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
What is Hemiballismus
Severe proximal Chorea | - Violent flinging movements of one side of the body
26
Cause of Hemiballismus
infarction/SOL/ haemorrhage in the contralateral (opposite) subthalamic nucleus
27
Treatment of Hemiballismus
not always required, settles spontaneously, may need dopamine blockers or surgery in extreme cases
28
Describe Tics
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
Explain Types of tics
``` 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
Causes of Tics
``` 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
What is Tourette's Syndrome
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
Treatment of Tourette's
Psychobehavioural therapy, reassurance, rarely | neuroleptic medication needed
33
Describe Myoclonus
Brief involuntary twitch (lighting fast jerk) of a muscle or group of muscles - not a tremor , no rhythm , irregular - stimulus sensitivity
34
Causes of Myoclonus
- Neuro & metabolic disorders e.g. Epilepsy, CJD, ureamia, liver failure - Often drug-induced - may not be a disease but just a normal jerk
35
Positive myoclonus vs negative
Positive : contractions | Negative : relaxations
36
Drugs most commonly to induce Dyskinesia
Salbutamol , Lithium , Valproate , Levodopa
37
Explain presentation of Tardive dyskinesia , what is the cause
* Mouthing & smacking of lips * Grimaces with contortion of face & neck * Occurs after several years of treatment with Neuroleptics
38
Explain presentation of Acute Dystonic reactions, what is the cause and treatment
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
Describe Athetosis
* 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
Describe Akathisia
unpleasant sensation of restlessness, manifests as inability to sit still or remain motionless - feeling not involuntary movement
41
Describe Wilson's disease
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
Treatment of Wilson's disease
copper chelating drugs (e.g. penicillamine, trietene)
43
Describe ataxia
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
Describe Sensory ataxia
* 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
Cerebellar ataxia presentation
* 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
What causes ipsilateral limb ataxia
Cerebellar hemisphere lesions
47
What causes Truncal & gait ataxia
Midline ( vermis ) cerebellar lesions
48
Causes of cerebellar ataxia
``` 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
Circumducting gait causes
Post stroke and Hemiballism
50
To little movement , touching opposing fingers slows down , little facial expression, resting tremor What is the likely diagnosis
Parkinson's disease : Bradykinesia
51
If person's movement is slow but speed and amplitude is not decreasing by increments is it Bradykinesia ?
No , might be slowing of movements due to MND