Movement disorder tutorial

Surat Tanprawate, MD, MSc (London), FRCP(T)Division of Neurology, Chaingmai University

NNCMUThe Northern Neuroscience Centre Chiangmai University

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

Neurological diseases

Congenital Trauma Tumor Infection/inflammation Degeneration Demyelination

Vascular Metabolic/Toxic/Drugs Genetic Cryptogenic Idiopathic

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

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

Anatomy of movement control

Direct and Indirect pathway of movement control

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?

Movement disorder

Hypokinetic Hyperkinetic

Hypokinetic rigid syndrome

Hyperkinetic rigid syndrome

Pattern of movement disorder

Classify by anatomy, distribution, cause,

age

Hyperkinetic movement disorder

Rhythmic, sustained, intermittent, speed, suppressibility, complex movement

Tremor, Chorea, athetosis, dystonia, myoclonus, ballism, tic

Chorea = danceirregular, nonrhythmic, unsustained involuntary movement that flows from one part of the body to another

motor impersistence

Dancing lady

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

Generalised dystonia

Myoclonussudden, brief, jerky, and shock-like involuntary movements involving face, trunk, and extremities

positive myoclonusnegative myoclonus

Generalised myoclonus

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

Tremor

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

He have had tic since he was 10 y.o.

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

Athetosis = without fixed position

involuntary, convoluted, writhing, slow movements of the arms, fingers and legs

Pseudoathetosis in a patient with severe axonal polyneuropathy

Common movement disorder

Tremor

Step approach- MDS consensus

1.Inspection the tremor2.Specific examination for assessment of

signs related to tremor3.Syndrome classification of tremor

Terminology for tremor and the hierarchical relation of the terms as

indicated by the numbers

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

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)

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

Syndrome classification of

tremor

Tremor description (activated by, location,

frequency)+

Specific s/s

Characteristics of Essential Tremor and Parkinsonian Tremor

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

Essential tremor

Secondary criteria for identifying ET

Long duration (>3 years) Family history: reported in > 50% of the

patients

Beneficial response to ethanol

Essential Tremor

Achimedes spiral

Achimedes spiral

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)

Holmes tremor midbrain tremor rubral tremor

thalamic tremor

predominately proximal limb (

Wing Beating Tremor in Wilsons disease

Dystonic tremor

Parkinsons disease

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

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)

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

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

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

Pathology

Normal substantia nigra

Extensive loss of pigmented neurons

Surviving neuron contains a Lewy body

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

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%

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

Pill rolling tremor

Finger tapping

Bradykinesia

Micrographia

Micrographia

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

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

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)

Diagnostic studies

MRI/CT brain: using for exclude other cause of parkinsonism

In PD, the MRI brain usually reveals normal

PD disease progression-treatment response

Modality of treatment

Symptoms based treatment Pharmacologic vs Non-pharmacologic Motor vs Non-motor symptoms

Neuro-protection Prevention

Dopamine Acetylcholine

Motor symptoms of Parkinsons disease

Symptomatic based treatment

Enhance dopaminergic transmission L-dopa, dopamine agonist, drug that

decrease dopamine destruction

Drug manipulating other neurotransmitter

Anti-cholinergic drug

Dose of the preparations of Sinemet and Madopar

Levodopa + DDI

Madopar (levodopa+benserazide)Sinemet (levodopa+carbidopa)

Madopar HBSSinemet CR

39

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

Parkinson Plus Syndrome

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

PSP described by Richardson

Dystonia

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

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.)

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

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

Blephalospasm+oromandibular dystonia= Meiges syndrome

Treatment Levodopar should be tried to exclude

DRD

Anti-cholinergic: Clonazepam, baclofen,

benzodiazepine, carbamazepine, tizanidine

Botulinum toxin infection

Myoclonus

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

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

Negative myoclonus (flapping tremor or asterixis) in patient with hepatic encephalopathy

Post hypoxic myoclonus - cortical myoclonus

Hemifacial spasm

Most common peripheral myoclonus

Chorea

the dancing

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

Acute right side chorea in acute basal ganglia infarction

On

Off

A Parkinsons disease patient with motor

dyskinesia (chorea) during on L-dopa

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)

Thank you for your kind attention

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