Tremor and Hyperkinetic Disorders Flashcards
Name 5 types of hyperkinetic movement disorder.
Tremor. Tics. Chorea. Myoclonus. Dystonia
Define tremor.
Rhythmic sinusoidal oscillation of a body part
Define tics.
Involuntary stereotyped movements or vocalizations
Define chorea.
Brief irregular purposeless movements which flit and flow from one body part to another
Define myoclonus.
Brief electric-shock like jerks
Define dystonia.
Abnormal posture of the affected body part
Describe tremor.
Rhythmic sinusoidal oscillation of a body part, usually due to alternate activation of agonist and antagonist muscles
Outline the 4 ways in which tremor can be classified.
Position: at rest, on posture, during movement (kinetic tremor).
Distribution: body part affected.
Frequency: measured in Hertz.
Amplitude: ‘fine’ or ‘coarse.’
What should you ALWAYS ask a patient who presents with a tremor?
When they notice the tremor most
Name the 6 main types of tremor.
- Resting
- Postural
- Kinetic
- Head
- Jaw
- Palatal
Name the 3 causes of a resting tremor.
- Parkinson’s disease.
- Drug-induced parkinsonism.
- Psychogenic tremor
Name the 3 causes of a postural tremor.
- Essential tremor.
- Enhanced physiological tremor.
- Tremor associated with neuropathy.
Name the 2 causes of a kinetic tremor.
- Cerebellar disease (demyelination, haemorrhage, degenerative, toxic).
- Wilson’s disease.
Name the 2 causes of a head tremor.
- Dystonia.
* Cerebellar disease.
Name the 2 causes of a jaw tremor.
- Dystonia.
* Parkinson’s disease.
Outline the key things which must be discussed when taking a history from a patient with a tremor.
- Age at onset
- Body part(s) affected
- Any precipitating factors at onset?
- Drug / toxin exposure
- Exacerbating and relieving factors
- Associated neurological symptoms
- Associated systemic symptoms
- Family history
What are the important features which should be included in the examination of a patient with a tremor?
- Examine at rest, on posture and during movement.
- Ask patient to write something and copy a spiral.
- Complete physical and neurological examination.
What tests looks at cerebellar function?
Get pt to touch their nose, then touch your finger
Tremor due to stress is usually symmetrical
TRUE
An asymmetrical tremor suggests a problem with the brain
TRUE
What investigations should be done in a patient who presents with a tremor?
- Guided by presentation.
- TFT’s.
- Copper and coeruloplasmin in young patients, <45.
Describe the treatment for a patient with a tremor.
Usually symptomatic, if at all.
in selected cases, deep brain stimulation
What is a tremor associated with dystonia?
A tremor in a body part that is not dynamic, but there is dystonia elsewhere
A dystonic tremor can occur at…….
Rest, in sustained postures and in voluntary movement
Focal and task specific
Describe an essential tremor.
- Abnormal tremor in bilateral upper limbs, with absence of neurological signs elsewhere
Essential tremors are _________
BILATERAL
What is the duration of an essential tremor like?
Long
An essential tremor is not what?
A diagnosis, but a syndrome of many different types of tremor – dystonia predominates
What medications can be used to treat a dystonic tremor/
1st Line: Propanolol + Primidone
Also: atenolol, gabapentin, sotalol, alpraxolam, topiramate, clonazepam, nadolol, nimodipine
What type of drug in Primidone?
An anticonvulsant
What can be used in the treatment of a severe dystonic tremor?
Deep brain stimulation
What is dystonia?
A disorder of movement, specifically of involuntary sustained muscle contraction, which leads to twisting and repetitive movement or abnormal posturing
Give 2 examples of dystonia.
- Writers cramp
* Torticollis
There are 3 main physiological abnormalities that have been found in patients with dystonia. What are these?
- Loss or reduction in reciprocal inhibition
- Alterations in brain plasticity
- Alterations in sensory function
What is the main treatment for dystonia?
- Botox
2. Deep brain stimulation
What is the definition of chorea?
Brief irregular purposeless movements that flit and flow from one body part to another. Patients appear constantly restless or fidgety. Often generalized, but it may be confined to one region (e.g. face)
In some cases, it can be difficult to distinguish between chorea and . . .
Myoclonus (short and not flying around) or tics (suppressible
Chorea is a _______
Symptom
What is the main cause of inherited chorea?
Huntington’s disease
What is the major autoimmune cause of chorea?
SLE
Suggest a cause of paroxysmal chorea.
Parkinson’s disease
Outline the important features to cover when taking a history from a patient with chorea.
- Age of onset
- Acute or gradual
- Drug exposure
- Family history
- Psychiatric/behavioural disturbances
- Systemic symptoms
What should be covered when examining a patient with chorea?
- Examine at rest, with arms out and while walking
- Note distribution of chorea
- Other neurological signs
- Systemic signs
What investigations should be carried out in a patient who presents with chorea?
- Brain imaging
- Blood tests
- Antibodies
What is given for symptomatic treatment of chorea?
Terabenazine or dopamine receptor blocking drugs
What is Huntington’s disease?
An autosomal dominant inherited neurodegenerative disorder characterized by progressive behavioural disturbance, dementia, and movement disorder, usually chorea
What is the inheritance pattern of Huntington’s disease?
Autosomal dominant
Who gets Huntington’s disease?
- 4-8 per 100,000.
- Affects males and females equally.
- Some communities have high prevalence rates (Moray Forth in Scotland + Venezuela).
- Some communities have low rates (Japanese + African Americans).
When does Huntington’s usually onset?
4th decade, although it is variable
Describe the onset of Huntington’s disease.
Psychiatric, cognitive or behavioural disturbances, neurological signs or mixed.
- EYE MOVEMENT ABNORMALITIES
Describe the genetics of Huntington’s disease.
CAG triplet-repeat expansion on chromosome 4
Tics can be either motor or vocal
TRUE
Tics can be either motor or complex
TRUE
When do primary tic disorders start and what is the cause?
Childhood
Idiopathic
When do secondary tic disorders start?
Adulthood
What should you always ask when taking a history from a patient with tics?
Associated psychopathology e.g OCD, ADHD, anxiety, self harm
What should always be noted on examination of a patient with tics?
Are the tics suppressible?
What is the inheritance of Tourettes like?
Autosomal dominant inheritance is seen in some families, but no genes have been identified yet
What is the diagnostic criteria for Tourettes syndrome?
- Multiple motor tics + one or more vocal tics
- Tics must occur many times a day
- Age of onset <18
- Exclusion of obvious secondary causes
What is the treatment for Tourettes syndrome?
Symptomatic – clonidine + tetrabenazine for tics.
+
Associated psychopathology (inc CBT).
What is the definition of myoclonus?
Brief electric shock-like jerks.
- Hiccups or hypnic jerks (when falling asleep) are common and normal forms of myoclonus
What is myoclonus caused by?
Brief activation of a group of muscles, leading to a jerk of the affected body part.
What can muscle activation in myoclonus be caused by?
The cortex, subcortical structures, spinal cord or nerve root and plexus
What is negative myoclonus caused by?
A temporary cessation of muscle activity.
Eg: asterixis (liver flap) in pts with liver failure
When is the onset of Juvenile Myoclonus Epilepsy?
In teenage years
What are the 2 features of JME?
Myoclonic jerks + generalized seizures.
What are the typical precipitants of myoclonic jerks and seizures?
Alcohol and sleep deprivation
When do symptoms of JME tend to be worse?
In the mornings
What will an EEG of someone with JME show?
Characteristic 3-5Hz polyspike and wave pattern
Name 2 drugs which are affective in the treatment of JME?
Sodium valproate + Levetiracetam
What drug can aggravate JME and thus should never be given?
Carbamazepine
Longterm, what is the treatment for JME?
AED