Transcript EKSTRAPİRAMİDAL SİSTEM VE BAZAL GANGLİA

Burcu Ormeci,MD
Department of Neurology
Extrapyramidal System
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 2 main system manage motor functions
 Pyramidal system
 Corticospinal tract
 Extrapyramidal system
 Basal ganglia
 caudate
 putamen
 globus pallidus
Neurotransmitters/Neuromodulators
at Basal Ganglia
 GABA  inhibitory
 Glutamate  excitatory
 Dopamine 
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 D1 receptors  excitatory
 D2 receptors  inhibitory
 Acetylcholine modulator
 Not excitatory or inhibitory
 It changes excitability
Motor Functions
of the Basal Ganglia

 The initiation and planning of movements
 Adjusting speed and magnitude of movement
 Automatically implementation of learned motor
programs (walking, cycling, etc.)
 Implementation of consecutive or simultaneous
movements
 Adjustment of muscle tone
 Truncal stability
Classification of The
Movement Disorders
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 Movement disorders are classified according to
dominant abnormal or involuntary movements
 hypokinetic (reduced movement)
 hyperkinetic (increased movement)
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HYPOKINETIC MOVEMENT
DISORDERS
Hypokinetic Movement
Disorders
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 It is named «Akinetic-rigid syndromes»
 Parkinson's syndrome is prototype of Akinetic-rigid
syndromes
Parkinson’s Syndrom
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 Symptoms
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Bradykinesia-akinesia
Rigidity
Postural instability
Resting tremor
 may be accompanied, but it is not necessary
Parkinson’s Syndrom
DEGENERATİVE
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1 - Idiopathic Parkinson's disease (IPD)
2 - Parkinson's plus syndromes
multisystem atrophy, Progressive supranuclear palsy
corticobasal degeneration, Lewy Body Dementia
3 - Parkinsonism associated other degenerative diseases
Spinocerebellar ataxia type 2,3,17
Huntington's Disease
Striapallidodentat bilateral calcinosis (Fahr's Disease)
Frontotemporal dementia-parkinsonism complex
Dentatorubropallidolusian atrophy
Nöroakantositoz ect
Underlying pathology of these diseases are degenerative cell loss and
not be detected in another reason
Parkinson’s Syndrom
Symptomatic
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1 - Vascular (small vessel disease-related subcortical encephalopathy, multiple
lacunar infarcts, basal ganglia and Brainstem hemorrhages and infarcts)
2 - Normal pressure hydrocephalus
3 - Tumors
4 - Drug-induced (neuroleptics, dopamine exhausting drugs, calcium channel
blockers, antiemetics, valproic acid, lithium)
5 - Intoxications (carbon monoxide, manganese, potassium permanganate,
ephedrine abuse, mercury and other heavy metals, organic solvents, paint
thinner, carbon disulfide, MPTP, cyanide)
6 - Infections (encephalitis, prion disease, neurosyphilis, toxoplasmosis)
7 - Metabolic causes (hypoxia, hypoparathyroidism, extra-pontine miyelinozis,
chronic liver disease, Wilson's disease)
8 - Head injury, dementia pugilistica (boxer)
9 - Demyelinating Diseases
10 - Psychogenic Parkinsonism
IDIOPATIC PARKINSON’S
DISEASE
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 Constitutes 80-85% of parkinsonism
 The average age of onset is 50-60 years
 Early-onset <40 years
 Juvenile <20 years
 Loss of dopaminergic cells in the substantia nigra pars
compacta
 Lewy bodies
 When dopaminergic cell loss reach to 60-70%, clinical
symptoms start to occur
IDIOPATIC PARKINSON’S
DISEASE
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 The cardinal symptoms of Parkinson's disease
 Bradykinesia
+
 Rigidity
 Resting tremor
 Postural instability
*Not need to be all-in-one
*The disease often begins from half of the body
asymmetrically
IDIOPATIC PARKINSON’S
DISEASE
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 Other clinical symptoms of Parkinson's disease
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Anteflexion posture
Freezing phenomenon
Gait disorders
Speech disorders
Micrography, dysphagia
Autonomic disorders
Mental-emotional symptoms
Sleep disorders
Odor and visual perception disorders
IDIOPATIC PARKINSON’S
DISEASE
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 Resting Tremor
 Often, the first clinical finding is motor
symptoms, 15% never seen
 Frequency is 4-6 Hz
 Usually emerges on the hands, sometimes on the
feet, tongue, jaw and lip
 Audio and head tremor never emerges
 Increases with stress, mental activity and walking
IDIOPATIC PARKINSON’S
DISEASE
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 Bradykinesia / Akinesia
 The most characteristic symptom of basal ganglia
disorders
 Presence is necessary to diagnosis
 Bradimimi, micrography, decrease of associate
movements and sialorrhoea develop because of
this symptom
 The most basic symptom of disability
IDIOPATIC PARKINSON’S
DISEASE
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 Rigidity
 Due to agonist and antagonist muscles have
increased tone simultaneously
 Called cogwheel phenomenon
 Emerges by passive motion
 Increases with other extremity motion (Froment's
sign)
 Patients describe as stiffness or hardness
IDIOPATIC PARKINSON’S
DISEASE
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 Postural instability
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The most common cause of disability
Occurs in later stages of the disease
Due to loss of postural reflex
The most common cause of falls
Pull test is used for diagnosis
Patients fall down while sitting as a block
TREATMENT of
PARKINSON’S DISEASE
 Dopaminergic drugs
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 Levodopa (dopamine precursor)
 Dopamine agonists
 These drugs mimic the effect of dopamine as binding to
dopamine receptors
 Anticholinergic agents
 Re-provide deteriorated balance between dopamine and
acetylcholine in the basal ganglia
 MAO-B inhibitors
 Main enzyme which destroyed the dopamine
 Dopamine stays in the synaptic gap for longer time
 COMT inhibitors
 Amantadine (PK-Mertz)
PARKINSON’S DISEASE
TREATMENT
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 SURGICAL
 Destruction of problematic areas
 Stereotactic surgery
 Gamma-knife
 Deep brain stimulation
 High-frequency electrical impulses via deep electrodes
reduce the activity of the cells in problematic areas
PARKINSON PLUS SYNDROMS
 Common features
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 Classic signs of Parkinson's syndrome (bradykinesia,
rigidity, tremor, postural reflex abnormalities)
+
 Additional signs and symptoms
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HYPERKINETIC
MOVEMENT DISORDERS
DYSTONIA
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 Characterized by involuntary, continuous, twisting,
rotating muscle contraction
 Recurrent
 Results with temporary or permanent abnormal
postures
 Disappears during sleep
 Dystonic contractions temporarily loss with touch
 Sensory trick
Type of Dystonias
 Hemifasyal Spasm
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 Only on half of the face
 May be continuous or intermittent
 Semi-rhythmic contractions
 Blepharospasm
 Increasing of eye blink frequency functional blindness
 Oromandibulolingual Dystonia
 Dystonia around the mouth-jaw-tongue
 Laryngeal Dystonia (pasmodic Dysphonia)
 Difficulty of voicing and speech
Type of Dystonias
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 Cervical Dystonia (spasmodic torticollis)
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Torticollis
Laterocollis
Anterocollis
Retrocollis
 "Task Specific” dystonia
 Occurs only during selective motor functions
 The most common is "writer's cramp"
CHOREA and BALLISM
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 Arrhythmic, rapid, saltatory or smooth, simple or
complex movements
 Chorea
 Amplitude is smaller
 Usually seen in distal of extremity
 Ballism
 Amplitude is greater
 Usually seen in proximal of extremity
TREMOR
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 Tremor,
 is involuntary, rhythmic oscillations
 occur as a result of synchronous contraction of the
reciprocal muscles
1- Kinetic tremor
- Intensiyenel tremor (cerebellum)
2- Postural Tremor
3-Resting tremor
Tremor
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Physiological tremor
 10-12 hz, postural tremor with low-amplitude
 Etiology
 Mental reasons (anxiety, stress, fatigue),
 Metabolic causes ( fever, thyrotoxicosis, hypoglycaemia,
ect.),
 Drugs (neuroleptics, beta-adrenergic agonists, valproic acid,
lithium, steroids, dopamine agonists, antidepressants),
 Toxins (mercury, lead, arsenic)
 Some food ingredients (caffeine)
May lead to increased physiological tremor
MYOCLONUS
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Sudden, short-term (<100ms), as fast as
lightning
 Form of throwing, jumping
 Positive myoclonus
 The sudden contraction of a particular muscle group
 Negative myoclonus (asterixis)
 Sudden relaxation of the muscles while maintain a
posture
MYOCLONUS
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 Physiological myoclonus
 While falling asleep, after excessive exercise , in anxiety
 Essential myoclonus
 There is no pathological signs and symptoms related to
another disorders
 Reduce the symptoms with Alcohol is typically
 Symptomatic myoclonus
 During the course of diseases such as Liver failure, uremia,
electrolyte imbalance, poisoning, head trauma, hypoxicischemic ensafalopati (Lance-Adams syndrome),
neurodegenerative diseases, central nervous system
infections, prion diseases… etc
Sleep-related
Movement Disorders
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Restless Legs Syndrome
 It is identified leg discomfort at rest or before sleep
Periodic limb movements of sleep
 Semirhythmic involuntary movements during sleep
especially in the feet
Drug-related
Movement Disorders
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 Acute Dystonic Reactions
 Within a few hours after drug intake
 Especially around the head, neck, face, tongue and
mouth
 Form of dyskinetic and dystonic involuntary
movements
 Subacute Parkinsonism or Akathisia
 Neuroleptic useing for last 1-3 months
 Usually reversible
 Tardive Syndromes
 As a result of long-term use of neuroleptic
 Sometime irreversible
PSYCHOGENIC MOVEMENT
DISORDERS
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 Can mimic all kinds of movement disorders such as dystonia,
myoclonus, tremor, tic disorder
 Symptoms onset suddenly
 Emerge after psychic trauma
 There is serious handicaps
 It is bizaare, doesn’t like normal forms
 If the patient's attention is disrupted, symptoms ameliorate
 There are some periods of self-recovery
 Positive response to placebo treatment
 The presence of secondary gain
 Neurological examination is completely normal
 The presence of symptoms that suggestive psychopathology
Treatment of Hyperkinetic
Movement Disorders
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 Treatment is symptomatic
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Anticholinergics
Antidopaminergics (neuroleptics)
Baclofen
Benzodiazepines
Botulinum toxin