Movement Disorders Flashcards

(93 cards)

1
Q

Case 1
 This 23 yo woman has become demented over a few months and developed jerks of her body as
seen in the video. She comes from poor socio-economic background and did not get childhood
vaccinations. However, she did get measles, chickenpox and mumps as a child. She was well until a
few months ago when she slowly became encephalopathic and then developed these movements
and eventually became mute and bed bound.
 What type of movement disorder does she have?

A

Myoclonic jerks

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2
Q

Case 1
 This 23 yo woman has become demented over a few months and developed jerks of her body as
seen in the video. She comes from poor socio-economic background and did not get childhood
vaccinations. However, she did get measles, chickenpox and mumps as a child. She was well until a
few months ago when she slowly became encephalopathic and then developed these movements
and eventually became mute and bed bound.
 What type of movement disorder does she have?
o Myoclonic jerks
 What diagnosis can you suggest?

A

o In a young person (23 years old) with rapid cognitive decline, myoclonic jerks, and a history
of measles infection(without vaccination), the most likely diagnosis is Subacute Sclerosing
Panencephalitis (SSPE).
o SSPE is a rare, fatal complication of measles virus infection occurring years after the acute
infection due to persistent measles virus in the brain, leading to progressive
neurodegeneration.

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3
Q

Supporting Clues for SSPE:

A

 Young age (typically develops between 10-30 years old)
 History of measles (especially without vaccination)
 Progressive encephalopathy (cognitive decline, behavioral changes‚ motor
dysfunction, coma)
 Myoclonic jerks
 EEG: Periodic complexes (“Radermecker complexes”)
 CSF: Raised measles antibody titers

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4
Q

Case 1
 This 23 yo woman has become demented over a few months and developed jerks of her body as
seen in the video. She comes from poor socio-economic background and did not get childhood
vaccinations. However, she did get measles, chickenpox and mumps as a child. She was well until a
few months ago when she slowly became encephalopathic and then developed these movements
and eventually became mute and bed bound

 If she had the same clinical syndrome, but was 60 yo, can suggest a different diagnosis?.

A

o “ Creutzfeldt-Jakob Disease (CJD) (Sporadic CJD - sCJD)
- Rapidly progressive dementia
- Myoclonus
- Ataxia
- EEG: Periodic sharp wave complexes
- CSF: 14-3-3 protein or RT-QuIC positivity

o Autoimmune/Paraneoplastic Encephalitis
- Anti-NMDA receptor or other paraneoplastic antibodies
- Associated with tumors (e.g., small cell lung cancer, ovarian teratomas)

o Limbic Encephalitis (Paraneoplastic or Autoimmune)
- Subacute cognitive decline
- Myoclonus, seizures

o Toxic-Metabolic Causes
- Hepatic or uremic encephalopathy, Wilson’s disease in younger cases

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5
Q

Case 2
 This teenage boy had a sore throat some weeks ago and his teacher and parents are complaining
that he can’t keep still. He is fully orientated and can otherwise function well at home and at
school.
 What is the movement disorder type?

A

The boy has multiple fast short movements his right arm & leg. The face and left side
appears reasonably quiet and normal. This type of movement will satisfy the chorea type
and could even be called right hemi-chorea

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6
Q

Case 2
 This teenage boy had a sore throat some weeks ago and his teacher and parents are complaining
that he can’t keep still. He is fully orientated and can otherwise function well at home and at
school.
 What is the movement disorder type?
o The boy has multiple fast short movements his right arm & leg. The face and left side
appears reasonably quiet and normal. This type of movement will satisfy the chorea type
and could even be called right hemi-chorea.

What is his likely diagnosis?

A

As a teenager he could have one of many conditions which could cause this.

o First consideration should be rheumatic fever (also called Sydenhams’ chorea)
o Other options include SLE, drugs, infective & inflammatory brain disease, a whole spectrum
of neurodegenerative/metabolic conditions and a young stroke ?

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7
Q

This teenage boy had a sore throat some weeks ago and his teacher and parents are complaining
that he can’t keep still. He is fully orientated and can otherwise function well at home and at
school.

What would your work-up be?

A

o Start with search for Rheumatic fever. Then metabolic/ inflammatory screen ?

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8
Q

Case 3
 This teenage girl presented with abnormal movements including sniffing and shrugging movements.
 Her family tolerated this, but her boyfriend got upset when he saw her winking at other boys. She
says she had similar movements when she was little girl, but they went away on treatment. She can
stop them, but only for a short while, then they start again.
 What is the movement disorder type?

 What is her likely diagnosis?

A

o These are tics. The movements are as fast as chorea, but the actions are normal type of
behaviours such as pursing lips shrugging etc.

Tourette syndrome

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9
Q

Case 4
 This 50 yo woman is complaining of involuntary mouth movements. She was previously treated for
depression.
 What is the movement disorder type?

 What is her likely diagnosis?

 How would you confirm the diagnosis?

A

o Oro-bucco-facial fast movements. Dyskinesia.

o Tardive dyskinesia

o Find a history of previous use of phenothiazines.

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10
Q

Case 5
 This 47 yo woman has been causing trouble at home, arguing with everyone. She doesn’t seem fully
orientated and appropriate, but most problematic of all is her restless movements all the time.
 What is the movement disorder type?

 What is her likely diagnosis?

 How would you confirm the diagnosis?

A

o Chorea-whole body including face limbs, body.

o Huntington’s chorea

o Family history and referral to neuro-genetics clinic

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11
Q

Case 6
 This 50 yo man has a tremor of his head and hands. He is otherwise fine. He says a cold beer helps
settle the shakes, but it has been getting worse over the years. His mother had it too.
 What is the movement disorder type?

 What is his likely diagnosis?

 Can it be treated?

A

o Spontaneous head tremor, postural (action) when drinking water.

o Essential tremor (familial)

o Yes. Beta blocker (or primidone)

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12
Q

What is a movement disorder?

A

A neurological condition that affects a person’s ability to move.

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13
Q

How do movement disorders affect movement?

A

They may cause involuntary movements (e.g., tremors) or make voluntary movements difficult.

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14
Q

What type of symptoms can movement disorders cause?

A

Uncontrolled movements, slowed movements, or problems initiating movement.

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15
Q

What are the two main categories of movement disorders?

A
  1. Hypokinetic – too little movement (e.g., Parkinsonism).
    1. Hyperkinetic – too much movement (e.g., tremor, chorea, dystonia).
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16
Q

What is Parkinsonism?

A

A group of disorders with bradykinesia, rigidity, tremor, and postural instability.

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17
Q

What is bradykinesia?

A

Slowness in initiating and performing voluntary movements.

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18
Q

What is a tremor?

A

An involuntary, rhythmic oscillatory movement of a body part.

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19
Q

What is dystonia?

A

Sustained or intermittent muscle contractions causing abnormal postures or twisting movements.

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20
Q

What is chorea?

A

Irregular, rapid, unpredictable, dance-like movements that flow from one muscle group to another.

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21
Q

What is athetosis?

A

Slow, writhing, continuous movements, often of the hands and feet.

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22
Q

What is hemiballismus?

A

Sudden, violent, flinging movements of one side of the body, usually due to subthalamic nucleus damage.

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23
Q

What is myoclonus?

A

Sudden, brief, shock-like muscle jerks

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24
Q

What is tics?

A

Sudden, rapid, recurrent, stereotyped movements or vocalizations, often suppressible.

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25
What is Tourette’s syndrome?
A tic disorder with both motor and vocal tics, starting in childhood.
26
What is akathisia?
A feeling of inner restlessness with the need to move, often caused by antipsychotics.
27
What part of the brain is most often involved in movement disorders?
The basal ganglia and its connections.
28
What are common symptoms of movement disorders?
Involuntary muscle jerking/spasms, shaking, stiffness, unsteady or shuffling gait, and difficulty performing intentional movements.
29
What is ataxia?
Loss of balance or coordination.
30
What is chorea?
Brief, repetitive, involuntary movements that may look like fidgeting
31
What conditions can cause chorea?
Huntington’s disease, acute rheumatic fever (Sydenham’s chorea), and hyperglycaemic hemichorea.
32
What is dystonia?
Involuntary, sustained muscle contractions causing twisting or abnormal postures.
33
What is essential tremor?
A common movement disorder with excessive rhythmic shaking, often during voluntary movements (action tremor).
34
What is Huntington’s disease?
An inherited neurodegenerative disorder where nerve cells in the brain waste away, causing chorea, psychiatric symptoms, and dementia.
35
What is Parkinson’s disease?
A progressive disorder with bradykinesia, resting tremor (“pill-rolling”), rigidity (“lead-pipe”), and postural instability, often starting unilaterally.
36
Which brain structures are most often involved in movement disorders?
Basal ganglia, midbrain, and cortex.
37
What is the role of the basal ganglia in movement?
They regulate initiation and smooth execution of voluntary movements through excitatory and inhibitory circuits.
38
How does basal ganglia dysfunction present?
With hyperkinetic disorders (e.g., chorea, dystonia) or hypokinetic disorders (e.g., Parkinsonism).
39
Which basal ganglia structure is primarily affected in Parkinson’s disease?
Substantia nigra pars compacta (dopamine-producing neurons).
40
Which basal ganglia structure is affected in hemiballismus?
Subthalamic nucleus (lesion causes violent flinging movements).
41
Which basal ganglia structure degenerates in Huntington’s disease?
The caudate nucleus and putamen (striatum)
42
What is the role of the midbrain in movement?
Contains the substantia nigra and red nucleus, crucial for motor control.
43
How does midbrain pathology cause movement disorders?
Loss of dopaminergic neurons (Parkinson’s), or disruption of motor pathways (tremor, rigidity).
44
What is the role of the cerebral cortex in movement?
Initiates voluntary movements and integrates motor planning with sensory feedback.
45
How does cortical dysfunction contribute to movement disorders?
Can cause abnormal motor planning, seizures with motor symptoms, or worsen basal ganglia dysfunction.
46
What is the main function of the cerebellum?
To coordinate voluntary movements, maintain balance, and ensure smooth, accurate motion.
47
What type of movement disorder results from cerebellar dysfunction?
Ataxia
48
What are the main features of cerebellar ataxia?
Loss of coordination, wide-based gait, intention tremor, dysmetria (overshooting/undershooting), and nystagmus
49
What is dysmetria?
Inability to control the range of movement, causing overshooting or undershooting a target.
50
What is dysdiadochokinesia?
Difficulty performing rapid alternating movements, e.g., pronation-supination of the hand.
51
What is an intention tremor?
Tremor that appears or worsens during purposeful movement toward a target.
52
What are common causes of cerebellar ataxia?
Stroke, tumors, multiple sclerosis, chronic alcohol use, inherited ataxias, and drug toxicity (e.g., phenytoin).
53
How does cerebellar gait ataxia present?
A broad-based, unsteady, staggering gait (resembles “drunken” walking
54
What is the difference between cerebellar tremor and Parkinsonian tremor?
Cerebellar tremor is intention tremor (during movement); Parkinsonian tremor is resting tremor.
55
What is Parkinson’s disease?
A neurodegenerative disorder that affects dopamine-producing neurons in the substantia nigra
56
Which neurotransmitter is most affected in Parkinson’s disease?
Dopamine
57
What are the cardinal features of Parkinson’s disease?
Bradykinesia, resting tremor, rigidity, and postural instability.
58
What is vascular Parkinsonism?
Parkinsonism due to extensive small vessel disease, usually affecting the lower limbs more.
59
What is Parkinson’s Plus?
A group of primary brain disorders that mimic Parkinson’s but do not respond to dopamine.
60
Which conditions are included in Parkinson’s Plus syndromes?
Multisystem atrophy, Lewy body dementia, progressive supranuclear palsy, and Parkinson’s dementia.
61
What are distinguishing features of Parkinson’s Plus syndromes?
Postural instability, axial symptoms, and poor response to dopamine therapy.
62
What is drug-induced Parkinsonism?
Parkinsonian symptoms caused by medications or toxins that block or affect dopamine pathways.
63
Which drugs commonly cause drug-induced Parkinsonism?
Typical antipsychotics (causing EPSEs), antiemetics, antihistamines (e.g., chlorpromazine).
64
Which substances or exposures can cause Parkinsonism?
Manganese exposure (miners), cocaine, methamphetamines.
65
What are the main motor signs of Parkinson’s disease?
Rigidity, bradykinesia, tremor, and postural instability.
66
What is lead-pipe rigidity?
Uniform stiffness of a limb throughout its range of movement.
67
What is cogwheel rigidity (wrist sign
Rigidity with a ratchet-like quality due to tremor superimposed on stiffness.
68
What is bradykinesia and how does it present?
Slowness of movement, often seen as slow walking and reduced arm swing.
69
How is speech affected in Parkinson’s disease?
Speech becomes monotone and reduced in volume (hypophonia).
70
What sleep problems are common in Parkinson’s disease?
REM sleep behaviour disorder (yelling, screaming, or acting out dreams) and difficulty turning in bed due to rigidity.
71
What is anosmia and why is it important in Parkinson’s?
Loss of smell (and taste); it is often an early sign of Parkinson’s disease.
72
What is Parkinson’s dementia?
Cognitive decline that can occur in later stages of Parkinson’s disease.
73
What gastrointestinal symptoms are associated with Parkinson’s?
Constipation and gastro-oesophageal reflux disease (GORD), reflecting involvement of the enteric nervous system.
74
What autonomic dysfunctions are seen in Parkinson’s?
Postural instability, orthostatic hypotension, and falls (often within the first 3 years).
75
What are the main components to test when assessing for aphasia?
Fluency, naming, repetition, reading, and writing.
76
How do you test fluency in aphasia?
Ask the patient to speak spontaneously and observe sentence length, grammar, and effort.
77
How do you test naming in aphasia?
Show the patient common objects or pictures and ask them to name them.
78
How do you test repetition in aphasia?
Ask the patient to repeat simple and complex phrases.
79
How do you test reading in aphasia?
Give the patient written words or sentences and ask them to read aloud or follow a written instruction
80
How do you test writing in aphasia?
Ask the patient to write their name, a sentence, or copy written material.
81
What is Levodopa?
A central nervous system agent that is converted to dopamine in the brain to improve Parkinson’s symptoms.
82
What is Carbidopa?
A decarboxylase inhibitor that prevents levodopa from being broken down before reaching the brain.
83
Why is Carbidopa given with Levodopa?
It allows lower doses of levodopa to be used, reducing side effects such as nausea and vomiting.
84
What is the main benefit of Carbidopa/Levodopa therapy?
Improves motor symptoms by restoring brain dopamine levels.
85
86
What is Bromocriptine?
A dopamine agonist that directly stimulates dopamine receptors in the brain.
87
How do dopamine agonists (like Bromocriptine) differ from Levodopa?
They don’t need to be converted into dopamine and have a longer duration of action but more side effects (e.g., impulse control disorders, hallucinations).
88
What is the inheritance pattern of Huntington’s disease?
Autosomal dominant.
89
What type of movement disorder is seen in Huntington’s disease?
Chorea affecting the face, hands, feet, and torso.
90
What cognitive problems occur in Huntington’s disease?
Difficulty learning new things, memory impairment, and poor concentration.
91
What emotional changes are common in Huntington’s disease?
Mood swings, irritability, depression, and apathy
92
What behavioural problems may occur in Huntington’s disease?
Social withdrawal, impulsiveness, and antisocial behaviour.
93
What other neurological difficulties are seen in Huntington’s disease?
Difficulty speaking, swallowing, walking, and maintaining balance.