basic movement disorder approach
DESCRIPTION
I teach the medical student with a slide set on movement disorder-the basic approach.TRANSCRIPT
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Movement disorder tutorial
Surat Tanprawate, MD, MSc (London), FRCP(T)Division of Neurology, Chaingmai University
NNCMUThe Northern Neuroscience Centre Chiangmai University
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Neurological symptoms
Conscious and cognitive functions
alteration of conscious higher cortical function
disorder
dementia Cranial nerve functions
anosmia, visual loss, diplopia, CN V dysfunction, facial weakness, hearing loss, tinnitus, dysphagia, tongue weakness
Dysfunction of motor system weakness ataxia movement disorder
Dysfunction of sensory system numbness pain
Autonomic dysfunction Episodic disorder
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Neurological diseases
Congenital Trauma Tumor Infection/inflammation Degeneration Demyelination
Vascular Metabolic/Toxic/Drugs Genetic Cryptogenic Idiopathic
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What is movement disorder?
The term : movement disorders: originally refer to basal ganglia or extrapyramidal diseases
slowness or poverty of movement (bradykinesia or hypokinesia (e.g. parkinsonian disorders)
abnormal involuntary movements (hyperkinesias) such as tremor, dystonia, athetosis, chorea, ballism, tics, myoclonus, restless legs syndrome, stereotypies, akathisias, and other dyskinesias
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What is movement disorder?
Some may similar movement disorder abnormalities in muscle tone (e.g.rigidity,
spasticity, and stiff man syndrome),
incoordination (cerebellar ataxia) apraxia seizure
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Anatomy of movement control
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Direct and Indirect pathway of movement control
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Step approach3 question should be asked
1.Is it hypokinetic or hyperkinetic movement disorder?
2.What is the pattern of movement disorder?
3.What is the classification of such movement disorder?
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Movement disorder
Hypokinetic Hyperkinetic
Hypokinetic rigid syndrome
Hyperkinetic rigid syndrome
Pattern of movement disorder
Classify by anatomy, distribution, cause,
age
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Hyperkinetic movement disorder
Rhythmic, sustained, intermittent, speed, suppressibility, complex movement
Tremor, Chorea, athetosis, dystonia, myoclonus, ballism, tic
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Chorea = danceirregular, nonrhythmic, unsustained involuntary movement that flows from one part of the body to another
motor impersistence
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Dancing lady
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Dystoniasyndrome of sustained muscle contractions, frequently causing twisting, repetitive movements, or abnormal postures
sustained contractions, consistent directional or patterned character (predictable), and
exacerbation during voluntary movementssensory trick
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Generalised dystonia
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Myoclonussudden, brief, jerky, and shock-like involuntary movements involving face, trunk, and extremities
positive myoclonusnegative myoclonus
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Generalised myoclonus
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Tremora rhythmic oscillation of a body part produced by alternating or synchronous contraction of opposing muscles
other movement clinical symptoms can be act like tremor: dystonic tremor, myoclonic tremor
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Tremor
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Ticsrepetitive, stereotyped, involuntary, sudden, inopportune, non-propositional, and irresistible movement
unpleasant feelingnot absolutely clear as patients can exert some control
on the movementcan be simple or complex
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He have had tic since he was 10 y.o.
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Ballism=dacinginvoluntary, flinging motions of the extremities, the movement are often violent and have wide amplitude of motion, continuous and random, can involve proximal or distal
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Athetosis = without fixed position
involuntary, convoluted, writhing, slow movements of the arms, fingers and legs
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Pseudoathetosis in a patient with severe axonal polyneuropathy
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Common movement disorder
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Tremor
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Step approach- MDS consensus
1.Inspection the tremor2.Specific examination for assessment of
signs related to tremor3.Syndrome classification of tremor
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Terminology for tremor and the hierarchical relation of the terms as
indicated by the numbers
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Inspection
Frequency Low (7 Hz)
Location Head: chin, face, tongue,
palate
Upper extremity: shoulder, elbow, wrist, fingers
Trunk Lower extremity: hip,
knee, ankle joint, toes
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Specific examination for assessment of:
Akinesia/bradykinesia Muscle tone (including Froments sign for the upper and lower extremity and coactivation sign for
psychogenic tremor) Postural abnormalities Dystonia Cerebellar signs Pyramidal signs Neuropathic signs Systemic signs (thyrotoxicosis and so forth) Gait and stance (orthostatic tremor)
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Syndrome Activity Specific S/S Cause
Physiologic tremor Postural No Physiologic response
Enhance physiologic tremor Postural, Kinetic
Hyperthyroid, tachycardia Hyperthyroid, drugs
Essential tremor Postural, Kinetic No No
Parkinsonian tremor RestBradykinesia,
postural instability, rigidity
Neuro-degeneration
Cerebellar tremor Postural, kinetic, intention AtaxiaVarious cause
affected cerebellar pathway
Syndrome classification of tremor
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Syndrome classification of
tremor
Tremor description (activated by, location,
frequency)+
Specific s/s
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Characteristics of Essential Tremor and Parkinsonian Tremor
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Essential tremorCore criteria for identifying ET
Bilateral action tremor of the hands and forearms
Absence of other neurological signs, with the exception of the cogwheel phenomenon
May have isolated head tremor with no abnormal posture
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Essential tremor
Secondary criteria for identifying ET
Long duration (>3 years) Family history: reported in > 50% of the
patients
Beneficial response to ethanol
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Essential Tremor
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Achimedes spiral
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Achimedes spiral
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Treatment ET
First line Propranolol start at 10 mg x 3 => 240-320 mg/d Primidone
Second line Gabapentin, topiramate, clozapine, long acting
benzodiazepine (clonazepam)
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Holmes tremor midbrain tremor rubral tremor
thalamic tremor
predominately proximal limb (
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Wing Beating Tremor in Wilsons disease
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Dystonic tremor
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Parkinsons disease
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James Parkinson, London
(1755 1824)
An Essay on the Shaking Palsy(1817)
Shaking Palsy(Paralysis agitans)
He identified 6 cases, 3 of whom he personally examined; 3 he observed on the streets of London
J Neuropsychiatry Clin Neurosci 2002;14:22336
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Parkinsonism
clinical syndrome of bradykinesia, resting tremor, cogwheel rigidity, and postural instability
Parkinsons disease
clinical syndrome of asymmetrical parkinsonism, usually with rest tremor, in association with the specific pathological findings of depigmentation of the SN as a result of loss of melanin-laden dopaminergic neurons containing eosinophilic cytoplasmic inclusions(Lewy bodies)
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Epidemiology
Community based series prevalence 360 per 100,000 and an
incidence of 18 per 100,000 per year
PD is an age-related disease gradually increase after age 50 years, and
disease before age 30 years is rare
Female: Male=1:1de Lau and Breteler. Lancet Neurol 2006; 5: 525-535
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Pathophysiology
Structural change Loss of pigmented neurons in the SNc and
other pigmented neuron
Histopathology: Lewy bodies Neurotransmitter change Depletion of dopamine containing cells in
the substantia nigra leads to decreased dopamine n the striatal
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Pathology
Gross: loss of pigmented cell in substantial nigra(SN) and other pigmented nuclei(locus ceruleus(LC), dorsal motor nucleus of the vagus)
http://www.uhmc.sunysb.edu/pathology/neuropathhttp://www.babraham.ac.uk/images/research/SAS/emson/fig1.jpg
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Pathology
Normal substantia nigra
Extensive loss of pigmented neurons
Surviving neuron contains a Lewy body
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Group of Parkinsonism Primary or idiopathic parkinsonism
Parkinsons disease Secondary parkinsonism
hydrocephalus, vascular parkinsonism, encephalitis Parkinson plus syndrome
Progressive supranuclear palsy(PSP), corticobasal degeneration(CBD), multiple system atrophy(MSA)
Hereditary parkinsonism Wilsons disease, Dopa-responseive dystonia,
Huntingtons disease(HD)
TYPICAL OR
CLASSIC
ATYPICAL
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United Kingdom Parkinson's Disease Society(UKPDS) Brain Bank Diagnostic
Criteria for PD
Step 1: Diagnosis of Parkinsonism Step 2: Features tending to exclude
Parkinsons disease as the cause of Parkinsonism
Step 3: Features that support a diagnosis of Parkinsons disease (three or more required for diagnosis of definite Parkinsons disease)
Hughes AJ, Daniel SE, Kilford L, Lees AJ. JNNP 1992 Mar;55(3):181-4Diagnostic accuracy to 82%
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Step 1: Diagnosis of Parkinsonism
Bradykinesia and at least one of the following:
Muscular rigidity 46 Hz resting tremor Postural instability not caused by primary
visual, vestibular, cerebellar or proprioceptive dysfunction
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Pill rolling tremor
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Finger tapping
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Bradykinesia
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Micrographia
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Micrographia
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Step 2: Features tending to exclude Parkinsons disease as the cause of Parkinsonism
History of repeated strokes with stepwise progression of parkinsonian features
History of repeated head injury
History of definite encephalitis Neuroleptic treatment at
onset of symptoms
>1 affected relatives Sustained remission Strictly unilateral features after
3 years
Supranuclear gaze palsy Cerebellar signs Early severe autonomic involvement Early severe dementia with
disturbances of memory, language and praxis
Babinski's sign Presence of a cerebral tumour or
communicating hydrocephalus on computed tomography scan
Negative response to large doses of levodopa (if malabsorption excluded)
MPTP exposure
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Step 3: Features that support a diagnosis of PD (three or more required)
Unilateral onset Rest tremor present Progressive disorder Persistent asymmetry affecting the side of onset most Excellent (70100%) response to levodopa Severe levodopa-induced chorea Levodopa response for 5 years Clinical course of 10 years
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Non-motor symptoms
Loss of sense of smell, constipation REM behavior disorder (a sleep
disorder)
Mood disorders Orthostatic hypotension (low blood
pressure when standing up)
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Diagnostic studies
MRI/CT brain: using for exclude other cause of parkinsonism
In PD, the MRI brain usually reveals normal
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PD disease progression-treatment response
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Modality of treatment
Symptoms based treatment Pharmacologic vs Non-pharmacologic Motor vs Non-motor symptoms
Neuro-protection Prevention
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Dopamine Acetylcholine
Motor symptoms of Parkinsons disease
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Symptomatic based treatment
Enhance dopaminergic transmission L-dopa, dopamine agonist, drug that
decrease dopamine destruction
Drug manipulating other neurotransmitter
Anti-cholinergic drug
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Dose of the preparations of Sinemet and Madopar
Levodopa + DDI
Madopar (levodopa+benserazide)Sinemet (levodopa+carbidopa)
Madopar HBSSinemet CR
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As the disease progress, the Therapeutic window narrow
symptoms and side effects occur as the levodopa therapeutic window diminishes
Dyskinesia threshold
Efficacy threshold
Smooth, extend response
Absent or infrequent dyskinesia
Diminished duration Increased incidence
of dykinesia
Short, unpredictable response
on time is associared with dyskinesia
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Parkinson Plus Syndrome
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Parkinson-plus syndrome
Multiple system atrophy cerebellar sign + ve, autonomic dysfunction
Progressive supranuclear palsy vertical gaze palsy
Corticobasal degeneration limb apraxia
Dementia with lewy bodies Vivid visual hallucination with dementia
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PSP described by Richardson
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Dystonia
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Dystonia classification Age of onset
early-onset: age < 26 year late-onset: age > 26 year
Distribution focal (single body reion) segmental (contiguous region) multifocal (eg. hemidystonia) Generalized
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Dystonia classification-by etiologyPrimary dystonia
AD: early-onset limb dystonia (DYT1), Mixed dystonias (DYT6, DYT13), Late-onset craniocervical dystonia (DYT7)
Idiopathic (cervical dystonia, writer cramp, generalised dystonia etc)
Secondary dystonia
Dystonia-plus: Dopa-responsive dystonia(DRD), rapid onset dystonia parkinsonism (RDP), Myoclonus-dystonia(M-D)
Heredodegerative dystonias: AD (HD, SCA,3, DRPLA), AR (Wilsons disease, MLD)
Acquired cause: drug induced, basal ganglia lesions From other degenerative disorder (PD, PSP etc.)
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Classification of dystonia by distribution
5 categories: focal, segmental, multifoacl, hemi-, generalized
Focal dystonia: 2/3 of dystonic patients Focal dystonia: cervical dystonia(most
common), oromandibular dystonia, blemphalospasm, laryngeal dystonia, limb dystonia
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2 cervical dystoniaCervical dystonia
patterned, repetitive, clonic (spasmodic), or tonic (sustained) muscle contractions resulting in abnormal movements and postures of the the head and neck
Symptoms: pain, headache, abnormal posture, tremor, orthopedic or neurological complications
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Blephalospasm+oromandibular dystonia= Meiges syndrome
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Treatment Levodopar should be tried to exclude
DRD
Anti-cholinergic: Clonazepam, baclofen,
benzodiazepine, carbamazepine, tizanidine
Botulinum toxin infection
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Myoclonus
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Classification Etiology
physiological, essential, epileptic, symptomatic
Anatomical distribution focal, segmental,
multifocal, generalize
Provocative factor spontaneous, reflex,
action
Contraction pattern rhythmic, arrhythmic,
oscillaroty
Clinical neurophysiology testing
cortical, cortical-subcortical, subcortical-supraspinal, spinal, peripheral
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Step Describe distribution of myoclonus-
focal, generalize
Anatomical localization: cortical, subcortical, spinal, peripheral
Describe type - negative vs positive Look for other neurological vs physical
sign
Identify cause
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Negative myoclonus (flapping tremor or asterixis) in patient with hepatic encephalopathy
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Post hypoxic myoclonus - cortical myoclonus
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Hemifacial spasm
Most common peripheral myoclonus
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Chorea
the dancing
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Choreairregular, nonrhythmic, unsustained involuntary movement that flows from one part of the body to another
step to identify chorea- Most important Identify body part of chorea
- focal, hemibody: structural lesion in the brain- generalized: diffuse brain lesion (acquire vs congenital) or Toxic/Metabolic/Drug
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Acute right side chorea in acute basal ganglia infarction
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On
Off
A Parkinsons disease patient with motor
dyskinesia (chorea) during on L-dopa
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Conclusion Movement disorder: hypo-hyperkinetic Each type of hyper kinetic - description the movement
disorder pattern
Identify distribution, associated neurological finding and possible cause (for work up)
Very common movement disorder you should know : Parkinsons disease, essential tremor, structural lesion in the brain (mostly cause focal, hemi-body movement disorder), Generalised movement disorder (look for metabolic/drug)
Some may have genetic cause (ask for the family members)
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Thank you for your kind attention
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