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Neurology- Clinical > Movement Disorders > Flashcards

Movement Disorders Flashcards

1
Q

What is the difference between hypo and hyperkinetic movement disorders?

A

Hypo = too little movement, hyper = too much movement

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2
Q

What are some examples of hypokinetic movement disorders?

A

Parkinson’s disease and akinetic rigid syndrome

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3
Q

What are some examples of symptoms of hyperkinetic movement disorders?

A

Tics, tremore, myoclonus, dystonia, chorea

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4
Q

Describe the features of primary hyperkinetic movement disorders?

A

Usually no underlying structural brain lesion, non-progressive and often inherited

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5
Q

What is the defining feature of a secondary hyperkinetic movement disorder?

A

Presence of a precipitating factor i.e. brain injury, infection, toxin

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6
Q

Describe the features of heredo-degenerative hyperkinetic movement disorders?

A

Typically late onset and progressive

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7
Q

Describe the features of psychogenic movement disorders?

A

Unusual physical features with psychological disturbance

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8
Q

What is another term used to describe hyperkinetic movement disorders?

A

Dyskinesias

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9
Q

What is the physiological cause for a tremor?

A

Alternate activation of agonist and antagonist muscles

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10
Q

What are the main causes of a resting tremor?

A

Parkinson’s disease/Parkinsonism, psychogenic

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11
Q

What is the main cause of a postural tremor?

A

Essential tremor

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12
Q

What are the main causes of a kinetic tremor?

A

Cerebellar disease and Wilson’s disease

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13
Q

How should patients presenting with a tremor be examined?

A

At rest, on posture and while moving. They should also be asked to write something and to draw a spiral, as well as a complete neurological exam

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14
Q

What are some investigations you should do on a young person presenting with a tremor or dystonia?

A

TFTs and copper/ceruloplasmin levels

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15
Q

Describe an essential tremor?

A

A symmetrical postural or kinetic tremor with high frequency

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16
Q

What are the core criteria of essential tremor?

A

Bilateral action tremor of the hands and forearms / absence of other neurological signs / may have isolated head tremor with no dystonia

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17
Q

What are some secondary criteria of essential tremor?

A

Long duration (> 3 years), positive Fx, beneficial response to alcohol

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18
Q

Essential tremor is commonly inherited - what pattern does it follow? What is its mean age of onset?

A

Autosomal dominant / 15

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19
Q

What are the first line treatments for essential tremor?

A

Propranolol and primidone

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20
Q

What is a last line treatment for patients severely affected by essential tremor?

A

Deep brain stimulation of the ventralis intermedius nucleus

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21
Q

What are tics?

A

Involuntary, stereotyped movements or vocalisations

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22
Q

Are tics suppressible?

A

Yes, but this creates internal tension within the patient

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23
Q

Describe how a patient with a tic disorder may feel whilst trying to suppress a tic?

A

A growing feeling of anxiety and discomfort

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24
Q

When do primary tic disorders almost always start?

A

In childhood

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25
Q

What investigation is usually done for a tic disorder?

A

Usually none, but should be guided by the history

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26
Q

What are some things that tic disorders can occur secondary to?

A

Neurodegenerative disorders, developmental syndromes, structural abnormalities, infection, drugs/toxins

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27
Q

Patients with Tourette’s syndrome often also have what else?

A

Obsessive compulsive behaviours and decreased impulse control

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28
Q

Which gender is more commonly affected with Tourette’s syndrome?

A

Males (4: 1)

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29
Q

Though no candidate gene has been identified, Tourette’s syndrome is often inherited. What is the pattern of this?

A

Autosomal dominant

30
Q

With regards to the tics themselves, what is the criteria for Tourette’s syndrome?

A

Multiple motor tics and one or more vocal tics must be present

31
Q

How often must the tics occur to be diagnosed with Tourette’s syndrome?

A

Many times a day, nearly every day OR intermittently for more than 1 year with no longer than 3 months tic free

32
Q

To be diagnosed with Tourette’s syndrome, what is important about the age of onset?

A

Has to be aged < 18 at the time of onset

33
Q

What are some treatment options for Tourette’s syndrome?

A

Clonidine/tetrabenazine and CBT

34
Q

What is chorea?

A

Brief, irregular, purposeless movements that flit and flow from one body part to another

35
Q

How will patients with chorea appear?

A

Constantly restless and fidgitey

36
Q

What are some groups of investigations that may be done for patients presenting with chorea?

A

Brain imaging, autoantibodies, copper studies, genetic testing

37
Q

What is the ideal treatment of chorea? What can be used as symptomatic treatment?

A

Treat the underlying cause / tetrabenazine, or dopamine receptor blocking drugs

38
Q

How is Huntington’s disease inherited?

A

Autosomal dominant

39
Q

What are the two characteristic features of Huntington’s disease?

A

Behavioural disturbance (dementia) and movement disorder (usually chorea)

40
Q

When is the typical onset of Huntington’s disease?

A

In the 4th decade

41
Q

What is the genetic cause for Huntington’s disease?

A

CAG trinucleotide repeats affecting the Huntingtin gene on chromosome 4

42
Q

The number of CAG repeats has what effect on Huntington’s disease prognosis?

A

The more repeats, the earlier onset and greater severity

43
Q

How many CAG repeats are needed to diagnose Huntington’s?

A

36

44
Q

What test is used to confirm a diagnosis of Huntington’s? What investigation can be done while this is awaited?

A

Genetic testing / MRI brain

45
Q

What is myoclonus?

A

Brief, electric shock like jerks

46
Q

What are some examples of common and normal forms of myoclonus?

A

Hiccups, and hypnic jerks (when falling asleep)

47
Q

What causes myoclonus?

A

Brief activation of a group of muscles leading to a jerk of the affected body part

48
Q

How is negative myoclonus produced? Give an example?

A

Temporary cessation of muscle activity / liver flap

49
Q

Investigation of myoclonus is guided by presentation but what can be used to characterise it?

A

Electrophysiological tests

50
Q

Treatment of myoclonus is often symptomatic and involves what? What is the side effect of this?

A

Using multiple drugs / sedation

51
Q

When does juvenile myoclonic epilepsy tend to begin? What does it begin with?

A

Teenage years / myoclonic jerks and seizures

52
Q

What are some typical precipitants of myoclonic jerks and seizures in juvenile myoclonic epilepsy?

A

Sleep deprivation and alcohol

53
Q

When are symptoms of juvenile myoclonic epilepsy typically worst?

A

In the morning

54
Q

What are some effective treatments for juvenile myoclonic epilepsy? What treatment should not be used as it can exacerbate the condition?

A

Sodium valproate or leviteracitam / carbamazepine

55
Q

What is dystonia?

A

Involuntary muscle spasms which lead to sustained abnormal postures of the affected body part

56
Q

In dystonia, typically the abnormal posture is not fixed. What does this lead to?

A

Slow, writhing movements (athetosis)

57
Q

Dystonia can often be associated with what affecting the same body part?

A

Tremor

58
Q

Dystonia is often improved with what? And made worse by what?

A

Sensory tricks / particular positions or tasks

59
Q

Dystonia is generally classified by what? Describe the categories?

A

Age of onset, early onset aged < 26 and late onset aged > 26

60
Q

What is primary dystonia?

A

Dystonia +/- tremor are the only features

61
Q

What are dystonia plus syndromes?

A

Other features apart from only dystonia and tremor may occur, but there is no secondary cause or neurodegeneration

62
Q

What is paroxysmal dystonia?

A

Episodic attacks, with no clinical signs between attacks

63
Q

Where in the body is affected by each of these types of dystonia: a) focal? b) segmental? c) hemibody? d) multifocal? e) generalised?

A

a) single body region b) continuous body region c) ipsilateral arm and leg d) 2 non-contiguous body parts e) whole body

64
Q

What can play an important role in causing focal dystonia?

A

Botox

65
Q

What is the treatment for dystonia?

A

Symptomatic, if at all

66
Q

When do primary young onset dystonias typically occur?

A

Late childhood/early teends

67
Q

Describe the typical onset of a primary young onset dystonia?

A

Limb onset with the spread of symptoms over months to two years

68
Q

50-60% of patients with primary young onset dystonias will have a mutation where?

A

DYT1 gene on chromosome 9

69
Q

What is the inheritance pattern of primary young onset dystonias?

A

Autosomal dominant

70
Q

What are some differentials of primary young onset dystonias?

A

Dopa-responsive dystonia (always do a Levodopa trial) or a young onset of other degenerative disease

Neurology- Clinical (31 decks)

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