Review of Movement
Disorders
Cherian Abraham Karunapuzha, M.D.
Assistant Professor
Movement Disorders Division
Department of Neurology
The University of Oklahoma Health Sciences Center
OU Neurology
Cherian Abraham Karunapuzha, MD
 Assistant Professor of
Neurology
 Sub Specialty – Movement
disorders
 Areas of Interest –
Botulinum toxin , Deep
brain stimulation, levodopa
pump, Parkinsonian
disorders
OU Neurology
DISCLOSURES
FINANCIAL DISCLOSURE
Consultant & Speaker for Teva neuroscience
and UCB
UNLABELED/UNAPPROVED USES
DISCLOSURE
The speaker has nothing to disclose
OU Neurology
LEARNING OBJECTIVES
 Identify and characterize the clinical significance of
bradykinesia, rigidity, and resting tremor – Parkinson
disease
 Distinguish and localize the lesions responsible for
essential tremor, resting tremor, and intention “tremor”
 Identify and describe:
– chorea, hemiballismus, and dyskinesia
– dystonia, tic, and myoclonus
OU Neurology
What is a “movement disorder” ?
Light Blue – ventricles
Red – Caudate nucleus
Green – Putamen
Dark Blue – Globus Pallidus
Black – Substantia Nigra
Brown - Thalamus
 Depending
Conditions
Most
diseases
arising
from
areBG
a
not
dysfunction
but
of signs
the
can can
be be
Not
“weakness”
on of
locus
-BG
more
of
like
damage,
a“fixable”
switchbox
the
with
alleviated,
extrapyramidal
Parkinson’s,
system
(basal
essential
ganglia)
tremors,
–
different Oneg.
bradykinesia,
and
rigidity,
Off switches
tremor,
chorea,
or programs.
dystonia,
tic,
Huntington’s, torticollis,
extrapyramidal
diseases Tourette’s
myoclonus…
OU Neurology
Basal Ganglia Circuit
D1
D2
Substantia
Nigra
Cortex
Thalamus
Striatum
Direct
Pathway
GPi
Striatum
Indirect
Pathway
GPe
STN
GPe – Globus Pallidus externa /lateral
GPi – Globus Pallidus interna /medial
STN – Subthalamic Nucleus
D1, D2 – types of dopamine receptors
OU Neurology
Common phenomenonology and
associated conditions

Parkinsonism











Bradykinesia
Freezing
Rigidity
Tremor – resting
Tremor – action/postural
Chorea, ballism
Stereotypy
Dystonia
Tic
Myoclonus













Parkinson disease
Multiple System atrophy
PSP
Drug induced parkinsonism
NPH
Essential Tremor
Drug induced tremor
Huntington disease
Sydenham chorea
Levodopa dyskinesias
Tardive dyskinesias
Torticollis, blepharospasm
Tourette syndrome
Metabolic, CJD
OU Neurology
Tremor
 Oscillatory, rhythmic and regular movement that affects
one or more body parts, such as the limbs, neck,
tongue, chin, or vocal cords.
 Distribution :
 Arms, head, legs, larynx…
 Unilateral or asymmetric or symmetric
 Context :
 Resting – 3-6 Hz
 Postural – 5-10 Hz
 Action (kinetic) – 5-10 Hz
 Terminal (intention) – 2-4 Hz
OU Neurology
Postural/Action Tremor
OU Neurology
Essential Tremor
Action or postural tremor of arms,
sometimes head and voice, occasionally
legs
Family history common
Improves with alcohol
Worsens with Caffeine
Mild tremor may first appear in teens or
young adulthood
Gradually worsen over decades
OU Neurology
Essential Tremor (Contd.)
Check for hyperthyroidism
Check for drug induced – albuterol, SSRI,
Depakote, antipsychotics
MRI brain for mimics - white matter
lesions brainstem & cerebellum
Rx: Propranolol 10 mg tid -> 60mg tid or
Primidone 25 mg qhs -> 250 mg tid
Surgery: deep brain stimulation (DBS) in
thalamus for refractory tremors
OU Neurology
Intention Tremor – usually associated cerebellar
findings of dysmetria, dysdiadochokinesia , ataxia etc.
OU Neurology
Psychogenic tremor ?? – improves with
distraction - Counseling/clinical psychology
OU Neurology
Resting Tremor
present in the distal parts of the
extremities and the lips while the involved
body part is “at rest” & ceases on active
movement of the limb
“Pill-rolling” tremor of the fingers
flexion-extension or pronation-supination
tremor of the hands
OU Neurology
Brady/hypokinesia – slowness or
decreased amplitude of movement
a loss of automatic movements
slowness in initiating movement on
command
reduction in amplitude of the voluntary
movement
masked facies, decreased frequency of
blinking, soft speech, drooling, small
handwriting, shuffling gait, decreased
armswing
OU Neurology
Rigidity - Increased resistance to
passive motion ..
..present equally in all directions of the
passive movement, equally in flexors and
extensors & throughout the range of
motion – extrapyramidal lesions
Stiffness, heaviness, or aching muscle
Cogwheel and Leadpipe rigidity
 Spasticity – Another hypertonic state – Damage
to the primary motor cortex or the corticospinal
tract - velocity dependent, clasp knife
phenomenon
OU Neurology
Parkinson Disease
A clinical syndrome resulting from
degeneration of nigro-striatal dopaminergic
neurons
Asymmetric onset
TRAP : Tremor (resting), Rigidity, Akinesia
(bradykinesia), Postural instability
Diagnosis – clinical exam & response to
levodopa (L-dopa) supplementation
OU Neurology
Parkinson disease (contd.)
 MRI brain to look for mimics - enlarged ventricles,
subdural hemorrhage, tumor, multiple subcortical
strokes
OU Neurology
Parkinson disease (contd.)
Incidence: 4.5-21 cases/100,000 per year
Cause: combination of genetic and
environmental factors
Pathology: Lewy
body – abnormal
aggregates of protein
(alpha synuclein)
OU Neurology
Parkinson disease (contd.)
slow progression - 10-25 years
independence usually not reduced in first
5-10 years
Later signs: postural instability, freezing,
levodopa induced dyskinesia, fluctuating
response to meds, dysphagia,
incontinence, postural hypotension,
dementia
OU Neurology
Freezing = arrest in initiation or
continuation of movement
OU Neurology
Parkinson disease (contd.)
 MAO B inhibitors – Rasagiline, Selegiline – mild
symptomatic effect, increase ON time,?disease modifying
 Dopamine agonists – Ropinirole, Pramipexole, Rotigotine
– moderate symptomatic effect
 Dopamine precursor – Carbidopa/Levodopa (Sinemet)
25/100 one tablet TID to 1200mg/day – MOST Robust
symptomatic effect
 COMT inhibitors – Entacapone – increase ON time
 NMDA antagonist – Amantadine - dyskinesias
 DBS (deep brain stimulation) or Surgical ablation
 PT/OT , speech therapy – Lee silverman voice therapy
OU Neurology
Choreioform Movements
Chorea - involuntary, irregular,
purposeless, nonrhythmic, abrupt, rapid,
unsustained movements that seem to
move unpredictably from one body part to
another
Ballism – larger amplitude and rapidproximal parts of limb
Athetosis - more sinuous slow, writhing
OU Neurology
Sydenham Chorea
 Post-strep reaction
 Demographics: teens,
female > male
 Chorea; sometimes
behavioral (OCD-like)
 Testing: ASOT, Anti
DNA-aseB, infection
usually resolved
 Tends to subside even without Rx in Weeks to Months
 Benzos, Valproate can reduce the chorea. Severe –
neuroleptics. Benzathine penicillin PPX till age 26
OU Neurology
Huntington’s Disease
OU Neurology
Huntington’s Disease
Neurodegeneration, initially of striatal
‘medium spiny’ neurons to GPe
 Caused by expanded CAG repeat in Huntingtin
Gene (autosomal dominant), normal < 35 repeats
Onset young adult, though can be any age
Presents with gradually worsening chorea,
cognitive, mood & behavioral changes
Management: antipsychotics,
antidepressant, Tetrabenazine, behavioral
OU Neurology
L-Dopa induced Dyskinesias – usually peak
dose effect 40 minutes after ingestion of L-Dopa
OU Neurology
Stereotypy
‘Coordinated’ movements that repeat
continually and identically
When they occur at irregular intervals,
stereotypies may not always be easily
distinguished from motor tics,
compulsions, gestures & mannerisms
Tics - occur paroxysmally out of a
background of normal motor behavior and
usually associated with an urge
OU Neurology
Tardive Dyskinesia
 Dopamine Antagonists (D2):
 Typical > atypical
antipsychotics
 metoclopramide (Reglan),
promethazine (Phenergan)
 ? D2 receptor hypersensitivity
 Appears after years of use
(sometimes even months)
 Risk factors: dose, older age,
female sex
 Classic : Oro-Bucco-Lingual
 Rx – Klonopin, Depakote,
stereotypy (lipsmacking)
Tetrabenazine 12.5 mg tid
 Anticholinergics - Artane,
 Remove offending drug, or switch
Cogentin or even Benadryl
to a more atypical antipsychotic
worsen it.
OU Neurology
Tics
Brief, Stereotyped abnormal movements or
sounds
In response to urge (psychic/physical ‘itch’)
Can be complex (sequence, muscles)
Can be stopped on command, but there is
ensuing buildup of tension to do it
The diversity of motor tics is one feature
that sets their phenomenology apart from
stereotypies
OU Neurology
Tourette Syndome
Onset < 18 . For > 1 year
Multiple motor tics and vocal tics –
coprolalia. Tics evolves over time.
Comorbidities: ADHD, OCD, impulse
control disorder
M > F (female relatives may have OCD)
OU Neurology
Tourette Syndome (contd.)
 Adrenergic - Guanfacine,
Clonidine (0.1 - 0.3mg
tid)
 Antipsychotics - Pimozide,
Haloperidol, Risperidone
(1-16 mg/day div qd-bid)
 Dopamine Depletor –
Tetrabenazine
 Stimulants – Adderall,
Ritalin SR (20 – 60 mg/day
div qd-bid)
 Antidepressants
 Behavioral therapy
 ? DBS
OU Neurology
Dystonia
 Twisting movements that tend to be sustained at
the peak of the movement
 Frequently repetitive in the same group of muscles
(patterned – unlike Chorea)
 Often progresses to prolonged abnormal postures
 Can be focal, segmental or generalized
 Primary or Secondary
 Often induced by action, sometimes task-specific
such as writing etc.
 Have sensory tricks which minimise the dystonia
temporarily
OU Neurology
Adult onset focal dystonia (more
common)
 Cervical dystonia
(torticollis)
 Blepharospasm
(forced eyelid
closure)
 Usually idiopathic
but sometimes a
triggering insult
(eg. Injury, Antidopaminergic
meds)
OU Neurology
Dystonia (contd.)
Stop anti-dopaminergics
Anticholinergic: Cogentin, Artane(2 -16mg
per day div. TID titrated gradually over 23 months).
Muscle relaxants – Benzos, Baclofen
Botulinum toxin injection – focal dystonias
– last 3-4 months
DBS - “Humanitarian Device Exemption”
OU Neurology
Myoclonus
Sudden lightning-like movement
Can be repetitive but Not rhythmic (if it
was, it would be a tremor!)
Can be focal or regional or multifocal or
generalized; epileptic and non epileptic.
Like tremor, myoclonus can arise from
disease of many parts of CNS, not just
basal ganglia
OU Neurology
Myoclonus (contd.)
 Physiologic
(Hypneic, Hiccups)
 Serotonin
Syndrome
 Dementias –
Creutzfeld Jakob
Disease (CJD)
 Multiple Sclerosis
 JME (epilepsy)
 Rx: clonazepam (0.25-0.5 mg PO bid-tid) or valproic
acid or levetiracitam
OU Neurology
Restless Legs Syndrome (RLS) or
Willis Ekbom syndrome
Diagnostic criteria:
 Voluntary urge to move the limb usually
secondary to an uncomfortable sensation
Worse during periods of rest
Relieved with movement of the limb
Worse in the evening/night **
Family history
Response to dopaminergic meds
Periodic limb movements in Sleep (PLMS)
OU Neurology
RLS (Contd.)
 Secondary RLS:
 Uremia
 Pregnancy
 Iron deficiency
 Iron supplementation if
Ferritin levels <50µg/l
 Exacerbating drugs:
 Antihistamines
 Antipsychotics
 Antidepressants (except
Bupropion or Trazodone)
 Preventive & Rescue
Treatment:
Coping strategies
Dopamine agonists
(Requip, Mirapex,
Neupro)
Antiepileptics
(Horizant)
Opioids **
Sinemet **
OU Neurology
Thank You
Questions and Comments
Contact or Refer - OU Movement disorders clinic
www.wemove.org
OU Neurology