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?
Myoclonic jerks
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?
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.
Supporting Clues for SSPE:
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
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?.
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
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?
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
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?
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 ?
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?
o Start with search for Rheumatic fever. Then metabolic/ inflammatory screen ?
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?
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
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?
o Oro-bucco-facial fast movements. Dyskinesia.
o Tardive dyskinesia
o Find a history of previous use of phenothiazines.
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?
o Chorea-whole body including face limbs, body.
o Huntington’s chorea
o Family history and referral to neuro-genetics clinic
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?
o Spontaneous head tremor, postural (action) when drinking water.
o Essential tremor (familial)
o Yes. Beta blocker (or primidone)
What is a movement disorder?
A neurological condition that affects a person’s ability to move.
How do movement disorders affect movement?
They may cause involuntary movements (e.g., tremors) or make voluntary movements difficult.
What type of symptoms can movement disorders cause?
Uncontrolled movements, slowed movements, or problems initiating movement.
What are the two main categories of movement disorders?
What is Parkinsonism?
A group of disorders with bradykinesia, rigidity, tremor, and postural instability.
What is bradykinesia?
Slowness in initiating and performing voluntary movements.
What is a tremor?
An involuntary, rhythmic oscillatory movement of a body part.
What is dystonia?
Sustained or intermittent muscle contractions causing abnormal postures or twisting movements.
What is chorea?
Irregular, rapid, unpredictable, dance-like movements that flow from one muscle group to another.
What is athetosis?
Slow, writhing, continuous movements, often of the hands and feet.
What is hemiballismus?
Sudden, violent, flinging movements of one side of the body, usually due to subthalamic nucleus damage.
What is myoclonus?
Sudden, brief, shock-like muscle jerks
What is tics?
Sudden, rapid, recurrent, stereotyped movements or vocalizations, often suppressible.