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, 6a 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 ( fibers) extrafusal
muscles contraction (restoration of length)
Active contraction (muscle tension) Golgi organ
sensory fibers alpha motor neurons ( fibers)
(inhibitory effect)
Suprasegmental (reticulospinal) mechanism
Central impulses gamma motoneurons ( 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.