Movement Disorders Flashcards

1
Q

What is a tremor?`

A

Rhythmic sinusoidal oscillation of a body part

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2
Q

What are tics?

A

Involuntary stereotyped movements or vocalisations

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3
Q

What is chorea?

A

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

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4
Q

What is myoclonus?

A

Brief electric-shock like jerks

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5
Q

What is dystonia?

A

Abnormal posture of the affected body part

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6
Q

How are tremors classified?

A

Position(rest, posture, movement), distribution, frequency, amplitude

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7
Q

What are some causes for a resting tremor?

A

Parkinson’s disease
Drug-induced parkinsonism
Psychogenic tremor

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8
Q

What are some causes for a postural tremor?

A

Essential tremor
Enhanced physiological tremor
Tremor associated with neuropathy

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9
Q

What are some causes for a kinetic tremor?

A
Cerebellar disease (demyelination, haemorrhage, degenerative, toxic)
Wilson's disease
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10
Q

What are some causes for a head tremor?

A

Dystonia

Cerebellar disease

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11
Q

What are some causes for a jaw tremor?

A

Dystonia

Parkinson’s disease

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12
Q

What are some causes for a palatal tremor?

A

With ataxia
Symptomatic
Essential tremor

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13
Q

What investigations should be done in patients with tremor?

A

Possibly TFTs and in young patients (<45) copper and coeruloplasmin

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14
Q

What is a tremor associated with dystonia?

A

Tremor in a body part that is not dystonic but there is dystonia elsewhere

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15
Q

What is essential tremor?

A
Abnormal tremor in bilateral upper limbs with absence of neuro signs elsewhere.
Long duration
No rest tremor
\+ve FHx
Alcohol responsive
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16
Q

What are the first line treatments for dystonic tremor?

A

Propanolol and primidone

Deep brain stimulation for severely affected patients

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17
Q

Which brain region is affected in dystonia?

A

Basal ganglia

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18
Q

What 3 main physiological abnormalities have been found in patients with dystonia?

A

Loss of reduction in reciprocal inhibition
Alterations in brain plasticity
Alterations in sensory function

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19
Q

What does the gene DYT1 cause?

A

Torsion Dystonia

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20
Q

Describe torsion dystonia

A

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

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21
Q

What is the best option for cervical dystonia?

A

Botox A or B(both same effect, low dose has fewer side effects and works as well as high)

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22
Q

What is good at treating writer’s cramp?

A

Botox

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23
Q

When is surgery required in cervical dystonia?

A

When botox is ineffective

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24
Q

What are some AI causes of chorea?

A
Anti-phospholipid syndrome
Bechet syndrome
Coeliac disease
Sydenham’s chorea
Hashimotos’ thyroiditis
25
Q

What are some inherited/degenerative causes of chorea?

A
HD/HD-like syndromes
Wilson's disease
Neuroacanthocythosis
Benign hereditary chorea
Ataxia telangiectasia
Spinocerebellar ataxia Typ 17
26
Q

What are some infectious causes of chorea?

A

HIV

27
Q

What are some drug-based causes of chorea?

A

Dopamine-receptor blocking drugs, levodopa, stimulants, pill, anticonvulsants

28
Q

What are some paroxysmal chorea causes of chorea?

A

PD

29
Q

What are some metabolic causes of chorea?

A

Chorea gravidarum, glucose, thyroid, parathyroid, sodium, magnesium metabolism

30
Q

What investigations should be carried out in chorea?

A
Possibly brain imaging
Bloods for acanthocytes
ds-DNA
antiphospholipid autoantibodies
Copper
Genetic testing for HD
31
Q

What is the treatment for chorea?

A

Treat underlying cause where possible

Symptomatic treatment is usually with terabenazine or dopamine receptor blocking drugs

32
Q

How are tics classified?

A

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

33
Q

What are some features of ticks other than the usual motor or vocal ones?

A

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

34
Q

When do primary tic disorders nearly always start?

A

Childhood

35
Q

What is adult onset of tics most likely due to?

A

A secondary cause

36
Q

What investigations are required for tics?

A
Usually none
Possible copper studies
Blood film for acanthocythosis
ASO titre
Uric acid
Genetic testing for HD
Brain imaging
37
Q

What are some examples of primary tic disorders?

A

Simple transient tics of childhood
Chronic tics of childhood
Gilles de la Tourette’s syndrome
Adult onset Tourettism

38
Q

What neurodegenerative disorders can cause secondary tic disorders?

A
Huntington’s disease
Wilson’s disease
Neuroacanthocythosis
Neuronal brain iron accumulation syndrome
Rett’s syndrome
Lesch-Nyhan syndrome
39
Q

What developmental syndromes can cause secondary tic disorders?

A

Down syndrome and other Ch abnormalities
Fragile X
Autism
Non-specific mental retardation

40
Q

What structural abnormalities can cause secondary tic disorders?

A

Post-encephalopathy

Basal ganglia lesions (usually caudate nucleus)

41
Q

What infective causes can cause secondary tic disorders?

A

Sydenham’s chorea

PANDAS

42
Q

What drugs and toxins can cause secondary tic disorders?

A

CO poisoning
Cocaine
Amphetamines
Anticonvulsants

43
Q

What is the inheritance for Tourette’s?

A

AD inheritance seen in some families, no genes identified yet

44
Q

What is the M:F ratio for Tourette’s?

A

4:1

45
Q

What is the diagnostic criteria for Tourette’s?

A

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

46
Q

What is the treatment for Tourette’s?

A

Symptomatic treatment (clonidine, tetrabenazine) for tics and also associated psychopathology (including CBT)

47
Q

What is myoclonus caused by?

A

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

48
Q

What is negative myoclonus produced by?

A

A temporary cessation of muscle activity e.g. asterixis (liver flap)

49
Q

What are some types of myoclonus?

A

Primary
Myoclonus with epilepsy
Progressive myoclonic epilepsy and ataxia

50
Q

What are some causes of symptomatic myoclonus with encephalopathy?

A
Liver failure
Renal failure
Drug Intoxication (alcohol, lithium)
Toxins (lead)
Post hypoxia
Progressive encephalomyelitis with rigidity
51
Q

What are some causes of symptomatic myoclonus without encephalopathy plus dementia?

A

Alzheimer’s
Dementia with Lewy bodies
Creutzfeldt-Jakob disease

52
Q

What are some causes of symptomatic myoclonus without encephalopathy plus Parkinsonism?

A

Corticobasal degeneration
Multiple system atrophy
Spinocerebellar ataxis

53
Q

What are some causes of symptomatic myoclonus without encephalopathy that are focal/segmental related?

A

Spinal cord, root, plexus injury

Palatal myoclonus

54
Q

What are some other causes of symptomatic myoclonus without encephalopathy?

A

Whipple disease
Coeliac’s disease
PNP
Drugs

55
Q

What are investigations are required in myoclonus?

A

Electrophysiological tests to characterise myoclonus

56
Q

What treatment is required in myoclonus?

A

Usually symptomatic
Often combination of drugs
S/Es include sedation

57
Q

What is juvenile myoclonus epilepsy?

A

Onset in teenage years of myoclonic jerks and generalized seizures

58
Q

Describe juvenile myoclonus epilepsy

A

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

59
Q

What is the treatment for juvenile myoclonus epilepsy?

A

Sodium valproate and levetiracetam are effective; carbamazepine can aggravate the epilepsy syndrome
AED treatment is usually required longterm