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Movement Disorders
Basal ganglia
Functional circuits
of the basal ganglia
Classification
of movement disorders
• Hypokinetic-hypertonic movement disorders
– parkinsonism
• Hyperkinetic-hypotonic movement disorders
(dyskinesias)
– tremor
– chorea
– dystonia
– tics
– myoclonus
– ballism
Classification
of parkinsonism
1. Primary, idiopathic parkinsonism
Parkinson’s disease - 80 %
2. Secondary parkinsonism
• drug-induced
• toxic
• vascular
• post-hypoxic
• post-encephalitic
• post-traumatic
3.Parkinsonism-plus syndromes
• Progressive supranuclear palsy
• Levy body dementia
• Multiple system atrophy
• Cortico-basal degeneration
Pathophysiology
of parkinsonism
Pathogenesis of Parkinson’s disease
Dopamine loss
basal ganglia
Neurodegeneration
of substantia nigra
Neurodegeneration in Parkinson’s disease
enviromental factors ageing genetic factors
oxydative stress excitotoxity
lack of protective mechanisms
mitochondrial damage
loss of nigral cells
- loss of dopamine
loss of other cell populations
- loss of other
neurotransmitters
APOPTOSIS
• hypokinesia
• rigidity
• rest tremor
• postural
instability
Clinical features
of parkinsonism
Movement disorders
Diagnostic criteria
of Parkinson's disease
 Hypokynetic-hypertonic
syndrome
 Unilateral beginning of
the disease
(+) effect of levadopa
 The absence of postural
instability, autonomic
symptoms, cognitive
disturbances at early stage
Dopaminergic treatment
of Parkinson’s disease
COMT
catabolism
catabolism
МАО-В
hemato-encephalic barrier
L - DOPA
decarboxylase inhibitor
reuptake
dopamine agonists
Treatment of Parkinson’s disease
• Levadopa drugs (nakom, madopar)
• Dopamine agonists (mirapex, pronoran)
• MAO-B inhibitors (selegiline)
• Amantidine (midantan)
• COMT – inhibitors
• Anticholinergic drugs (ciclodol)
Motor fluctuations
"OFF" state
"ON" state
Motor fluctuations
Drug-induced dyskinesias
Surgical treatment of Parkinson's disease
• Stereotactic destruction of
– thalamus
– pallidum
• Deep brain stimulation of
– thalamus
– internal segment of pallidum
– subthalamic nucleus
• Transplantation of dopamine-producing cells
– embrional cells
tremor
chorea
dystonia
tics
myoclonus
ballism
Hyperkinesis
Tremor
involuntary
rhytmic oscillating movements
in limbs or the whole body due to
alternating contraction of agonist and
antagonist muscles
• Rest (pill-rolling) tremor
• Action tremor
– postural (occurs during maintenance of a
posture )
– kinetic (occurs during voluntary movement )
– intentional (appears in movements and
increases near of the aim)
Types of tremor
• Physiological
• Pathological: essential tremor
Classification of tremor
• Fatigue
• Anxiety
• Cold
• Hypoglycemia
• Hyperthyriodism
• Drugs (coffein, steroids)
Increased physiological tremor
Essential tremor
• It’s type of postural tremor
• Rhythmic, usually symmetric movement in the
hands, may involve the head
• Autosomal-dominant inheritance
• Onset may be in adolescence or early adult
years; if it’s develops late – senile tremor
• Aggravated by emotional stress, disappears
with a small amount of alcohol
• Neurological examination is normal except for
tremor
Essential tremor
• β-adrenoblockers (propranolol)
• Anti-epileptic drugs (primidone,
clonazepam)
Treatment of tremor
sustained muscle contractions
frequently causing twisting and
repetitive movement or abnormal
postures
Dystonia
Dystonia
Focal (affects only one region of
the body)
Segmental (two adjacent
regions)
Multifocal (two or more
nonadjacent regions)
Hemidystonia
Generalized
Dystonia
Focal dystonia
blepharospasm
oromandibular dystonia
cervical dystonia: torticollis (head
rotation), laterocollis (head tilt to
one side), anterocollis,
posterocollis (flexion or extension
of the neck)
graphospasm (writer’s cramp)
spastic dysphonia
toy or foot dystonia
Cranial
distonia
• clonazepam
• Myorelaxants (baclofen, tizanidin)
• Anticholinergic drugs
• Botulotoxin (botox, disport)
Treatment of dystonia
is irregular, arrhythmic,
unpredictable, jerky movement,
which involves the extremities and
the face in continuous, random
sequence
Chorea
• Hereditary: Huntington disease, Wilson disease
• Secondary: Sydenham chorea (rheumatic),
infections, encephalitis, systemic lupus
erythematosus
• Drug-induced chorea: antipsychotic, estrogens,
levadopa
• Metabolic and endocrine: hyperthyroidism,
hyperglycemic non-ketotic encephalopathy
• Senile chorea
Classification of chorea
Huntington disease
• Autosomal-dominant inheritance
• Abnormal irregular rapid jerky movements
• Progressive dementia
• The gait is unsteady with a tendency to bob
and weave
• Facial grimacing with involuntary movements
of the tongue
Chorea
• Psychotic drugs
– Haloperidol 3-6 mg daily
– sulpiride
– sonopax
Treatment of chorea
is involuntary,
brief, sudden
(till 0,1 sec) muscle contractions
producing visible movement
Myoclonus
Essential
Symptomatic (Alzheimer disease,
Huntington disease, liver disease,
paraneoplastic syndromes)
Physiological (sleep myoclonus, hiccups)
Epileptic
Classification of myoclonus
Myoclonus
• clonazepam
• Valproat acid (depakin)
• Carbamazepin, hexamidin
• Nootropic drug (pyracetam) in high dosage
- 12 g i/v (in posthypoxic myoclonus)
Treatment of myoclonus
- are repetitive, rapid, usually brief
involuntary non-rhitmic stereotyped
movements, which involve the face,
axial muscles and proximal limbs.
Tics interrupt normal voluntary motor
activity
Tics
• Simple motor (blinking, twitching,
shrugging of the shoulders)
• Complex motor (handshaking,
scratching, kicking, toughing)
• Simple vocal (moaning, grunting,
hissing, clicking, shouting, throat
clearing, sniffing, coughing)
• Complex vocal (echolalia, coprolalia)
• Generalized (Tourette syndrome)
Classification of tics
Tics
• Clonazepam, baclofen
• Neuroleptics (sonopax, tiaprid)
• Haloperidol 0.25-10 mg daily
Treatment of tics
-sudden onset of violent involuntary flinging
movements of the limbs. Usually affects only one
side of the body (hemiballism).
The appearance is similar to a baseball pitcher’s
windup.
Caused by a lesion of the subthalamic nucleus,
usually as a result of stroke
Ballism
Ballism
Treatment: neuroleptic drugs

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