1. EXTRAPYRAMIDAL SYSTEM and BASAL GANGLIA
Burcu Ormeci,MD
Department of Neurology

2. Extrapyramidal System
2 main system manage motor functions
Pyramidal system
Corticospinal tract
Extrapyramidal system
Basal ganglia
caudate
putamen
globus pallidus
EPS is created by the basal ganglia
Consist of: -den oluşmaktadır

4. Neurotransmitters/Neuromodulators at Basal Ganglia
GABA  inhibitory
Glutamate  excitatory
Dopamine 
D1 receptors  excitatory
D2 receptors  inhibitory
Acetylcholine modulator
Not excitatory or inhibitory
It changes excitability

5. 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
Ball is played, ball was played
This plan is made by the basal ganglia

6. Classification of The Movement Disorders
Movement disorders are classified according to dominant abnormal or involuntary movements
hypokinetic (reduced movement)
hyperkinetic (increased movement)

7. HYPOKINETIC MOVEMENT DISORDERS

8. Hypokinetic Movement Disorders
It is named «Akinetic-rigid syndromes»
Parkinson's syndrome is prototype of Akinetic-rigid syndromes
Cogwhile rigidity

9. Parkinson’s Syndrom Symptoms Bradykinesia-akinesia Rigidity
Postural instability
Resting tremor
may be accompanied, but it is not necessary
Fall down

10. Parkinson’s Syndrom DEGENERATİVE 2 - Parkinson's plus syndromes
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

11. Parkinson’s Syndrom Symptomatic
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

12. IDIOPATIC PARKINSON’S DISEASE
Constitutes 80-85% of parkinsonism
The average age of onset is 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

13. IDIOPATIC PARKINSON’S DISEASE
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

14. IDIOPATIC PARKINSON’S DISEASE
Other clinical symptoms of Parkinson's disease
Anteflexion posture
Freezing phenomenon
Gait disorders
Speech disorders
Micrography, dysphagia
Autonomic disorders
Mental-emotional symptoms
Sleep disorders
Odor and visual perception disorders

15. IDIOPATIC PARKINSON’S DISEASE
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

16. IDIOPATIC PARKINSON’S DISEASE
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

17. IDIOPATIC PARKINSON’S DISEASE
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

18. IDIOPATIC PARKINSON’S DISEASE
Postural instability
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

20. TREATMENT of PARKINSON’S DISEASE
Dopaminergic drugs
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)

21. PARKINSON’S DISEASE TREATMENT
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

22. PARKINSON PLUS SYNDROMS
Common features
Classic signs of Parkinson's syndrome (bradykinesia, rigidity, tremor, postural reflex abnormalities) +
Additional signs and symptoms

23. HYPERKINETIC MOVEMENT DISORDERS

24. DYSTONIA
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

25. Type of Dystonias Hemifasyal Spasm Blepharospasm
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

26. Type of Dystonias Cervical Dystonia (spasmodic torticollis)
Laterocollis
Anterocollis
Retrocollis
"Task Specific” dystonia
Occurs only during selective motor functions
The most common is "writer's cramp"

27. CHOREA and BALLISM
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

28. TREMOR 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
Aksiyon Tremoru (kinetik tremor)
Bir ekstremiteyi aktif olarak kullanırken, hareket esnasında ortaya çıkar. Genelde yüksek frekanslıdır ancak genliği az ya da çok olabilir, genlik hareket esnasında hep aynı kalır. Postüral tremor gibi aksiyon tremoru da en sık esansiyel tremor hastalığında görülür.
İntansiyon Tremoru
Bu tip tremorun en büyük özelliği istemli hareket esnasında ortaya çıkması ve hedefe yaklaşırken tremorun genliğinin çok artmasıdır. Örneğin parmak-burun testinde hasta parmağını burnuna yaklaştırdıkça tremorun genliği artar ve hasta hedefi bulamaz. İntansiyon tremoru düşük frekanslı, kaba bir tremordur ve kural olarak serebellum ve bağlantılarının tutulması sonucu ortaya çıkar. Dizartri ve nistagmus tremora eşlik edebilir.

29. Tremor 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

30. MYOCLONUS 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

31. MYOCLONUS Physiological myoclonus Essential 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, hypoxic-ischemic ensafalopati (Lance-Adams syndrome), neurodegenerative diseases, central nervous system infections, prion diseases… etc

32. Sleep-related Movement Disorders
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

33. Drug-related Movement Disorders
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

34. PSYCHOGENIC MOVEMENT DISORDERS
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

35. Treatment of Hyperkinetic Movement Disorders
Treatment is symptomatic
Anticholinergics
Antidopaminergics (neuroleptics)
Baclofen
Benzodiazepines
Botulinum toxin