session 5: movement disorders. vignette 55 yo accountant: –frozen right shoulder/stiffness...
TRANSCRIPT
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Session 5:Movement Disorders
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Vignette
• 55 yo accountant:– Frozen right shoulder/stiffness– Slowly progressive– Impaired fine motor function– Right hand tremor– Reduced right arm swing– Slow overall– ? Sexual function– ? Bladder function– ? Cognitive function– ? Falls– ? Hallucinations– ? Medications or drug exposure– ? Family history
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Examination
• No orthostatic hypotension• Hypomimia• Hypophonia• Normal eye movements• RUE and neck rigidity• Right hand and jaw tremor• Reduced right arm swing• Slight impairment in postural stability
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Some Causes of ParkinsonismSome Causes of Parkinsonism
MSA
PDPSP
DLBD
VPPPFG
CBD
FTD
MedsNPH
AD
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Parkinsonism DDX• Parkinson’s disease (PD)• Multiple System Atrophy (MSA)• Progressive Supranuclear Palsy (PSP)• Medication-induced parkinsonism
– Antipsychotics– Metoclopramide– Others
• Parkinsonism with Alzheimer’s Disease (AD)• Diffuse Lewy Body Disease (DLBD)• Vascular parkinsonism (VP)• Corticobasal Degeneration (CBD)• Normal Pressure Hydrocephalus (NPH)• Gait disorder of the elderly• Toxins: CO, CN, Manganese, MPTP, rotenone?, CS2,
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Red Flags if Seen Early
• Falls
• Cognitive changes
• Prominent sexual or bladder dysfunction
• Lack of response to adequate Rx trial
• Symmetric onset
• Early bulbar dysfunction
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Red Flags at Any TimeRed Flags at Any Time
Cortical Sensory SignsCortical Sensory Signs
Alien Limb/unilateral apraxiaAlien Limb/unilateral apraxia
No response to L-dopaNo response to L-dopa
Impaired vertical eye movements Impaired vertical eye movements
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Dementia
Parkinsonism
Dementia
Dementia
DementiaParkinsonism
Parkinsonism
Initial Symptoms
AD
DLBD
PDD
Years Later
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Diffuse Lewy Body Disease Video
Click here to view movie
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Progressive Supranuclear Palsy
Dudley Moore
• Supranuclear palsy• Falls early in course• Dysarthria• Cognitive impairment• Emotional
incontinence• Abducted arms when
ambulating: “gun-slinger’s gait”
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PSP: Abnormal PSP: Abnormal Voluntary Eye MovementsVoluntary Eye Movements
Baylor U
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ParkinsonismParkinsonism
TTremorremor = rhythmical; typically a rest tremor = rhythmical; typically a rest tremor
RRigidityigidity = resistance to movement = resistance to movement
AAkinesiakinesia = slowness/absence of movement = slowness/absence of movement
PPostural changesostural changes = stooped posture = stooped posture
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Resting TremorClick here to view movie
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RigidityClick here to view movie
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Akinesia/BradykinesiaClick here to view movie
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Postural Changes
Stooped Posture
www.artandmedicine.com
Impaired Postural Reflexes
Click here to view movie
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PD:Preferential loss of
putamenal dopamine projections
Dauer and PrzedborskiNeuron 2003
Lewy bodies containsynuclein, ubiquitin and other proteins
Normal PD
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Increased Tone• Rigidity
– Increased tone– Usually bidirectional
• Paratonia (Gegenhalten)– Inability to relax one’s limb manifesting as
resistance to movement
• Spasticity– Velocity dependent increase in tone– Usually unidirectional– Other upper motor neuron signs are often present:
hyper-reflexia, Babinski sign and pyramidal distribution of weakness
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Definitions• Chorea: derived from the Greek word “to dance” to
describe the arrhythmic, involuntary flitting movements• Athethosis: involuntary writhing movements• Hemiballism: large amplitude involuntary movement
restricted to one side of the body; usually involves proximal upper limb.
• Myoclonus: sudden brief jerk or shock-like movements• Dystonia: abnormal sustained posture resulting from
simultaneous co-contraction of agonist and antagonist muscles.
• Tremor: rhythmic oscillation of a body part due to alternating or synchronous contractions of opposing muscles
• Tics: sudden, brief, purposeless, stereotyped simple or complex movements or vocalizations
• Akathisia: inner restlessness; often associated with external signs of restless behavior
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ET TreatmentsET Treatments
MedicationsMedicationsPropranolol (InderalPropranolol (Inderal®®))
Mysoline (PrimidoneMysoline (Primidone®®))
Clonazepam (KlonopinClonazepam (Klonopin®®))
SurgeriesSurgeriesThalamotomyThalamotomy
Thalamic Deep Brain Stimulation Thalamic Deep Brain Stimulation (DBS)(DBS)
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Tremor
• Cerebellar disorders– Tremor with action with accompanying dysmetria.
Note cerebellar features on exam: asynergia, ataxia, dysarthria, dysdiadochokinesia, dysmetria, gait disturbance, hypotonia, nystagmus, rebound, etc.
• Essential tremor– Postural and action tremor without parkinsonism;
typically life-long and tremor responds to alcohol; may have family history
• Parkinsonism– Typically has a rest tremor, but may also be present
with posture and action or solely the latter
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Acute Neuroleptic-induced A/Es
• Acute dystonic reaction– Cervical dystonia (torticollis)– Opisthotonus– Oculogyric crisis– Other
• Neuroleptic malignant syndrome
• Akathisia (restlessness)
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Acute Dystonic Reaction
• Usually occurs within 48 hours; but sometimes may be up to 5 days after Rx
• More common with parenteral than oral medications
• More common in males than females; particularly young males
• Features: neck, jaw, tongue, oculogyric, opisthotonus, etc.
• Rx: anticholinergics, antihistaminics
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Acute Dystonic Reaction
http://www.haveinc.com/eps/
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Opisthotonus
www.pharmacology2000.com/ Central/sedhyp/opis1.jpg
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Akathisia: Restlessness
http://www.haveinc.com/eps/
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Chronic Neuroleptic Exposure
• Tardive dystonia– Axial more common– Appendicular (limb)
• Tardive dyskinesia
• Medication induced parkinsonism
• Tardive tics/akathisia/tremor, etc.
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73 yo woman with action tremor
• DDx– Essential tremor (ET)
• Life-long, progressive tremor• Alcohol responsiveness• Absence of bradykinesia, rigidity, etc.
– Parkinson syndrome with action tremor– Medication induced tremor– Could be both ET and PD
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56 yo man with progressive chorea, dementia and positive family history
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Huntington’s DiseaseHuntington’s Disease
Features:Features:ChoreaChorea
Cognitive declineCognitive decline
Cause:Cause:
Autosomal dominant Autosomal dominant
Amplification of CAG (cytosine adenosine Amplification of CAG (cytosine adenosine guanine) repeats in the IT-15 gene on guanine) repeats in the IT-15 gene on chromosome 4chromosome 4
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SOME TRINUCLEOTIDE REPEAT DISORDERS
HD occurs with > 36-38 CAG repeats in the IT15 gene
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ANTICIPATION: EARLIER ONSET WITH MORE CAG REPEATS
AUTOSOMALDOMINANT
TRANSMISSION
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32 yo man with recurrent grunting, OCD and unusual involuntary limb
movements
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Tourette’s Syndrome (TS)Tourette’s Syndrome (TS)Tics:
Involuntary stereotypic movements or vocalizationsTS Features:
Presence of both vocal and motor tics Onset before age 18/21 yearsTics may be temporarily suppressibleWith suppression of tics there is often inner tension which is relieved upon performing ticsCoprolalia (foul language): only ~17% of casesCo-morbidities: OCD, ADHD, learning disability
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54 year-old man who reacts to metaclopramide
• Age
• Gender
• Other factors