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Neuromuscular management > Lecture 6 - Neuromuscular Diseases > Flashcards

Lecture 6 - Neuromuscular Diseases Flashcards

1
Q

The type of neuropathy a patient has depends on the ___________

A

type of fibers affected

are they myelinated or unmyelinated fibers?

Are they A Delta, A beta, …..?

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2
Q

You’re more likely to have a ______ injury than an injury at the plexus

A

nerve root/nerve injury

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3
Q

What is ALS?

Does it have UMN or LMN degeneration?

Who is more commonly affected?

A

Progressive fatal disease that has degen of both UMN and LMN

More men than women

10% familial

Average life expectancy after diagnosis: 3 years

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4
Q

What is a common way of diagnosing ALS?

A

You see atrophy of tongue muscles, fasciulations of tongue muscles

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5
Q

T or F: ALS also causes cognitive problems

A

F, no cognitive probems

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6
Q

C9orf72 ALS vs SOD1 ALS

A

SOD1 is a rare type that is actually treatable

C9orf72 is the common form that is not treatable

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7
Q

What are the most common symptoms of ALS?

A

Fatigue

Muscle stiffness

SOB

muscle cramps

increased saliva

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8
Q

How is the west nile virus transmitted

A

Mosquito bites

note: peak transmission between july-october

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9
Q

T or F: most cases of west nile virus are asymptomatic

A

T, 80% asymptomatic

20% have a fever

Less than 1% are neuroinvasive

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10
Q

What is acute flaccid paralysis

A

Usually after viral illness

acute onset and rapid progression of asymmetric flaccid weakness and hypoactive/absent reflexes, respiratory insufficiency, bowel and bladder problems

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11
Q

What is poliomyelitis

A

Virus that is usually aymptomatic

minor disease- flu like symptoms

major disease- CNS invasion (less than 1% fo cases)

Has been eradicated in north america

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12
Q

What is the diagnostic criteria for post-polio syndrome?

A

History of paralytic poliomyelitis

period of partial or complete recovery

gradual onset of progressive and presistent muscle weakness

symptoms for +1 year

exclude other causes

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13
Q

Radiculopathy is most common in what nerve root?

A

L5

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14
Q

Symptoms of radiculopathy

A

Pain, numbness, tingling in dermatome

weakness in myotome

reduced reflexes

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15
Q

__% of cervical radiculopathies due to herniated disc improve without surgical intervention

A

95

note: surgery is need if: Not responding to PT, significant weakness

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16
Q

How do you differentiate between radiculopathy and lower motor neuron lesion?

A

Radiculopathy is in a dermatomal distribution AND shooting pain down neck

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17
Q

If you’ve got more than one nerve distribution affected by radiculopathy in one arm or leg, what is a possible cause?

A

Plexopathy

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18
Q

What are the signs and symptoms of plexopathy? What is plexopathy most commonly caused by?

A

typically painful, restricted to single limb, more widespread than single nerve root

most commonly casued by trauma, tumor, or thoracic outlet syndrome

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19
Q

What is mononeuropathy

what are the signs and symptoms

what is it caused by?

A

Neuropathy of single peripheral nerve

Signs and symptoms: restricted to anatomic distribution of one nerve

etiology: usually compression, entrapment, trauma

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20
Q

What is polyneuropathy?

A

generalized process affecting (many) peripheral nerves

signs and symptoms: depending on which nerves are involved

commonly isometric and length dependent weakness, sensory loss, loss of DTR

stocking/glove distribution

hundreds of reasons you could have this

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21
Q

How do you characterize polyneuropathy?

A

Is it sensory, motor, or autonomic?

is it large or small fibers?

demyelinating or axonal?

hereditary or accquired?

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22
Q

What nerves are myelinated?

Which ones arent?

A

Myelinated: Motor, sensory A-Beta A-Delta, Some autonomic

Unmyelinated: Sensory C fibers, some autonomic

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23
Q

Most myelinated fibers are _____ diameter and mainly __________

A

large

mainly motor

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24
Q

What is the main kind of neuropathy we can treat w/ medication (neurologists can)

A

demyelinating

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25
Q

What are the suggestive features that someone has demyelinating neuropathy (polyneuropathy)

A

weakness without atrophy (at first)
length independent
patchy
asymmetric

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26
Q

What are the suggestive features that tell you it’s an axonal problem vs a demyelinating problem?

A

Suggestive features of an axonal problem:

Distal > proximal

Legs > arms (longer axons more susceptible)

muscle wasting early (demyelinating doesnt have early muscle wasting)

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27
Q

What are two common causes of axonal problems?

A

Diabetes (most common)

Alcohol (second most common)

28
Q

What are 2 examples of “small” nerve fibers

A

Sensory nerves (specifically pain and temp)

Autonomic nerves

29
Q

Patient’s with small fiber neuropathy will experience what symptoms?

A

Autonomic dysfunction

allodynia

neuropathic pain

discoloration of skin, reduced sweating, hairloss

reduced ability to feel pain, hot/cold

30
Q

what are examples of large diameter nerve fibers?

A

Motor Nerves

Sensory nerves that carry pressure/vibration/proprioception

31
Q

Patients that have “large fiber neuropathy” will present w/…..

A

Numbness, tightness, balance problems

“walking on foam/cobblestone” (no proprioception)

32
Q

Orthostatic hypotension

arrythmias

severe constipation, ED, Urinary Retention

abnormal sweating

early satiety, light headedness w/ meals

These are signs of what kind of problem?

A

Autonomic neuropathy

33
Q

What are 2 of the most common causes of polyneuropathy?

A
  1. Diabetes
  2. Alcohol

Others: Thyroid disease, vitamin deficiency, systemic problems, infections

34
Q

What is an example of a rapidly progressing inflammatory demyelinating polyneuropathy?

What is an example of a slowily progressive inflammatory demyelinating polyneuropathy

A

Rapid: Acute inflammatory demyelinating polyneuropathy (AIDP) or Guillain Barre syndrome.
-progression ends in majority by 4 weeks

Slow: Chronic inflammatory demyelinating polyneuropathy (CIDP)
-Weakness progresses for over 2 months

35
Q

What are common causes of Acute inflammatory demyelinating polyneuropathy (AIDP)

A

preceding illness, surgery, vaccination in some

36
Q

How does Acute inflammatory demyelinating polyneuropathy (AIDP) progress?

A
  1. Tingling paresthesia w/ little sensory loss
  2. severe radicular back pain
  3. Weakness (mild to complete quadriplegia)
37
Q

How is Acute inflammatory demyelinating polyneuropathy (AIDP) usually diagnosed with an exam?

A

Exam:
-symmetric weakness,
-facial weakness
-sensory loss variable
-areflexia in most after 1 week

note: also diagnosed w/ CSF and EMG

38
Q

What is the progression of Guillain Barre syndrome

A

Monophasic

39
Q

How do you treat Guillain Barre syndrome?

A

Supportive care: monitor/treat respiratory failure, autonomic dysfunction

-plasma exchange and IVIG (anti immune system drug)

NO STEROIDS

Rehab starts early

40
Q

at the start of demyelinating conditions you do not see muscle __________, but you do see muscle ___________

A

atrophy

weakness

41
Q

Who is normally affected by Chronic inflammatory demyelinating polyneuropathy (CIDP)?

A

Male > female

Average onset 50+

Usually relapsing-remittent or can be progressive

42
Q

How does Chronic inflammatory demyelinating polyneuropathy (CIDP) progress?

A

slowily over months

Motor > sensory

symmetric proximal and distal weakness which progresses to atrophy

hypo or areflexive

sensory loss mainly in legs, rarely disabling

43
Q

What condition is slowily progressive

with symmetric weakness in proximal and distal muscles

hypo/areflexive

A

CIDP

44
Q

What is the normal length dependent pattern in polyneuropathy

A

stocking/glove

45
Q

How does diabetic neuropathy usually present?

A

Distal symmetric sensory polyneuropathy

“stocking/glove”

usually affects LE/UE

slowily progressive

no weakness

autonomic problems

46
Q

diabetic neuropathy affects what % of diabetics

A

65%

47
Q

What condition is associated w/ large weight loss

begins abruptly

begins w/ sharp aching pain asymmetrically in hip and thigh, spreads to leg and foot

pain -> progressive weakness of thigh and knee -> weakness progresses distally

often becomes bilateral

A

Diabetic lumbosacral radiculoplexus neuropathy AKA diabetic amyotrophy

note: usually affects DM2 in middle or older age

48
Q

Hereditary neuropathies:

Charcot-marie-tooth disease

Hereditary sensory autonomic neuropathy

amyloid

HNPP

These commonly have an insidious onset when??

A

Under 20 years old

49
Q

What condition presents w/

distal weakness and atrophy

length dependent sensory loss

foot deformities: pes cavus, pes plannus, hammer toe

abscent or decreased reflexes

chronic slow progression

A

Charcot-Marie-Tooth-disease

50
Q

What can clue you in that a demyelinating condition is hereditary

A

delayed early milestones

slow progression

foot deformities, injuries, surgeries

family history

hip problems/socliosis

abscence of positive sensory symptoms but prominent sensory loss on exam

51
Q

Charcot Marie Tooth disease clinical phenotypes:

Early onset:

Childhood onset:

Late onset:

A

Early onset: delayed onset of walking (>15 months)

Childhood onset: Normal onset of walking
slow runner as a child
develops symptoms between 5-20
most common presentation

late onset group:
20+

52
Q

Supportive treatment for Charcot Marie Tooth disease

A

PT and OT

orthotics

screen for scoliosis and hip dysplasia

surgery for skeletal and soft tissues abnormalities

53
Q

Lambert eaton myasthenic syndrome and botulism are forms of __________ disorders

myasthenia gravis is a ___________ disorder

A

Presynaptic

Post synaptic

54
Q

How can you differentiate myasthenia gravis from MS?

A

Pt improves w/ rest

55
Q

What disease presents as:

Flucuating/fatiguable (can test fatigue w/ eye muscles)

asymmetric ptosis/diplopia

difficulty chewing

dysarthra,dysphasia, nasal speech

hoarseness

head drop

limb weakness

A

Myasthenia gravis

56
Q

Who does Myasthenia gravis mainly affect?

A

Younger woman and older men

57
Q

What are common triggers for Myasthenia gravis

A

Emotional Upset

Viral Illness/systemic illness

heat or hyperthyroidism

surgery

menstral cycle

drugs

58
Q

What drugs make Myasthenia gravis worse?

A

D-Penicillamine

Botox

Magnesium Salts

IFN

Succinylcholine

59
Q

How to diagnose Myasthenia gravis?

A

Tensilon test- The test involves injecting the drug Tensilon (edrophonium chloride) intravenously into a patient’s arm or hand, and then observing their response to repetitive physical movements:

Ocular cooling - Their eye works better after u put a cold pack on it because cold makes the NM junction work better

antibody panel serum

EMG

60
Q

Symptoms of Myasthenia gravis

A

fatigue

exercise intolerance

weakness

myalgia

cramps

stiffness

atrophy

61
Q

How do you identify myopathy on a physical exam

A

Usually proximal weakness
-identify w/ GOWER’S SIGN (they have to get up from the ground using their hands to post) \
DTR can be normal or slightly reduced

62
Q

Polymyositis, dermatomyositis, and inclusion body myositis are all examples of…

A

inflammatory myopathies

63
Q

How do polymyositis and dermatomyositis usually present?

A

Subacute/chronic onset of proximal weakness

variable pulmonary and cardiac involvement

usually involve a malignancy (cancer)

autoimmune disorder

HIV

Dermatomyositis: Inflammation of the skin aswell, theyre all red and inflamed in different places

64
Q

What supplementation is important for bonehealth for patients w/ dermatomyositis

A

Calcium and vitamin D

65
Q

Who most commonly gets inclusion body myositis?

A

50+ men

66
Q

How does inclusion body myositis usually present?

A

Proximal leg and distal arm weakness (finger flexors and quad)

asymmetric

muscle atrophy

dysphagia

sometimes associated w/ autoimmune disease

note: 56% need an AD after 7 years (20% WC)

Neuromuscular management (19 decks)

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