Peripheral Neuropathies and ALS Flashcards

1
Q

Mononeuropathy

A

Focal involvement of a single nerve

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2
Q

Examples of mononeuropathy

A
Median: Carpal tunnel
Ulnar: cubital tunnel
Radial: wristdrop
Peroneal: foot drop
(compression, trauma or entrapment)
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3
Q

Mononeuropathy multiplex

A

Damage to one or more peripheral nerves (at random and noncontigous)
Pattern early on is asymmetric

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4
Q

Polyneuropathy

A

Several peripheral nerves affected at same time

Presents as distal and symmetric deficit

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5
Q

How to classify the problem

A

(based on structure affected)
Axon (axonal or neuronal)
Myelin (demyelinating)

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6
Q

Most common neuropathy

A

Diabetic polyneuropathy

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7
Q

Neuropathy

A

Axon is target

May see motor and sensory deficits but SENSORY usually precedes motor

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8
Q

Neuronal neuropathy

A

Affects nerve cell bodies in anterior horn of spinal cord or dorsal root ganglion

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9
Q

Examples of neuronal neuropathy

A

Type 2 Charcot Marie tooth hereditary

Vitamin B6 toxicity

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10
Q

Demyelinating neuropathies

A

Involve myelin sheath surrounding axon

Often autoimmune or inherited (Guillain Barre, chronic inflammatory demyelinating polyneuropathy or charcot marie tooth)

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11
Q

What is Guillain Barre Syndrome?

A

Idiopathic inflammatory neuropathy

Demyelinating

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12
Q

When do you see Guillain Barre more

A

After infection (campylobacter jejuni!!)

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13
Q

Presentation of Guillain Barre Syndrome

A

Ascending weakness (symmetric) usually beginning in legs
Sensory usually
Absent DTRs frequently
Maybe autonomic dysfunction

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14
Q

What is chronic inflammatory demyelinating polyneuropathy?

A

Idiopathic inflammatory neuropathy (slowly progressive or relapsing)

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15
Q

Presentation of CIDP

A

Diffuse hyporeflexia or areflexia
Diffuse weakness
Generalized sensory loss

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16
Q

What is Charcot-Marie-Tooth?

A

Hereditary motor and sensory neuropathies (genetically heterogenous group of disorders with similar clinical phenotype)

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17
Q

Presentation of Charcot Marie Tooth

A

Weakness
Wasting of distal muscles in limbs (with or without sensory loss)
Pes cavus
Reduced or absent DTRs

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18
Q

Timing types

A

Acute (days to 4 wks)
Subacute (4-8 wks)
Chronic (>8 wks)

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19
Q

Cause of acute mononeuropathy

A

Probably traumatic or ischemia

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20
Q

Cause of chronic mononeuropathy

A

-More common

Entrapment or recurrent minor trauma

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21
Q

Cause of acute polyneuropathy

A

Think inflammatory process (Guillain-Barre, neoplasms, infection, toxins)

22
Q

Cause of chronic polyneuropathy

A

Hereditary or metabolic, CIDP (gradual evolution over yrs)

23
Q

Peripheral vs motor vs sensory nerves

A

Peripheral: sensory, motor and autonomic elements
Motor: large myelinated fibers
Sensory: large for proprioception and small for pain and temp

24
Q

Sxs associated with motor nerve problems

A

Weakness
Fatigue
Cramps
Muscle twitches

25
Q

Signs of motor nerve problems

A

Weakness
Muscle wasting
Fasciculations

26
Q

Sxs associated with sensory nerve probs

A

Numbness or loss of sensation
Altered sensation
Neuropathic pain (burning, dull, poorly localized or sharp)
Loss of coordination/imbalance

27
Q

Signs of sensory nerve probs

A
Sensory loss (sharp/dull, light touch, temp, vibration etc)
Loss of proprioception
28
Q

First thing to be lost in pts with polyneuropathies

A

Ankle reflexes

29
Q

Autonomic sxs

A
Postural hypotension
Tachycardia
Cold extremities
Impaired thermoregulatory sweating
Bowel and bladder dysfunction
Impotence
30
Q

Example of chronic axonal polyneuropathy

A

Diabetes

31
Q

History seen with chronic axonal polyneuropathies

A

Sxs begin in LE
Sensory sxs usually before motor
Slowly progressive, dysesthesias, mild gait probs
Stocking glove distribution

32
Q

PE for chronic axonal polyneuropathies

A

Distal loss of sensation to pin prick, light touch, vibrations, temp or proprioception
May see muscle wasting of feet or lower leg
Hypoactive or absent reflexes

33
Q

Most common acute demyelinating polyneuropathy

A

Guillain Barre

34
Q

History seen with acute demyelinating polyneuropathy

A

Mostly affecting motor nerve fibes
Weakness is usually the early sign
Dysesthesias distally later on
May have gait difficulties or hand clumsiness

35
Q

PE with acute demyelinating polyneuropathy

A
Generalized weakness (distal muscles)
Abnorm vibratory and proprioception (out of proportion to loss of pin prick or temp sensation)-mostly large myelinated fibers
36
Q

Myopathy vs neuropathy

A

Myopathies have PROXIMAL motor weakness while neuropathies have DISTAL motor weakness (if any sensory loss or change in reflexes than neuropathy)

37
Q

Radiculopathy

A

Different distributions of motor and sensory deficits (segmental pattern)
Neck or back pain that radiates to extremities in radicular distribution is probably root lesion

38
Q

B12 deficiency

A
Symmetrical neuropathy (LEGS more than arms)
Paresthesias and ataxia first with loss of vibration and proprioception
39
Q

Motor neuron diseases

A

Sxs of upper and/or lower motor neuron dysfunction WITHOUT sensory sxs

40
Q

What can electrodiagnostic testing do?

A

Differentiate axonal or demyelinating in character

See is primary nerve or muscle disorder

41
Q

Nerve conduction study

A

Permit conduction velocity to be measured in motor and sensory fibers

42
Q

Electomyography

A

May show denervation of affected muscles

43
Q

Tx of Guillain Barre

A

Plasmapheresis and IVIG

No steroids!!!!

44
Q

Tx of CIDP

A

Steroids and IVIG (consider plasma exchange)

45
Q

Pharm tx for neuropathic pain

A

NSAIDs (mild)
*Gabapentin, pregabalin, TCAs
Maybe duloxetine, venlafaxine or carbamazepine

46
Q

Most common form of motor neuron disease

A

Amyotrophic lateral sclerosis (ALS)

47
Q

Presentation of ALS

A

Relentlessly progressive, incureable neurodegenerative disorder (muscle weakness and disability)
Mixed upper and lower motor neuron deficit in limbs
Normal sensory exam!!
Usually presents in 1 of 4 body segments (cranial/bulbar, cervical, thoracic and lumbosacral) and then spreads
Involuntary muscle wasting and weight loss

48
Q

PE to distinguish lower and upper motor neuron

A

Lower: weakness, atrophy, muscle cramps, hypo-reflexia, fasciculations!
Upper: slowness, incoordination, increased tone, hyperreflexia, spasticity, clonus

49
Q

How to diagnose ALS

A

Clinically (NCS normal, EMG might show diffuse ongoing chronic denervation)

50
Q

Median survival of ALS

A

3-5 yrs (survival beyond 20 very rare)