Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Semester 5 PMNM Exam 10 > Anterior Horn Cell - Spinal Muscle Atrophy > Flashcards

Anterior Horn Cell - Spinal Muscle Atrophy Flashcards

1
Q

Spinal muscle atrophies primarily impact

A

Anterior horn cell

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

SMA - include

A

A number of disorders that are characterized by progressive weakness and atrophy of certain mm groups
Almost always will be a symmetrical pattern of progressive weakness though

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

SMA - pathologic condition of

A

the motor unit

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

SMA - most common

A

LMN disorder in children

Second most common genetic pathology that we see (first is CF)

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

SMA - genetic

A

ALL are genetic

Most are autosomal recessive - chromosome 5

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

SMA - what is it

A

Disorder of the LMN and motor nuclei of the brainstem

Degeneration of the AHC (motor neuron) and the CN nuclei

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

SMA - more common in

A

M than F (2:1)

The later the onset, the less likely F will be affected

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

SMA - classification

A

Arbitrary - usually comes down to age of diagnosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Common findings - EMG

A

EMG - evidence of degeneration and regeneration, decrease in compound motor unit action potentials (CMAPs)

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

Common findings - MM biopsy

A

MM biopsy - small fiber atrophy interspread with large fiber hypertrophy

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

Common findings - Lab studies

A

Lab studies are generally normal

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

Common findings - Nerve conduction velocities

A

Nerve conduction velocities are generally normal

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

SMA classification

A

Adult onset (IV)
Mild (III)
Intermediate (II)
Severe (I)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

SMA classification Adult Onset - Type

A

IV

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

SMA classification - Adult onset Type IV - general presentation

A

Subtle presentation
Mm initially affected are pelvic and shoulder girdle (proximal mm)
Problems with standing, STS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

SMA classification - Mild - Type

A

III

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

SMA classification - Mild Type III- General presentation

A

Able to stand and walk unaided

Usually discovered in adolescence

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

SMA classification - Intermediate - Type

A

II

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

SMA classification Intermediate Type II - General presentation

A

Able to sit unsupported, unable to stand or walk unaided

Might not be able to achieve sitting but once there they might be fine

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

SMA classification - Severe - Type

A

I

21
Q

SMA classification - Severe Type I - general presentation

A

Unable to sit unaided

Trunk has to be supported

22
Q

SMA Type I (Severe) - Table
Symptom onset:
Course:
Death:

A

0 to 6 m
Never sits
Less than 2 yrs

23
Q

SMA Type II (intermediate) - Table
Symptom onset:
Course:

A

7 to 18 m
Never stands
More then 2 years

24
Q

SMA Type III (mild) - Table
Symptom onset:
Course:

A

More than 18 m
Stands alone
Adult

25
Q

SMA Type IV (adult)- Table
Symptom onset:
Course:

A

Over 30
Relatively benign
Normal life span

26
Q

Mild - type III AKA

Characterized by

A

Kugel Welander
Autosomal recessive
Proximal mm weakness, primarily LEs

27
Q

Mild - Type III - clinical deatures

A

Difficulty with running, jumping
Waddling gait
Hand tremors
Hypermobile joints - hypotonicity issue

28
Q

Mild - Type III - Prognosis

A

Long term survival dependent on resp. function

Bulbar function impairment often comes about late in the disease process

29
Q

Mild - Type III - - Intervention focus

A 
Encourage activity
Promote ambulation as long as possible
Minimize risk of infection
Join protection 
Promote nutrition
30
Q

Intermediate - Type II - age of onset usually between

A

6 and 12 months

31
Q

Intermediate - Type II - prognosis

A

Long term prognosis is dependent on resp. function

32
Q

Which is the most common type

A

Intermediate - Type II - accounting for about 50% of cases

33
Q

Intermediate - Type II - Clinical features

A 
Symmetrical weakness of LE
Tongue fasciculations
Hand tremors
Absent or dec DTRs
Facial mm spared
Kyphoscoliosis
Intercostal mm involvement
Hypotonia
Significant jt laxity
34
Q

Intermediate - Type II - prognosis

A

Dependent on resp. system
MM weakness is relatively static
Functional improvement with intervention
May be prone to hip issues (sublux/disloc)

35
Q

Intermediate - Type II - Intervention

A 
Prevention of scoliosis
Standing posture is important
Promote ambulation if possible 
Prevent infections
Assistive technologies
36
Q

Severe (type I) - age of onset

A

In utero or within first few months of life

1/10,000 births

37
Q

Severe (type I) - clinical features

A 
Hypotonia (floppy child)
Significant mm weakness (frog)
Sucking and swallowing problems
Resp. difficulties
Poor head control
Costal recession
Weak cry
38
Q

Severe (type I) - prognosis

A

Very prone to resp infections

Life expectancy for most is 1-3 years without ventilatory support - even with they wont live past 5 or 6 years

39
Q

Severe (type I) - Intervention

A

Resp care including suctioning

Pattern of breathing is diaphragmatic but with inappropriate thoracic expansion

40
Q

Methods of tube feeding

A

Used to provide nutritional support
Hydration
Medication delivery
Temporary or Permanent

41
Q

Types of naso

A

Nasogastric
Nasoduodenal
Nasojejunal

42
Q

Issues for NG tubes

A 
Uncomfortable
Tape
Easier to displace
Less volume possible  so you need more frequent feeding schedule 
More spit up
43
Q

Operative feeding tubes

A

Gastrostomy (GT) feeding

Jejunostomy (JT) feeding

44
Q

GI tubes - open gastrostomy

A

Open G tube that they will go in and suture

45
Q

Percutaneous endoscopic gastrostomy tube - PEG tube

A

More common

Go in and place it - the tube has a balloon at the end that goes into the stomach

46
Q

Buttons

A

Make sure to check them for leakage or drainage, for infection, and just make sure to continuously monitor them

47
Q

Issues for GI tube

A 
Infections
Drainage/Leakage
Malfunctions can occur 
Tube can interfere with activity 
Balloon buttons may not inflate 
Valves may get stuck
48
Q

Good things with GI tube

A

Can give a larger bolus - more volume
Easier to replace
Less GI upset

49
Q

Floppy infant

A

Dec mm activity secondary to hypotonicity issue
Res positions are unusual (frog leg)
Developmental delays

Semester 5 PMNM Exam 10 (6 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions