OU Neurology Parkinson Disease & other Movement Disorders Cherian Abraham Karunapuzha, M.D....
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Transcript of OU Neurology Parkinson Disease & other Movement Disorders Cherian Abraham Karunapuzha, M.D....
OU NeurologyOU Neurology
Parkinson Disease & other Movement Disorders
Cherian Abraham Karunapuzha, M.D.Assistant Professor
Movement Disorders DivisionDepartment of Neurology
The University of Oklahoma Health Sciences Center
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DISCLOSURES
FINANCIAL DISCLOSUREThe speaker is on the speaker bureau
for Teva neuroscience and UCB.
UNLABELED/UNAPPROVED USES DISCLOSUREThe speaker has nothing to disclose
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LEARNING OBJECTIVES
Learn to recognize the phenomenology of various abnormal movements
Learn about common movement disorders associated with such abnormal movements
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What is a “movement disorder” ?
Most diseases of BG are not “fixable” but can be alleviated, eg. Parkinson’s, essential tremors, Huntington’s, torticollis, Tourette’s
Light Blue – ventriclesRed – Caudate nucleusGreen – PutamenDark Blue – Globus PallidusBlack – Substantia NigraBrown - Thalamus
Not “weakness” - more like a switchbox with different On and Off switches or programs.
Depending on locus of BG damage, the signs can be bradykinesia, rigidity, tremor, chorea, dystonia, tic, myoclonus…
Conditions arising from a dysfunction of the extrapyramidal system (basal ganglia) – extrapyramidal diseases
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Basal Ganglia Circuit
Cortex
StriatumDirectPathway
StriatumIndirectPathway
GPe STN
GPi
Thalamus
Substantia Nigra
D1
D2
GPe – Globus Pallidus externa /lateralGPi – Globus Pallidus interna /medialSTN – Subthalamic NucleusD1, D2 – types of dopamine receptors
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Common phenomenonology and associated symptoms
BradykinesiaBradykinesia FreezingFreezing RigidityRigidity Tremor – Tremor – restingresting
Tremor – Tremor – action/posturalaction/postural
Chorea, ballismChorea, ballism StereotypyStereotypy DystoniaDystonia TicTic MyoclonusMyoclonus
Parkinsonism Parkinsonism Parkinson diseaseParkinson disease Multiple System atrophyMultiple System atrophy PSPPSP Drug induced Drug induced
parkinsonismparkinsonism NPHNPH
Essential TremorEssential Tremor Drug induced tremorDrug induced tremor Huntington diseaseHuntington disease Sydenham choreaSydenham chorea Levodopa dyskinesiasLevodopa dyskinesias Tardive dyskinesiasTardive dyskinesias Torticollis, Torticollis,
blepharospasmblepharospasm Tourette syndromeTourette syndrome Metabolic, CJDMetabolic, CJD
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Tremor
Oscillatory, rhythmic and regular movement that affects one or more body parts, such as the limbs, neck, tongue, chin, or vocal cords.
Distribution : Arms, head, legs, larynx… Unilateral or asymmetric or symmetric Context : Resting – 3-6 Hz Postural – 5-10 Hz Action (kinetic) – 5-10 Hz Terminal (intention) – 2-4 Hz
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Essential Tremor
Action or postural tremor of arms, sometimes head and voice, occasionally legs
Family history common Improves with alcohol. Worsens with
Caffeine. Mild tremor may first appear in teens or
young adulthoodGradually worsen over decades
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Essential Tremor (Contd.)
Check for hyperthyroidismCheck for drug induced – albuterol, SSRI,
Depakote, antipsychoticsMRI brain for mimics - white matter
lesions brainstem & cerebellumRx: Propranolol 10 mg tid -> 60mg tid or
Primidone 25 mg qhs -> 250 mg tidSurgery: deep brain stimulation (DBS) in
thalamus for refractory tremors
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Cerebellar Tremor – associated findings of dysmetria, dysdiadokinesia , ataxia etc.
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Psychogenic tremor ?? – improves with distraction - Counseling/clinical psychology
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Resting Tremor
present in the distal parts of the extremities and the lips while the involved body part is “at rest” & ceases on active movement of the limb
“Pill-rolling” tremor of the fingersflexion-extension or pronation-
supination tremor of the hands
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Brady/hypokinesia – slowness or decreased amplitude of movementa loss of automatic movements slowness in initiating movement on
command reduction in amplitude of the
voluntary movementmasked facies, decreased frequency
of blinking, soft speech, drooling, small handwriting, shuffling gait, decreased armswing
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Rigidity - Increased resistance to passive motion ....present equally in all directions of the
passive movement, equally in flexors and extensors & throughout the range of motion – extrapyramidal lesions
Stiffness, heaviness, or aching muscle Cogwheel and Leadpipe rigidity Spasticity – Another hypertonic state –
Damage to the primary motor cortex or the corticospinal tract - velocity dependent, clasp knife phenomenon
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Parkinson Disease
A clinical syndrome resulting from degeneration of nigro-striatal dopaminergic neurons
Asymmetric onsetTRAP : Tremor (resting), Rigidity,
Akinesia (bradykinesia), Postural instability
Diagnosis – clinical exam & response to levodopa (L-dopa) supplementation
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Parkinson disease (contd.)
MRI brain to look for mimics - enlarged ventricles, subdural hemorrhage, tumor, multiple subcortical strokes
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Parkinson disease (contd.)
Incidence: 4.5-21 cases/100,000 per year
Cause: combination of genetic and environmental factors
Pathology: Lewy body – abnormal aggregates of protein (alpha synuclein)
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Parkinson disease (contd.)
slow progression - 10-25 yearsindependence usually not reduced in
first 5-10 yearslater: postural instability, freezing,
levodopa induced dyskinesia, fluctuating response to meds, dysphagia, incontinence, postural hypotension, dementia
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Parkinson disease (contd.)
MAO B inhibitors – Rasagiline, Selegiline – mild symptomatic effect, increase ON time,?disease modifying
Dopamine agonists – Ropinirole, Pramipexole, Rotigotine – moderate symptomatic effect
Dopamine – Carbidopa/Levodopa (Sinemet) 25/100 one tablet TID to 1200mg/day – Robust symptomatic effect
COMT inhibitors – Entacapone – increase ON time NMDA antagonist – Amantadine - dyskinesias DBS (deep brain stimulation) or Surgical ablation PT/OT , speech therapy – Lee silverman voice therapy
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Choreioform Movements
Chorea - involuntary, irregular, purposeless, nonrhythmic, abrupt, rapid, unsustained movements that seem to move unpredictably from one body part to another
Ballism – larger amplitude and rapid-proximal parts of limb
Athetosis - more sinuous slow, writhing
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Sydenham Chorea
Post-strep reactionDemographics: teens, female > maleChorea; sometimes behavioral (OCD-
like) Testing of limited use: ASOT false pos
and neg, infection usually resolvedTends to subside even without Rx in
weeks or monthsValproate can reduce the chorea
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Huntington’s Disease
Neurodegeneration, initially of striatal ‘medium spiny’ neurons to GPe
Caused by expanded CAG repeat in Huntingtin Gene (autosomal dominant), normal < 35 repeats
Onset young adult, though can be any agePresents with gradually worsening chorea,
cognitive, mood & behavioral changesManagement: antipsychotics,
antidepressant, tetrabenazine, behavioral
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L-Dopa induced Dyskinesias – usually peak dose about 40 minutes after ingestion of L-Dopa
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Stereotypy
‘Coordinated’ movements that repeat continually and identically
When they occur at irregular intervals, stereotypies may not always be easily distinguished from motor tics, compulsions, gestures & mannerisms
Tics - occur paroxysmally out of a background of normal motor behavior and usually associated with an urge
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Tardive Dyskinesia
Dopamine Antagonists (D2):
Typical > atypical antipsychotics
metoclopramide (Reglan), promethazine (Phenergan)
? D2 receptor hypersensitivity
Appears after years of use (sometimes even months)
Risk factors: dose, older age, female sex
Classic - Oro-Bucco-Lingual stereotypy (lipsmacking).
Anticholinergics - Artane, Cogentin or even Benadryl worsen it.
Rx - Tetrabenazine 12.5 mg tid Remove offending drug, or
switch to a more atypical antipsychotic
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Tics
Brief, Stereotyped abnormal movements or sounds
In response to urge (psychic/physical)Can be complex (sequence, muscles)The diversity of motor tics is one
feature that sets their phenomenology apart from stereotypies
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Tourette Syndome
Onset < 18 . For > 1 yearMultiple motor tics and vocal tics –
coprolalia. Tics evolves over time.Comorbidities: ADHD, OCD, impulse
control disorderM > F (female relatives may have
OCD)
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Tourette Syndome (contd.)
Adrenergic - Guanfacine, Clonidine (0.1 - 0.3mg tid)
Antipsychotics - Pimozide, Haloperidol, Risperidone (1-16 mg/day div qd-bid)
Dopamine Depletor –Tetrabenazine
Stimulants – Adderall, Ritalin SR (20 – 60 mg/day div qd-bid)
Antidepressants Behavioral therapy ? DBS
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Dystonia
Twisting movements that tend to be sustained at the peak of the movement
Frequently repetitive in the same group of muscles (patterned – unlike Chorea)
Often progresses to prolonged abnormal postures
Can be focal, segmental or generalizedOften induced by action, sometimes
task-specific such as writing etc.
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Adult onset focal dystonia (more common)
Cervical dystonia (torticollis)
Blepharospasm (forced eyelid closure)
Usually idiopathic but sometimes a triggering insult (eg. injury)
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Primary Torsion Dystonia
Generalized - gradual onset, usually starts in the foot
Juvenile Onset CAG deletion in
DYT1 gene resulting in Torsin A mutation
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Dystonia (contd.)
Anticholinergic: Cogentin, Artane(2 -16mg per day div. TID titrated gradually over 2-3 months).
Muscle relaxants – Benzos, Baclofen Botulinum toxin injection – focal
dystonias – last 3-4 monthsDBS - “Humanitarian Device
Exemption”
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Myoclonus
Sudden lightning-like movementCan be repetitive but Not rhythmic (if
it was, it would be a tremor!)Can be focal or regional or multifocoal
or generalized; epileptic and non epileptic.
Like tremor, myoclonus can arise from disease of many parts of CNS, not just basal ganglia
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Myoclonus (contd.)
Physiologic (Hypneic, Hiccups)
Serotonin Syndrome
Dementias – Creutzfeld Jakob Disease (CJD)
Multiple Sclerosis JME (epilepsy) Rx: clonazepam (0.25-0.5 mg PO bid-tid) or
valproic acid or levetiracitam