Neuromuscular Multiple Sclerosis (MS) and ALS Flashcards

1
Q

What is the Epidemiology of MS?

A
  • Onset typcically 20-40 years, more likely affecting woman
  • Caucasions highest risk
  • Living above 40° latitue (unknown reason)

These are important for Differential Diagnosis

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2
Q

What is MS?

What may increase the chances of getting MS?

A

A chronic demyelinating disease of the CNS
- An autoimmune disease
- Viral infection triggers immune response
- This can happen in the brain or in the spinal cord

  • May be genetic susceptibility
  • Increased risk with Vitamin D deficiency and smoking
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3
Q

With MS, lesions (plaques) can occur anywhere in the CNS. What are common structures that are attacked by this autoimmune disease?

A

MS typically attacts white matter

  • Optic pathway
  • Corticospinal tract
  • Dorsal Column of spinal cord
  • Cerebellar peduncles

These are all susceptible to attacts

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4
Q

What is the Pathophysioligy of MS?

A

When a person is exposed to a virus, this sets off an immune response. This inadvertently fights off or attacks the myelin in the CNS.
- Due to this nerve conduction is impaired and slow, if the myelin is greatly damaged it can block conduction
- Oligodendrocytes will also be affected by the immune response

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5
Q

What are common impairments of MS?

This is based on the sturcutres typically attacked, optic pathway, corticospinal tract, dorsal column, and cerebral peduncels

A
  • Optic pathway: Blurred vision, altered acuity
  • Corticospinal Tract: Paresis/Plegia, spasticity
  • Dorsal Column: Proprioception, Parestesias (Pins and needles), dysesthesias
  • Cerebral Peduncels: Balance, coordination, tremor, ataxia, hypotonia, vestibular disorders, dysmetria, dysdiadochokinesia
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6
Q

What are Sx of MS?

(Theres a lot)

This is based on the sturcutres typically attacked, optic pathway, corticospinal tract, dorsal column, and cerebral peduncels

A
  • Sensory
  • Pain
  • Visual
  • Motor
  • Fatigue
  • Coordination and Balance
  • Gait/mobility
  • Speech and swallowing
  • Depression
  • Emotional
  • Cognitive
  • Bladder and Bowel (may have incontience, increases risk of infection)
  • Sexual

Bolded are more specific to MS

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7
Q

MS

What are Exacerbations?

A

These are new and recurrent MS symptoms lasting more than 24 hours
- Multiple bouts of exacerbations over a 1 year period is needed for diagnosis

Factors that are linked to increased risk of exacerbations or relapse:
- Viral or bacterial infection (common cold, FLU, UTI, sinus infection, etc)
- Disease of major organs systoms (Hepatitis, pancreatitis, asthma attacts,etc)
- Stress

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8
Q

MS

What are Pseudoexacerbations?

A

These is a temporary worsening of symptoms; resovled within 24 hours

  • The most common one is called Uthoff’’s syndrome: Heat sensitivity - Patients will have temporary worsening of Sx (Overexertion, exposure to heat)

Considerations for those in south FL
Precautions in warm gyms and aquatic therapy

A large number of individuals suffer from this

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9
Q

Clinical Subtype of MS

What is Relapsing-Remitting MS (RRMS)?

A

This is the most common (85% of pts have this type)

  • This is characterized by acute attacks or relapses, followed by partial or full revovery or remissions.
  • Of all the subtypes this has the best prognosis
  • However at some point, the oligodendrocytes get wiped out and they are unable to fully remelinate the nerves, and the patients ability to fully recover goes down.
  • At this point when the pt relapses and remites they cant go to baseline anymore, this is called permanent deficit
  • These patient then progress to Secondary Progresive MS

Remission can last weeks, months , or years. Symptoms may be a litle worse then the 1st relapse.
Ex. if a patient goes through their first relapse and they have blurred vision and then they remit back to baseline. After the pt remits after a period of time they may relapse again but this time their sx are worse they may have foot drop and paresthesia in addition to the blurred vision. But after some time they remit once again back to baseline. However, once the patient losses all the oligodendrocytes they cannot full recover or get back to baseline (right side of pic). So when they have a relapse and remission they do not get back to baseline anymore. So now if the patient may have permanent foot drop

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10
Q

How does Secondary Progressive Relapse (SPMS) begin?
How is it characterized?

A
  • It begins with a Relapsing Remitting Course, followed by a progression to Secondary Progressive MS
  • This is characterized by a steady and irreversible decline with or without acute attacks (relapse)
  • Whether the person has those acute attacks or not, they are never recover/remit and they continue to lose function over time
On the (L) is RRMS, on the (R) is SPMS
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11
Q

What is Primary Progressive MS?

A

This type is not as common ~10% have this subtype

  • This is characterized by a steady functional decline from onset
  • In this subtype there are no attacks or exacerbations and there are no periods of remission
  • Overtime the Sx get worse and worse at a steady decline
  • There are periods of platues, where the patient does not get worse but after the platues are over they continue to get further away from the baseline
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12
Q

What is Progressive Relapsing MS (PRMS)?

A

Least common: 5%, however most severe

  • Its characterized by a steady deterioration from onset
  • Is this subtype the patient are in a steady decline from onset and they have occasional acute attacks and the Sx get way worse (or heightened) and after the relapse they continue on their steady decline without remission (recovery)
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13
Q

How is Relapse-Remitting MS (RRMS) clincally Diagnosed?

A

When the patient has experienced at leat 2 attacks (exacerbations) or relapses that last more than one day and are separated by more than 1 month

For example, if a pt complains of blurred vision that lasted 2 days and then a month and a half later complained of foot drop that lasted for a few days will be RRMS.

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14
Q

**

How is Primary Progressive MS Clinically Diagnosed?

A

The pts impairments need to be present for greater than 6 months

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15
Q

What test are used to Diagnose MS?

A
  • Lumbar Puncture: This is the CSF analysis. Doctors look for evidence of myeline proteins and elevated IgG proteins in the CSF cells. (You’ll get little bits of myelin floating around in the CSF as well as IgG bands, if those are present the test would be positive)
  • Evoked Potentials (EP): This test measures the electrical activity of the brain in response to stimulation of a specific sensory nerve pathway. It can detect the slowing of electrical conduction caused by demyelination. (Often looking at the optic pathway, because its a common finding with MS)
  • MRI: This is the perferred imaging method, to help with diagnosis and help monitor the course of the disease. Pts with MS can have both acute lesions, which are areas of inflammation on their brains and spinal cord and chronic plaques, which are scars in the myline sheath
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16
Q

What are the 3 categories of Medical Management of MS? What is the role of each category?

A
  • The first category includes meds. that are used to help slow down disease progression by treating and helping prevent attacks

-These are Anit-inflammatory and immunosuppressive drugs, they are administered during acute exacerbations/attacks

  • Second category is disease modifying or immunomodulating drugs, these help slow the progression of neurological disablilty and reduce overall disease activity. Administerd at time of remission
  • The third category are for Sx relief, this varies patient to patient
17
Q

What is the Medical Management of Acute Relapses for MS?

A

First we have to suppress the immune system, second we have to reduce the inflammation repsonse, by doing this we try to shorten the duration of the attack and lessen its effects

  • Typically Corticosterioids, which are anti-inflammatory and immunosuppressive are given at high doses.
    -They are administered through IV for a brief course usually, 3 to 5 days
    -Followed by a taper dosage of oral medication over a period of 1 to 3 weeks
  • Also the use of Plasmapheresis are also used to treat acute attacks
    -However its only for those pts who have Relapse Remitting MS (RRMS)
    -The pt blood is drained and pulled out of their body and plasma is separated out, the goal with this is to filter out the immune cells in the plasma. After, plamsma is put back into the blood and back into the pts body
18
Q

What is the Medical Management of Disease Modifying for MS?

A
  • This is the use of Immunomodulating drugs (synthetic interferon) and they are given during times of remission and act to prevent worsening of disease progression.
    (It modulates the immune system, does not suppress it)
    -If a patient is on this med. while an attack, they can help reduce the severity of the attack
19
Q

What is the Medical Management of Symptom for MS?

A

Symptom relief will vary patient to patient. This account for intensity of Sx and the type of Sx.

Sx treated can be for:
- Spasticty
- Pain
- Fatigue, etc.

20
Q

What is the General Framework for Rehabilitation for MS?

A

MS is chroic, neurodegenerative. We must consider several approaches and the stage of the disease

  • Restorative Approach: This is used a lot with RRMS to restore function to the origional functional level (Baseline)
  • Compensation Approach: This is better for the other 3 subtypes when the patient is progressively getting worse
21
Q

What questions should be asked with MS patients?

A
  • What subtype of MS do you have?
  • When was the first exacerbation? How often do you get exacerbations and what do they look like?
  • What was functionality before the exacerbation vs after remission?
  • How do you do in Heat? At what temp. do you get uncomfortable to exercise?
  • What causes your fatigue?
  • What Meds. are you taking and what is the schedule for said meds?
22
Q

What Outcome Measure are used for MS?

A
  • Expanded Disability Status Scale (EDSS): Gold Standard
  • MS Functional Scale (MSFC)
  • MS Quality of Life-54 (MSQOL-54)
  • MS Impact Scale (MSIS-29)
23
Q

What type of disease is ALS?

A

Its a Motor Neuron Disease
- It can either be inherited or Sporadic
- This is a disorder of UMN, LMN or a combination of both
- This is incurable

Limb ALS is more common than Bulbar ALS

24
Q

What are Known Risk Factors for ALS?

A
  • Male
  • Age (Late 50s)
  • Family Hx
  • Disease-causing Mutations
  • Clusters (Western pacific)
25
Q

What is the Pathophysiology of ALS?

A

This is a progressive degeneration and loss of motor neurons in the SC, brainstem, and motor cortex

26
Q

Pathophysiology

What does ALS typically affect?

A
  • UMN in cortex
  • Corticospinal tracts
  • Brainstem nuclei for CN (V, VII, IX, X, XII)
  • Anterior Horn cells in SC
  • Sensory system and spinocerebellar tract
27
Q

What are the Clincial Manifestations for ALS?

A
  • Depends on extent of motor neuron loss, degree and combination of UMN and LMN loss, pattern of onset and porgression, body regions affected stage of disease
  • Typically associated with symtoms presenting distal to proximal
28
Q

What are impairments related to LMN Pathology?

A
  • Muscle weakness
  • Hyporeflexia
  • Fatigue
  • Hypotonicity
  • Atrophy
  • Muscle cramps
  • Fasciculations
29
Q

What are impairments related to UMN Pathology?

A
  • Spasticity
  • Hyperreflexia
  • Clonus
  • Pathological Reflex (Babinski)
  • Weakness
30
Q

What are impairments related to Bulbar Pathology?

A
  • Bulbar Muscle weakness
  • Spastic/flaccid bulbar palsy
  • Dysarthria (Inability to articulate)
  • Dysphagia
  • Sialorrhea (Increased salivation)
  • Pseudobulbar (PBA) (affects emotions)
31
Q

With ALS, what are other impairmets that may happen as the disease moves proximally?

A
  • There may be respiratory issues
  • There may be cognitive issues
32
Q

What is required in order to be diagnosed with ALS? What must there be a absense of?

A

Requires presence of:
- LMN signs
- UMN signs
- Progression of disease within a region or to other regions (not local)

Absence of:
- Electrophysical and pathological evidence of other disease
- Neuroimaging evidence of other disease processes

33
Q

What is the Disease course and prognosis of ALS?

A
  • Variable: Average duration 27-32 months
  • Death within 3-5 years, usually from respiratory failure
  • < 35-40 years have better 5 year survival rates
  • Survival time greater with Limb onset than bulbar onseet
34
Q

With the Framework for Rehabilitation, what may you see in the Late Stage of ALS in terms of impairments, Activity Limitations and Participation Restrictions?

A

Impairments: Numerous and Severe
Activity Limitations: Becomes dependent in all aspects or mobility and self-care
Participation Restrictions: Dependent

35
Q

With the Framework for Rehabilitation, what may you see in the Early Stage of ALS in terms of impairments, Activity Limitations and Participation Restrictions?

A

Impairments: Varitey of abnormal signs and symptoms
Activity Limitations: Minor limitations present
Participation Restrictions: None

36
Q

With the Framework for Rehabilitation, what may you see in the Middle Stage of ALS in terms of impairments, Activity Limitations and Participation Restrictions?

A

Impairments: Increased number and severity
Activity Limitations: Minimal to moderate limitations
Participation Restrictions: Begins to develop

37
Q

What are the Disease-Specific Outcome Measures for ALS?

A
  • ALS Functional Rating Scale (ALSFR)
  • ALS Assessment Questionnaire (ALSAQ-40)