The Northern Neuroscience Centre Chiangmai University

NNC MU
Movement disorder
tutorial
Surat Tanprawate, MD, MSc (London), FRCP(T)
Division of Neurology, Chaingmai 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 approach
3 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
rigid syndrome
Hypokinetic
rigid syndrome

Hyperkinetic
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 = dance
irregular, nonrhythmic, unsustained
involuntary movement that flows from
one part of the body to another
motor impersistence

Dancing lady

Dystonia
syndrome of sustained muscle
contractions, frequently causing twisting,
repetitive movements, or abnormal
postures
sustained contractions, consistent directional
or patterned character (predictable), and
exacerbation during voluntary movements
sensory trick

Generalised dystonia

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

positive myoclonus
negative myoclonus

Generalised myoclonus

Tremor
a 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

Tics
repetitive, stereotyped, involuntary,
sudden, inopportune, non-propositional,
and irresistible movement
unpleasant feeling
not absolutely clear as patients can exert some control
on the movement
can be simple or complex

He have had tic since he

was 10 y.o.

Ballism=dacing
involuntary, 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 tremor

2.Specific examination for assessment of
signs related to tremor

3.Syndrome classification of tremor

Terminology for tremor and the

hierarchical relation of the terms as
indicated by the numbers

Inspection
Frequency
Low (<4 Hz)
Medium (4-7 Hz)
High (>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
tone (including Froments sign for the upper
Muscle
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 classification of 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

Rest

Bradykinesia,
postural instability,
rigidity

Neuro-degeneration

Cerebellar tremor

Postural, kinetic,
intention

Ataxia

Various cause
affected cerebellar
pathway

Syndrome
classification of
tremor
Tremor description
(activated by, location,
frequency)
+
Specific s/s

Characteristics of Essential Tremor and Parkinsonian Tremor

Essential tremor
Core 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)

Beneficial response to ethanol

Family history: reported in > 50% of the

patients

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 (<4.5 Hz)

during postural in nature

Upper brain stem, thalamus,

cerebellum, interrupting pathways in the

midbrain tegmentum

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=1:1
de 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.babraham.ac.uk/images/research/SAS/emson/fig1.jpg

http://www.uhmc.sunysb.edu/pathology/neuropath

Pathology
Normal substantia nigra

Surviving neuron contains a Lewy body

Extensive loss of pigmented neurons

Group of Parkinsonism

Primary or idiopathic parkinsonism

hydrocephalus, vascular parkinsonism, encephalitis

Parkinson plus syndrome

Parkinsons disease

Secondary parkinsonism

TYPICAL
OR
CLASSIC

Progressive supranuclear palsy(PSP), corticobasal

degeneration(CBD), multiple system atrophy(MSA)

Hereditary parkinsonism

ATYPICAL

Wilsons disease, Dopa-responseive dystonia,

Huntingtons disease(HD)

United Kingdom Parkinson's Disease

Society(UKPDS) Brain Bank Diagnostic
Criteria for PD

Step 1: Diagnosis of Parkinsonism

Step 3: Features that support a diagnosis of

Parkinsons disease (three or more required
for diagnosis of definite Parkinsons disease)

Step 2: Features tending to exclude

Parkinsons disease as the cause of
Parkinsonism

Diagnostic
accuracy to 82%

Hughes AJ, Daniel SE, Kilford L, Lees AJ. JNNP 1992 Mar;55(3):181-4

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

Babinski's sign

Negative response to large doses of

levodopa (if malabsorption
excluded)

MPTP exposure

Cerebellar signs
Early severe autonomic involvement
Early severe dementia with
disturbances of memory, language
and praxis

Presence of a cerebral tumour or

communicating hydrocephalus on
computed tomography scan

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 progressiontreatment response

Modality of treatment

Symptoms based treatment

Pharmacologic vs Non-pharmacologic
Motor vs Non-motor symptoms

Neuro-protection
Prevention

Motor symptoms of Parkinsons

disease

Dopamine

Acetylcholine

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 HBS
Sinemet CR

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
39

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 etiology

Primary 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

Cervical
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

2 cervical dystonia

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, corticalsubcortical, subcorticalsupraspinal, spinal,

peripheral

Step
Describe distribution of myoclonusfocal, 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

Chorea
irregular, 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

A Parkinsons disease
patient with motor
dyskinesia (chorea)
during on L-dopa
On

Off

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)

NNC MU
Thank you for your
kind attention
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