Movement

    Clinical Feature (1)

    Parkinsons Disease - Paralysis Agitans

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    Clinical Feature (2)

    Resting Tremor

    Parkinsonian PostureRigidity-Cogwheel Rigidity

    Parkinsons Disease

    Paralysis Agitans