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s6- neuro 2 > Week 6- Amyotrophic Lateral Sclerosis (ALS) > Flashcards

Week 6- Amyotrophic Lateral Sclerosis (ALS) Flashcards

1
Q

PART 1: INTRODUCTION

A

PART 1: INTRODUCTION

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2
Q
  • Amyotrophic = _______
  • Lateral = ________
  • Sclerosis = _________
A
  • Amyotrophic = A-without, Myo-muscle, trophic-nourishment
  • Lateral = defines area in spinal cord where portions of nerve cells that signal and control muscles are located
  • Sclerosis = scarring/hardening in region
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3
Q
  • ALS is the most common and devastatingly fatal of all _____ _______ diseases.
  • Mortality typically occurs in __-__ years with highly variable symptoms.
A
  • motor neuron diseases

- 2-5 years

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4
Q
  • ALS is characterized by progressive degeneration and loss of motor neurons in _________, __________, and ____________
  • Does it involve the destruction of UMN or LMN?
  • Degeneration of anterior horn cells and descending _________ and ________ tracts.
A
  • spinal cord, brain stem, and motor cortex
  • Both UMN and LMN
  • corticobulbar and corticospinal tracts
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5
Q
  • Onset of ALS is often >___ years but can affect younger people.
  • Does it occur in men or women more often?
A
  • > 50 years

- Men > Women (1.5:1)

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6
Q

Describe the pathophysiology of ALS.

A
  • Demyelination and gliosis of the motor cortex, corticospinal and corticobulbar tracts resulting from degeneration of UMN cell bodies in the motor cortex.
  • As disease continues the degeneration continues in areas adjacent to original site of onset adjacent to contralateral sites of onset.
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7
Q

ALS only targets ______ neurons.

A

-motor neurons

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8
Q

What is the cause of ALS?

A

-Ultimately unknown

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9
Q

What is the biggest pathophy etiology related to ALS?

A

-Glutamate excitotoxicity resulting in increased levels in CSF, plasma, post-mortum tissue.

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10
Q

_______ ALS makes up about 90% of cases, however, 5-10% are genetic.

A

-Sporatic ALS

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11
Q

ALS Risk Factors:

  • ______ > ______
  • Caucasian, non-Hispanic minorities
  • Geographical clusters
  • Family Hx
  • Age (__-__)
  • Prior trauma or TBI
A
  • Males > Females

- Age (55-75)

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12
Q

Diagnosis of ALS is very difficult and is largely diagnosed by _________ due to lack of biomarkers.

A

-exclusion

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13
Q

Clinical diagnosis of ALS requires a pattern of observed and reported symptoms of both _____ and _____ as well as a _________ ________ in physical function that cannot be attributed to other disorders.

A
  • UMN and LMN

- persistent decline in physical function

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14
Q

Diagnostic Criteria (El Escorial criteria):

Exclusion of all other Dx + Progressive Functional Decline +
-Progressive UMN and LMN deficits in at least one limb or region of the human body
OR
-LMN deficits in at least one region as defined by clinical examination and/or by EMG in two body regions

A

1

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15
Q

PART 2: ALS CLINICAL PRESENTATION AND SUBTYPES

A

PART 2: ALS CLINICAL PRESENTATION AND SUBTYPES

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16
Q

What is the cardinal sign of ALS?

A

Muscle weakness

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17
Q

Muscle Weakness:

  • Most common impairment: focal, __________ muscle weakness beginning in LE or UE or bulbar weakness.
  • ____ weakness&raquo_space; ____ weakness
  • ____ and __&__ muscles normally sparred until terminal stages.
A
  • asymmetrical
  • LMN weakness&raquo_space; UMN weakness
  • Eye and B&B muscles spared
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18
Q

What are some other impairments that may be seen following a diagnosis of ALS? (6)

A

-UMN Impairments (Spasticity, hyperreflexia, clonus, pathological reflexes, weakness)
-LMN Impairments (Hyporeflexia, hypotonicity, atrophy, fasciculations, weakness)
-Bulbar impairments (Spastic bulbar palsy or flaccid bulbar palsy; dysarthria, dysphagia, sialorrhea)
-Respiratory Impairments (Decreased respiratory muscle strength, reduction of VC up to 50% typically when symptoms arise)
-Cognitive Impairments (VARIABLE EVIDENCE ON PREVALENCE. Verbal fluency, language comprehension, memory, abstract reasoning)
-Additional Impairments
(Pain, fatigue; ANS symptoms (1/3 of cases))

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19
Q

_______ pathways, _______ motor function, and ______________ control are spared for majority of disease course

A

-Sensory pathways, ocular motor function, and sphincter (bowel/bladder) control spared until late stages.

20
Q

1

A

1

21
Q

1

A

1

22
Q

1

A

1

23
Q

1

A

1

24
Q

11

A

1

25
Q

1

A

1

26
Q

What are the (3) clinical subtypes of ALS?

A
  • Classical ALS (limb-onset vs bulbar-onset)
  • Primary Lateral Sclerosis (PLS)
  • Progressive Spinal Muscular Atrophy (SMA)
27
Q

What are 2 other lesser types of ALS?

A
  • ALS with Frontotemporal Dementia (FTD)

- Familial ALS

28
Q

Classic ALS:

  • __/__ of ALS population, more common in men.
  • Involvement of UMN or LMN?
  • Either _____-onset or ______-onset.
  • What are the early signs of Classic ALS?
  • With additional bulbar involvement, there is wasting of the _________ and muscles for speech and swallowing.
A
  • 2/3rds
  • UMN and LMN
  • limb-onset or bulbar-onset
  • progressive limb weakness, reduced dexterity, wasting of hands, fasciculations
  • tongue
29
Q

Is limb-onset or bulbar-onset Classic ALS more common?

A

-Limb-Onset

30
Q

Limb-Onset Classic ALS:

  • __-__% of cases (most common presentation of ALS)
  • First symptoms are a result from damage to motor neurons in _________ specifically ________ tracts.
  • Are distal or proximal muscles affected first?
A
  • 70-80%
  • damage to motor neurons in motor cortex, specifically corticospinal tracts
  • Distal (fasciculations, atrophy, weakness, spasticity)
31
Q

Bulbar Onset Classic ALS:
-__-__% of cases.
-Does it progress faster or slower than limb-onset ALS?
First symptoms are a result of damage to motor neurons in _________, specifically __________ tracts (face, head, neck muscles).
-Affects CN ___-___ causing dysarthria and dysphagia.
-More common in older _______.

A
  • 20-30%
  • progresses faster than limb-onset
  • damage to motor neurons in brainstem, specifically corticobulbar tracts
  • CN IX-XII
  • older females (2:3)
32
Q

Primary Lateral Sclerosis (PLS):

  • Rare condition affecting __% of those with ALS.
  • ____ S/Sx only.
  • If the LMNs are not affected within __ years, the disease usually remains a pure upper motor neuron disease.
  • Key examination findings can include spasticity, UMN pattern weakness, and pseudobulbar findings.
  • What does the prognosis of these patients look like?
A
  • 5%
  • UMN
  • 2 years
  • Relatively good with life expectancy comparable to normal!!
33
Q

Progressive Spinal Muscular Atrophy (SMA IV):

  • __-__% of cases
  • ___ S/Sx only.
  • Mainly ____ involvement.
  • Does it affect men or women greater?
  • May be limited to one region, examples include ___________ syndrome and _________ syndrome.
  • Progression is highly variable (slow or rapid) and the median survival duration after onset is ___ months longer that patients with ALS.
A
  • 5-10%
  • LMN
  • limb
  • Men > women (2:1)
  • flail arm syndrome (cervical), flail leg syndrome (lumbosacral)
  • 12 months
34
Q
  • Flail Arm Syndrome = _______

- Flail Leg Syndrome = _______

A
  • Flail Arm Syndrome = bilateral symmetrical

- Flail Leg Syndrome = bilateral asymmetrical

35
Q

ALS with Frontotemporal Dementia (ALS-FTD):

  • Cognitive symptoms and dementia once thought to be uncommon ALS symptom. Recent studies suggest that roughly ___% of patients with ALS demonstrate mild-mod cognitive and/or behavioral impairments without dementia and ___% of individuals with ALS will develop FTD.
  • What are some common deficits with these patients?
  • This can adversely affect compliance. Clinical course is usually more rapid in patients with ALS-FTD.
A
  • 30% cog/behavioral impairments, 20% develop FTD
  • Common deficits involve executive dysfunction (language or personality) and can cause problems with judgement, impulsivity, decreased ability to handle routine tasks.
36
Q

Familial ALS:

  • __-__% of cases
  • Most cases are inherited in an autosomal _______ pattern.
  • What is the common onset?
  • No difference between Familial ALS and _______ ALS on neurological examination.
A
  • 5-10%
  • autosomal dominant pattern
  • 40s-50s
  • Sporadic ALS
37
Q

PART 3: STAGING, MEDICAL MANAGEMENT, PROGNOSIS

A

PART 3: STAGING, MEDICAL MANAGEMENT, PROGNOSIS

38
Q

How many stages of ALS are there?

A

-6 Stages

39
Q

Describe the progression of ALS from stage I-III.

A

Stage I: early disease, mild focal weakness, asymmetrical distribution, symptoms of hand cramping and fasciculations

Stage II: moderate weakness in groups of muscles, some wasting (atrophy) of muscles; modified independence with assistive devices

Stage III: severe weakness of specific muscles, increasing fatigue, mild to moderate functional limitations, ambulatory

40
Q

Describe the progression of ALS from stage IV-VI.

A

Stage IV: severe weakness and wasting of LEs, mild weakness of UEs; moderate assistive and assistive devices, wheelchair user

Stage V: progressive weakness with deterioration of mobility and endurance, increased fatigue, moderate to severe weakness of whole limbs and trunk, spasticity, hyperreflexia, moss of head control, maximal assist

Stage VI: bedridden, dependent ADLs, FMS; progressive respiratory distress

41
Q

Is there an effective treatment for ALS?

A

-NO

42
Q

Where does most of treatment go if there is no effective treatment/cure?

A

-Symptomatic treatment

43
Q
  • What is the #1 disease modifying drug used to prolong survival by at least a few months and slows progression of ALS?
  • This drug is specifically thought to help slow _______-onset ALS.
A
  • Riluzole

- bulbar-onset

44
Q

What is the main outcome measure used for ALS?

A

-ALS Functional Rating Scale (ALSFRS-R)

45
Q

ALSFRS-R:

  • Measures _____ and ______ function across 12 functional categories.
  • Used to assess what 2 things?
  • Total possible score 48 with higher scores reflecting _______ performance.
A
  • ADL and global function
  • Used to assess response to treatment or disease progression
  • higher scores = worse performance
46
Q

Prognosis:

  • Average mortality rate __-__ years with ultimately respiratory failure.
  • What are some negative prognostic indicators?
  • What are positive prognostic indicators?
A
  • 2-5 years
  • bulbar-onset, early respiratory signs
  • patients who participate in multidisciplinary clinics

s6- neuro 2 (18 decks)

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