Movement Disorder for Resident
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Movement disorder tutorial
Surat Tanprawate, MD, FRCPTDivision of Neurology, Chaing Mai University
Monday, December 26, 2011
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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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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 movements”
“sensory trick”
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Myoclonussudden, brief, jerky, and shock-like involuntary movements involving face, trunk, and extremities
“positive myoclonus”
“negative 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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Ticsrepetitive, 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”
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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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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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Tremor
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Step approach- MDS consensus
1. Inspection the tremor
2. Specific examination for assessment of signs related to tremor
3. 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 (<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
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Specific examination for assessment of:
• Akinesia/bradykinesia
• Muscle tone (including Fromentʼs 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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Froment's (muscle tone) sign
• Increase in resistance to passive movements of a limb about a joint that can be detected specifically when there is a voluntary activity of another body part.
• Comment: This phenomenon may be seen in a wide variety of tremulous disorders including ET and PD
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Characteristics of Essential Tremor and Parkinsonian Tremor
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Syndrome classification of
tremor
Tremor description (activated by, location,
frequency)+
Specific s/s
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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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Essential Tremor
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Achimedes spiral
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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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Holmeʼs 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
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Wing Beating Tremor
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Dystonic tremor
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Palatal tremor
• previously term “palatal myoclonus”
• from brainstem or cerebellar lesion or essential
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Palatal tremor
• previously term “palatal myoclonus”
• from brainstem or cerebellar lesion or essential
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Triangle of Guillain-Mollaret
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Parkinsonʼs disease and other Parkinsonism
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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;14:223–36
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Rigidity
Stooped posture
Hips and kneesslightly flex
Tremor
Short shuffling steps
Reduce arm swing
Paralysis agitan (shaking palsy)
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Parkinsonism
• clinical syndrome of bradykinesia, resting tremor, cogwheel rigidity, and postural instability
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Parkinsonism
• clinical syndrome of bradykinesia, resting tremor, cogwheel rigidity, and postural instability
Parkinsonʼs 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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Group of Parkinsonism• Primary or idiopathic parkinsonism
• Parkinsonʼs disease
• Secondary parkinsonism
• hydrocephalus, vascular parkinsonism, encephalitis
• Parkinson plus syndrome
• Progressive supranuclear palsy(PSP), corticobasal degeneration(CBD), multiple system atrophy(MSA)
• Hereditary parkinsonism
• Wilsonʼs disease, Dopa-responseive dystonia, Huntingtonʼs disease(HD)
TYPICAL OR
CLASSIC
ATYPICAL
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Parkinsonʼs disease
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Gibb et al, 1988, Table from Litvan et al, 2003Monday, December 26, 2011
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PD- diagnostic criteria
Gibb et al, 1988, Table from Litvan et al, 2003
Diagnostic accuracy to 82%
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Pill rolling tremor
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Finger tapping
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Bradykinesia• Slowly movement
• Impairment of repetitive movement: diminish
• Speed, amplitude, rhythm
• Test by
• Finger and foot tapping
• Writing
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Bradykinesia
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Micrographia
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Micrographia
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Micrographia
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PD is a progressive
disease
Not just motor, but also
non-motor symptoms
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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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Christine CW, Aminoff MJ, Am J Med. 2004;117: 412–419.
Differential diagnosis
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PD disease progression-treatment response
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The natural history of response to levodopa in PD
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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 Parkinson’s 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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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
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Parkinson Plus Syndrome
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Parkinson-plus syndrome
• Multiple system atrophy
• Progressive supranuclear palsy
• Corticobasal degeneration
• Dementia with lewy bodies
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Multiple system atrophy (MSA)
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MSASecond consensus statement on the diagnosis of MSA, Gilman et al Neurology 2008
Definite MSA– Pathologic findings of widespread and abundant CNS α-synuclein-positive glial cytoplasmic inclusionsAND– Neurodegenerative changes in striatonigral or olivopontocerebellar structures
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Second consensus statement on the diagnosis of MSA, Gilman et al Neurology 2008
Table 2 Criteria for possible MSA • A sporadic, progressive, adult (>30 y)–onset diseasecharacterized by ● Parkinsonism (bradykinesia with rigidity, tremor, or posturalinstability) or ● A cerebellar syndrome (gait ataxia with cerebellar dysarthria,limb ataxia, or cerebellar oculomotor dysfunction) and● At least one feature suggesting autonomic dysfunction (otherwise unexplained urinary urgency, frequency or incomplete bladder emptying, erectile dysfunction in males, or significant orthostatic blood pressure decline that does not meet the level required in probable MSA) and● At least one of the additional features shown in table 3
Gilman et al Neurology 2008
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Table 4 Features supporting (red flags) and not supporting a diagnosis of MSA
Supporting features● Orofacial dystonia● Disproportionate antecollis● Camptocormia (severe anterior flexion of the spine) and/or • Pisa syndrome (severe lateral flexion of the spine) ● Contractures of hands or feet ● Inspiratory sighs● Severe dysphonia ● Severe dysarthria● New or increased snoring● Cold hands and feet● Pathologic laughter or crying● Jerky, myoclonic postural/action tremor
Nonsupporting features● Classic pill-rolling rest tremor● Clinically significant neuropathy• Hallucinations not induced by drugs● Onset after age 75 y● Family history of ataxia orparkinsonism● Dementia (on DSM-IV)● White matter lesions suggesting multiple sclerosis
Gilman et al Neurology 2008
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MSA-P vs MSA-C
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Progressive Supranuclear Palsy
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• Richardson syndrome(54%)
• early onset of postural instability, supranuclear gaze palsy, and cognitive dysfunction
• PSP-Parkinsonism(32%)
• features more typical of idiopathic PD, including a moderate response to levodopa
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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 etiology
• Primary dystonia
• AD: early-onset limb dystonia (DYT1), Mixed dystonias (DYT6, DYT13), Late-onset craniocervical dystonia (DYT7)
• 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 (Wilsonʼs disease, MLD)
• Acquired cause: drug induced, basal ganglia lesions
• Unknown etiology (PD, CBD, PSP)
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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 พี่น้อง cervical 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
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Sensory tricks in cervical dystonia
• “Geste antagoniste” “Gegendruckphenomen”
• Maneuvers which reduce or abolish dystonic posture in CD
• Unknown mechanism
• May sensorimotor integration
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Blephalospasm+oromandibular dystonia= Meigeʼs 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
• Where is the lesion?• Physiological classification
• What is the lesion?• Etiological classification
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CorticalSubcortical
SpinalPeripheral
Anatomic distribution
Contraction pattern
Provocative factor
Etiology-physiologic-essential-epileptic myoclonus-symptomatic
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Rhythmic Provocative
cortical +/- +
subcortical +/- ++
spinal + -
peripheral - -
Focal, segmentalcortical, spinal, peripheral
Multifocal, generalized
cortical, subcortical
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Fortum induced myclonus
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Post hypoxic myoclonusMonday, December 26, 2011
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Hemifacial spasm
Most common peripheral myoclonusMonday, December 26, 2011
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See you next
lecture !!!
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Monday, December 26, 2011