Miscellaneous Neurological Disorders Flashcards

1
Q

What is the most common acquired disease of myelin?

A

multiple sclerosis

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

What is the main job of the myelin sheath?

A

The main job of a myelin layer (or sheath) is to increase the speed at which impulses propagate along the myelinated fiber.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q
  1. What is the problem in the pathophysiology of MS?
  2. What does this result in?
    2
A
  1. Body mistakenly directs antibodies and white blood cells against proteins in the myelin sheath.
    • Results in inflammation and injury to the sheath and ultimately to the nerves that it surrounds.

-The result may be multiple areas of scarring (sclerosis).

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q
  1. These areas of demyelination are found scattered in the? 3
  2. Eventually, damage can slow or block the nerve signals that control what? 4
  3. Initiating cause is what?
A
    • white matter of the brain,
    • spinal cord and
    • optic nerve (off up to disc ratio/afferent pupillary defect)
    • muscle coordination,
    • strength,
    • sensation and
    • vision.
  1. unknown!!
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Pathogenesis of MS involves what?

2

A
  1. autoimmune-mediated inflammatory demyelination

2. axonal injury

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Pathogenesis involves autoimmune-mediated inflammatory demyelination and axonal injury: Specifically what happens at the microscopic level?
3

A
  1. Peri-vascular infiltrates by lymphocytes and monocytes
  2. MHC (major histocompatibility complex) antigen expression.
  3. HLA-DR2 – increases risk
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What is MS characterized by?

2 (early and late)

A

Is characterized by

  • relapses, followed in most cases by some degrees of recovery.
  • Relapsing-remitting

Eventually progresses to continual disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

MS: Areas commonly affected (and what they control)?

7

A
  1. Optic nerve
  2. Corticobulbar tracts (speech & swallowing)
  3. Corticospinal tracts (muscle strength)
  4. Cerebellar tracts (gait & coordination)
  5. Spinocerebellar tracts (balance)
  6. Longitudinal fasciculus (conjugate gaze, EOM’s)
  7. Posterior cell columns of the spinal cord
    - –(position and vibratory sense)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

MS occurs in what ages?

A

Usually occurs between ages 15-50

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Geographically what is the risk?

A

Geographical factors:
More common in countries with temperate climates, including Europe, southern Canada, northern United States, and southeastern Australia. The reason is unknown.

Northern latitude populations have a very high incidence.
No population with a high risk for MS exists between latitudes 40N and 40S.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Potential environmental factors?

1

A
  1. Many viruses and bacteria have been suspected of causing MS, most recently the Epstein-Barr virus.

Some studies have suggested that developing infection at a critical period of exposure may lead to conditions conducive to the development of MS a decade or more later.

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

MS symtpoms?

10

A
  1. Weakness, numbness, tingling or unsteadiness in a limb
    - –“Lhermitte’s symptom’s”
  2. Unilateral visual impairment
  3. Fatigue
  4. Spastic paraparesis (what’s paraparesis??)
  5. Diplopia
  6. Disequilibrium
  7. Muscle weakness
  8. Sphincter disturbance such as urinary urgency or hesitancy
  9. Dysarthria
  10. Mental disturbance
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

MS signs?

7

A
  1. Optic neuritis (can often be the initial episode for a patient who develops MS)
  2. Opthalmoplegia
  3. Nystagmus
  4. Spasticity or hyperreflexia
  5. Babinski sign
  6. Absent abdominal reflexes
  7. Labile or changed mood
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q
MS
Younger patients
Subacute or acute onset of focal neurologic symptoms and signs reflecting?
5
(signs)5
A
  1. Optic nerve
  2. Pyramidal tracts
  3. Posterior columns
  4. Cerebellum
  5. Central vestibular system
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

MS
Older patients
Insidiously progressive myelopathy?
(signs) 3

A
  1. Spastic leg weakness
  2. Axial instability
  3. Bladder impairment
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

MS

Majority of patients have resolution of their initial symptoms, then fall into the following pattern:

A
  • -Relapsing-remitting disease
    1. Seen in majority of patients
    2. Interval of months to years after initial episode before new symptoms develop or original ones reoccur.
17
Q

What can trigger exacerbation?
3

Relapses are more likely during the 2 or 3 month following what?

A
  1. Infection
  2. fever
  3. trauma

pregnancy

18
Q

As disease progresses, increasing disability with what symtpoms?
5

A
  1. weakness,
  2. spasticity,
  3. ataxia,
  4. impaired vision
  5. urinary incontinence.
19
Q

19

A

19

20
Q

19

A

19

21
Q

How do we diagnosis Multiple Sclerosis???

2

A

MRI IV gadolinium enhances acute lesions

LP

22
Q

MS diagnosis components?

4

A
  1. Complete history and physical
  2. Signs and symptom complaints
  3. Neurological exam
  4. Neuroimaging is an adjunct to clinical information:

CT scans are NOT helpful and not sensitive!!

23
Q

What do we image in MS?

A
  1. MRI of the head or cervical cord

85% clinically definite in MS patients

24
Q

What are the two types of lesions in MS?

Occur predominantly where? 6

A
    • Multifocal
    • Hyperintense
  1. Occur predominantly in:
    - Peri-ventricular white matter
    - Corpus callosum
    - Cerebellum
    - Cerebellar peduncles
    - Brainstem
    - Spinal cord
25
Q

CSF can be helpful where MRI is not confirmatory: Usually normal but they might have what?
5

Test results can be altered in a variety of inflammatory neurologic disorders and are not specific for MS.

A
  1. Protein elevations
  2. Lymphocytosis
  3. Elevated IgG
  4. Myelin antibodies
  5. Oligoclonal bands
26
Q

How do we definitively diagnosis MS?

2

A

Definitive diagnosis requires

  1. intermittent or progressive CNS symptoms supported by evidence of
  2. two or more CNS white matter lesions occurring in an appropriately aged patient.

Clinical picture must indicate involvement from different part of the CNS at different times.

27
Q

Probable diagnosis of MS?

3

A
  1. Multifocal white matter disease but only
  2. one clinical attack, or
  3. with a history of at least two clinical attacks but signs of only one lesion.
28
Q

What is is used as a measure of disease progression by assigning a severity score (0-10) to the patient’s clinical status?

A

The Kurtzke Expanded Disability Status Scale (EDSS)

29
Q

MS Goal of therapy?

A

Treatment: Directed at modifying the course and managing primary symptoms.

30
Q

Treatment for minimally affeted patients?

6

A
1. Require no specific treatment
Encourage to maintain healthy lifestyle
2. Avoid excessive fatigue
3. Emotional stress
4. Viral infections
5. Extremes of temperatures
6. Physical therapy
31
Q

Pharmacologic treatment categories: Goals?

4

A
  1. Treat acute symptoms
  2. Modify the course of disease
  3. Interrupt progressive disease
  4. Treat continuing symptoms
32
Q

Mainstay of treatment for acute exacerbations?

What do these accomplish? 3

A

Corticosteriods

  1. Reduce inflammation
  2. Improve nerve conduction
  3. Long term administration does not alter the course of disease and can have harmful side effects.
33
Q

Drugs used to modify course of MS: One category and three drugs

A

Immune modifiers

  1. Interferon 1a
  2. Interferon 1b
  3. Glatiramer acetate
34
Q

Drugs used for progressive MS:

One category and 4 drugs

A

Immunosuppressants

  1. Methotrexate (Rheumatrex)
  2. Cyclophosphomide (Cytoxan)
  3. Mitoxantrone (Novantrone)
  4. Azathioprine (Imuran)
35
Q
  1. What is Tysabri (Natalzumab)?
  2. What does it accomplish in treatment?
  3. In what patients, would you use this in?
A
  1. a laboratory-produced
  2. monoclonal antibody
    designed to hamper movement of potentially damaging immune cells from the bloodstream, across the “blood-brain barrier” into the brain and spinal cord
  3. Because Tysabri increases the risk of progressive multifocal leukoencephalopathy (PML) it is generally recommended for patients who have had an inadequate response to, or cannot tolerate, an alternate MS therapy
36
Q

Symptomatic treatment:

  1. Spasticity? 2
  2. Fatigue? 2
  3. Bladder problems? 1
  4. Depression?
A
  1. Spasticity
    - Dantrolene (Muscle relaxant)
    - Baclofen (Muscle relaxant, antispasmodic)
  2. Fatigue
    - Modofinil (Provigil) (CNS stimulant)
    - Amantadine (anti viral)
  3. Bladder problems
    -Cholinergics
    –Bethanechol (Duvoid, Urecholine)
    These drugs contract the bladder, thus allowing complete emptying.
  4. Depression
    Antidepressants
37
Q
  1. What is Cerebral Palsy caused by?
  2. What is it not caused by? 2
  3. The early signs of cerebral palsy usually appear before a child reaches what age?
A
  1. IS caused by abnormalities in parts of the brain that control muscle movements/damage to the young developing brain
  2. NOT caused by problems in the muscles or nerves.
  3. 3 yrs of age
38
Q

Definition of Cerebral palsy?

A

A non-progressive disorder, but secondary orthopedic deformities, such as hip dislocation and scoliosis of the spine, are common.

39
Q
  1. Caused by damage to the young developing brain.
    can occur during pregnancy (about 75%). How? 5
  2. During childhood? (5%) 1
  3. After Birth? (15%) 3
A
  1. can occur during pregnancy (about 75%)
    - Infections during pregnancy
    - Insufficient perinatal oxygen
    - Prematurity
    - Rh incompatibility
  2. during childbirth (about 5%)
    - Asphyxia during labor and delivery
  3. after birth (about 15%)
    - brain injuries that occur during the first two years of life.
    - brain infections (such as meningitis)
    - head injuries (trauma, child abuse)