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Flashcards in Movement Disorders Deck (59):
1

What is a tremor?`

Rhythmic sinusoidal oscillation of a body part

2

What are tics?

Involuntary stereotyped movements or vocalisations

3

What is chorea?

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

4

What is myoclonus?

Brief electric-shock like jerks

5

What is dystonia?

Abnormal posture of the affected body part

6

How are tremors classified?

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

7

What are some causes for a resting tremor?

Parkinson’s disease
Drug-induced parkinsonism
Psychogenic tremor

8

What are some causes for a postural tremor?

Essential tremor
Enhanced physiological tremor
Tremor associated with neuropathy

9

What are some causes for a kinetic tremor?

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

10

What are some causes for a head tremor?

Dystonia
Cerebellar disease

11

What are some causes for a jaw tremor?

Dystonia
Parkinson's disease

12

What are some causes for a palatal tremor?

With ataxia
Symptomatic
Essential tremor

13

What investigations should be done in patients with tremor?

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

14

What is a tremor associated with dystonia?

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

15

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

16

What are the first line treatments for dystonic tremor?

Propanolol and primidone
Deep brain stimulation for severely affected patients

17

Which brain region is affected in dystonia?

Basal ganglia

18

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

19

What does the gene DYT1 cause?

Torsion Dystonia

20

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

21

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)

22

What is good at treating writer's cramp?

Botox

23

When is surgery required in cervical dystonia?

When botox is ineffective

24

What are some AI causes of chorea?

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

25

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

26

What are some infectious causes of chorea?

HIV

27

What are some drug-based causes of chorea?

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

28

What are some paroxysmal chorea causes of chorea?

PD

29

What are some metabolic causes of chorea?

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

30

What investigations should be carried out in chorea?

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

31

What is the treatment for chorea?

Treat underlying cause where possible
Symptomatic treatment is usually with terabenazine or dopamine receptor blocking drugs

32

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

33

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

34

When do primary tic disorders nearly always start?

Childhood

35

What is adult onset of tics most likely due to?

A secondary cause

36

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

37

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

38

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

39

What developmental syndromes can cause secondary tic disorders?

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

40

What structural abnormalities can cause secondary tic disorders?

Post-encephalopathy
Basal ganglia lesions (usually caudate nucleus)

41

What infective causes can cause secondary tic disorders?

Sydenham's chorea
PANDAS

42

What drugs and toxins can cause secondary tic disorders?

CO poisoning
Cocaine
Amphetamines
Anticonvulsants

43

What is the inheritance for Tourette's?

AD inheritance seen in some families, no genes identified yet

44

What is the M:F ratio for Tourette's?

4:1

45

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

46

What is the treatment for Tourette's?

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

47

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

48

What is negative myoclonus produced by?

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

49

What are some types of myoclonus?

Primary
Myoclonus with epilepsy
Progressive myoclonic epilepsy and ataxia

50

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

51

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

Alzheimer's
Dementia with Lewy bodies
Creutzfeldt-Jakob disease

52

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

Corticobasal degeneration
Multiple system atrophy
Spinocerebellar ataxis

53

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

Spinal cord, root, plexus injury
Palatal myoclonus

54

What are some other causes of symptomatic myoclonus without encephalopathy?

Whipple disease
Coeliac's disease
PNP
Drugs

55

What are investigations are required in myoclonus?

Electrophysiological tests to characterise myoclonus

56

What treatment is required in myoclonus?

Usually symptomatic
Often combination of drugs
S/Es include sedation

57

What is juvenile myoclonus epilepsy?

Onset in teenage years of myoclonic jerks and generalized seizures

58

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

59

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