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EXTRAPYRAMIDAL SYSTEM and BASAL GANGLIA

EXTRAPYRAMIDAL SYSTEM and BASAL GANGLIA. Burcu Ugurel,MD Department of Neurology. Extrapyramidal System. Motor functions are managed by 2 main system Pyramidal system Corticospinal tract Extrapyramidal system Basal ganglia caudate putamen globus pallidus.

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EXTRAPYRAMIDAL SYSTEM and BASAL GANGLIA

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  1. EXTRAPYRAMIDAL SYSTEM andBASAL GANGLIA Burcu Ugurel,MD Department of Neurology

  2. ExtrapyramidalSystem • Motor functionsaremanagedby 2 main system • Pyramidalsystem • Corticospinaltract • Extrapyramidalsystem • Basalganglia • caudate • putamen • globuspallidus

  3. Neurotransmitters/Neuromodulators at BasalGanglia • GABA inhibitory • Glutamateexcitatory • Dopamine • D1 receptors excitatory • D2 receptors inhibitory • Acetylcholinemodulator • Not excitatoryorinhibitory • Itchangesexcitability

  4. Motor Functionsof theBasalGanglia • Theinitiationandplanning of movements • Adjustingspeedandmagnitude of movement • Automaticallyimplementation of learned motor programs (walking, cycling, etc.) • Implementation of consecutiveorsimultaneousmovements • Adjustment of muscletone • Truncalstability

  5. CLASSIFICATION of the MOVEMENT DISORDERS • Movementdisordersareclassifiedaccordingto dominant abnormalorinvoluntarymovements • hypokinetic (reducedmovement) • hyperkinetic (increasedmovement)

  6. HISTORY at the MOVEMENT DISORDERS • Determine of theaffected body regionorregions • Inoneregion • focal • Multipleregionsadjacenttoeachother • segmental • multipleregionsindependentfromeachother • multifocal • Ifentire body is affected • generalized • Predominantlydistalorproximal

  7. HISTORY at the MOVEMENT DISORDERS • Does it occur at rest or on movement • Essential tremor typicallyoccursduringthemotion, disappears at rest • OppositeforParkinson's tremor

  8. HISTORY at the MOVEMENT DISORDERS • speed of movement • Myoclonusfast as lightning • Koreafast • Atetozslow-flowing • Rhythmicityandcontinuity • Tremor rhythmic • Koreaandmyoclonus arrhythmic

  9. HISTORY at the MOVEMENT DISORDERS • Theemergenceoraggravationwithcertainpostures • orthostatic tremor occurswith a fixedstanding • Most of themdisappearduringsleep • Some of themcontinueduringsleep • palatal tremor • Some of themoccurwhenjustpassingofftosleeporduringsleep • Restlesslegssyndrome • Periodiclimbmovements of sleep

  10. HISTORY at the MOVEMENT DISORDERS • The presence of sensorysymptomsbeforeinvoluntarymovement • Suppressesinvoluntarymovement as a voluntary • The presence of factorsthatstarting, worseningorcorrecting of involuntarymovements • Ticdisorders

  11. HAREKET BOZUKLUKLARI • Thefirst step at diagnosis; • Basic movementdisordershould be identifiedandnamed as a phenomenological • Tremor, dystonia, bradykinesia, etc.

  12. HYPOKINETIC MOVEMENT DISORDERS

  13. HYPOKINETIC MOVEMENT DISORDERS • Akinetic-rigidsyndromes • Clinicalfeatures • Decreases of motion • Difficulties on motion • Slowdown • Increasedmuscletone • Parkinson'ssyndrome is prototype of Akinetic-rigidsyndromes

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

  15. Parkinson’s Syndrom AetiologicalClassification • Degenerative • IdiopathicParkinson'sDisease (IPD), • Parkinson'splussyndromes • otherdegenerativediseasesassociatedwithparkinsonism Underlyingpathology of thesediseasesaredegenerativecelllossand not be detected in anotherreason • Symptomatic

  16. 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) Hemiparkinson-hemiatrofi syndrome Frontotemporal dementia-parkinsonism complex Dentatorubropallidolusian atrophy Pallidal degeneration Striatal necrosis associated mitochondrial diseases Pantothenate kinase associated neurodegeneration Nöroakantositoz

  17. Parkinson’s Syndrom Symptomatic 1 - Vascular (smallvesseldisease-relatedsubcorticalencephalopathy, multiplelacunarinfarcts, basalgangliaandBrainstemhemorrhagesandinfarcts) 2 - Normal pressurehydrocephalus 3 - Tumors 4 - Drug-induced (neuroleptics, dopamineexhaustingdrugs, calciumchannelblockers, antiemetics, valproicacid, lithium) 5 - Intoxications(carbonmonoxide, manganese, potassiumpermanganate, ephedrineabuse, mercuryandotherheavymetals, organicsolvents, paintthinner, carbondisulfide, MPTP, cyanide) 6 - Infections (encephalitis, priondisease, neurosyphilis, toxoplasmosis) 7 - Metaboliccauses (hypoxia, hypoparathyroidism, extra-pontinemiyelinozis, chronicliverdisease, Wilson'sdisease) 8 - Headinjury, dementiapugilistica (boxer) 9 - DemyelinatingDiseases 10 - PsychogenicParkinsonism

  18. 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 dopaminergiccellscontainingmelanin in thesubstantianigra pars compacta • Lewybodies • Whendopaminergiccelllossreachto 60-70%, clinicalsymptoms start tooccur

  19. Pathology of Parkinson'sdisease

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

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

  22. IDIOPATIC PARKINSON’S DISEASE • Non-motor symptoms • REM sleepbehaviordisorder • Loss of smell sense (anosmia) • constipation Thesesymptoms can occurmanyyearsbeforefromthe start of the motor symptoms • Parkinson'sdiseasedementiareachesto 50-80% on fifteenth-twentiethyears

  23. IDIOPATIC PARKINSON’S DISEASE • Tremor • Can be seen as resting/postural /re-emergen tremor • Often, thefirstclinicalfinding is motor symptoms, 15% neverseen • Frequency is 4-6 Hz • Usuallyemerges on thehands, sometimes on thefeet, tongue, jawandlip • Audioandhead tremor neveremerges • Increaseswithstress, mentalactivityandwalking

  24. IDIOPATIC PARKINSON’S DISEASE

  25. IDIOPATIC PARKINSON’S DISEASE • Bradykinesia / Akinesia • Themostcharacteristicsymptom of basalgangliadisorders • Presence is necessaryforthediagnosis • Bradimimi, micrography, decreaseof associatemovementsandsialorrhoeadevelopbecause of thissymptom • Themostbasicsymptomthatcreatesdisability

  26. IDIOPATIC PARKINSON’S DISEASE • Rigidity • Develops as a result of simultaneousincreasedtoneof agonistand antagonist muscles • May be located in theproximalordistal • Showscogwheelrigidity • Determinesbypassivemotion • Increaseswithotherextremitymovement (Froment'ssign) • Patientsdescribe as a stiffnessorhardness

  27. IDIOPATIC PARKINSON’S DISEASE • Posturalinstability • Theleastspecificsymptombut themostcommoncause of disability • Occurs in laterstages of thedisease • Occursduetoloss of posturalreflex • Themostcommoncause of falls • Pulltest is usedfordiagnosis • Leadstositting as a block

  28. PARKINSON’S DISEASETREATMENT • Dopaminergic Treatment • Levodopa (dopamine precursor) • Dopamine can not pass the blood brain barrier, levodopa may • Used with peripheral type inhibitors of dopa decarboxylase (benserazide or carbidopa) in order to prevent peripheral side effects of dopamine and increase the amount of levodopa pass through to the brain • These substances can not pass the blood brain barrier so do not prevent translation of Levodopa to dopamine in the brain • Due to the long-term side effects may not prescribed initially • Dyskinesias, such as on-off period

  29. PARKINSON’S DISEASETREATMENT • Dopamine agonists • These drugs mimic the effect of dopamine binding to dopamine receptors • Bromocriptine (Parlodel) • Lisuride (Dopergin) • Pergolide (Permax) • Piribedil (Trivastal) • Cabergoline (Cabaser) • Ropinirole (Requip) • Piramipexola (Pexola, Parkyn)

  30. PARKINSON’S DISEASETREATMENT • Anticholinergic agents • Re-provide deteriorated balance between dopamine and acetylcholine in the basal ganglia • Biperiden (Akineton) • Bornaprin (Sormodren) • Triheksifenidil (Artane)

  31. PARKINSON’S DISEASETREATMENT • MAO-B inhibitors • Main enzyme which destroyed the dopamine • Dopamine stays in the synaptic gap for longer time • Selegiline (Moverdin, seldepar) • Rasajilin (Azilect)

  32. PARKINSON’S DISEASETREATMENT • COMT inhibitors • Entacapone (Comtan) • Tolcapone • Amantadine (PK-Mertz)

  33. PARKINSON’S DISEASETREATMENT • SURGICAL • The target part varies according to symptoms on the foreground • Thalamus • The globus pallidus internus • Subthalamic nucleus • Destruction of these regions • Stereotactic surgery • Gamma-knife • Deep brain stimulation • High-frequency electrical impulses via deep electrodes reduce the activity of the cells in these regions

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

  35. PARKINSON PLUS SYNDROMS • Multisystem Atrophy • MSA-P • MSA-C • Progressive Supranuclear Palsy • Corticobasal Degeneration • Lewy Body Dementia

  36. HYPERKINETIC MOVEMENT DISORDERS

  37. DYSTONIA • Characterized by involuntary, continuous, twisting, rotating muscle contraction • Develops as a result of simultaneous contractions of agonist and antagonist muscles • Recurrent • Results with temporary or permanent abnormal postures • Disappears during sleep • Dystonic contractions temporarily loss with touch • Sensory trick

  38. Primary (Idiopathic) Dystonia • Idiopathic torsion dystonia (most common) • Dystonias of childhood • Usually begins in one extremity often in leg, • Spreads to the other limbs and sometimes cranial muscles within months or years • Dystonias which start over the age of 40 • Often focal or segmental, • Primarily keeps cranio-cervical muscles

  39. Primary (Idiopathic) Dystonia DopaResponsedDystonia ( Segawadisease) • Autosomal dominant (30-40% penetrance) • Usuallybegins in childhood in thelowerextremitywithdystonia • There is noinvolvement of thecranialregions • Showsdiurnalfluctuation (lesscomplaint in themorning, dopaminestoresrunout in theeveningandcomplaintincreases) • Goodresponsewithlow-dose L-dopa (50-250 mg) • L-dopatherapyshould be triedforallkinds of primarydystonia in childhood

  40. Primary (Idiopathic) DystoniaFocal Dystonia • 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

  41. Primary (Idiopathic) DystoniaFocal Dystonia • Focal Dystonia (more) • Cervical Dystonia (spasmodic torticollis) • Torticollis • Laterocollis • Anterocollis • Retrocollis • "Task Specific” dystonia • Characterized by excessive muscle contractions producing abnormal postures during selective motor functions • The most common "writer's cramp"

  42. SymptomaticDystonias Wilson'sdisease (hepatolenticulardegeneration) • Autosomalrecessive • Usuallystartsbetweentheage of 10-20 • Depends on disorder of coppermetabolism • Copperprecipitates on liver, brainandkidney • Allkinds of movementdisordersseenduetoinvolvement of basalganglia • Behavioralandmentalproblems • Kayser-fleischer ring

  43. SymptomaticDystonias • Wilson's Disease (more) • Should be investigated WD all kinds of movement disorders in childhood and adolescence • Laboratory investigations • Level of serum ceruloplasmin is low • Level of free copper in serum is high • Total amount of copper in serum decreased • 24-hour urine copper excretion is increased

  44. Treatment of Dystonia • Treatment of symptomatic dystonias aimed at reason • idiopathic dystonia • Anticholinergics • Antidopaminergics (neuroleptics) • Baclofen • Benzodiazepines • Botulinum toxin • In general, the first choice is drug for generalized dystonias, and botulinum toxin for focal dystonias

  45. CHOREA and BALLISM • Arrhythmic, rapid, saltatory or smooth, simple or complex properties • Chorea • Amplitude is smaller • Usually seen in distal of extremity • Ballism • Amplitude is greater • Usually seen in proximal of extremity

  46. CHOREA and BALLISM Huntington's disease • Clinical features; • Korea is the dominant movement disorder • chorea, dystonia, parkinsonism • Psychiatric findings • Dementia • Autosomal dominant • Increased number of CAG repeat • Predictive, and prenatal diagnosis is possible

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