Movement Disorders Flashcards
What is a tremor?`
Rhythmic sinusoidal oscillation of a body part
What are tics?
Involuntary stereotyped movements or vocalisations
What is chorea?
Brief irregular purposeless movements which flit and flow from one body part to another
What is myoclonus?
Brief electric-shock like jerks
What is dystonia?
Abnormal posture of the affected body part
How are tremors classified?
Position(rest, posture, movement), distribution, frequency, amplitude
What are some causes for a resting tremor?
Parkinson’s disease
Drug-induced parkinsonism
Psychogenic tremor
What are some causes for a postural tremor?
Essential tremor
Enhanced physiological tremor
Tremor associated with neuropathy
What are some causes for a kinetic tremor?
Cerebellar disease (demyelination, haemorrhage, degenerative, toxic) Wilson's disease
What are some causes for a head tremor?
Dystonia
Cerebellar disease
What are some causes for a jaw tremor?
Dystonia
Parkinson’s disease
What are some causes for a palatal tremor?
With ataxia
Symptomatic
Essential tremor
What investigations should be done in patients with tremor?
Possibly TFTs and in young patients (<45) copper and coeruloplasmin
What is a tremor associated with dystonia?
Tremor in a body part that is not dystonic but there is dystonia elsewhere
What is essential tremor?
Abnormal tremor in bilateral upper limbs with absence of neuro signs elsewhere. Long duration No rest tremor \+ve FHx Alcohol responsive
What are the first line treatments for dystonic tremor?
Propanolol and primidone
Deep brain stimulation for severely affected patients
Which brain region is affected in dystonia?
Basal ganglia
What 3 main physiological abnormalities have been found in patients with dystonia?
Loss of reduction in reciprocal inhibition
Alterations in brain plasticity
Alterations in sensory function
What does the gene DYT1 cause?
Torsion Dystonia
Describe torsion dystonia
Starts before 28yo- usually childhood
Starts in a limb- usually legs
Progresses over 5-10y to become generalised/multifocal
Often +ve FHx, but gene not highly penetrant
Trunk and neck in minority, head/face very rare
What is the best option for cervical dystonia?
Botox A or B(both same effect, low dose has fewer side effects and works as well as high)
What is good at treating writer’s cramp?
Botox
When is surgery required in cervical dystonia?
When botox is ineffective
What are some AI causes of chorea?
Anti-phospholipid syndrome Bechet syndrome Coeliac disease Sydenham’s chorea Hashimotos’ thyroiditis
What are some inherited/degenerative causes of chorea?
HD/HD-like syndromes Wilson's disease Neuroacanthocythosis Benign hereditary chorea Ataxia telangiectasia Spinocerebellar ataxia Typ 17
What are some infectious causes of chorea?
HIV
What are some drug-based causes of chorea?
Dopamine-receptor blocking drugs, levodopa, stimulants, pill, anticonvulsants
What are some paroxysmal chorea causes of chorea?
PD
What are some metabolic causes of chorea?
Chorea gravidarum, glucose, thyroid, parathyroid, sodium, magnesium metabolism
What investigations should be carried out in chorea?
Possibly brain imaging Bloods for acanthocytes ds-DNA antiphospholipid autoantibodies Copper Genetic testing for HD
What is the treatment for chorea?
Treat underlying cause where possible
Symptomatic treatment is usually with terabenazine or dopamine receptor blocking drugs
How are tics classified?
Motor or vocal
Motor- simple (one discrete movement) or complex
Vocal- simple (singe unarticulated sound) or complex (sterotyped utterance of words or phrases)
Primary (idiopathic) or secondary
What are some features of ticks other than the usual motor or vocal ones?
Copropraxia – production of obscene gestures
Echopraxia – copying the movements of others
Coprolalia – saying of obscene words
Echolalia – copying the words of others
Palilalia – repetition of the same phrase, word or syllable
When do primary tic disorders nearly always start?
Childhood
What is adult onset of tics most likely due to?
A secondary cause
What investigations are required for tics?
Usually none Possible copper studies Blood film for acanthocythosis ASO titre Uric acid Genetic testing for HD Brain imaging
What are some examples of primary tic disorders?
Simple transient tics of childhood
Chronic tics of childhood
Gilles de la Tourette’s syndrome
Adult onset Tourettism
What neurodegenerative disorders can cause secondary tic disorders?
Huntington’s disease Wilson’s disease Neuroacanthocythosis Neuronal brain iron accumulation syndrome Rett’s syndrome Lesch-Nyhan syndrome
What developmental syndromes can cause secondary tic disorders?
Down syndrome and other Ch abnormalities
Fragile X
Autism
Non-specific mental retardation
What structural abnormalities can cause secondary tic disorders?
Post-encephalopathy
Basal ganglia lesions (usually caudate nucleus)
What infective causes can cause secondary tic disorders?
Sydenham’s chorea
PANDAS
What drugs and toxins can cause secondary tic disorders?
CO poisoning
Cocaine
Amphetamines
Anticonvulsants
What is the inheritance for Tourette’s?
AD inheritance seen in some families, no genes identified yet
What is the M:F ratio for Tourette’s?
4:1
What is the diagnostic criteria for Tourette’s?
Both multiple motor tics and one or more vocal tics must be present
The tics must occur many times a day, nearly every day, or intermittently for more than 1 year with no longer than 3 months interval of tic-freeness
Age of onset
What is the treatment for Tourette’s?
Symptomatic treatment (clonidine, tetrabenazine) for tics and also associated psychopathology (including CBT)
What is myoclonus caused by?
Brief activation of a group of muscles leading to a jerk of the affected body part. This activation can arise from the cortex, subcortical structures, spinal cord, or nerve root and plexus
What is negative myoclonus produced by?
A temporary cessation of muscle activity e.g. asterixis (liver flap)
What are some types of myoclonus?
Primary
Myoclonus with epilepsy
Progressive myoclonic epilepsy and ataxia
What are some causes of symptomatic myoclonus with encephalopathy?
Liver failure Renal failure Drug Intoxication (alcohol, lithium) Toxins (lead) Post hypoxia Progressive encephalomyelitis with rigidity
What are some causes of symptomatic myoclonus without encephalopathy plus dementia?
Alzheimer’s
Dementia with Lewy bodies
Creutzfeldt-Jakob disease
What are some causes of symptomatic myoclonus without encephalopathy plus Parkinsonism?
Corticobasal degeneration
Multiple system atrophy
Spinocerebellar ataxis
What are some causes of symptomatic myoclonus without encephalopathy that are focal/segmental related?
Spinal cord, root, plexus injury
Palatal myoclonus
What are some other causes of symptomatic myoclonus without encephalopathy?
Whipple disease
Coeliac’s disease
PNP
Drugs
What are investigations are required in myoclonus?
Electrophysiological tests to characterise myoclonus
What treatment is required in myoclonus?
Usually symptomatic
Often combination of drugs
S/Es include sedation
What is juvenile myoclonus epilepsy?
Onset in teenage years of myoclonic jerks and generalized seizures
Describe juvenile myoclonus epilepsy
Typical precipitants of the myoclonic jerks and seizures are alcohol and sleep deprivation
Symptoms tend to be worse in the mornings
EEG shows characteristic 3-5Hz polyspike and wave pattern
What is the treatment for juvenile myoclonus epilepsy?
Sodium valproate and levetiracetam are effective; carbamazepine can aggravate the epilepsy syndrome
AED treatment is usually required longterm
