Basal Ganglia

Parkinsons Disease
Disease of mesostriatal dopaminergic system

PD

Muhammad Ali in Alanta Olympic

normal

Parkinsons Disease - Paralysis Agitans

Substantia Nigra, Pars Compacta (SNc) DOPAminergic Neuron

Clinical Feature (1) Slowness of Movement

- Difficulty in Initiation and Cessation of Movement

Parkinsons Disease - Paralysis Agitans

Substantia Nigra, Pars Compacta (SNc) DOPAminergic Neuron

Clinical Feature (1) Slowness of Movement

- Difficulty in Initiation and Cessation of Movement

Parkinsons Disease Paralysis Agitans

Clinical Feature (2)

Resting Tremor Parkinsonian Posture Rigidity-Cogwheel Rigidity

HUNTINGTONS CHOREA Clinical Feature

- Predominantly autosomal dominantly inherited chronic fatal disease (Gene: chromosome 4) - Insidious onset: Usually 40-50 - Choreic movements in onset - Frequently associated with emotional disturbances - Ultimately, grotesque gait and sever dysarthria, progressive dementia ensues.

Principal Pathologic Lesion:

Corpus Striatum (esp. caudate nucleus) and Cerebral Cortex

HEMIBALLISM

Clinical Feature
- Usually results from CVA (Cerebrovascular Accident) involving subthalamic nucleus - sudden onset - Violent, writhing, involuntary movements of wide excursion confined to one half of the body - The movements are continuous and often exhausting but cease during sleep - Sometimes fatal due to exhaustion - Could be controlled by phenothiazines and stereotaxic surgery

Lesion: Subthalamic Nucleus

SYDENHAMS CHOREA

Clinical Feature
- Complication of Rheumatic Fever - Fine, disorganized , and random movements of extremities, face and tongue - Accompanied by Muscular Hypotonia - Typical exaggeration of associated movements during voluntary activity - Usually recovers spontaneously in 1 to 4 months

Principal Pathologic Lesion: Corpus Striatum

Basal Ganglia

Introduction

Traditional Concepts of Basal Ganglia

Corpus Striatum Caudate Nucleus Lenticular Nucleus Putamen Globus Pallidus Corpus Amygdaloideum Paleostriatum Archistriatum Pallidum Neostriatum Striatum

Lateral surface of basal ganglia

1. Putamen 2. Tail of caudate nucleus 3. Caudatolenticular gray bridge 4. Amygdaloid body 5. thalamus

1. head of caudate nucelus 2. body of caudate nucelus 3. caudatolenticular gray bridge 4. putamen 5. tail of caudate nucleus 6. external segment of globus pallidus 7. internal segment of globus pallidus 8. amygdaloid body

9. nucleus accumbens septi

Medial surface of basal ganglia

Basal Ganglia Components of Basal Ganglia

Components

Corpus Striatum Striatum ----- Caudate Nucleus & Putamen Pallidum ----- Globus Pallidus (GP) Substantia Nigra Pars Compacta (SNc) Pars Reticulata (SNr) Subthalamic Nucleus (STN) Ventral Striatum and Ventral Pallidum Nucleus Accumbens Septi Non cholinergic portion of Substantia Innominata

Basal Ganglia

Components

STRIATUM
Caudate Nucleus
Head, (Corpus), Tail caudatolenticular gray bridge

Putamen Ventral Striatum: Nucleus Accumbens (Septi)

Basal Ganglia Striatal Compartments

(Mosaic or Modular Organization)

Components

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

Spiny I neuron

Spiny II neuron
Aspiny I neuron

Aspiny II neuron
Aspiny III neuron Neurogliform cell

Basal Ganglia

Components

Striosome and Matrix compartment

AchE

Basal Ganglia

Components Inputs to the spiny striatal neurons

distal extrinsic proximal - intrinsic

Basal Ganglia

Components

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

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

Subthalamus Subthalamic Nucleus (STN) zona incerta Field of Forel

H H1 H2 ansa lenticularis thalamic fasciculus lenticular fasciculus

Components of Basal Ganglia

Putamen Globus pallidus external segment internal segment Subthalamic Nucleus Substantia nigra Internal capsule

Basal Ganglia

Components

Subthalamic Nucleus
1. epithalamus 2. dorsal thalamus 3. subthalamus 3-1. subthalamic nucleus 3-2. zona incerta 3-3. globus pallidus 4. hypothalamus 5. putamen arrow: internal capsule Origin of subthalamic nucleus and putamen (Kuhlenbeck)

Basal Ganglia

Connections

Input Portion

STRIATUM
(Caudate Nucleus and Putamen)

Output Portion
1. PALLIDUM (Globus Pallidus) 2. SNr (Substantia Nigra, Pars Reticulata)

Basal Ganglia
STRIATUM

Connections

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

Afferent Connections of Basal Ganglia

Input-Output Relationship of Basal Ganglia

1. striopallidal fiber 2. strionigral fiber 3. strionigral fiber from striosome to SNc 4. ventrostrioventro-pallidal fiber

Basal Ganglia
PALLIDUM (Globus Pallidus)

Connections

Afferents from 1. Striatum [Striatopallidal Projection] 2. STN <GPi & GPe> [Subthalamopallidal Projection] Efferents to 1. Thalamus (VLo, VApc, CM) <GPi> [Pallidothalamic Projection] 2. STN <GPe> [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)

Efferent Connections of Basal Ganglia

Basal Ganglia Pallidothalamic Fibers

from GPi to VLo, VApc, CM

Connections

Ansa Lenticularis
from outer (lateral) portion of GPi fibers sweep ventromedially and rostrally around the posterior limb of the internal capsule enters Forels field H

Lenticular Fasciculus
from inner (medial) portion of GPi perforate internal capsule enters Forels field H2

Thalamic fasciculus
ansa lenticularis, lenticular fasciculus and cerebellothalamic fibers Forels field H1

Connections of the Basal Ganglia

Cerebral Cortex amygdaloid body raphe STRIATUM Thalamus STN SNc

Pallidum

SNr

habenular nucleus

tectum
(superior colliculus)

PPN
(pedunculopontine nucleus)

Basal Ganglia (Main Motor Circuit)

Connections

Primary Motor Area (M I)

Supplementary Motor Area (SMA)

STRIATUM (Putamen)

pyramidal tract
ansa lenticularis

THALAMUS (VLo, VApc, CM)

LMN

PALLIDUM (GPi)

lenticular fasciculus

Basal Ganglia (Prefronatal Complex Loop)

Connections

Primary Motor Area (M I)

Prefrontal Association Cortex

STRIATUM (Caudate Nucleus)

pyramidal tract

THALAMUS
(VLm, VAmc, MD)

SNr
(Substantia Nigra, pars reticulata)

LMN

Basal Ganglia (Microcircuitary)

Connections

Motor Cortex
+
glutamate ?

glutamate

Striatum

glutamate

GABA

GABA

VA-VL complex

GP, SNr

Basal Ganglia (Limbic Loop)

Connections

Orbitofrontal Cortex Anterior Cingulate Gyrus Hippocampal Formation

Ventral Striatum
Caudate Nucleus

THALAMUS
(VAmc, MD)

Ventral Pallidum GPi, SNr

Basal Ganglia (Oculomotor Loop)

Connections

Primary Motor Area (M I)

Frontal Eye Field (area 8)

STRIATUM (Caudate Nucleus)

pyramidal tract
THALAMUS
(VLm, VAmc, MD)

SNr
(Substantia Nigra, pars reticulata)

LMN

Tectum

Basal Ganglia (SNc and CM-PF nuclear complex)

Connections

Striatum

Striatum

SNc

THALAMUS (CM-PF)

Pallidum

Pallidum

Basal Ganglia (Brain Stem Efferents)

aves (birds)

Phylogeny
Neostriatum Intermediale (motor cortex)

DIP (thalamus) GPi SpL nDCP SNr

Tectum
(superior colliculus)

mammals

GPi

VA-VL complex (thalamus) nPC

motor cortex
Tectum
(superior colliculus)

SNr

Basal Ganglia (STN)

Subthalamic Nucleus

Connections
Cerebral Coretx

STN
subthalamic fasciculus

Thalamus (CM-PF)
PPN

Output Portion of Basal Ganglia

GPe

GPi

SNr

Basal Ganglia (Ventral Striatum and Ventral Pallidum)

Connections

Hippocampal Formation

Basolateral Amygdala Thalamus (MD) Temporal & Frontal Cortex

Basolateral Amygdala

Ventral Striatum

Ventral Pallidum

Thalamus (MD)

PPN & VTA

SNc, VTA & Dorsal Raphe

Basal Ganglia (Brain Stem Efferents)

Connections

Brain Stem & Spinal Cord Striatum

(caudate nucleus)

Tectum
(superior colliculus)

tectospinal tract

GPi, SNr PPN

(midbrain locomotor region)

Lower Motor Neuron via reticular formation

Basal Ganglia

Basal Ganglia and Pyramidal Tract

SMA
(supplementary motor area)

upper motor neuron

UMN

pyramidal tract

lower motor neuron

BASAL GANGLIA CIRCUIT

LMN

Basal Forebrain Area

BASAL FORBRAIN AREA (Heimer)
Ventral Striatum, Part of Ventral Pallidum Basal Nucleus of Meynert (Ch4) Extended Amygdala

Components

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

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 routines Cerebral Palsy ------------- Disordered motor program

SYDENHAMS CHOREA

Clinical Feature
- Complication of Rheumatic Fever - Fine, disorganized , and random movements of extremities, face and tongue - Accompanied by Muscular Hypotonia - Typical exaggeration of associated movements during voluntary activity - Usually recovers spontaneously in 1 to 4 months

Principal Pathologic Lesion: Corpus Striatum

HUNTINGTONS CHOREA Clinical Feature

- Predominantly autosomal dominantly inherited chronic fatal disease (Gene: chromosome 4) - Insidious onset: Usually 40-50 - Choreic movements in onset - Frequently associated with emotional disturbances - Ultimately, grotesque gait and sever dysarthria, progressive dementia ensues.

Principal Pathologic Lesion:

Corpus Striatum (esp. caudate nucleus) and Cerebral Cortex

HEMIBALLISM

Clinical Feature
- Usually results from CVA (Cerebrovascular Accident) involving subthalamic nucleus - sudden onset - Violent, writhing, involuntary movements of wide excursion confined to one half of the body - The movements are continuous and often exhausting but cease during sleep - Sometimes fatal due to exhaustion - Could be controlled by phenothiazines and stereotaxic surgery

Lesion: Subthalamic Nucleus

Parkinsons Disease
Disease of mesostriatal dopaminergic system

PD

Muhammad Ali in Alanta Olympic

normal

Parkinsons Disease - Paralysis Agitans

Substantia Nigra, Pars Compacta (SNc) DOPAminergic Neuron

Clinical Feature (1) Slowness of Movement

- Difficulty in Initiation and Cessation of Movement

Parkinsons Disease Paralysis Agitans

Clinical Feature (2)

Resting Tremor Parkinsonian Posture Rigidity-Cogwheel Rigidity