EXTRAPYRAMIDAL SYSTEM

I.Velcheva
University Hospital
“St. Naum”
 DEFINITION
The extrapyramidal system
includes all motor gray
structures and pathways that are
not included in the pyramidal
system. It is a set of subcortical
circuits and pathways and
includes the basal ganglia, red
nucleus, brain stem reticular
formation and other brainstem
nuclei.
 EXTRAPYRAMIDAL
STRUCTURES
BASAL GANGLIA (5)
Corpus striatum
NEOSTRIATUM
 Caudate nucleus(1)
 Putamen(2)
PALEOSTRIATUM
 Globus pallidus(3)
 Substantia nigra(4)
 Subthalamic
nucleus(5)
Putamen + Globus pallidus
= Lenticular nucleus
 EXTRAPYRAMIDAL STRUCTURES
Cerebral cortex
(premotor frontal, cingulate gyrus)

Basal ganglia
Thalamus (ventral nuclei)
Motor nuclei of the
brainstem:
 Red nucleus
 RF of pons and medulla
oblongata
 Pontine nuclei
 Superior colliculi
 Lateral vestibular nucleus
 Inferior olive

Cerebellum
 CONNECTIONS OF THE
EXTRAPYRAMIDAL SYSTEM
Afferent connections
Cortical ipsilateral
projections from frontal
motor areas 4, 6aα and
6a fields to striatum
(inhibitory)
Thalamic projections
from the cenromedian
nucleus (facilitatory)
Connections between
basal ganglia
Caudate nucleus 
Substantia nigra
(GABA) (inhibitory)
Substantia nigra
Caudate nucleus
(dopaminergic)
 CONNECTIONS OF THE
EXTRAPYRAMIDAL SYSTEM

Extrapyramidal
circuits
Cerebral cortex 
Striatum  Pallidum 
Thalamus  Cerebral
cortex (4, 6aa, 6a
frontal fields)
Striatum  Pallidum
 Thalamus 
Striatum
Pallidum  Nucl.
Subthalamicus 
Pallidum
Striatum  Substantia
nigra  Striatum
 CONNECTIONS OF THE
EXTRAPYRAMIDAL SYSTEM
Efferent connections
Striatum  Pallidum
 Thalamus
Hypothalamus 
Subthalamic nuclei
Subcortical
descending system
Cortico – striate, -
rubral, - nigral, -
reticular tracts to
brainstem and
spinal cord via
descending chain of
neurons
NEUROTRANSMITTERS
Striatum – acetylcholine +
dopamine, serotonin, GABA
Acetylcholine – dopamine balance
Globus pallidus – GABA
Brainstem – NE, serotonin,
enkephalin, glutamate
CLINICAL – PATHOLOGICAL
CORRELATIONS
Degeneration of the substantia
nigra – Parkinsonism
Destructive lesions of the
subthalamic nucleus –
hemibalism
Degeneration in caudate nucl.
and putamen – chorea,
athetosis
 CLINICAL – PATHOLOGICAL
CORRELATIONS
Degeneration of
the substantia
nigra –
Parkinsonism
 Rigidity
 Hypokinesia (slowness of
voluntary movement)
 Tremor (static, resting with
a frequency of 4-6Hz),
asymmetric, “pill-rolling”
movement
 Abnormal gait and posture
(flexed posture on
standing and walking)
 CLINICAL – PATHOLOGICAL
CORRELATIONS

Degeneration in caudate
nucl. and putamen – chorea
 Choreic involuntary movements
 Muscular hypotonia
Irregular gait and speech.
Chorea dissapears during sleep.
Etiology: Sydenham`s (rheumatic)
chorea,Huntington`s chorea
 INVOLUNTARY MOVEMENTS
Tremor (rhythmic oscillatory movement, which
occurs at rest, during maintenance of a particular
posture, during movement)
 Static (resting)
 Postural (physiological during sustained posture -8-
12-20Hz, enhanced physiological during fear or
anxiety, benign essential- rhythmic, symmetric,
involves hands and head, familial)
 Intention tremor during activity
Tics (sudden abrupt coordinated abnormal
movements, repeated at short intervals)
(transient simple, chronic simple,
persistent simple or multiple,
chronic multiple motor and vocal – Gilles de la
Tourette syndrome)
INVOLUNTARY MOVEMENTS
Chorea (rapid, irregular muscle jerks in different parts
of the body, combined with muscular hypotonia)

Hemibalism (violant unilateral involuntary jerks with

involvement of the proximal muscles)

Athetosis (slow sinuous abnormal movements in

the limbs)

Myoclonus (sudden rapid twitchlike involuntary

muscle contractions, which do not change the position of
the affected body part)
(physiological, essential, epileptic)
INVOLUNTARY MOVEMENTS
Dystonia (twisting repetitive movements of
axial and limb muscles, abnormal postures)
 Focal : blepharospasm, spasmodic torticolos,
occupational cramps (writer`s cramp,violinist`s
cramp ect.)
 Segmental – one leg and trunk
 Multifocal – more than one limb
 Generalized – all muscles of limbs and
trunk
 Hemidystonia – unilateral limbs
INVOLUNTARY MOVEMENTS
Chorea
INVOLUNTARY MOVEMENTS

Dystonia (generalized)
INVOLUNTARY MOVEMENTS

Dystonia (focal)
INVOLUNTARY MOVEMENTS

Myoclonus
(physiological,
essential, epileptic)
 MUSCLE TONE
A state of permanent muscle
contraction in order to support posture (resistance of
the muscles
to passive movements).
2 components: static (at rest) and dynamic (during
stretch)
Regulation of the muscle tone
The normal muscle tone is defined by nonreflex (visco-
elastic properties of the muscles) and reflex
mechanisms.
Segmental mechanism – 2 reflex feedback systems
 Stretched muscle at rest → muscle spindle → sensory
fibers → alpha motor neurons (α fibers) → extrafusal
muscles → contraction (restoration of length)
 Active contraction (muscle tension) → Golgi organ →
sensory fibers → alpha motor neurons (β fibers)
(inhibitory effect)
Suprasegmental (reticulospinal) mechanism
Central impulses → gamma motoneurons (γ fibers) →
intrafusal muscle (increase of tension)
 MUSCLE TONE
Regulation of the muscle tone
Segmental - stretch reflex
Suprasegmental (reticulospinal)
 MUSCLE TONE
Regulation of the muscle tone
Segmental - stretch reflex
 SYNDROMES OF DISORDERED
MUSCLE TONE
Decreased muscle tone (hypotonia, flaccidity)
Indicates:
 lower motor neuron lesion (efferent arm of the
reflex arc)
 Acute stage of pyramidal lesion (neural shock)
 Neostriatal lesion (chorea)
 Cerebellar lesion
 Acute stage of spinal cord lesion (spinal shock)
 Primary muscle diseases
 SYNDROMES OF
DISORDERED MUSCLE TONE
Increased muscle tone (hypertonia)
 Spasticity - increased tone in certain muscle
groups (flexors of the arm and extensors of the
legs) and increase of resistance linearly in
relation to velocity of stretch.
Spasticity indicates upper motor neuron
lesions.
 Rigidity - increased muscle tone with continuous
“lead pipe rigidity” or intermitent “cogwheel
rigidity” muscle tension.
Rigidity indicates extrapyramidal dysfunction.