EXTRAPYRAMIDAL SYSTEM and BASAL GANGLIA

1. EXTRAPYRAMIDAL SYSTEM andBASAL 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

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

6. Classification of The Movement Disorders • Movementdisordersareclassifiedaccordingto dominant abnormalorinvoluntarymovements • hypokinetic (reducedmovement) • hyperkinetic (increasedmovement)

7. HYPOKINETIC MOVEMENT DISORDERS

8. HypokineticMovementDisorders • It is named «Akinetic-rigid syndromes» • Parkinson's syndrome is prototype of Akinetic-rigid syndromes

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

10. Parkinson’s Syndrom DEGENERATİVE 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 • Theaverageage of onset is 50-60 years • Early-onset <40 years • Juvenile <20 years • Loss of dopaminergiccellsin thesubstantianigra pars compacta • Lewybodies • Whendopaminergiccelllossreachto 60-70%, clinicalsymptoms start tooccur

13. IDIOPATIC PARKINSON’S DISEASE • Thecardinalsymptoms of Parkinson'sdisease • Bradykinesia + • Rigidity • Resting tremor • Posturalinstability *Not needto be all-in-one *Thediseaseoftenbeginsfromhalf of the body asymmetrically

14. IDIOPATIC PARKINSON’S DISEASE • Otherclinicalsymptoms of Parkinson'sdisease • Anteflexionposture • Freezingphenomenon • Gaitdisorders • Speech disorders • Micrography, dysphagia • Autonomicdisorders • Mental-emotionalsymptoms • Sleepdisorders • Odorandvisualperceptiondisorders

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 ofPARKINSON’S DISEASE • Dopaminergicdrugs • Levodopa (dopamine precursor) • Dopamineagonists • Thesedrugsmimictheeffect of dopamineas bindingtodopaminereceptors • Anticholinergicagents • Re-providedeterioratedbalancebetweendopamineandacetylcholine in thebasalganglia • MAO-B inhibitors • Main enzymewhichdestroyedthedopamine • Dopaminestays in thesynapticgapforlongertime • COMT inhibitors • Amantadine (PK-Mertz)

21. PARKINSON’S DISEASETREATMENT • 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 • Characterizedbyinvoluntary, continuous, twisting, rotatingmusclecontraction • Recurrent • Resultswithtemporaryorpermanentabnormalpostures • Disappearsduringsleep • Dystoniccontractionstemporarilylosswithtouch • Sensorytrick

25. Type of Dystonias • HemifasyalSpasm • Only on half of theface • May be continuousorintermittent • Semi-rhythmiccontractions • Blepharospasm • Increasing of eyeblinkfrequencyfunctionalblindness • OromandibulolingualDystonia • Dystoniaaroundthemouth-jaw-tongue • LaryngealDystonia (pasmodicDysphonia) • Difficulty of voicingandspeech

26. Type of Dystonias • Cervical Dystonia (spasmodic torticollis) • 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, rhythmicoscillations • occur as a result of synchronouscontraction of thereciprocalmuscles 1- Kinetic tremor - Intensiyenel tremor (cerebellum) 2- Postural Tremor 3-Resting tremor

29. Tremor Physiological tremor • 10-12 hz, postural tremor withlow-amplitude • Etiology • Mentalreasons (anxiety, stress, fatigue), • Metaboliccauses ( fever, thyrotoxicosis, hypoglycaemia, ect.), • Drugs (neuroleptics, beta-adrenergicagonists, valproicacid, lithium, steroids, dopamineagonists, antidepressants), • Toxins (mercury, lead, arsenic) • Somefoodingredients (caffeine) May leadtoincreasedphysiological 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 • Physiologicalmyoclonus • Whilefallingasleep, afterexcessiveexercise , in anxiety • Essentialmyoclonus • There is nopathologicalsignsandsymptomsrelatedtoanotherdisorders • ReducethesymptomswithAlcohol is typically • Symptomaticmyoclonus • Duringthecourse of diseasessuch as Liverfailure, uremia, electrolyteimbalance, poisoning, headtrauma, hypoxic-ischemicensafalopati (Lance-Adams syndrome), neurodegenerativediseases, centralnervoussysteminfections, priondiseases… 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 • SubacuteParkinsonism or Akathisia • Neuroleptic useingforlast 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’tlike 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