Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Neurology > Clinical Features of Demyelinating Diseases > Flashcards

Clinical Features of Demyelinating Diseases Flashcards

1
Q

What are common MS symptoms at onset?

A
  1. Sensory disturbance (34%)
  2. Weakness (22%)
  3. Visual loss (13%)
  4. Ataxia (11%)
  5. Diplopia (8%)
  6. Vertigo (4.3%)
  7. 2% or less=fatigue, facial pain, headache, bladder dysfunction, facial weakness, dysarthria, hearing loss, cramps, los of consciousness, psychiatric symptoms, poor memory, dysphagia, loss of taste
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

List the top 4 MS Symptoms during entire disease course:

A
  • Weakness (89%)
  • Sensory disturbance (87%)
  • Ataxia (82%)
  • Bladder dysfunction (71%)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

How can the clincal course of MS be described (4)?

A
  1. Relapsing-remitting MS (RRMS)
  2. Secondary progressive MS (SPMS)
  3. Primary progressive MS (PPMS)
  4. Progressive relapsing MS (PRMS)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Describe the RRMS course:

A
  • Relapses (“Flares”, “attacks”, “exacerbations”): acute/subacute symptoms caused by plaque formation
  • Remissions: periods of recovery of neurologic function
  • Approximately 85% of patients
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Describe the SPMS course:

A
  • 60-85% of patients with RRMS develop SPMS
  • Median time from initial flare to secondary progression = 19 years (highly variable)
  • Relapses can still occur; frequency declines (eventually stop in most patients)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Describe the PPMS and PRMS courses:

A
  • PPMS
    • 10% of patients
    • Progressive from onset without relapses/remissions
  • PRMS
    • 5% of patients
    • Progressive symptoms from onset and also rare relapses
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

How is MS diagnosed?

A
  • No specific test
  • Relies largely on clinical history and documentation of consistent signs on neurologic exam
    • “Dissemination in time and space”
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

McDonald Criteria

A

McDonald Criteria

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q
  • What is Clinically Isolated Syndrome (CIS)?
  • What is the risk of developing MS?
A
  • Clinically Isolated Syndrome (CIS) = the first clinical demyelinating event that is suggestive of MS
    • Some patients with CIS will go on to have further attacks (and therefore develop MS) while others will have no further attacks (and therefore won’t develop MS)
  • Risk of developing MS after CIS (after 14 years):
    • 88% if there are any lesions on initial MRI
    • 19% if there are 0 lesions on initial brain MRI
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

How is MS treated?

A
  • No cure currently
  • Available treatments are only partially effective in reducing MS symptoms and disability
  • Available treatments are approved for relapsing MS:
    • Interferon ß (SC and IM preparations)
    • Glatirameracetate (SC)
    • Natalizumab(IV)
    • Fingolimod(PO)
    • Teriflunomide(PO)
    • Dimethyl fumarate(PO)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What is the most common inflammatory optic neuropathy?

A

Demyelinating Optic Neuritis (DON)

  • Association with MS
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What are the demographics of DON?

A
  • Mean age = 31.8
  • F>M (3:1)
  • More common in Caucasians
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

How is DON diagnosed?

A

Clinical diagnosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What are the characteristic symptoms of DON?

A
  • Acute-subacute vision loss
  • Pain (92%) typically worse with eye movements
  • Decreased color vision
  • Phosphenes (lights, sparkles, shifting squares)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What are the characteristic signs of DON?

A
  • Optic neuropathy
    • Decreased visual acuity (any level)
    • Dyschromatopsia
    • Visual field defect
    • Decreased contrast sensitivity
    • Relative afferent defect
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q
  • What is the recovery time of DON?
  • How is DON treated?
  • What is the long term prognosis?
A
  • Recovery typically fairly prompt and nearly complete
    • begins within 1 month, with slower recovery over 12-18 months
  • IV steroids increase the rate at which vision recovers
    • Oral prednisone increased risk of recurrent optic neuritis
    • Long-term visual prognosis same with or without steroids​
  • Excellent long-term prognosis
17
Q

How is DON related to MS?

A
  • IV steroids may impart a short-term protective effect against MS
    • Protective effect lost beyond 2 years
  • Long-term MS risk stratification based on brain MRI
  • Other risk factors:
    • CSF OCBs, CSF IgG synthesis, abnormal SSEPs, abnormal VEPs
18
Q

Acute Disseminated Encephalomyelitis (ADEM):

  • Definition:
  • Onset:
  • Demographics:
  • Patients are often ….
A
  • Acute or subacute polysymptomatic onset that affects multifocal areas of the CNS and must include encephalopathy (behavioral change or alteration in consciousness)
  • Typically monophasic, but may be recurrent or multiphasic
  • More common in children
  • Patients are often very ill (coma common)
19
Q
  1. ADEM usually follows ….
  2. What does MRI show?
  3. What is seen in the CSF?
A
  1. Often follows an acute infectious illness or vaccination
    • Believed to be induced by an immune reaction directed at a cross-reacting myelin antigen
  2. MRI usually shows multiple/extensive lesions
    • Usually both cerebral hemispheres
    • Involvement of deep gray matter common (more so than in MS)
    • Optic nerves and spinal cord may be involved
  3. CSF usually shows increased WBCs
20
Q

How is ADEM diagnosed?

A

Diagnosis of exclusion

21
Q

ADEM

  • Brain Biopsy:
  • Treatment:
  • Prognosis:
A
  • Brain Biospy: perivenous demyelination, typically distributed in sleeves surrounding areas of perivascular inflammation
  • Treatment: high-dose IV corticosteroids
  • Prognosis: generally favorable
22
Q
A
23
Q

Define Transverse Myelitis:

A
  • Syndrome of acute or subacute myelopathy accompanied by indicators of inflammation (either radiologically or based on spinal fluid)
  • Heterogeneous collection of disorders
    • May occur as a stand-alone syndrome (idiopathic), a relapse of MS or NMO, a component of ADEM, or a nondemyelinating syndrome (ex. infectious, granulomatous)
24
Q

What are the clinical s/s in transverse myelitis?

A
  • Most patients present with a combination of sensory, motor, and bladder/bowel-related symptoms
  • Lhermitte Sign: electrical or dysesthetic sensation in the spine or limbs elicited by neck flexion
  • Paroxysmal tonic spasms: repeated, brief (30-90 sec) stereotyped attacks of painful limb or truncalmuscle spasms, often triggered by limb movements
25
Q

How is transverse myelitis classified and what can the classes suggest?

A
  1. Complete = relatively symmetric moderate-severe loss of motor and sensory modalities caudal to the level of the lesion
    • Suggests a monophasic disorder or relapsing NMO
  2. Incomplete = partial or patchy involvement of at least one spinal segment with mild to moderate weakness and asymmetric or dissociated sensory symptoms
    • More likely to herald MS, with high risk for future relapses
26
Q

What is the treatment and prognosis of transverse myelitis?

A
  • Treatment (non-infectious):
    • High dose IV corticosteroids
      • optional oral prednisone taper
    • Severe attacks: Plasma exchange
  • Prognosis: depends on etiology
    • Partial cord syndromes in MS are typically mild to moderate, plateau after days to 4 weeks, and patients typically recover well
    • Complete cord syndromes are more likely to result in substantial residual deficits
27
Q

Define Central Pontine Myelinolysis:

  • What is the most common cause?
A

Demyelinating condition due to severe osmotic stress

  • Most commonly caused by overly-rapid correction of hyponatremia in patients with conditions predisoposingto nutritional/electrolyte stress
28
Q

What are the symptoms of central pontine myelinolysis?

A
  • Neuropsychiatric
    • emotional lability, disinhibition, bizarre behaviors
  • Neurologic
    • ​confusion, impaired cognition, dysarthria, dysphasia, gait instability, weakness/paralysis, seizures
  • Demyelination can occur outside of the pons (“extra-pontine myelinolysis”)
29
Q

Define Neuromyelitis Optica(NMO, “Devic disease”):

A

Severe demyelinating disease which preferentially involves the optic nerves and spinal cord

30
Q

How does NMO present?

A
  • **Typical presentation **= Acute optic neuritis preceded or followed by myelitis
  • Vision loss often severe, 20% bilateral
  • Periorbital pain less common than in idiopathic ON
  • May be monophasic or relapsing (70%)
31
Q

NMO

  • Optic disc may appear ….
  • What is typically seen on MRI?
  • What is shown on spinal MRI?
  • What is seen in the CSF?
A
  • Optic disc may appear:
    • normal or swollen acutely
    • pallor chronically
  • MRI typically shows scattered areas of demyelination,
    • concentrated in the optic nerves, chiasm, spinal cord
  • Spinal MRI:
    • lesions are longitudinally extensive
    • ≥3 vertebral segments
  • CSF:
    • may be moderate pleocytosis (>50 cells)
    • OCBs in 20-30% (70-90% in MS)
32
Q

How is NMO diagnosed?

A
  • No definitive diagnostic test
  • Aquaporin-4 IgG autoantibody (73% sensitivity, 94% specificity serum)
    • Antibody targets aquaporin channel located in astrocyticfoot processes of the BBB

Neurology (26 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions