Session 5: Movement Disorders. Vignette 55 yo accountant: –Frozen right shoulder/stiffness...
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Transcript of Session 5: Movement Disorders. Vignette 55 yo accountant: –Frozen right shoulder/stiffness...
Session 5:Movement Disorders
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
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
Some Causes of ParkinsonismSome Causes of Parkinsonism
MSA
PDPSP
DLBD
VPPPFG
CBD
FTD
MedsNPH
AD
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,
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
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
Dementia
Parkinsonism
Dementia
Dementia
DementiaParkinsonism
Parkinsonism
Initial Symptoms
AD
DLBD
PDD
Years Later
Diffuse Lewy Body Disease Video
Click here to view movie
Progressive Supranuclear Palsy
Dudley Moore
• Supranuclear palsy• Falls early in course• Dysarthria• Cognitive impairment• Emotional
incontinence• Abducted arms when
ambulating: “gun-slinger’s gait”
PSP: Abnormal PSP: Abnormal Voluntary Eye MovementsVoluntary Eye Movements
Baylor U
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
Resting TremorClick here to view movie
RigidityClick here to view movie
Akinesia/BradykinesiaClick here to view movie
Postural Changes
Stooped Posture
www.artandmedicine.com
Impaired Postural Reflexes
Click here to view movie
PD:Preferential loss of
putamenal dopamine projections
Dauer and PrzedborskiNeuron 2003
Lewy bodies containsynuclein, ubiquitin and other proteins
Normal PD
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
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
ET TreatmentsET Treatments
MedicationsMedicationsPropranolol (InderalPropranolol (Inderal®®))
Mysoline (PrimidoneMysoline (Primidone®®))
Clonazepam (KlonopinClonazepam (Klonopin®®))
SurgeriesSurgeriesThalamotomyThalamotomy
Thalamic Deep Brain Stimulation Thalamic Deep Brain Stimulation (DBS)(DBS)
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
Acute Neuroleptic-induced A/Es
• Acute dystonic reaction– Cervical dystonia (torticollis)– Opisthotonus– Oculogyric crisis– Other
• Neuroleptic malignant syndrome
• Akathisia (restlessness)
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
Acute Dystonic Reaction
http://www.haveinc.com/eps/
Opisthotonus
www.pharmacology2000.com/ Central/sedhyp/opis1.jpg
Akathisia: Restlessness
http://www.haveinc.com/eps/
Chronic Neuroleptic Exposure
• Tardive dystonia– Axial more common– Appendicular (limb)
• Tardive dyskinesia
• Medication induced parkinsonism
• Tardive tics/akathisia/tremor, etc.
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
56 yo man with progressive chorea, dementia and positive family history
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
SOME TRINUCLEOTIDE REPEAT DISORDERS
HD occurs with > 36-38 CAG repeats in the IT15 gene
ANTICIPATION: EARLIER ONSET WITH MORE CAG REPEATS
AUTOSOMALDOMINANT
TRANSMISSION
32 yo man with recurrent grunting, OCD and unusual involuntary limb
movements
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
54 year-old man who reacts to metaclopramide
• Age
• Gender
• Other factors