1. Movement disorders such as tremor, chorea, athetosis, dystonia, tics, and parkinsonism involve disturbances in the basal ganglia circuitry between the cortex, thalamus, and basal ganglia. 2. Parkinson's disease is characterized by rigidity, bradykinesia, tremor, and postural instability due to loss of dopaminergic neurons in the substantia nigra, leading to impaired inhibition of the striatum. 3. The progression of Parkinson's disease and accumulation of alpha-synuclein aggregates in the brainstem and forebrain can be staged according to Braak staging, beginning with the medulla oblongata in stage I and

1. Introduction to movement
disorders and
parkinsonism
Domina Petric, MD

2. Tremor
I.

3. • Tremor consists of a rhythmic oscillatory
movement around a joint.
• It is best characterized by its relation to
activity.
• Tremor at rest is characteristic of
parkinsonism, when it is often associated
with rigidity and an impairment of
voluntary activity.
Tremor

4. • Tremor may occur during
maintenance of sustained posture
(postural tremor) or during
movement (intention tremor).
• A conspicious postural tremor is the
cardinal feature of benign essential
or familial tremor.
Tremor

5. • Intention tremor occurs in patients
with a lesion of the brainstem or
cerebellum, especially when the
superior cerebellar peduncle is
involved.
• It may also occur as a manifestation
of toxicity from alcohol or certain
other drugs.
Tremor

6. Chorea
II.

7. • Chorea consists of irregular, unpredictable,
involuntary muscle jerks that occur in
different parts of the body and impair
voluntary activity.
• The proximal muscles of the limbs are
usually most severely affected.
• Abnormal movements of proximal muscles
are particularly violent: BALLISMUS.
Chorea

8. Chorea may be hereditary or
may occur as a complication
of number of general
medical disorders and of
theraphy with certain drugs.
Chorea

9. Athetosis, dystonia
III.

10. www.5minuteconsult.com
Athetosis

11. Sites.google.com
Dystonia

12. Perinatal brain damage with focal or generalized cerebral lesions
Acute complication of certain drugs
Accompaniment of diverse neurologic disorders
Isolated inherited phenomenon of uncertain cause (idiopathic torsion
dystonia, dystonia musculorum deformans)
Athetosis, dystonia

13. Tics
IV.

14. • Tics are sudden coordinated abnormal
movements that tend to occur
repetitively, particularly about the face
and head, especially in children.
• Tics can be suppressed voluntarily for
short periods of time.
• Common tics include repetitive sniffing
or shoulder shruging.
Tics

15. Tics may be single or multiple and
transient or chronic.
Gilles de la Tourette´s syndrome is
characterized by chronic mutliple tics.
Tics

16. • Many of the movement disorders have
been attributed to disturbance of the
basal ganglia.
The basic circuitry of the basal ganglia
involves three interacting neuronal loops:
• cortex
• thalamus
• basal ganglia
Basal ganglia

17. Parkinsonism
V.

18. • It is characterized by a combination of
rigidity, bradykinesia, tremor and
postural instability.
• It is usually idiopathic: Parkinson´s
disease or paralysis agitans.
• Cognitive decline occurs in many
patients as the disease advances.
Parkinsonism

19. • Personality changes
• Affective disorders: anxiety, depression
• Abnormalities of autonomic function: sphincter or
sexual functions, choking, sweating abnormalities,
disturbances of blood pressure regulation
• Sleep disorders
• Sensory complaints, pain
Nonmotor symptoms

20. The disease is generally
progressive, leading to
increasing disability unless
effective treatment is
provided.
Parkinsonism

21. impaired degradation of protiens
intracellular protein accumulation and aggregation
oxidative stress
mitochondrial damage
inflammatory cascades
apoptosis
Pathogenesis

22. • Recognized genetic abnormalities
accout for 10-15% of cases.
• Mutations of the α-synuclein gene at
4q21 or duplication/triplication of
the normal synuclein gene:
synucleinopathy.
Pathogenesis

23. • Mutations of the leucine-rich repeat
kinase 2 (LRRK2) gene at 12cen, and the
UCHL1 gene may also cause autosomal
dominant parkinsonism.
• Mutations in the parkin gene (6q25.2-
q27) cause early-onset, autosomal
recessive, familial parkinsonism or
sporadic juvenil-onset parkinsonism.
Pathogenesis

24. • Cigarette smoking, coffee, anti-
inflammatory drug use and high serum
uric acid levels may be protective.
• The incidence of the Parkinson´s disease
is increased in those working in teaching,
health care, farming and in those with
lead or manganase exposure and with
vitamin D deficiency.
Pathogenesis

25. Intracellular inclusion
bodies containing
α-synuclein.
Lewy bodies

26. Stage II
Higher brainstem
Stage I
Olfactory nucleus and
lower brainstem
Stage IV
The mesocortex and
thalamus
Stage V and VI
Neocortex
Stage III
Substantia nigra: motor
features develop
21 3 4 5, 6
Braak stages: Lewy bodies

27. • The disease begins in structures of the lower
brainstem and the olfactory system.
• In particular, the dorsal motor nucleus of the
vagus nerve in the medulla
oblongata and anterior olfactory nucleus are
affected.
• Lewy neurites, thread-like alpha-synuclein
aggregates, are more prevalent than globular
Lewy bodies in this stage.
Stage I

28. • It is characterized by additional lesions in
the raphe nuclei and gigantocellular
reticular nucleus of the medulla oblongata.
• The disease then moves up the brainstem,
traveling from the medullary structures to
the locus ceruleus in the pontine
tegmentum.
• Lewy neurites outnumber Lewy bodies.
Stage II

29. • At the beginning of stage III, the disease has
entered the substantia nigra and Lewy body
lesions begin to form in the pars compacta.
• The latter half of this stage involves disease
progression into the basal nucleus of Meynert,
a cluster of acetylcholine-rich neurons in the
basal forebrain.
• Further, structures affected in stages I and II
begin to develop more Lewy bodies.
Stage III

30. • It is characterized by severe dopaminergic cell
destruction in the pars compacta.
• There is also mesocortex and allocortex
involvement.
• The neocortex remains unaffected.
• In particular, pathology can be observed in the
amygdala and in the subnuclei of the thalamus.
• There is significant damage done to the
anterior olfactory nucleus.
Stage IV

31. • The disease has started to invade the
neocortex and spreads into the structures
of the temporal, parietal, and frontal
lobes.
• Cell death can be observed in the
substantia nigra, the dorsal motor nucleus
of the vagus nerve, the gigantocellular
reticular nucleus and the locus ceruleus.
Stage V

32. The disease has fully
invaded the neocortex,
affecting the motor and
sensory areas in the brain.
Stage VI

33. Wikimedia Commons
Braak stage I:
olfactory nucleus,
lower brainstem
Braak stage II:
higher brain stem
Olfactory and autonomic
disturbances
Braak stage III: substantia nigra
Motor disturbances
Braak stage IV:
mesocortex,
thalamus
Sleep disturbances
Braak stage V, VI: neocortex
Emotional and cognitive
disturbances

34. • The normally high concentration of
dopamine in the basal ganglia of the brain is
reduced in parkinsonism.
• Levodopa and dopamine agonists alleviate
many of the motor features.
• Restoration of the normal balance of
cholinergic and dopaminergic influences on
the basal ganglia: antimuscarinic drugs.
Pathogenesis

35. • In idiopathic parkinsonism, dopaminergic
neurons in the substantia nigra that normally
inhibit the output of GABAergic cells in the
corpus striatum are lost.
• Drugs that induce parkinsonian syndromes are
dopamine receptor antagonists (antipsychotic
agents) or lead to the destruction of the
dopaminergic nigrostriatal neurons (MPTP).
Pathogenesis

36. Norepinephrine is
also depleted in the
brain in parkinsonism.
Pathogenesis

37. • Katzung, Masters, Trevor.
Basic and clinical
pharmacology.
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• Wikipedia.org
Literature