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Neurology > Hyperkinetic movement disorders > Flashcards

Hyperkinetic movement disorders Flashcards

1
Q

Name some examples of hyperkinetic and hypokinetic movement disorders respectively

A

Hyperkinetic - myoclonus, tremor, tics, chorea, dystonia

Hypokinetic - parkinsonism/akinetic rigid syndrome

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2
Q

What is tremor?

A

Rhythmic sinusoidal oscillation of a body part. Usually due to alternate activation of agonist and antagonist muscles

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3
Q

What are tics?

A

Involuntary stereotyped movements or vocalisations. Suppressible by the patient for a short period of time. Typically the patient experiences a growing feeling of anxiety and discomfort during tic suppression and when allowed to relax will respond with a flurry of tics

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4
Q

What is chorea?

A

Brief irregular purposeless movements which flit and flow from one body part to another. Patient appears constantly restless. Often generalised but it may be confined to one region

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5
Q

What is myoclonus?

A

Brief electric shock like jerks. Hiccups or hypnic jerks are common

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6
Q

What is dystonia?

A

Involuntary muscle spasm which leads to sustained abnormal posture of the affected body part.
Typically the abnormal posture is not fixed and slow writhing movements can occur where the dominant muscle activity switches back and forth from agonist to antagonist

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7
Q

What are some types of tremor?

A

Resting tremor, postural tremor, kinetic tremor, head tremor, jaw tremor, palatal tremor

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8
Q

Name causes of resting tremor

A

Resting tremor - Parkinson’s disease, drug-induced parkinsonism, psychogenic tremor

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9
Q

Name causes of postural tremor

A

Postural tremor - essential tremor, enhanced physiological tremor, tremor associated with neuropathy

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10
Q

Name causes of kinetic tremor

A

Kinetic tremor - cerebellar disease (demyelination, haemorrhage), Wilson’s disease

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11
Q

Name causes of head tremor

A

Dystonia, cerebellar disease

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12
Q

Name causes of jaw tremor

A

Dystonia, Parkinson’s disease

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13
Q

Name causes of palatal tremor

A

With ataxia, symptomatic, essential tremor

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14
Q

How is a patient with tremor investigated?

A

History - age at onset, body part affected, precipitating factors, drug/toxin exposure, exacerbating and alleviating factors, associated systemic and neurological symptoms, family history
Examination - at rest, on posture and during movement., ask patient to write something and copy a spiral., complete physical and neurological examination
Investigations - guided by presentation, possibly thyroid function test and in young patients copper and coeruloplasmin

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15
Q

How is a patient with tremor treated?

A

Usually symptomatic if at all. Deep brain stimulation in selected cases

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16
Q

What are the core criteria for an essential tremor?

A

Bilateral action tremor, absence of other neurological signs, may have isolated head tremor with no signs of dystonia

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17
Q

How is essential tremor treated?

A

Propanolol or primidone first line. Other treatments include; atenolol, gabapentin, sotalol, topiramate

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18
Q

What is important to ask when taking a history from a patient with tics?

A

Age at onset, what are the movements/vocalisations?, precipitating factors, family history, associated psychopathology e.g. OCD, neurological history or symptoms, extent of disability associated with tics

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19
Q

What is important when examining someone with tics?

A

Are the tics suppressible? Motor, vocal or both? Simple, complex or both? Other neurological signs

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20
Q

How are tics investigated?

A

Guided by presentation, usually none. Possibly copper studies, blood film for acanthocytosis, ASO titre, uric acid, genetic testing for HD, brain imaging

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21
Q

What are some causes of primary tic disorder?

A

Simple transient tics of childhood, chronic tics of childhood, Gilles de la Tourette’s syndrome, adult onset tourettism

22
Q

What are some causes of secondary tic disorder?

A

HD, Wilson’s disease, neuroacanthocytosis, down syndrome, fragile X syndrome, autism, post-encephalopathy, non-specific mental retardation, basal ganglia lesions (usually caudate nucleus), carbon monoxide poisoning, cocaine, amphetamines

23
Q

How is Gilles de la Tourette syndrome (TS) inherited and who does it typically affect?

A

0.5-1% of the population
Males > females (4:1)
Autosomal dominant inheritance

24
Q

What are the diagnostic criteria for TS?

A

Both multiple motor tics and one or more vocal tics must be present.
Tics must occur many times a day, nearly every day, or intermittently for >1year with no more than 3 months tic-freeness.
Age of onset<18
Exclusion of obvious secondary cause

25
Q

How is TS treated?

A

Symptomatically - clonidine, tetrabenazine for tics. CBT for associated psychopathology

26
Q

What are some broad categories of causes of chorea?

A

Inherited/degenerative disorders, autoimmune disorders, infectious disorders (HIV), drugs, paroxysmal chorea (PD) and metabolic

27
Q

What are some examples of inherited/degenerative causes of chorea?

A

HD, Wilson’s disease, neuroacanthocytosis, benign hereditary chorea

28
Q

What are some examples of autoimmune causes of chorea?

A

SLE, anti-phospholipid syndrome, Bechet syndrome, coeliac disease, Hashimoto’s thyroiditis

29
Q

What drugs can cause chorea?

A

Dopamine receptor blocking drugs e.g. haloperidol, levodopa, stimulants, OCP

30
Q

What are some examples of metabolic causes of chorea?

A

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

31
Q

What is important when examining a patient with chorea?

A

Examine at rest, with arms outstretched and when walking. Note distribution of chorea.
Systemic signs - rash, arthopathy, Kayser-Fleischer rings, signs of thyroid disease

32
Q

How is chorea treated?

A

Treat underlying cause where possible. Symptomatically with terabenazine or dopamine receptor blocking drugs e.g. haloperidol

33
Q

What is Huntington’s disease?

A

Autosomal dominant inherited neurodegenerative disorder characterised by progressive behavioural disturbance, dementia and movement disorder, usually chorea

34
Q

What are the clinical features of HD?

A

Age of onset variable, but usually in fourth decade.
Onset with psychiatric, cognitive or behavioural disturbances, neurological signs or mixed.
Eye movement abnormalities

35
Q

What is the underlying genetic defect in HD?

A

CAG-triplet expansion disorder affecting the Huntington gene on chromosome 4.
Number of CAG repeats determines whether disease will occur (>/=40) or not (=35).
Uncertainty between 36-39

36
Q

What mechanism causes myoclonus?

A

Caused by brief activation of a muscle group leading to a jerk of the affected body part. This activation can arise from the cortex, sub-cortical structures, spinal cord or nerve root plexus

37
Q

What is negative myoclonus?

A

Produced by a temporary cessation of muscle activity. For example, asterixis (liver flap) in patients with liver failure

38
Q

What are some causes of primary myoclonus?

A

Hypnic jerks, benign infantile myoclonus with feeding/sleep, essential myoclonus, myoclonus dystonia, cortical tremor

39
Q

What are some causes of myoclonus with epilepsy?

A

Benign myoclonic epilepsy of infancy, juvenile myoclonic epilepsy, West’s syndrome, early infantile myoclonic encephalopathy

40
Q

What are some causes of progressive myoclonic epilepsy and ataxis?

A

Lafora body disease, sialidosis,

41
Q

What can cause symptomatic myoclonus?

A

With encephalopathy - liver failure, renal failure, alcohol, lithium, lead, post hypoxia
Without encephalopathy:
Plus dementia - AD, Creutzfeldt-Jakob disease, dementia with Lewy bodies
Plus Parkinsonism - multiple system atrophy, corticobasal degeneration
Focal/segmental - spinal cord, root, plexus injury, palatal myoclonus

42
Q

How does juvenile myoclonic epilepsy present?

A

Onset in teenage years of myoclonic jerks and generalised seizures.
Typical precipitants of the myoclonic jerks and seizures are alcohol and sleep deprivation.
Symptoms tend to be worse in the mornings.
EEG shows characteristic polyspike and wave pattern

43
Q

How is juvenile myoclonic epilepsy treated?

A

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

44
Q

How is dystonia classified by age?

A

Early onset dystonia <26 years

Late-onset dystonia >26 years

45
Q

What is primary dystonia?

A

Dystonia +/- tremor are the only symptom and sign

46
Q

What is secondary dystonia?

A

Clear secondary cause is present e.g. brain injury, drug exposure

47
Q

How do primary young onset dystonias present?

A

Onset usually late childhood/early teens.
Limb onset is typical with spread of symptoms over months to 2 years.
50-60% of patients will have a mutation in the DYT1 gene on chromosome 9q

48
Q

How is young onset dystonia managed?

A

A trial of levodopa is appropriate in all patients with young onset dystonia

49
Q

Name some examples of primary adult onset FOCAL dystonias

A 
Cervical dystonia
Blepharospasm
Oromandibular dystonia
Spasmodic dysphonia 
Limb dystonia
Axial dystonia
50
Q

What treatment plays an important role in focal dystonias?

A

Botulinum toxin

Neurology (18 decks)

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