movement disorder for resident

Movement disorder tutorial

Surat Tanprawate, MD, FRCPTDivision of Neurology, Chaing Mai University

Monday, December 26, 2011

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,

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 movements”

“sensory trick”

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

“positive myoclonus”

“negative 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

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”

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

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

• 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)

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

Characteristics of Essential Tremor and Parkinsonian Tremor

Syndrome classification of

tremor

Tremor description (activated by, location,

frequency)+

Specific s/s

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

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)

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

Wing Beating Tremor

Dystonic tremor

Palatal tremor

• previously term “palatal myoclonus”

• from brainstem or cerebellar lesion or essential

Palatal tremor

• previously term “palatal myoclonus”

• from brainstem or cerebellar lesion or essential

Triangle of Guillain-Mollaret

Parkinsonʼs disease and other Parkinsonism

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

Rigidity

Stooped posture

Hips and kneesslightly flex

Tremor

Short shuffling steps

Reduce arm swing

Paralysis agitan (shaking palsy)

Parkinsonism

• clinical syndrome of bradykinesia, resting tremor, cogwheel rigidity, and postural instability

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)

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

Parkinsonʼs disease

Gibb et al, 1988, Table from Litvan et al, 2003Monday, December 26, 2011

PD- diagnostic criteria

Gibb et al, 1988, Table from Litvan et al, 2003

Diagnostic accuracy to 82%

Pill rolling tremor

Finger tapping

Bradykinesia• Slowly movement

• Impairment of repetitive movement: diminish

• Speed, amplitude, rhythm

• Test by

• Finger and foot tapping

• Writing

Bradykinesia

Micrographia

PD is a progressive

disease

Not just motor, but also

non-motor symptoms

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)

Christine CW, Aminoff MJ, Am J Med. 2004;117: 412–419.

Differential diagnosis

PD disease progression-treatment response

The natural history of response to levodopa in PD

Modality of treatment

• Symptoms based treatment

• Pharmacologic vs Non-pharmacologic

• Motor vs Non-motor symptoms

• Neuro-protection

• Prevention

Dopamine Acetylcholine

Motor symptoms of Parkinson’s 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

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

• Progressive supranuclear palsy

• Corticobasal degeneration

• Dementia with lewy bodies

Multiple system atrophy (MSA)

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

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

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

MSA-P vs MSA-C

Progressive Supranuclear Palsy

• 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

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)

• 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)

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 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

Sensory tricks in cervical dystonia

• “Geste antagoniste” “Gegendruckphenomen”

• Maneuvers which reduce or abolish dystonic posture in CD

• Unknown mechanism

• May sensorimotor integration

Blephalospasm+oromandibular dystonia= Meigeʼs syndrome

Treatment• Levodopar should be tried to exclude

• 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

• Where is the lesion?• Physiological classification

• What is the lesion?• Etiological classification

CorticalSubcortical

SpinalPeripheral

Anatomic distribution

Contraction pattern

Provocative factor

Etiology-physiologic-essential-epileptic myoclonus-symptomatic

Rhythmic Provocative

cortical +/- +

subcortical +/- ++

spinal + -

peripheral - -

Focal, segmentalcortical, spinal, peripheral

Multifocal, generalized

cortical, subcortical

Fortum induced myclonus

Post hypoxic myoclonusMonday, December 26, 2011

Hemifacial spasm

Most common peripheral myoclonusMonday, December 26, 2011

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movement disorder for resident

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