Movement Disorders Flashcards
What does ‘movement disorders’ divide into?
Hypokinesia
Hyperkinesia
What is hypokinesia?
Slowed movement, increased tone
What is hyperkinesia?
Excessive movement
What is idiopathic Parkinson’s Disease?
Condition of progressive reduction of dopamine in the basal ganglia leading to hypokinetic movement disorder
What are the risk factors for idiopathic Parkinson’s Disease?
Age >65 Male sex Pesticide exposure Occupation: farmer Family history
What is a protective factor for idiopathic Parkinson’s Disease?
Smoking
What is the underlying pathology for idiopathic Parkinson’s Disease?
Degeneration of dopamine neurones within the substantial nigra
Accumulation of Lewy bodies, initially within the substantial migration then the rest of the brain
What are the main clinical features of idiopathic Parkinson’s Disease?
Tremor
Rigidity
Akinesia (bradykinesia)
Postural changes
Are features of idiopathic Parkinson’s Disease symmetric or asymmetric?
Asymmetrical
The initially affected side will always be worse
Describe the tremor seen in idiopathic Parkinson’s Disease?
Resting tremor (can re-emerge after few secs when hands held outstretched)
Pill rolling tremor
Made worse by emotion and stress
Describe muscle tone in idiopathic Parkinson’s Disease?
Increased tone
Cog wheel rigidity (rating tremor + rigidity)
Positive Froment’s manoeuvre (rigidity increases in examined body segment by voluntary movement of other body parts)
Describe bradykinesia in idiopathic Parkinson’s Disease?
Difficulty initiating movement
Progressive reduction in speed amplitude with attempted rapid alternating movement of body segments
Reduced blinking, facial expression, volume of voice
Progressive reduction in size of handwriting
Describe postural changes in idiopathic Parkinson’s Disease?
Difficulty standing up
Slow to start walking
Small shuffling steps with reduced arm swing
Stooped posture
Difficulty turning and navigating objects
Falls in late disease
What are the non-motor symptoms in idiopathic Parkinson’s Disease?
Drooling
Reduced sense of smell
REM sleep disorders
Autonomic (constipation, urinary urgency, frequency)
Psychiatric (depression, dementia, psychosis, personality change, visual hallucinations)
What is the usual progression of symptoms in idiopathic Parkinson’s Disease?
Constipation, hyposmia, depression (prodromal)
Bradycardia, rigidity, tremor, pain, fatigue (first few years)
Fluctuations, dyskinesia
Urinary symptoms, dementia, dysphagia, postural instability, falls(10 years)
Psychosis (20 years)
What is the diagnostic investigation in idiopathic Parkinson’s Disease?
Clinical examination
What is assessed in clinical examination of idiopathic Parkinson’s Disease?
Tremor Gait Upper and lower limb: tone, reflexes Eye movements Speech Writing
What investigations other than clinical can be done for idiopathic Parkinson’s Disease?
Levodopa challenge test
Genetic testing if very young or very strong family history
Structural brain imaging if doubt
What is the gold standard management for idiopathic Parkinson’s Disease?
Levodopa + Carbidopa
How does Levodopa work, and how does Carbidopa improve its effects?
Levodopa crosses the BBB and is converted into dopamine in the brain
Carbidopa prevents peripheral breakdown of dopamine
What are the side effects of Levodopa + Carbidopa as treatment for idiopathic Parkinson’s Disease?
Nausea, vomiting
Dyskinesia/dystonia
Sudden off states
Psychiatric: psychosis, hallucinations, reduced impulse control
What is used in idiopathic Parkinson’s Disease to manage the nausea that can be caused by medication, and why?
Domperidone
Used as it is selective to peripheral dopamine receptors so won’t prevent activity of levodopa in the brain
Avoid Metoclopramide
When is levodopa used in idiopathic Parkinson’s Disease, and why then?
Use is delayed until significant disability
It has diminishing effects, with action lasting 5-10 years
What drugs are used in idiopathic Parkinson’s Disease until levodopa used?
Dopamine agonists: Ropinirole, Pramipexole, Apomorphine)
COMT inhibitors: Entacapone
MOAB inhibitors: Selegeline, Rasagiline
What are Parkinsonian disorders?
Word used to describe a cluster of disorders associated with symptoms of idiopathic Parkinson’s but have a defined underlying cause
How are Parkinsonian disorders differentiated from actual Parkinson’s?
Symmetrical presentation Absence of tremor Levodopa unresponsiveness Fast progression Sensory disturbance Pyramidal signs Early falls
What are examples of Parkinsonian disorders?
Progressive supranuclear palsy Multi-System Atrophy Cortico-basal degeneration Vascular Parkinson's Dementia with Lewy bodies Drug-induced Parkinson's Wilson's disease Fragile X-tremor ataxia syndrome
Describe progressive supranuclear palsy
Vertical gaze palsy Lack of tremor Early postural instability and falls Speech and swallowing problems Symmetrical truncal rigidity more prominent than limb rigidity
Describe multi-system atrophy
Onset in 6th or 7th decade
Core triad: Early autonomic disturbance (postural hypotension, bladder dysfunction), ataxia, cerebellar signs
Pyramidal signs (spasticity, weakness, slowing of rapid alternating movements, hyperreflexia)
Jerky, postural tremor
MRI: hot cross bun sign due to cerebellar and pontine atrophy
Describe cortico-basal degeneration
Rigidity and weakness in one limb Myoclonus Dementia Apraxia - loss of purposeful movements Sensory disturbance, with severe cases causing alien limb phenomenon
Describe vascular Parkinson’s
Caused by multiple small infracts
Predominately affects lower limbs
Associated with prominent gait disturbance
Pyramidal signs and additional brain dysfunction seen due to infarcts
Structural brain imaging for diagnosis
Describe dementia with Lewy bodies
Cognitive decline with visual hallucinations
Differentiated based on onset of cognitive decline before the symptoms of Parkinson’s and not the other way around if the decline is caused by actual Parkinson’s
Describe drug-induced Parkinson’s
Responsible drugs: metoclopramide, antipsychotics, amiodarone
Drugs block the action of dopamine
Symmetrical Parkinsonism
Series of events important - symptoms emerging after drug exposure
Improvement/resolution within a few months of complete drug withdrawal
Describe Wilson’s disease
Hepatic and renal failure
Behavioural changes and cognitive decline
Keyser fisher rings in the eyes
Diagnosed by checking ceruloplasmin level
Describe fragile X-tremor ataxia syndrome
Late-onset (>50 years)
Cerebellar signs, dyssutonomia, cognitive decline of frontal type, peripheral neuropathy, Parkinsonism
Slow progression
Milder symptoms in females with permutation - premature ovarian failure and menopause
MRI shows hyper intensities in middle cerebellar peduncles (MCP)
Molecular testing
What is a tremor?
Rhythmic wave-like oscillations of a body part
What are the types of tremor?
Resting
Postural
Intention
What are the features of a resting tremor?
When muscles relaxed Slow Asymmetrical Abolished by movement Made worse by distraction Re-emergent
What can cause a resting tremor?
Parkinson’s
What are the features of a postural tremor?
Absent at rest, present on maintained posture
May persist during movement but key - doesn’t get worse with movement
Variable characteristics
What are causes of a postural tremor?
Anxiety Alcohol withdrawal Benign essential tremor Thyrotoxicosis Drugs (beta agonists - salbutamol, caffeine, lithium, valproate, tricyclics)
What is a benign essential tremor?
Autosomal dominant
Bilateral, fast, low amplitude tremor
Symptoms improve with alcohol consumption
What is the character of a thyrotoxicosis tremor?
Postural
Bilateral, symmetrical, fast
What are the features of an intention tremor?
Task orientated tremor
Made worse throughout range of movement
How does a cerebellar intention tremor present, and how is it assessed?
Gets worse as hand moves towards target
Assessed by finger nose test
What are causes of an intention tremor?
Cerebellar disease (MS, stroke, haemorrhage) Wilson's disease
What is dystonia?
Involuntary, sustained muscle spasms that result in abnormal posturing and repetitive movements in the context of an associated tremor
Movements are typically patterned, twisting, tremulous
Often worsened by voluntary action
What is the difference between focal and generalised dystonia?
Focal - limited to single muscle groups
Generalised - occur all over the body but typically start in one limb and then spread
What are examples of focal dystonias?
Cervical neck: torticollis Eyes: blepharospasm (rapid blinking) and forced eye closure Jaw: clenching of the jaw Vocal cords: coarse, strained voice Upper and lower limbs
What are causes of focal dystonias?
Cervical: muscle strain, OA Eyes: myasthenia, dry eyes Jaw: myasthenia, TMJ dysfunction Vocal cords: laryngitis, polyps Limbs: overuse, nerve entrapment
What are causes of generalised dystonias?
Genetic: dopa responsive dystonia, primary torsion dystonia
Drug induced: levodopa, anti psychotics, metoclopramide/Cyclizine
What is the presentation of drug-induced dystonia?
Torticollis
Trismus (jaw clenching)
Oculogyric crisis (eyes drawn up)
What is the management of dystonia?
Anti-spasmodics: Bacolfen
Botox (focal dystonia)
Anti cholinergic: Procyclidine (acute dystonia)
What is chorea?
Non-rhythmical irregular purposeless movements that flit and flow from one body part to another
What are causes of chorea?
Infection: HIV
Neurological: Parkinson’s
Genetic: Wilson’s, Huntington’s
Autoimmune: SLE, anti-phospholipid, Sydenham’s
Metabolic: thyrotoxicosis, sodium or magnesium disturbance
Drugs: anti psychotics, anti convulsants, levodopa, OCP
What is Huntington’s Disease?
Autosomal dominant neurodegenerative disease associated with CAG expansion in chromosome 4, which results in the production of a neurotoxin
What is the penetrance for Huntington’s Disease?
100%
What is meant by Huntington’s Disease showing ‘acceleration’?
Gets worse as it is passed down though the generations
Who presents with Huntington’s Disease?
Usually 4th decade (although age of onset variable)
M=F
What is the presentation of Huntington’s Disease?
Triad: emotional, cognitive, motor disturbance
Early: clumsiness, chorea, irritability, agitation, depression
Late: myoclonus, rigidity, fits, cognitive decline, dementia, loss of ability to speak or swallow
How is Huntington’s Disease diagnosed?
Genetic testing
What is the management of Huntington’s Disease?
Supportive only
What is the prognosis of Huntington’s Disease?
Average lifespan after diagnosis is 15 years
What is Sydenham’s chorea?
Self limiting chorea that is usually seen in children
Develops after a strep throat infection
What is ballism?
Extreme variant of chorea
Particularly involves proximal joints resulting in large amplitude flinging movements
Most often in a hemi-body distribution (‘Hemibalism/Hemichorea’)
Strongly suggestive of a contralateral lesion typically involving the striatum of subthalamic nucleus
What is myoclonus?
Sudden, involuntary focal or generalized muscle jerks
What is defined as abnormal myoclonus?
Over 5 muscle jerk movements
What are causes of myoclonus?
Genetic: benign essential myoclonus
Neurological: stroke, head injury, SOL
Infection: CJD (dementia, ataxia, myoclonus)
Drugs: levodopa, anti convulsants, alcohol withdrawal
Metabolic dysfunctions: liver failure, renal failure, hyponatremia
What are tics?
Involuntary stereotyped movements or vocalizations
Unable to be suppressed and attempts to suppress cause anxiety and discomfort, which is relieved upon completing the tic
What is the main primary tic disorder?
Tourette’s
What are causes of secondary tic disorders?
Autism
Huntington’s
Wilson’s
Haemochromatosis
