movement disorders diseases of the extrapyramidal...
TRANSCRIPT
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MOVEMENT DISORDERS
DISEASES OF THE EXTRAPYRAMIDAL
SYSTEM
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The extrapyramidal system ...
includes all that centers and
pathyways, wich responsible for the
organisation of movements with the
exception of the pyramidal tract.
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The function of the extrapyramidal system
• coordination of themovement behavior
• ensurance of the orderand „smooth” ofmovements
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Parts of the extrapyramidal system
nucleus lentiformis
striatum
• cortical centers: there are centers in all the corticallobes
• subcortical centers:
• caudate nucleus
• putamen
• pallidum
• subthalamical nucleus (Luys)
• nucleus ruber
• substantia nigra
• reticular formation
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There are two main clinically different groupscaused by the disease of the extrapyramidal
movement system:
• hypokinetic-hypertonic
(rigid)
• hyperkinetic-hypotonic
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hypokinetic-hypertonic (rigid)syndrome
Parkinsonism
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The etiology of the Parkinsonism
• primer, idiopathic form: Parkinson’s disease- the second most frequent progressive neurodegenerative
disease- the prevalence is 1% in the 65 year old population and it
increases to 4-5% in the 85 year old population- it is most frequently sporadic- approximately 10% of the cases are familiar (there are 10
indentified genes, wich cause autosomal dominant, orrecessive forms of Parkinson’s disease)
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The etiology of the Parkinsonism
• symptomatic: Parkinson’s syndrome– trauma– tumor– inflamation (encephalitis)– stroke (ischemic, hemorrhagic)– toxic agents (CO, neuroleptic drugs)
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The neuropathologic basis of Parkinson’s disease
• degeneration of the substantia nigra and thestriatum (Lewy bodies- citoplasmatic protein aggregations)
• disintegration of the dopamin-acetilcholinbalance in the central nervous system
• decrease of the dopamin level and cholinergicdominance
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the main symptoms of Parkinsonism
• rigidity• tremor• hypo-, and bradykinesis• postural instability
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rigidity• the tone of the agonist és antagonist (flexor and
extensor) muscles increases paralelly• the trunk and the extremities are in semiflected
position• during passive movement of the extremities we
can feel permanent resistance (cogwheelphenomenon)
• very often assimetrical distribution
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tremor• the agonistic and antagonistic (flexor és
extensor) muscles contract with rhytmic, 6-8/sec. frequency
• the tremor is most prominent in resting position, the stress could provoke it
• the intended movements decrease the tremorand it is stopped during sleep
• very often assimetrical distribution
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hypo-, and bradykinesis
• movements and walking become slow, sluggishand difficult
• shuffling gait with decreased excursion of legs• difficulties in turning• freezing: sudden stop in walking and movements
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other signs• on-off phenomenon• retropulsion• hypomimia, or blank facies• hypersalivation• monotonous speech• micrographia• hyposmia, hypogeusia• constipation• depression
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the Parkinsonic crisis could be lifethreatening !!
• complete immobility• aphagia and anarthria• extreme rigidity• fever• cardiovascular inssufficiency• bedsores, pneumonia, deep vein thrombosis,
lung embolism
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treatmentDRUGS
• MAO B inhibitors (Selegilin, Jumex, Rasagiline) • Amantadin (Viregyt K, PK Merz) • Dopamin agonists (ergotamine type:Bromocriptin,
non ergotamin type: Requip,Mirapexin, Apomorphin)• L-DOPA substitution (L-DOPA+DOPA decarboxilase
inhibitor pl.Madopar, Sinemet, Duellin)• COMT inhibitors (Comtan)• L-DOPA+DOPA decarboxilase inhibitor+ COMT inhibitor
(Stalevo)• anticholinergic drugs (Tremaril, Kemadrin, Akineton)
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The scheme of the recommended treatment of Parkinson’s disease in 2004The scheme of the recommended treatment of Parkinson’s disease in 2004Parkinson’s disease
treatment with drugs „non drug” treatment
education
help and support
exercises, physical education
nutrition (low protein)
?neuroprotection
functional deficit
observation
no yes
Dopamin agonists 1-dopa (+/- COMT-1)
Dopamin agonists 1-dopa (+/- COMT-1)
COMT-1 (if there was not tried)
Motor complications
If the treatment with drugs is not enough= neurosurgical treatment
OLANOW
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treatmentSURGICAL (just in very severe cases, when the
antiparkinson drugs are uneffective)
• deep brain stimulation – DBS (uni-, or bilateral)
• thalamotomy, pallidotomy (uni-, or bilateral)
• neurotransplantation (inplantation of fetalmesencephalic cells into the caudat nucleus and
putamen region)
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treatment
• physicotherapy (training, special exercises)
• psychotherapy
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diseases characterized with hyperkinesisand hypotonia
• chorea• ballism• athetosis
• dystonias• myoclonus• tic
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Chorea• features: irregular, sudden onset, fast movements
wich involve first of all the distal part of extremities, but could appear in the truncal, facial and tonguemuscles as well
• the emotional effects, stress could provoke it
• the anatomical basis is the degeneration of theputamen and the caudat nucleus
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Chorea
• forms:
– inherited (Huntington chorea, autosomal dominant)
– symptomatic (chorea minor, chorea gravidarum,
caused by traumas, tumors, inflamation, stroke in theabove mentioned brain regions)
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ballism
• features: violent, big movements in the proximalmuscles of the extremities with sudden onset. Because of these movements the patient can falldown.
• anatomical basis: the degeneration of thesubthalamical nucleus (Luys)
• forms:– idiopathic– symptomatic ( caused by traumas, tumors,
inflamation, stroke in the above mentioned brainregions)
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athetosis
• features: slow, „vermin-like” movements in the distalmuscles of the limbs, but the facial muscles could be involved as well
• anatomical basis: the degeneration of the striatumand the pallidum
• forms:– idiopathic– symptomatic (caused by traumas, tumors,
inflamation, stroke in the above mentioned brainregions)
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treatment
• dopamin antagonist: tiaprid (Tiapridal)
• chlorpromazin (Hibernal)
• Haloperidol
• clonazepam (Rivotril)
• amantadin
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dystonias (torticollis, tortipelvis, antero-, retrocollis)
• features: dystorsion of the neck, limbs and trunk becauseof elevatedmuscle tone
• etiology: the degeneration of the putamen, psychogenic problems: anxiety, depression
• forms:
– idiopathic
– symptomatic (caused by traumas, tumors, inflamation, stroke in theabove mentioned brain regions, side effect of : neuroleptic drugs, thietylperazine (Torecan), metoclopramide (Cerucal), domperidone(Motilium)
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treatment• local infiltration of the involved muscles with botulinum
toxin• L-Dopa (in L-Dopa resposive forms)• Anticholinergic/antihistaminic agents: trihexyphenidyl
(Artane), Procyclidine (Kemadrin), ethopropazine(Parsidol)
• Baclofen• Clonazepam (Rivotril)• Antiepileptic drugs: Carbamazepine (Tegretol, Neurotop),
Gabapentin (Neurontin, Gordius)• Dopamine-depleting agents: Tetrabenazine, Reserpine• Dopamin antagonists: Tiapridal
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myoclonus
• features: fast, irregular movement of a muscle group, or a muscle region of the body. It involves first of all the distal part of the body and axial muscles, or the palatal muscles
• The anatomical basis could be cerebral (cortical, subcortical), and spinalis lesions, or the degeneration of the so called„myoclonic triangle”: nucl. ruber, nucl. dentatus, oliva inferior
• forms:– idiopathic– associated with epilepsy– symptomatic
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treatment
• clonazepam (Rivotril)
• carbamazepine (Tegretol, Neurotop)
• levetiracetam (Keppra)
• valproic acid (Convulex, Depakine)
• piracetam (Nootropil)
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tic• features: irregular, stereotyped, fast movement
of a muscle group with sudden onset, or couldbe
• stereotyped repetition of words, wich most frequently obscene words (Gilles de la Tourettesyndrome: both multiple motor and vocal tics, for at least 1 year, tics changing over time, theonset of tics before the age of 21 years)
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treatment
• dopamine receptor antagonists: Haloperidol, Risperidon (Risperdal), Ziprasidone (Ziprexa)
• α2 agonists: Clonidine• monoamin depletors: Tetrabenazine• clonazepam (Rivotril)• SSRI-s: Paroxetine, Citalopram,
Venlafaxine…stb