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als Flashcards

1
Q

als age onset

A

> 50

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2
Q

ALS is ____ of diseases

A

Most common and devastatingly fatal of all motor neuron diseases

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3
Q

gender als

A

male slightly

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4
Q

patho als

A

Demyelination and gliosis of the motor cortex, corticospinal and corticobulbar tracts

Degeneration of UMN cell bodies in motor cortex

Damage to alpha motor neurons (LMN) of spinal cord

= UMN AND LMN DAMAGE

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5
Q

etiology als

A

Unknown
Viral/autoimmune/toxic?
Glutamate Excitotoxicity
Increased levels in CSF, plasma, post-mortem tissue
5-10% genetic (autosomal dominant)

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6
Q

dx of exclusion by ruling out what

A

Differential diagnosis: cervical or lumbar myelopathy, syringomyelia, MS, LMN pathology, heavy metal toxicity, paraneoplastic syndromes, myopathies, and Lyme Disease must all be excluded before diagnosis of ALS is made!

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7
Q

what to look for in CSF als

A

Creatine phosphokinase levels higher in ~70% cases
Higher in patients with limb-onset

glutemate exitotoxicity

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8
Q

Clinical diagnosis of ALS requires a pattern of observed and reported symptoms of __________ as well as a _______

A

Clinical diagnosis of ALS requires a pattern of observed and reported symptoms of both UMN and LMN as well as a persistent decline

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9
Q

El escorial dx als

A

Exclusion of all other Dx + Progressive Functional Decline AND
Progressive UMN and LMN deficits in ≥ 1 region of the human body
or
LMN deficits in ≥ 1 limb/region as defined by clinical examination
And/or by EMG in 2 clinical regions

Region: defined as bulbar, cervical, thoracic, lumbosacral
Leads to either: suspected, possible, probable, and definite ALS

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10
Q

gold coat dx of als

A

The Gold Coast Criteria for Diagnosis of ALS
Investigations excluding other disease processes, AND

Progressive motor impairment documented by history or repeated clinical assessment, preceded by normal motor function, AND

Presence of UMN and LMN signs in ≥ 1 region (with UMN and LMN dysfunction noted in the same body region if only one body region is involved) or LMN dysfunction in ≥ 2 regions

Region: defined as either bulbar, UE, or LE 
Leads to either: + ALS or - ALS
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11
Q

cardinal sign of als

A

muscle weakness

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12
Q

weakness caused by als

A

Can be due to UMN and/or LMN involvement
LMN weakness&raquo_space; UMN weakness
Eye, bowel and bladder muscles normally spared until terminal stages

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13
Q

UMN impairments

A

Spasticity, hyperreflexia, clonus, pathological reflexes, weakness

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14
Q

LMN impairments

A

Hyporeflexia, hypotonicity, atrophy, fasciculations, weakness

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15
Q

bublar impariments

A

Spastic bulbar palsy or flaccid bulbar palsy; dysarthria, dysphagia, sialorrhea (excessive salivation)

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16
Q

respiratory impairments als

A

Respiratory Impairments
Decreased respiratory muscle strength, reduction of VC up to 50% typically when symptoms arise
VC of 25-35% = significant risk of impending respiratory failure or death

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17
Q

cog impairments als

A

Verbal fluency, language comprehension, memory, abstract reasoning
More common in bulbar onset

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18
Q

additional impairments als

A

Pain, fatigue; ANS symptoms (1/3 of cases

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19
Q

less common till end of disease course

A

*Sensory pathways, ocular motor function, and sphincter (bowel/bladder) control are spared for most of the disease course
^with longer survival rates, we CAN see dysfunction here towards the very end stages of the disease course

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20
Q

classical ALS early signs

A

Early signs: progressive limb weakness, reduced dexterity, wasting of hands, muscle fasciculations
With additional bulbar involvement, there is wasting of the tongue, and muscles for speech and swallowing

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21
Q

Limb onset als

A

~70-80% of cases (most common presentation of ALS)
First symptoms are a result from damage to motor neurons in motor cortex, specifically corticospinal tracts
Distal  Proximal

22
Q

bublar onset als

A

~20-30% of cases. Progresses faster than limb-onset
First symptoms are a result of damage to motor neurons in brainstem, specifically corticobulbar tracts (face, head, neck muscles)
Affects cranial nerves IX to XII causing dysarthria and dysphagia
Tongue atrophy, tongue fasciculations
Vocal cord spasms
More common in older females with male:female

23
Q

primary lat sclerosis s/s

A

Upper motor neuron signs and symptoms ONLY
Key examination findings can include spasticity, upper motor neuron pattern weakness, and pseudobulbar findings

24
Q

primary lat sclerosis can _________

A

Can progress to Classical ALS
If the lower motor neurons are not affected within two years, the disease usually remains a pure upper motor neuron disease

25
Q

prognosis for primary lateral sclerosis

A

VERY different prognostic story: relatively good with life expectancy comparable to normal!

26
Q

SMA sex

A

Mainly affects men (male:female ratio: 2:1)

27
Q

SMA s/s

Hint what can develop later and regions

A

Lower motor neuron involvement ONLY
flaccid weakness, atrophy, fasciculation and hyporeflexia or areflexia
Mainly limb involvement
Bulbar palsy can develop later
May be limited to one region:
Flail arm syndrome (cervical): “Man-in barrel syndrome”
Flail leg syndrome (lumbosacral)

28
Q

SMA prognosis

A

Progression highly variable. Can be slow or very rapid.
Patients with slowly progressive spinal muscular atrophy have mean survival five to ten years
The median survival duration after onset in patients with PMA is about 12 months longer than in patients with classical ALS

29
Q

als ftd

A

20% of individuals with ALS will develop FTD

Common deficits involve executive function, either language or personality.

Causes problems with judgment, impulsivity, decreased ability to handle routine tasks

faster clinical course

30
Q

familial als
onset
disease progression

A

most cases are inherited in an autosomal dominant pattern
Onset: late forties or early fifties
No differences between familial and sporadic ALS on neurological examination

31
Q

stages of als I

A

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

32
Q

stages of als II

A

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

33
Q

stages. of als III

A

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

34
Q

stages of als IV

A

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

35
Q

stages of als V

A

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

36
Q

stages of als VI

A

bedridden,
dependent ADLs, FMS;
progressive respiratory distress

37
Q

medical management als

A

No effective treatment for disease

38
Q

disease modifying als

A

Riluzole (1995):. Glutamate antagonist. Prolongs survival by at least a few months and slows the progress of ALS, allowing the patient more time in the higher-functioning states.
Thought to especially help slow progress with bulbar-onset

39
Q

als functional rating scale

A

Measures ADL and global function across 12 functional categories

Used to assess response to treatment or disease progression

LOWER= worse

40
Q

prognosis als
positive
negative

A

Negative Prognostic Indicators
Bulbar-onset
Early respiratory signs
Positive Prognostic Indicators
According to the American Academy of Neurology, patients who participate in multidisciplinary clinics live longer and have an improved quality of life.

41
Q

PT goal rehab als

A

PT: Maintain independence as long as possible, prevent secondary complications, patient education, equipment requisition

42
Q

respiratory funciton als tx

A

Airway Clearance Techniques
Inspiratory Muscle Training
Found to be effective in prolonging muscle strength
Mean improvement in lifespan: 12 months!!
 PROVEN LONG TERM BENEFIT
Lung Volume Recruitment Training
Found to strongly enhance immediate cough efficacy with improved FVC
 PROVEN IMMEDIATE BENEFIT
Manual Assisted Cough Techniques
Secretion Mobilization (chest PT)
Chest stretching
Suctioning
Incentive spirometry
Long term mechanical ventilation
Huge decision for pt

43
Q

ex precaution als

A

Exercise Precautions:
Monitor fatigue levels closely
Avoid overwork injury
Avoid exercise if less than 1/3 of motor units are functioning
Limited positions with decreased pulmonary function

44
Q

ex program als

A

Best implemented in early stages of disease, less appropriate as disease progresses

Prevent further deconditioning and disuse atrophy while avoiding overwork damage in weakened, denervated muscle
Mild resistive exercises if muscle are good to normal ranges (3-5/5)

44
Q

prevention of indirect impairments als

A

Maintain activity levels as long as possible
PROM
Skin care
Positioning
Equipment requisition
PWC, hoyer, hospital bed, ramp, bathroom DME

45
Q

pt considerations on ALS stage 1

A

Continue normal activities
Stretching of affected joints
AROM, Resistive exercises to unaffected muscles
Aerobic activities – moderate intensity within patient tolerance ok

46
Q

pt considerations on ALS stage 2

A

Assess for use of assistive devices
Continue with stage 1 activities, however incorporate more conservation techniques
Increased need for caregiver assistance
Exercise 2-3 x per week with rest in between
Airway clearance techniques

47
Q

pt considerations on ALS stage 3

A

Paralysis occurs in some muscles, while other muscles may be weak or not affected.
Ex: Foot drop or hand paresis
Neck muscles become more involved
As more of the lower motor neurons are damaged, spasticity decreases.
Goal is to keep patient physically independent
Energy conservation techniques crucial
Use of splints, orthotics, AD

48
Q

pt considerations on ALS stage 4

A

Wheelchair level
Continue AROM and PROM to prevent contractures
Strengthening therex with focus on major muscle groups
Increased focus on preventing integumentary complications
Ordering all necessary equipment

49
Q

pt considerations on ALS stage 5

A

Inability to transfer
Pain contracture and cramping
Maintenance program: Stretching, splinting, STM, orthotics

50
Q

pt considerations on ALS stage 6

A

Max assist to dependent for all mobility
Skin care and prevention
Pain management
Postural drainage
Coughing techniques
Airway clearance

Neuro 2 (9 decks)

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