Multiple sclerosis Flashcards

(69 cards)

1
Q

Define what is multiple sclerosis

A

Chronic autoimmune demyelinating disease of the central nervous system (CNS), causing episodes of neurological dysfunction separated in time and space

= causing loss of the insulating myelin sheath

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2
Q

Which CNS cells are destroyed in MS?

A

Oligodendrocytes

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3
Q

What type of hypersensitivity reaction underlies MS?

A

Type IV (T-cell-mediated)

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4
Q

Which areas of the CNS are most commonly affected by MS plaques?

A
  1. Optic nerves
  2. Brainstem/cerebellar connections
  3. Cervical cord
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5
Q

Which populations are most affected by MS?

A

White populations, with increasing prevalence at higher latitudes (further from the equator)

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6
Q

Are peripheral nerves affected in MS?

A

No

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7
Q

What is the usual age of onset for MS?

A

20-40 years

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8
Q

Which is the most common form of MS?

A

Relapsing-remitting MS

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9
Q

How does relapsing-remitting MS present?

A

Symptoms occur in relapses (attacks) developing over days with partial or complete recovery over weeks

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10
Q

What is secondary progressive MS, and how common is it after 35 years of disease?

A

A phase of gradually worsening disability following RRMS

= occurs in about 75% of patients after 35 years

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11
Q

What proportion of patients have primary progressive MS, and how does it differ?

A

10–15%

= characterised by gradual disability progression without relapses or remissions

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12
Q

What pyramidal pattern of weakness is typical in MS?

A

Upper limb extensors and lower limb flexors are preferentially weakened

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13
Q

How does optic neuritis typically present?

A

Painful monocular visual loss over 1–2 weeks with reduced colour vision and a relative afferent pupillary defect

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14
Q

Which sensory modalities are commonly affected in MS due to dorsal column loss?

A

Proprioception and vibration sense

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15
Q

Which cranial nerve palsy most often causes diplopia in MS?

A

CN VI (abducens nerve palsy)

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16
Q

What clinical syndrome results from a lesion of the medial longitudinal fasciculus?

A

Internuclear ophthalmoplegia

= failure of adduction with nystagmus in the abducting eye

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17
Q

Name three classic cerebellar signs in MS

A
  1. Dysarthria
    = slurred or unclear speech due to problems with the muscles used for speaking
  2. Ataxia
    = loss of coordination of voluntary movements
  3. Intention tremor
    = shaking that occurs during purposeful movement (e.g. when reaching for something), but not at rest
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18
Q

What is Lhermitte’s phenomenon?

A

An electric shock-like sensation down the spine and into the limbs on neck flexion

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19
Q

What is the most common cause of lower urinary tract dysfunction in MS?

A

Detrusor overactivity
= urgency, frequency, nocturia, incontinence

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20
Q

What is the main purpose of blood tests in suspected MS?

A

To exclude differential diagnoses

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21
Q

What is the most sensitive imaging test for MS?

A

MRI brain and spine with gadolinium contrast

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22
Q

A patient with MS has failure of left eye adduction on right gaze and nystagmus in the right eye. What lesion does this indicate?

A

Left medial longitudinal fasciculus (MLF) → internuclear ophthalmoplegia

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23
Q

What is the characteristic CSF finding in MS?

A

Oligoclonal bands of IgG in CSF not matched in serum

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24
Q

On MRI, what demonstrates dissemination in space (DIS) vs dissemination in time (DIT)?

A

(1) DIS
= Multiple lesions in different CNS regions

(2) DIT
= New lesions on follow-up scan or simultaneous enhancing and non-enhancing lesions

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25
How are moderate to severe acute relapses in MS treated?
IV methylprednisolone 1 g daily for 3 days
26
Which biologic is increasingly used first-line in severe, rapidly evolving relapsing-remitting MS?
Natalizumab
27
Which injectable drugs are common first-line agents in mild–moderate relapsing–remitting MS?
Beta-interferon and glatiramer acetate
28
First line injectable disease-modifying therapies (DMTs) for multiple sclerosis
Beta interferon Glatiramer acetate
29
Second-line acute management of severe multiple sclerosis
Plasma exchange
30
What vitamin is associated with a lower incidence of Multiple Sclerosis?
Vitamin D
31
The only disease-modifying therapy (DMT) for multiple sclerosis that has shown efficacy in primary progressive disease is...
Ocrelizumab
32
What is the second most common ophthalmic presentation of multiple sclerosis?
Internuclear ophthalmoplegia
33
Internuclear ophthalmoplegia is due to an interruption between the communication of which two cranial nerve nuclei?
Third and sixth cranial nerve
34
Biologic disease-modifying therapies (DMTs) for multiple sclerosis
Natalizumab Alemtuzumab
35
What are the good prognostic factors in multiple sclerosis?
(1) Young-onset (2) Female (3) Sensory symptoms/signs at presentation (4) Long intervals between relapses (5) A few MRI lesions
36
What factors are associated with a worse prognosis of Multiple Sclerosis?
(1) Older (2) Male (3) Motor signs at onset (4) Early relapses (5) Many MRI lesions (6) Axonal loss
37
What are oligoclonal bands?
Intrathecally produced IgG = Intrathecally means produced or occurring within the cerebrospinal fluid (CSF) space around the brain and spinal cord
38
In the treatment of which neurological condition is interferon beta used?
Relapsing-remitting multiple sclerosis
39
A 22-year-old woman presents to ambulatory care with a 5-week history of progressively worsening double vision. She has also recently noticed an unpleasant, electric-like sensation radiating down her legs when taking a warm bath. On examination, when she looks to the left, her right eye fails to adduct, and there is nystagmus in her left, abducting eye Given the likely diagnosis, where is the lesion causing her ocular abnormality located?
Right medial longitudinal fasciculus
40
A 29-year-old female patient presents to the eye clinic complaining of a two-week history of double vision. On examination, she has a visual acuity of 6/36 in her left eye and red desaturation. Visual acuity 6/6 in her right eye. Fundoscopy shows a slightly pale left optic disc. On examination of her eye movements, you note that both eyes are normal in primary gaze and both eyes can look to the right. However, when she tries to look to her left, her right eye does not fully adduct, and she has a horizontal nystagmus in her left eye, which manages some abduction. Which investigation is most likely to reveal the underlying diagnosis?
MRI head
41
A 22-year-old woman with a clinical diagnosis of Multiple sclerosis presents with her third acute relapse with signs of weakness, optic neuritis and vertigo. Her cerebrospinal fluid shows oligoclonal bands, and a gadolinium-enhanced MRI brain scan shows periventricular plaques. What is the most appropriate pharmacotherapy for acute management to reduce the severity of her current symptoms?
Intravenous methylprednisolone = Methylprednisolone given over 3 days for acute Multiple Sclerosis can reduce duration and severity of attacks but it has no impact on long term outcome
42
A 37-year-old woman with a background of multiple sclerosis presents to the GP with blurred and double vision for the past two days. On examination, when she moves her eyes to either side, there is limited movement of the adducting eye and nystagmus in the abducting eye. The patient feels well in herself otherwise and has normal observations. What would be the best management of her symptoms?
Oral methylprednisolone
43
Multiple sclerosis may be divided into two groups Which are what?
1. Relapsing-remitting (which may become secondarily progressive) 2. Primary progressive
44
A 28-year-old software engineer had an episode of blurred vision and loss of colour vision in his left eye that resolved over a couple of weeks. A year later, he had a month of erectile dysfunction and urinary problems. After seeing a neurologist, he was given a diagnosis of multiple sclerosis. What would you expect to see on his lumbar puncture results?
1. Oligoclonal bands are distinct bands of Immunoglobulin (Ig) G 2. Total protein measures are often mildly raised
45
A 23-year-old woman presents with a 2-week history of worsening unsteadiness when walking and clumsiness in her right hand. On examination, she has a broad-based ataxic gait and is unable to heel-toe walk. She has dysmetria and an intention tremor on finger-nose testing and dysdiadochokinesia in the right upper limb. There is also an impairment of the heel-shin test on the right side. Power is normal throughout. What is the most likely cause of her presentation?
Demyelination
46
A 36-year-old woman presents to the neurology clinic with weakness. When asked about her family history, she informs you that her aunt takes a medication called Interferon beta, but she is unsure of her diagnosis. Interferon beta is a licensed treatment for what condition?
Relapsing remitting multiple sclerosis
47
A 37-year-old female patient from Sweden attends A&E complaining of a 2-day history of blurry vision and pain in her right eye. The visual acuity in her right eye is 1/60, and she has some loss of colour vision. She had a similar episode in her left eye a few years ago. Neurological examination is normal. Fundoscopy reveals a pale right optic disc. The left eye is normal. What is the most likely diagnosis? Explain why?
Multiple sclerosis = This patient has an optic neuritis = She has a sudden, painful loss of vision with reduced colour vision, very poor acuity, and a pale optic disc. The past similar episode in the other eye suggests a demyelinating cause, and in a young woman from Northern Europe, MS is the classic underlying condition
48
Explain what demyelination is?
Damage to the myelin sheath In multiple sclerosis (MS), the body’s immune system attacks this myelin in the brain, spinal cord, and optic nerves. When myelin is lost, nerve signals slow down or get blocked completely. This is why patients get symptoms such as vision problems (optic neuritis), weakness, numbness, or balance issues
49
A 34-year-old woman presents to the emergency department with loss of vision in her right eye. She reports initially the loss of the centre of her vision, which has now progressed to generalised visual loss. In addition, she reports pain on movement of the right eye. On further questioning, she reports an episode of sensory paraesthesia earlier that year that appeared transiently for 4-5 days, and she had attributed it to a combination of a “trapped nerve” and the stresses of work. What criteria can be used to help confirm the likely diagnosis?
McDonald criteria
50
A 33-year-old woman presents to her GP with intermittent neurological symptoms which have been ongoing for several months. She also had a recent episode of urinary incontinence, which has never happened before. Her GP refers her to a neurologist who performs a lumbar puncture, which is positive for oligoclonal bands, further supporting this diagnosis. MRI confirms the diagnosis. (1) What does she have and how do you know this? (2) What is the most appropriate first-line option for the chronic management of this condition?
(1) Relapsing-remitting multiple sclerosis = the patient has episodes of neurological symptoms that appear, improve, and then new symptoms appear later (2) First-line ; Interferon-beta Glatiramer acetate Second-line / high-efficacy (for highly active or refractory disease): Natalizumab Fingolimod Alemtuzumab Ocrelizumab
51
When is Natalizumab first line?
1. patients with highly active (>2 episodes a year) relapsing-remitting MS or 2. second-line therapy in patients who fail first-line treatments
52
A 35-year-old man presents to the Emergency Department due to a week of progressive bilateral leg weakness. He has no history of trauma or back pain. He suffered from trigeminal neuralgia 6 months ago, which resolved without treatment. He had a self-limited upper respiratory illness 14 days ago. His temperature is 37.2 degrees Celsius, his blood pressure is 132/78 mmHg, and his pulse is 82 beats per minute. Physical examination shows increased resistance to passive flexion and extension of the lower limbs. Deep tendon reflexes are 3+ with upgoing plantar reflexes bilaterally. There is decreased vibratory and positional sensation in his left upper extremity, but no other sensory loss. CSF analysis of this patient would most likely reveal what?
Oligoclonal bands
53
A 19-year-old woman with a history of eczema presented with right-sided blurring of vision and retro-orbital pain that had progressed over 7 days. She initially noticed that colours looked washed out, and now has a visual acuity of 6/15 and colour desaturation. Which investigation is the best to achieve a diagnosis?
MRI brain with contrast
54
A 34-year-old man presents with progressive limb weakness and numbness over the past two weeks. Last month, he noted episodes of visual disturbances that resolved spontaneously. His family history includes stroke and antiphospholipid syndrome. Physical examination reveals an impaired tandem gait and decreased muscle strength in the upper extremities. Deep tendon reflexes are 4+ bilaterally. Sensation is mildly decreased over the lower extremities. What best describes the primary pathological process of the underlying condition?
Autoimmune-mediated destruction of oligodendrocytes
55
A 28-year-old man presents to the emergency department with pain in his lower abdomen and a sensation of urinary retention - a bladder scan on admission confirms this. He also reports a gradual onset of leg weakness and patchy sensory disturbance in his lower limbs. On examination, there is a pyramidal distribution of weakness, with spastic hypertonia and brisk reflexes. An MRI of the brain and whole spine is performed, which reveals evidence of a single-segment transverse myelitis in the mid-thoracic cord. The optic nerves are normal. A lumbar puncture is performed, which reveals weakly positive oligoclonal banding. Testing for infectious causes of myelitis and anti-aquaporin 4 antibodies are negative, and IV methylprednisolone is administered. At 6 months follow-up, the patient is wheelchair dependent. What is the most likely diagnosis?
Primary progressive multiple sclerosis
56
Multiple white matter lesions in characteristic MS sites include what?
1. Periventricular (around the ventricles, often Dawson’s fingers) 2. Juxtacortical (next to the cortex) 3. Infratentorial (brainstem, cerebellum) 4. Spinal cord
57
An exchange student comes to his GP practice after a new diagnosis of multiple sclerosis. Before leaving his country, he was advised to seek out medical care as soon as possible. What option is a potential risk factor for this condition? A. Being male B. Individuals living in Brazil C. Individuals living in Canada D. Smoking vape E. Stressful lifestyle Explain why?
C = Multiple sclerosis is more common at higher latitudes. Brazil is closer to the equator and therefore has a lower latitude
58
A young woman presents after multiple episodes of optic neuritis, during which she develops unilateral eye pain. Upon examination, she is found to have decreased visual acuity and colour saturation on her affected eye. Her doctor suspects multiple sclerosis. Which features would be expected on a T2-weighted MRI?
(1) Multiple hyperintense (bright) white matter lesions in the brain and spinal cord (2) These bright spots represent areas of demyelination, which are typical of multiple sclerosis
59
A 31-year-old woman with a history of optic neuritis presents to the neurology clinic with left leg weakness for the past few days. This is associated with numbness. She reports having similar symptoms in her right arm about 8 months ago, although the symptoms resolved spontaneously over a few days back then. It is noticed that her symptoms are worse when she is in hot weather. Neurological examination, including cranial nerves, reveals weakness in her left leg movements, but otherwise is normal. Why is demyelination the likely underlying pathophysiology for her disease?
1. History of optic neuritis = classic sign of demyelination of the optic nerve 2. Episodes of neurological symptoms separated in time and space – different areas (arm, then leg) and times (8 months apart) 3. Symptoms worsen with heat (Uhthoff’s phenomenon) – typical of demyelinating diseases 4. Partial recovery between attacks – due to remyelination or compensation after inflammation subsides
60
What are first-line drugs for spasticity in multiple sclerosis?
Baclofen and gabapentin
61
A 30-year-old male was diagnosed with multiple sclerosis 3 years ago. He experienced symptoms such as increasing difficulty in walking, muscle fatigue and stiffness, losing balance, and numbness in his peripheries. The first episode of worsening symptoms had lasted about 3 months, followed by 7 months free of symptoms. The next episode was a longer period of 6 months of symptoms followed by only 1 month free of symptoms. He is currently experiencing symptoms again for the past 2 months. What classification of multiple sclerosis is being described?
Relapse-remitting
62
What does a multiple sclerosis diagnosis require?
demyelinating lesions that are separated in space and time
63
A 45-year-old woman with known multiple sclerosis for several years has been experiencing worsening muscle stiffness and difficulty in producing smooth movements. She has been taking baclofen for a year now but has had no effect. What is the next best medication to help with these symptoms?
Gabapentin
64
A 45-year-old female with multiple sclerosis complains of tingling in her hands, which comes on when she flexes her neck. What is this an example of?
Lhermitte's sign
65
What is Uhthoff’s phenomenon and when does it occur?
(1) Temporary worsening of neurological symptoms in people with multiple sclerosis when their body temperature rises (2) It can happen during exercise, in hot weather, with fever, or after a hot bath or shower, and symptoms improve once the person cools down
66
A 35-year-old woman presents with a variety of symptoms, including generalised skin tingling and headache. She is concerned she may have multiple sclerosis. What is the most common presentation of multiple sclerosis?
Optic neuritis
67
“most effective”, “most efficacious”, “highest relapse-reduction”, “best at preventing relapses” Drug name
natalizumab or alemtuzumab
68
“first-line”, “initial treatment”, “starting disease-modifying therapy” Drug name
beta-interferons or glatiramer acetate
69
A 40-year-old man presents to the emergency department, reporting episodes of blurred vision when reading. The episodes last between three and ten hours and are brought on shortly after, or during, exercise. The patient has a past medical history of relapsing-remitting multiple sclerosis, familial hypercholesterolaemia, and asthma. Neurological examination, including the eyes and vision, is unchanged from the patient's function described in previous consultations. What is the most likely explanation for this patient's presentation?
Uhthoff's phenomenon = sweating = increase in temperature