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Gwen - Neurology > Multiple sclerosis > Flashcards

Multiple sclerosis Flashcards

1
Q

What is multiple sclerosis?

A

https://www.youtube.com/watch?v=yzH8ul5PSZ8

MS is a chronic autoimmune T-cell mediated inflammatory disorder of the CNS. Multiple plaques of demyelination occur throughout the brain and spinal cord, occurring sporadically over years (dissemination in space and time which is crucial for diagnosis).

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2
Q

What gender does MS more commonly occur in?

A

Women

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3
Q

What is the mean age of onset of the disease?

A

Approximatley 30 years (range 20-40)

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4
Q

What immune cell is impliated in the development of MS?

A

T-cell mediated autoimmune disease

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5
Q

What parts of the nervous system does MS affect?

A

CNS - any part of the white matter of brain and spinal cord

  • Brain
  • Optic nerve
  • Spinal Cord
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6
Q

What neuronal structures are affected by MS?

A
  • Myelin sheaths
  • Oligodendrocytes (produce melanin)
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7
Q

What are the cardinal pathological features of of MS?

A
  1. Inflammation and oedema
  2. Plaques of demyelination (2-10mm) (which can heal causing relapsing and remitting symptoms)
  3. Prolonged demyelination causing axonal loss and gliotic scarring (clinically progressive symptoms)
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8
Q

Where do plaques most commonly occur in MS?

A

Can occur anywhere in CNS, but most commonly:

  • Optic nerves
  • Periventricular region
  • Corpus callosum
  • Brainstem and cerebellar connections
  • Cervical cord (corticospinal and posterior columns)
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9
Q

What does the focal acute inflammation cause in terms of nerve conduction?

A

Conduction block

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10
Q

What are the pathologial outcomes that can occur from acute demyelinating episodes?

A

Recovery and remyelination, or permanent axonal destruction

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11
Q

What is the pathological basis for the progression of MS as a disesase?

A

Progressive axonal damage

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12
Q

What proportion of individuals with relapsing remitting MS develop secondary progressive MS?

A

>80%

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13
Q

What are the different types of progression of MS?

A
  • Relapsing remitting MS
  • Secondary Progressive MS
  • Primary Progressive MS
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14
Q

What is relapsing remitting MS?

A

Symptoms occur in attacks (relapses) with a characteristic time course: onset over days and typically recovery, either partial or complete, over weeks. Patients may accumulate disability over time if relapses do not recover fully.

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15
Q

How often do relapses occur on average in relapsing remitting MS?

A

On average patients have one relapse per year but occasionally many years may separate relapses (benign MS – 10% of patients).

Relapses more common after infeciton

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16
Q

What is secondary progressive MS?

A

Late stage of MS consists of gradually worsening disability progressing slowly over years. 80% of those with RRMS progress to this stage by 35 years. Relapses may sometimes occur in this progressive phase (relapsing–progressive MS).

Initial relapsing/remitting then increased disability without remission.

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17
Q

What is primary progressive MS?

A

Characterized by gradually worsening disability without relapses or remissions. Typically presents later and is associated with fewer inflammatory changes on MRI.

Often evolving paraplesis.

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18
Q

What proportion of those with MS have primary progressive MS?

A

10-15%

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19
Q

How do those with MS most commonly present?

A

Wide variety of possible symptoms may occur depending on the anatomical site of lesions. Common ones include:

  • Optic neuritis
  • Brainstem demyelination
  • Spinal cord lesions
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20
Q

What are features of MS affecting the brainstem?

A
  • Diplopia
  • Vertigo with nystagmus
  • Facial numbness/weakness
  • Dysarthria
  • Dysphagia
  • Pyramidal signs
  • Bilateral internuclear opthalmoplegia - Pathognomonic of MS
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21
Q

What is internuclear opthalmoplegia?

A

Characterised by

  1. Impaired adduction of the eye on the abnormal side
  2. Horizontal jerk nystagmus in the opposite eye upon lateral gaze away from the side of the lesion

https://www.youtube.com/watch?v=_rXQmDZva8Y

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22
Q

What is the mechanism behind internuclear opthalmoplegia?

A

INO is caused by a lesion in the medial longitudinal fasciculus (MLF). The MLF connects the abducens nerve (CNVI) nuclei to the oculomotor nerve (CNIII) nuclei and facilitates conjugate eye movements during lateral gaze by coordinating adduction with abduction.

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23
Q

What are features of spinal cord lesions in MS?

A
  • Paraparesis - developing over days/weeks
  • Limb numbness/weakness + tingling
  • Lhermitte’s sign positive
  • Tight band sensation around chest - thoracic lesion
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24
Q

What is optic neuritis?

A

A demyelinating inflammation of the optic nerve. It is also known as optic papillitis (when the head of the optic nerve is involved) and retrobulbar neuritis (when the posterior part of the nerve is involved)

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25
What are causes of optic neuritis?
* **Idiopathic** * **MS** * **SLE** * **Sarcoidosis** * **Sjogren's syndrome** * **Behcets disease** * **Syphillis** * **Vitamin deficiency** * **Diabetes**
26
What are signs of optic neuritis?
* **Altered visual acuity** - may range from 6/6 to PL * **RAPD - relevant afferent pupillary defect** * **Lack of red reflex/retina obsured - large haemorrhage** * **Red desaturation** * **Central scotoma** * **ACUTE DISEASE: Swollen optic disc** - if inflamamtion anterior * **CHRONIC DISEASE: Optic atrophy**
27
What are the symptoms of optic neuritis?
Usually a woman, aged 20-40 * **Disturbance of vision of one eye** - scotoma, floaters, disruption of colour vision, blurred vision (typically distorsion of central vision and impoarment of colour perception) * **Pain that worsens on eye movement, unilateral** Improves over months
28
What are common symptoms seen in MS?
* **Visual changes** * **Sensory symptoms** * **Clumsy limbs** * **Ataxia/Unsteadiness** * **Urinary incontince - urgency/frequency** * **Constipation** * **Pain** * **Fatigue** * **Spasticity** * **Depression** * **Sexual dysfunction** * **Temperature sensitivity**
29
What sensory features are often seen in individuals with MS?
* **Dysaesthesia** * **Pins and needles** * **Trigeminal neuralgia** * **Reduction/Loss in proprioception in feet** * **Feeling of water trickling down skin** * **Uthoff's Phenomenon**
30
What eye signs might you see in MS?
* **Blurred vision** - optic neuropathy * **Diplopia** * **Hemianopia** * **Optic neuritis** - disc swelling * **Internuclear Opthalmoplegia** * **RAPD** * **Optic disc swelling** * **Argyll-Robertson pupil**
31
Why do individuals with MS become clumsy and ataxic?
Due to cerebellar lesions and loss of proprioception
32
Why do individuals with MS develop urinary urgency/frequency?
Hyper-reflexia of the bladder
33
What is Uthoff's Phenomenon?
Signs/symptoms worse (worsening of visual acuity) following increase in temp eg on hot day, after exercise, hot bath, fever
34
What is the cause of Uthoff's Phenomenon?
Heat slows conduction in nerve fibres, causing worsening of symptoms
35
What are argyle robertson pupils?
[https://www.youtube.com/watch?v=IbBYxGk1pUw](https://www.youtube.com/watch?v=IbBYxGk1pUw) Characterised by: * **Miosis (small pupils)** * **Absence of the pupillary light response** * **Brisk accommodation reaction** * **Bilateral involvement.**
36
Signs/symptoms of brainstem myelination
Diplopia (COMMON) Vertigo Dysphasia Nystagmus
37
Evidence of spinal cord lesions
Sensory - numbness, pins and needles (COMMON) Motor - spastic paraparesis (plaques in cervical or thoracic cord), limb weakness initially, hyperreflexia (UMN signs)
38
What is the mechanism behind Argyll-Robertson pupils?
Caused by a pretectal lesion in the dorsal midbrain affecting the fibres of light reflex, which spare the fibres of the accommodation pathway that innervate the Edinger–Westphal nuclei
39
What are motor features of MS?
* **Spasticity** * **Features of Myelitis**
40
What sexual/GU features can occur in MS?
* **Erectile dysfunction** * **Anorgasmia** * **Urine retention/incontinence**
41
What are GI features of MS?
* **Dysphagia** * **Constipation**
42
What features might suggest non-MS causes of some of the neurological features seen in MS?
* **Hyperthermia** * **Nausea/vomiting** * **Positional vertigo** * **Seizures** * **Aphasia** * **MEningism** * **Bilateral optic neuritis** * **CSF leucocytosis**
43
What are features of late stage MS?
* **Spastic tetraparesis** * **Ataxia** * **Optic atrophy** * **Nystagmus** * **Brainstem signs (e.g. bilateral INO)** * **Pseudobulbar palsy** * **Urinary incontinence** * **Cognitive impairment, often with frontal lobe features**
44
What criteria are used to diagnose MS?
The Mcdonald Criteria Evidence of demyelination separated in space and time - at least 2 separate occasions.
45
What investigations would you perform if you suspected MS?
* **MRI** * **LP** * **Visual evoked potentials**
46
What might you see on MRI in someone with
* **Demyelinating Plaques** - in 85% of clinical presentations * Esp in periventricular area and brainstem * Could enhance with gadolinium to see active lesions
47
If someone with MS was experiencing fatigue, what other causes of fatigue would you want to exclude?
* **Anaemia** * **Lack of sleep/apnoea/poor sleep hygeine** * **Hypothyroid** * **Medication** * **Depression**
48
What is Devic's Disease?
**Neuromyelitis optica** Considered a subtype of MS, presents with features of optic neuritis and Acute transverse myelitis
49
What do plaques signify in terms of disease activity in these areas?
There is active disease in areas where plaques are present
50
What would you be looking for on LP in someone with features of MS?
Oligoclonal bands of IgG on electrophoresis
51
What is rule of thumb with the presence of Oligoclonal bands in CSF and diagnosis of MS?
Present in CSF, but not in Serum Indicates intrathecal immunoglobulin synthesis. Can aoccur in other diseases but are normally also found in serum.
52
What is visual evoked potential testing?
**Detect lesions in visual pathway** - The patient has EEG probes on the skull the measure brain response to visual stimuli. They are then given a visual stimulus, and the time between the visual stimulus and the brain response (on EEG) is measured. If the response is delayed this is evidence of some sort of optic nerve lesion.
53
What other conditions can mimic MS lesions on MRI?
* **Sarcoidosis** (rule out with CXR) * **SLE** * **Bechet's syndrome**
54
What non-pharmacological apsects would you consider when managing someone with MS?
Discuss the following * **Education** * **Living arrangements** * **Job** * **General future plans** * **Lifestyle advice** - exercise, smoking, avoid stress * **MDT input** - physio, OT, psychiatry etc.
55
If someone had an acute relapse of MS, how would you manage them?
**IV/PO Methyprenisolone** - 3-5 days (shortens relapse)
56
What dose of methyprednisolone would you give someone having an acute relapse of MS?
0.5-1g/24 hrs
57
What disease modifying medications would you use in an attempt to reduce relapse rate in RRMS?
* **Beta Infterferons** * **​**Reduces relapses by 30% in active relapsing and remitting and decreases lesion accumulation. * **Natalizumab** * Monoclonal antibody, affects permeability of BBB inhibiting leucocytes from crossing * Only used in aggresive disease (risk of fatal mutlifocal leucoencephalopathy * **Alemtuzumab** * **​**Acts against T cells in relapsin remitting * **​Glatamiracetate** * ​Immunomodulating drug - acts as an 'immune decoy' * **Fingolimod** * ​sphingosine 1-phosphate receptor modulator, prevents lymphocytes from leaving lymph nodes. An oral formulation is available * **Teriflunomide** * **Cladribine** * **Dimethyl Fumarate** **B-interferon** and **Glatiramer Acetate** now no longer recommended by NICE in England and Wales [https://pathways.nice.org.uk/pathways/multiple-sclerosis#path=view%3A/pathways/multiple-sclerosis/disease-modifying-therapies-for-multiple-sclerosis.xml&content=view-node%3Anodes-beta-interferon-and-glatiramer-acetate](https://pathways.nice.org.uk/pathways/multiple-sclerosis#path=view%3A/pathways/multiple-sclerosis/disease-modifying-therapies-for-multiple-sclerosis.xml&content=view-node%3Anodes-beta-interferon-and-glatiramer-acetate)
58
Criteria for beta interferon use
relapsing-remitting disease + 2 relapses in past 2 years + able to walk 100m unaided secondary progressive disease + 2 relapses in past 2 years + able to walk 10m (aided or unaided) reduces number of relapses and MRI changes, however doesn't reduce overall disability
59
How would you manage symptoms of spasticity in someone with MS?
Medications * **1st line** - Baclofen, Gabapentin * **2nd line** - Tizanidine, dantrolene * **3rd line** - benzodiazepines Physiotherapy
60
How would you manage tremor symptoms in someone with MS?
**Botulinum toxin**
61
How would you manage symtpoms of urinary incontinence in someone with MS?
Important to get ulstrasound first to assess bladder emptying * **Antimuscarinic medications** - tolterodine, oxybutinin * **Teach self-Catheterisation** - if significant residual volume * Antichllinergics may improve urinary frequency
62
How would you manage symptoms of fatigue in someone with MS?
* **Amantadine** * **CBT** * **Exercise** Once other problems (e.g. anaemia, thyroid or depression) have been excluded NICE recommend a trial of amantadine other options include mindfulness training and CBT
63
How would you manage symptoms of constipation in someone with MS?
Laxatives
64
How would you manage symptoms of dysaesthesia/pain in someone with MS?
* **Amytriptilline** * **Gabapentin - also used for visual fields** * **Pregabalin**
65
How would you manage speech/swallowing difficulties in someone with MS?
SALT input
66
When should you commence treatment for urinary symptoms seen in MS?
Once residual bladder volume \> 100ml
67
Which individuals would be involved in an MDT managing someone with MS?
* **MS nurse** * **Physiotherapist** * **Occupational therapist** * **Speech and language therapist** * **Dietician** * **Rehab sepcialist** * **Continence advisor** * **Psychology/Psychiatry** * **Care workers**
68
How do individuals with MS most commonly die?
* **Bronchopneumonia** * **Renal failure**
69
How common is MS?
Affects 1 in 1000
70
Is there a genetic link with MS? Involved genes?
Minro genetic component - risk if relative is 2-4x greater than general population. HLA-DR2 HLA-DW2 (Increased liklihood northern hemispehre)
71
What is lhermitte's phenomenon?
Electric shock in arms and legs precipitated by neck flexion
72
Characteristic final stage symptoms
Spastic tetraparesis Ataxia Brainstem signs Blindness Incontience (detrouser sphincter dysnergia (loss of coordination of detrouser contraction) and sphincter relaxation Dementia
73
DDx optic neuritis
optic nerve compression
74
DDx spinal cord syndromes
Spinal crod compression, vitB12 deficiency
75
DDx brainstem syndromes
Tumours, brainstem encephalitis, cranial polyneuritis, vascular disease
76
DDx multifocal CNS disease
SLE, sarcoidosis, Behcets, sarcoid, Lymes, polyarteritis nodosa, syphillis (separated in space but not time!)
77
Good prognostic indicators
Female Touger age at onset Presents with optic neuritis Low early relapse rate
78
Bad prognostic indicators
Male Older age Motor/cerebellar signs Poor recorvery after first relapse rate High lesions load on MRI Early disability

Gwen - Neurology (38 decks)

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