Topic 2

The extrapyramidal system. The cerebellum.

Olena Rubanova
• The extrapyramidal system and the syndromes of its defect.
• The cerebellum, syndromes of defeat of the cerebellum.
• The kinds of ataxia.
• Practical skills.
 The extrapyramidal
system
• S. Wilson (1912)
• Disturbance of the movements that
coul’dn’t be explained in a lesion of the
pyramidal system or in disorders of
coordination of movements.
• The anatomofunctional complex, which
includes the majority of the basal nuclei, a
part of the gray matter of the midbrain
and diencephalon, and also numerous
communications of these formations with
other structures of the brain and spinal
cord.

Illustration of main motor pathways involved in the volitional control of movement. Th e two major efferent pathways under corticomotor control are the
α -motor neurons (light green) and the much smaller γ -motor neurons (dark green), located within the ventral horn of the spinal cord. Th e α -motor neurons innervate
the extrafusal muscle fi bres causing the muscle to contract. Th e γ -motor neurons attach to the intrafusal fibres found within the muscle spindle and provide stretch
sensitivity via the Ia afferent fibres (grey) which activate the α -motor neurons (gamma reflex loop). Th is loop helps regulate the muscle length and tone and is important
for reflexes. Th e Ia afferent, along with corticospinal projections, also excites associated interneurons (black) which regulate the reflex by inhibiting the α -motor neurons.
IMG.: ISBN 978–0–19–960953–6
The extrapyramidal system
All fibers that influence the motor activity without passing through the
pyramidal tract
• Cortex
• Basal Ganglia (caudate, putamen, globus pallidus, Subthalamic nucleus and
substantia nigra)
• Thalamus
• Cerebellum
• Red nucleus
• Reticular nucleus
 The basal
ganglia
• The basal ganglia include all of the
functionally interrelated nuclei
within then deep white matter of
the telencephalon
• The major nuclei of the basal
ganglia are:
• the caudate nucleus
• the lentiform nucleus
(putamen and globus palidus)
• the substantia nigra (compact
and reticular parts) Anatomy of the basal ganglia and their regulation of motor cortices.
(a) Position of the basal ganglia in the brain showing the major regions
• The subthalamic nucleus involved in the regulation of movement. (b) Human coronal sections through
the basal ganglia showing the major subdivisions. (c) Diagram showing the
main anatomical projections and regulatory interconnections involving the
basal ganglia. The basal ganglia can be subdivided, based on their interactions,
into input, output, and regulator nuclei (see text). BG, basal ganglia; CM,
central median; pre-SMA, pre-supplementary motor area; MD, medial dorsal
thalamus; SNc, substantia nigra pars compacta; SNr, substantia nigra pars
reticulata; VA, ventral anterior thalamus; VLa, ventral lateral anterior thalamus;
IMG.: ISBN 978–0–19–960953–6 VLp, ventral lateral posterior thalamus.
 The basal ganglia
• The caudate nucleus and the putamen - neostriatum (striatum,
corpus striatum)
• The globus palidus - paleostriatum (pallidum)

• the nuclei of the ventral part of the tegmen of mesencephalon, the

nuclei of the reticular formation, the nucleus ruber: efferent
pathways from the striopallidal complex to the motoneurons of the
s.c. – tectospinal, reticulospinal, rubrospinal tracts.

IMG.: ISBN 978–0–19–960953–6

Relation with other parts of NS
• Afferent and efferent pathways of the striopallidal system
• Cortico-subcortical neuronic circles and pathways
Afferent pathways
• The corpus striatum receives afferent input from extensive areas of the cerebral
cortex, particularly the motor areas of the frontal lobe, i.e., Brodmann areas 4, 6aα,
and 6aβ. These cortical afferents are derived from projection neurons of the
cerebral cortex, are glutamatergic, run ipsilaterally, and are topically organized.
• There are probably no reciprocal fibers running from the corpus striatum back to
the cortex.
• A further point-to-point afferent input to the corpus striatum is derived from the
centromedian nucleus of the thalamus, and is probably excitatory. This afferent
pathway transmits impulses from the cerebellum and the midbrain reticular
formation to the striatum.
• The substantia nigra sends dopaminergic afferent fibers to the striatum, whose
loss is the cause of Parkinson disease
• Finally, the striatum also receives a serotonergic input from the raphe nuclei.
Efferent pathways
Efferent pathways of the corpus striatum.
The major efferent projections of the corpus striatum go to the external
and internal segments of the globus pallidus.
Further efferent fibers travel to the pars compacta and pars reticulata
of the substantia nigra. The cells of origin of the striatal efferent fibers
are GABAergic spiny neurons, the most common cell type in the
striatum.
Cortico-basal ganglia-thalamo-cortical loop

• Regulation of movements:
• Sensomotor
• Oculomotor
• Regulation of cognitive functions
and behavioral reactions:
• Dorsolateral prefrontal
• Lateral orbitofrontal
• Anterior cingulated

The cortico- striato- thalamo- cortical loops follow a generic pattern, which is mirrored in parallel motor, oculomotor, cognitive, and limbic circuits.4
Functional differences are related to the specific subregions of cortex, striatum, pallidum, nigra, and thalamus. Note that this model of basal ganglia connectivity
emphasizes segregated information processing within each of the parallel loops. SMA, supplementary motor area; FEF, frontal eye fields; DLPFC, dorsolateral prefrontal
cortex; OFC, orbitofrontal cortex; ACC, anterior cingulate cortex; Gpi, globus pallidus internal; SNr, substantia nigra pars reticulata; and thalamic nuclei including
ventrolateral, VL, ventral anterior, VA, mediodorsal, MD.
Reproduced from Prog Brain Res., 85, Alexander GE, Crutcher MD, DeLong MR, Basal ganglia- thalamocortical circuits: parallel substrates for motor, oculomotor, ‘prefrontal’ and ‘limbic’ functions,
pp. 119– 46, Copyright (1991), Elsevier.
 IMG.: ISBN 978– 0– 19– 965594–
6

Corpus striatum - globus palidus

• Strionigral (direct) tract: bind of

the caudate nucleus and putamen
to the internal segment of the
globus palidus and reticular part
of the black substance.
• Striopallidal (indirect) tract: from
the corpus striatum to the
external segment of the globus
palidus, further to the subthalamic
nucleus and then to the internal
segment of the globus paliodus
and reticular part of the black
substance.
The left panel illustrates the influential ‘dual circuit model’, in which the output of the basal ganglia is determined by the balance between the direct pathway,
with striatonigral inhibitory connections that promote behaviour, and the indirect pathway, via the external globus pallidus (GPe) and subthalamic nucleus (STN), that
suppress behaviour. The balance between these two pathways is modulated by dopaminergic inputs from the substantia nigra pars compacta (SNc) and the ventral
tegmental area (VTA), which act on D1 and D2 dopamine receptors that are differentially expressed in the direct and indirect pathways. The right- hand panel brings
together the evidence for a more complex connectivity, including dopaminergic modulation at multiple sites, and reciprocal connections among the globus pallidus pars
externa, subthalamic nucleus, and globus pallidus pars interna (Gpi). SNr, substantia nigra pars reticulata. Modified from Nat Rev Neurosci. 11(11), Redgrave P, Rodriguez M, Smith Y, Rodriguez- Oroz MC, Lehericy S, Bergman H,
et al. Goal- directed and habitual control in the basal ganglia: implications
for Parkinson’s disease, pp. 760– 72, Copyright (2010), Nature Publishing Group.
Functions of the extrapyramidal system
• Realization and correction of the voluntary movements
• The myotatic function prepares muscles, provides the muscle tone for
making movements
• Maintenance of the segmentary apparatus in the constant readiness for
action
• Implementation of spontaneous motor actions of the indicative and
protective character (start-reflex)
• Participation in the expressive mimic movements
• Formation and choice of the motor program, initiation and realization of
the movement
Neurotransmitters
• Glutamate
• Aspartate
• GABA (gamma-aminobutyric acid)
• Norepinephrine
• Serotonin
• Epinephrine
• Dopamine
• Substance-P
Dopaminergic neuronic systems
• The nigrostriar dopaminergic pathway
• degeneration: depression of releasing dophamine from the nigrostriar terminals
in the corpus striatum (Parkinson’s disease)
• The mesolimbic pathway
• compact part of subst. nigra (mesencephalon) – nuclei of the forebrain’s limbic
area – ventral part of the corpus striatum - frontal lobe cortex
• Hypofunction (decrease of quantity of dopamine): the psychomotor akinesia,
emotional alienations
• The mesocortical pathway
• ventral layer of the tegment (mesencephalon) – cerebral cortex (prefrontal,
cingular, olfactory regions)
• Function: ingibitory controlling impact on the behavioral activity.
Types of
dopamine
receptors
• D1-D5
• D1 activation: dystonia
• D2 activation: choreic
hyperkinesia
Acetylcholine
• Promotes excition of neurons of the caudate nucleus

• The functional condition of the caudate nucleus is defined by balance

between the dopaminergic and acetylcholinergic systems
GABA
The striatal GABA-ergic neurons slow down the activity of dopaminergic
neurons of the substance nigre (control the dopamine level in the
corpus striatum)
The nigrostriar pathways are the complex functional system , non-
uniform on their physiological and biochemical components, wich
lesion leads to various motor disorders.
Examining the functions
1. General condition
2. Posture
3. Muscular tone
4. Facial expressions
5. Expressiveness of speech
6. Quality of performing the voluntary movements
7. Walking
8. * tremor
9. *hyperkinesia
10. *vegetative disturbances

NOT THE FORCE OF MUSCULAR CONTRACTION, BUT THE QUALITY OF MOVEMENTS

The syndromes of lesions of the
extrapyramidal system
• A. Strümpell (1920)

1. The hypokinetic-hypertonic syndrome

2. The hyperkinetic-hypotonic syndrome
The hypokinetic-hypertonic syndrome
(Parkinson’s syndrome, akinetic-rigid syndrome, amyostatic syndrome)

1. Hypokinesia
2. Muscular rigidity
3. Parkinsonic tremor
4. The vegetative disturbances
5. The mental disturbances
Hypokinesia

• The mask face

• Bradylalia
• The writing is slowed down
• Micrography
• Mannequin pose
Micrographia with decrementing script size in a patient with idiopathic Parkinson’s disease
• The symptom of a statue
• Small slow shuffling steps
• Acheirokinesis
• The postural instability
• Propultion/retropulsion/lateropultion
• The paradoxical kinesia (sometimes)
Muscular rigidity
(the muscular hypertonia)
• The same in the flexors and extensors
• Tone is increased already in the start
• “waxy rigidity” – the plastic muscular hypertonia
• “gear wheel”
• “bent posture”
• The symptom of “air pillow”

• The method of passive movements in joints

Parkinsonic tremor

(the tremor of rest)

• Rolling pills
• Account of coins

• no tremor during sleep and

movements
The vegetative disturbances
• Orthostatic hypotension
• Tachycardia in rest
• Arterial hypertension in the horizontal position
• Hypohidrosis / anhidrosis
• Impotency
• Constipations
• Diarrhea
• Urine incontinence
• The sebaceous face
• Hyperkeratosis
• Hypersalivation
The mental disturbances
• In later stages
• Bradyphrenia
• Viscosity of thinking
• Pedantism
• Acairia
The hyperkinetic-hypotonic syndrome
• Hyperkineses: the automatic violent involuntary movements, which
prevent performing the purposeful movements and are followed by
depression of the muscular tone

• Disappear during the sleep and intensifying at movements and under

the influence of emotions
The hyperkinetic-hypotonic syndrome
• Groups:
• H. of the level of the brain stem:
• Tremor
• Myoclonia
• Tics
• Spastic torticollis
• Facial paraspasm
• H. of the subcortical level:
• Chorea
• Athetosis
• Hemiballism
• Torsion dystonia
• The compound cortico-subcortical h. (the
myoclonus epilepsy)
Tremor
• The tremor of action:
• Postural
• Kinetic (the simple kinetic tremor
and the intension tremor)
• Isometric
• The tremor of rest
Dystonia
• Tonic or clonic-tonic movements
• Types:
• The focal dystonia:
• Oromandibular d.
• Blepharospasm
• Cervical d. (spastic torticollis):
• Torticollis
• Anterocollis
• Retrocollis
• Spasm of the lower extremities
• The segmentary dystonia
• The generalized dystonia (the torsion dystonia)
• The hemidystonia
• The multifocal dystonia
 Cervical Dystonia

Torticollis Anterocollis Retrocollis

Professional
dystonia
• The writer’s cramp
Musician’s Dystonia
• The typist’s spasm
• The pianist’s spasm
• etc.

Writer’s Cramp
Rower’s Dystonia
Myoclonia
• Myoclonus: brief, isolated,
involuntary, random, jerk
movement of a group of muscles.
Intermittent with distinct pause
between each movement.
• On the distribution:
• Generalized
• Multifocal
• Segmentary
• Focal
• On the mechanism:
• Spontaneous
• Reflex
• Kinetic
Myoclonia
• On the pathophysiological
mech.:
• Positive
• Negative
• Localization:
• Cortical
• Subcortical
• Brain stem
• Spinal
• Peripheral
The choreal
hyperkinesia
• The fast random consensual
motions with the expressed
locomotor effect
• Arises in different parts of the
body (either at rest or during
the movements)
• Constantly in motion
• Lesion of corpus striatum

Chorea Acanthocytosis with Head Drops and Trunk Flexions

Athetosis

• repetitive involuntary, slow, sinuous,

writhing movements, which are especially
severe in the hands. There are also elements
of postural disturbance. Usually combined
with chorea known as chorea-athetosis
• The neuropathological basis of athetosis is
loss of neurons in the striatum, the globus
pallidus, and, less commonly, the thalamus
Ballism
• This rare movement disorder is caused by lesions of the subthalamic
nucleus. It leads to large-amplitude flinging/throwing movements of
the limbs, proceeding from the proximal joints. In the vast majority of
cases it arises on one side only (hemiballism) contralateral to the
lesion.
• The neuropathological substrate of ballism is a lesion of the
contralateral subthalamic nucleus (corpus Luysii) and/or its fiber
connections to the thalamus.
 • Repetitive, stereotyped, semipurposeful movement.
• Patient could willingly suppress them at expense of mounting inner tension

Tics • There are 2 types of tics

• Simple tics of children: transient or chronic
• Complex tics: Tourette syndrome (tics, vocalization, obsessive behavior)
Akathisia
• Restlessness, irresistible requirement to move in order to reduce a
strain and feeling of discomfort
Statocoordinatory system
#cerebellum #functions_of_the_cerebellum
#the_posterior_spinocerebellar_tract #ataxia
#the_anterior_spinocerebellar_tract #finger_to_nose_test
#the_frontopontine_tract #heel_to_knee_test
#the_occipitotemporal_tract_of_the_pons #diadichokinesis_test
#the_efferent_pathways_of_the_cerebellum #the_test_on_proportionality_of_the_move
ments
#megalography
#nystagmus
Topic 3
The clinical classification of the sensitivity. Anatomy of sensitive tracts.
Reseasrch methods. Kinds and types of violations of the sensitivity (the
complexes of symptoms of sensitive violations in defeat of different
levels of sensitive tracts). Practical skills.
thank you for your attention