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 attacks 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?

What are factors that are linked to exacerbations or relapse?

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 attacks,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

Conider a fan when in the gym or a cooling vest. Or havig the pt workout first thing in the moring when their body temp is the lowest

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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 little 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 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)
18
Q

What type of disease is ALS?

A

Its a Motor Neuron Disease
- It can either be inherited or Sporadic (no family hx)
- This is a disorder of UMN, LMN or a combination of both
- This is incurable

Limb ALS is more common than Bulbar ALS

19
Q

What are Known Risk Factors for ALS?

A
  • Male
  • Age (Late 50s)
  • Family Hx
  • Disease-causing Mutations (Ex. SOD1)
  • Clusters (Western pacific form of ALS - Guam and Japan)
20
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

21
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 (These are generally spared)
22
Q

What takes place in the Early stages of ALS?

A
  • In the early stages of the disease the body can compensate with the muscles that are no longer recieveing innervation through a process called Collateral Sprouting. This is where a healthy axon surrounding the area begin to sprout and they innervate the synaptic sites that were previously activated by the damaged neurons. So the body preserves strength and function but only through a period of time
23
Q

As ALS progresses, the sprouting and re-innervation can’t compensate for the rate of degeneration, what occurs from this?

A
  • The progression of ALS is spread in a Contiguous Manner, meaning it’s spread locally within an area before moving to other areas
  • After this there is a Rostral/Caudal spread of this disease
    –Caudal to rostral progression in the spinal cord occurs faster that rostal to caudal
24
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, stage of disease
  • Typically associated with symtoms presenting distal to proximal
25
Q

What are impairments related to LMN Pathology?

A
  • Muscle weakness: Cardinal Sign, this is more significant that weakness of UMN loss
  • Hyporeflexia
  • Fatigue
  • Hypotonicity
  • Atrophy
  • Muscle cramps
  • Fasciculations
26
Q

What are impairments related to UMN Pathology?

A
  • Spasticity
  • Hyperreflexia
  • Clonus
  • Pathological Reflex (Babinski/Hoffmans)
  • Weakness
27
Q

What are impairments related to Bulbar Pathology?

A
  • Bulbar Muscle weakness
  • Spastic/flaccid bulbar palsy
  • Dysarthria (Inability to articulate)
  • Dysphagia
  • Sialorrhea (Increased salivation or drooling)
  • Pseudobulbar (PBA) (affects emotions)
28
Q

What is the difference between limb ALS and Bulbar ALS?

A

Limb ALS onset begins more distally and works it way proximally, Bulbar onset attacks speech and swallowing which is more dangerous for the patient

29
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
30
Q

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

A

No definitive diagnostic test for ALS

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

31
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
32
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

33
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

34
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

35
Q

What are the Disease-Specific Outcome Measures for ALS?

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