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Neurology Clinical > Hyperkinetic Movement Disorders > Flashcards

Hyperkinetic Movement Disorders Flashcards

1
Q

Define Hypokinetic and Hyperkinetic

A

Hypokinetic : excess movement

Hyperkinetic: lack movement

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2
Q

List Akinetic Rigid Syndromes

A
  • Parkinson’s Disease
  • Lewy body dementia
  • Atypical Parkinsonism : Multiple System Atrophy (MSA) , Progressive Supranuclear Palsy (PSP), Corticobasal Degeneration (CBD)
  • Drug-induced Parkinsonism
  • Vascular Parkinsonism
  • Chronic traumatic encephalopathy
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3
Q

What is Bradykinesia

A

Slowness of movement

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4
Q

Define Tardive

A

Late occurring

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5
Q

Define Dyskenesia

A

Difficulty or abnormality in movement.

Involuntary movement , fragmentary/incomplete

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6
Q

Define Akinetic

A

No movement

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7
Q

Signs of Hyperkinetic disorders ( Dyskenesia)

A
  • Tremor
  • Dystonia
  • Chorea
  • Hemiballismus
  • Tics
  • Myoclonus
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8
Q

What is a Resting tremor

A

Occurs in a body part that is not activated and completely supported against gravity

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9
Q

What is an Action tremor

A

Occurs with voluntary muscle contraction
• Postural
• Isometric
• Kinetic

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10
Q

Causes of tremor

A
  • Essential tremor
  • Genetic tremor disorders : Wilson’s
  • Degenerative disorders : PD
  • Metabolic diseases : Hypothyroid
  • Peripheral neuropathies
  • Drug-induced : salbutamol, lithium
  • Toxins : heavy metal
  • Functional
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11
Q

Explain essential tremor

A

Most common tremor syndrome in adults
• Bilateral UL action tremor -Hands, arms, head (titubation), trunk, speech
• Frequency 4-7Hz ( fast )
• diagnosed when No other neuro signs (dystonia, ataxia, parkinsonism)
• Slowly progressive, rarely very disabling
• Improves with alcohol, B-blockers, DBS
• Handwriting is shaky but not small
• Patients often have a family history

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12
Q

Describe Dystonia , give examples and how it’s classified

A

Involuntary sustained contractions of opposing muscles groups causing twisting movements or abnormal postures.
ex: head tilted to side / protruded forward , inverted foot
Classified by area affected, age of onset or secondary to degenerative disease

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13
Q

Causes of Dystonia

A

Genetic , birth trauma or secondary to disease

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14
Q

How does a patient with Dystonia present

A
  • Abnormal posture ( head twisting / spasming )
  • cramps
  • pain
  • may be associated with tremor
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15
Q

What is Laryngeal Dystonia

A

Spasmodic dysphonia
Affects muscles of larynx
- Adductor vocal cords slam together and stiffen , making it difficult for vibration = voice production , speech sounds strangled , words cut off
- Abductor : cords fold open and don’t vibrate = voice is weak and breathy

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16
Q

How is Laryngeal Dystonia diagnosed

A

ENT via Larynoscopy

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17
Q

Treatment for Laryngeal Dystonia

A

botulinum toxin

injections

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18
Q

What is Generalized dystonia and what is the cause

A 
Primary dystonia " torsion " all over the body 
- Usually onset <25 years of 
age
- Typically lower limb onset
- 50% have DYT1 mutation 
(AD) ( dominant inheritance)
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19
Q

What is Chorea

A

Rapid jerky movements that flit from one part of body to another (‘dancing hands and feet’)

  • generalized
  • short-lasting
  • distal : affects legs and hands more
  • asymmetrical
  • can’t stick out tongue for 10 seconds
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20
Q

Causes of Chorea

A
  • Huntington’s disease
  • Rheumatic fever (Sydenham’s chorea)
  • Drugs (OCP, L-dopa)
  • Pregnancy (chorea gravidarum)
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21
Q

Treatment of Chorea

A

dopamine receptor blockers (tetrabenazine

22
Q

Describe Huntington’s Disease

A

Hyperkinetic (chorea), psychiatric and cognitive neurodegenerative disorder.

  • Autosomal dominant caused by triple repeat disorder
  • loss of nerve cells in putamen and caudate nucleus = reduction in GABA
  • prognosis: 10-20 y from onset
23
Q

HD presentation

A
  • jerky uncontrollable movements (not just a tremor)
  • unsteady gait,
  • dysarthria
  • dysphagia
  • anxiety, depression, insomnia
  • turn head to saccade ( look at something ) = abnormal eye movements
24
Q

HD treatment

A
  • Tetrabenzine can reduce the chorea
  • antipsychotics often
    required
  • gene silencing ( still research )
25
Q

What is Hemiballismus

A

Severe proximal Chorea

- Violent flinging movements of one side of the body

26
Q

Cause of Hemiballismus

A

infarction/SOL/ haemorrhage in the contralateral (opposite) subthalamic nucleus

27
Q

Treatment of Hemiballismus

A

not always required, settles spontaneously, may need dopamine blockers or surgery in extreme cases

28
Q

Describe Tics

A

Sudden, repetitive, non-rhythmic, same movement over and over again or vocalization

  • preceded by urge : ex- need to yawn , sneeze , or scratch
    • not involuntary , suppressible but irresistible
  • build up of tension which patient consciously chooses to release , relieving urge
  • increases with stress, excitement
  • decrease when concentrating on absorbing activity
29
Q

Explain Types of tics

A 
Motor 
• simple: blinking, pouting, 
facial grimacing
• complex: kicking, jumping, 
hopping
Vocal 
• Simple (phonic): grunting, 
sniffing, coughing
• Complex: echolalia (repeating 
words spoken by someone 
else), coprolalia (spontaneous 
utterance of obscene words)
30
Q

Causes of Tics

A 
Primary:-
• Simple transient tics of 
childhood - goes away on it's on in 4 y old boys 
• Gilles de la Tourette’s 
syndrome
Secondary 
• Neurodegenerative disease 
(e.g. HD, Wilson’s disease)
• Associated with LDs (e.g. 
Down syndrome, Autism, 
Rett’s syndrome)
• Infection (e.g. Sydenham’s 
chorea, PANDAS)
31
Q

What is Tourette’s Syndrome

A

Severe expression of tic disorder
•Onset <18 years of age (usually 5-7 years old), M>F
•Multiple motor & at least one vocal tic, occuring many times
a day, nearly every day
- 10% have coprolalia
- Prognosis : improves with maturity, normal life expectancy and intelligence

32
Q

Treatment of Tourette’s

A

Psychobehavioural therapy, reassurance, rarely

neuroleptic medication needed

33
Q

Describe Myoclonus

A

Brief involuntary twitch (lighting fast jerk) of a muscle or group of muscles

  • not a tremor , no rhythm , irregular
  • stimulus sensitivity
34
Q

Causes of Myoclonus

A
  • Neuro & metabolic
    disorders e.g. Epilepsy, CJD, ureamia, liver failure
  • Often drug-induced
  • may not be a disease but just a normal jerk
35
Q

Positive myoclonus vs negative

A

Positive : contractions

Negative : relaxations

36
Q

Drugs most commonly to induce Dyskinesia

A

Salbutamol , Lithium , Valproate , Levodopa

37
Q

Explain presentation of Tardive dyskinesia , what is the cause

A
  • Mouthing & smacking of lips
  • Grimaces with contortion of face & neck
  • Occurs after several years of treatment with Neuroleptics
38
Q

Explain presentation of Acute Dystonic reactions, what is the cause and treatment

A

Spasmodic torticollis, trismus ( locked jaw ) , oculogyric crises (episodes of sustained upward gaze)
• Can occur after just a single dose, appearing rapidly
• Treated with IV Anticholinergic (e.g. Benztropine, Procyclidine)

39
Q

Describe Athetosis

A
  • Involuntary irregular, slow writing movements of the extremities, = exaggerated postures of the limbs
  • Pathology – injury to striatum, globus pallidus
  • Cause – birth trauma (cerebral palsy)
40
Q

Describe Akathisia

A

unpleasant sensation of restlessness, manifests as inability to sit still or
remain motionless
- feeling not involuntary movement

41
Q

Describe Wilson’s disease

A

Mixed movement disorder due to accumulation of Copper in brain , liver and cornea

  • presentation : wing beating tremor , dystonia, chorea
  • rigidity
  • dysarthria
  • cognitive and psychiatric impairment
  • Kayser Fleischer ring in blue eyes ( brown ring )
42
Q

Treatment of Wilson’s disease

A

copper chelating drugs (e.g. penicillamine, trietene)

43
Q

Describe ataxia

A

Lack of co-ordination of voluntary motor acts impairing their smooth performance (rate, range, timing, direction and force of movement maybe affected).

Causes : cerebellar , sensory , optic and frontal dysfunction

44
Q

Describe Sensory ataxia

A
  • Impaired proprioception
  • Cause : disease of the dorsal (posterior) columns of spinal cord, neuropathies or neuronopathies (dorsal root ganglia)
  • exacerbated by removal of visual cues (Romberg’s sign)
  • pseudoatheosis shown : loss of proprioception causing involuntary movements
45
Q

Cerebellar ataxia presentation

A
  • Dysarthria
  • Intention tremor
  • Dysmetria (past pointing)
  • Dysdiadochokinesia
  • Titubation (head tremor)
  • Gait ataxia
  • Abnormal eye movements (nystagmus, saccadic intrusions - side to side movement when fixating on point )
  • Rebound phenomenon
  • Often limb hypotonia
46
Q

What causes ipsilateral limb ataxia

A

Cerebellar hemisphere lesions

47
Q

What causes Truncal & gait ataxia

A

Midline ( vermis ) cerebellar lesions

48
Q

Causes of cerebellar ataxia

A 
Localised 
• Demyelination (Multiple sclerosis)
• Tumour
• Infarct
• Haemorhage

Global
• Infective – often kids (VZV
cerebellitis)
• Endocrine e.g. myxoedema
• Immune-mediated, paraneoplastic : Opsoclonus-myoclonus , Miller Fisher syndrome
• Degenerative : Multiple system atrophy (MSA), Spinocerebellar ataxias (SCAs), Episodic ataxias
• Toxic/Poisoning : DDT, mercury salts, 5-fluorouracil

49
Q

Circumducting gait causes

A

Post stroke and Hemiballism

50
Q

To little movement , touching opposing fingers slows down , little facial expression, resting tremor

What is the likely diagnosis

A

Parkinson’s disease : Bradykinesia

51
Q

If person’s movement is slow but speed and amplitude is not decreasing by increments is it Bradykinesia ?

A

No , might be slowing of movements due to MND

Neurology Clinical (14 decks)

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