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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
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

Neurology (27 decks)

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