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🧠📖💯 > Neuropathy/NMJ > Flashcards

Neuropathy/NMJ Flashcards

1
Q

Genetic abnormality in ALS

A

SOD1

C9orf72 * FTD

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2
Q

Age at presentation of ALS

A

7th decade

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3
Q

Genetic abnormality in SMA

A

SMN1

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4
Q

Genetic abnormality in Kennedy’s Disease

A

Androgen Receptor Gene

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5
Q

Age at presentation of Kennedy’s

A

3rd-4th decade

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6
Q

Epidemiology and presentation of Hirayama

A

18-25 yo
Athletic males

U/l hand weakness
C8-T1 myotomes
ALS-like

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7
Q

Genetic abnormality in HSMN1 / CMT1

A

PMP22 DUPLICATION

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8
Q

Genetic abnormality in HNPP

A

PMP22 DELETION

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9
Q

Mode of inheritance of HSMNs:

Except:

A

HSMN: AD

Except HSMN 10, X

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10
Q

Mode of inheritance of HSANs:

Except:

A

HSAN: AR

Except: HSAN 1: AD

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11
Q

Clinical presentation of lead toxicity

A

Motor predominant; wrist drop

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12
Q

Clinical presentation of mercury toxicity

A

Motor neuropathy; tremor

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13
Q

Clinical presentation of arsenic toxicity

A

Painful sensory neuropathy; GI tract upset

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14
Q

Clinical presentation of Thallium toxicity

A

Painful sensory neuropathy; alopecia

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15
Q

Clinical presentation of Vitamin B6 toxicity

A

Painful sensory neuropathy

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16
Q

What is POEMS syndrome

A 
Polyneuropathy
Organomegaly
Endocrinopathy
Monoclonal Protein
Skin changes
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17
Q

Painful, rapidly progressive polyradiculopathy with

weight loss, fever, night sweats

A

Neurolymphomatosis

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18
Q

Paraneoplastic neuropathy usually associated with wc cancer and antibodies?

A

Small cell lung CA

Anti-Hu (ANNA 1 and 2)

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19
Q

Painful sensory and autonomic neuropathy; can

present with carpal tunnel syndrome

A

Primary amyloidosis

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20
Q

What is Erb palsy?

A

Traumatic plexopathy
Involves upper trunk of the brachial plexus
Related to stretch injury at birth

C/p: Waiter’s tip sign (C5-C6)

For those w C8-T1 injury: Klmupke palsy -> clawhand

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21
Q

Traumatic plexopathies often involve the upper/lower trunk

A

Upper trunk

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22
Q

What is Erb Palsy?

A

Traumatic plexopathy

Involves upper trunk, brachial plexus

2/2 stretch injury at birth

C5-C6: waiter’s tip
C8-T1: clawhand

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23
Q

Neoplastic Plexopathies involve the upper/lower brachial plexus

A

Lower brachial plexus

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24
Q

Common causes of neoplastic plexopathies (2)

A

Pancoast tumor (direct extension of primary lung tumor)

Mets (breast, lung from LN)

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25
Q

Presentation of neoplastic plexopathies

A

Painful, rapidly progressive

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26
Q

Presentation of radiation-induced plexopathies

A

Painless, slowly progressive

(+) Myokymic discharges

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27
Q

Clinical presentation of inflamatory plexopathies

A

Acute-subacute onset of pain -> weakness, atrophy

Patchy
Pure motor nerves involved
-LTN, interosseous nerves

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28
Q

CSF and NCS findings of AIDP

A

CSF: cytoalbuminologic dissociation

NCS: loss of F waves

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29
Q

CSF and NCS findings of CIDP

A

CSF: cytoalbuminologic dissociation

NCS: demyelination, conduction block, temporal dispersion

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30
Q

NCS findings of multifocal motor neuropathy

A

NCS: conduction block

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31
Q

Who is affected by diabetic amyotrophy and how does it present?

A

Newly diagnosed or mild DM and weight loss

Nerve pain, asymmetric weakness and atrophy of proximal muscles

MC: quadriceps

Nerve biopsy: microvasculitis, ischemic injury

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32
Q

Most common cause of ulnar mononeuropathy

A

Mechanical compression or arthritis at the ulnar groove or cubital tunnel

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33
Q

Most common affected nerve in highly restricted forms of Parsonage Turner Syndrome

A

Long thoracic nerve -> serratus anterior palsy

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34
Q

Re peroneal mononeuropathy:

A) Usual cause?
B) Most commonly affected branch? Presentation?
C) If this muscle is abnormal on EMG, a sciatic neuropathy must be considered instead - this is proximal to the common peroneal nerve

A

A) Fibular neck 2/2 compression, trauma

B) Deep peroneal branch -> foot drop

C) Short head, biceps femoris

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35
Q

What are:

A) Most common nerve roots affected by intervertebral disk extrusions
B) Most common radiculopathy, cervical
C) Most common radiculopathy, lumbosacral

A

A) C5-C7
B) C7
C) L5

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36
Q

Re Spinal Stenosis, what are the:

A) Clinical presentation
B) Aggravating factors
C) Relieving factors

A

A) Neurogenic claudication and chronic back and leg pain

B) Walking, prolonged standing

C) Sitting or leaning forward

37
Q

Earliest symptoms in GBS

A

Paresthesias and slight numbness in the toes and fingers

38
Q

Distinct clinical and diagnostic findings of the Acute Axonal Form of GBS

A

Muscle atrophy apparent early

(+) Numerous electrically excitable motor nerves + extensive denervation

39
Q

Acute Axonal Forms of GBS are triggered largely by _____

A

C. Jejuni infections

40
Q

GBS with acute multifocal neuropathy w/ motor conduction block with intact reflexes has high titers of _____

A

Anti-GM1 antibody

41
Q

Ophthalmoplegia in GBS is associated with which antineural antibodies?

A

Anti-GQ1b

42
Q

Associated antibodies w/ GBS variants

A) Fisher syndrome or other ophthalmoplegic variants
B) Acute Motor Axonal Variant
C) Pharyngeal-cervical-brachial syndrome

A

A) Fisher syndrome or other ophthalmoplegic variants - GQ1b

B) Acute Motor Axonal Variant - GM1, GD1a

C) Pharyngeal-cervical-brachial syndrome - GT1a

43
Q

Which antibodies a/w GBS are usually seen in cases that follow Campylobacter infections?

A

Anti-GM1 antibodies

44
Q

Usual presentation of diabetic ophthalmoplegia

A

Isolated, PAINful 3rd nerve palsy w/ spared pupillary function

45
Q

Most frequently affected peripheral nerve of diabetic neuropathy

A

MC: Femoral nerve

2) Sciatic
3) Peroneal

46
Q

Focal degeneration of the myelin sheath with sparing of the axon

A

Segmental demyelination

47
Q

A reaction of BOTH the AXON and the MYELIN DISTAL to the site of disruption of the axon

A

Wallerian degeneration

48
Q

Dying back phenomenon

A

Axonal degeneration

49
Q

What is chromatolysis?

A

Highly characteristic histopathologic changes in the nerve cell body

Changes:
Swelling of cytoplasm
Marginalization, dissolution of Nissl substance

Cause:
Consequence of axonal interruption

50
Q

True or false. Recovery of function is SLOWEST in segmental demyelination.

A

False.

Segmental demyelination - rapid recovery of function

51
Q

The nutritional, metabolic, and toxic neuropathies assume a predominantly ________ pattern.

What is a known exception? (Adams)

A

Nutritional, metabolic, toxic neuropathies: Distal “axonal” pattern

Exception: porphyria - axonal, w/ proximal instead of distal weakness

52
Q

Maximum degree of denervation atrophy after an acute injury to the axons occurs in ______ days and reduces muscle volume by ____%

A

90-120 days

75-80%

53
Q

Treatment for LEMS

A

3,4 Diaminopyridine

blocks presynaptic K channels

54
Q

As a rule, neuropathies are associated w/ a reduction or loss of tendon reflexes. What is a known exception? (Adams)

A

Small-fiber neuropathies

55
Q

Which anti TB drug is known to cause peripheral neuropathy?

How do you prevent this?

A

Isoniazid

Administer pyridoxine daily

56
Q

Which aspect of having DM is the most important factor in the development of Diabetic Neuropathy?

A

Duration of diabetes

57
Q

Causes of radial nerve mononeuropathy?

A

Crutches
Humeral fracture
Saturday night palsy

58
Q

Match letters to numbers

A) MG
B) LEMS
C) Botulism

  1. Presynaptic
  2. Postsynaptic
  3. Presynaptic, ANS
A

MG - postsynaptic
LEMS - presynaptic
Botulism - presynaptic, ANS

59
Q

Immune mechanisms in

A) MG
B) LEMS
C) Botulism

A

MG - T-cell, ACh receptor antibodies, musk antibodies

LEMS - P/Q Ca channel antibodies

Botulism - botulinum toxin -> SNARE

60
Q

Epidemiology of MG

A

Bimodal

Young - F
Older - M

61
Q

Ca associated with LEMS

A

Small Cell Lung CA

62
Q

Types of botulism

A

Infantile
Foodborne
Wound
Adult infectious

63
Q

RNS findings in LEMS

A

Low stimulation: decrement in CMAP

high frequency stimulation: increment in CMAP

64
Q

In MG, those who are acherase negative and MuSK (+) are likely to be from which population?

A

Females and adolescents

65
Q

Focal degeneration of the myelin sheath with sparing of the axon

A

Segmental demyelination

66
Q

Reaction of both the axon and myelin distal to the site of disruption of the axon

A

Wallerian degeneration

67
Q

What is chromatolysis

A

Highly characteristic histopathologic changes in the nerve cell body

2/2 axonal interruption

Swelling of the cell cytoplasm
Marginalization and dissolution of the Nissl substance

68
Q

True or false: Recovery of function in segmental demyelination is slow.

A

False

69
Q

Nutritional, metabolic, and toxic neuropathies assume predominantly ______ patterns.

Exception:

A

Distal “axonal” patterns

Porphyria

70
Q

Maximum degree of denervation atrophy after an acute injury to the axons occurs in ____ days and reduces muscle volume by ____%

A

90-120 days

75-80%

71
Q

Neuropathies are associated with a reduction or loss of tendon reflexes. An exception is the group of ______

A

Small-fiber neuropathies

72
Q

Attack rates of GBS highest in what age group

A

50-74 years

73
Q

Characteristics of neuropathy associated with porphyria

A

Predominant motor neuropathy

Early bibrachial distribution of weakness, truncal sensory loss

74
Q

Most common form of neuropathy for paraneoplastic disorders

A

MC: Mixed sensorimotor PN

75
Q

Specific type of paraneoplastic neuropathy related to lung CA

A

Purely sensory PN

76
Q

Which TB drug commonly causes polyneuropathy?

How do you prevent this?

A

Isoniazid

Give IV Pyridoxine

77
Q

Most important factor for diabetics in developing diabetic PN?

A

Duration of DM

78
Q

Most common clinical syndrome of DM PN?

A

Distal, symmetrical, primarily sensory PN

79
Q

Which of the following is NOT true of DM PN’s pathophysiology

A) Ischemic 2/2 vasculopathy of the tunica media
B) Fascicular capillaries and epineural arterioles have thickened and hyalinized basement membranes
C) Reduction in trophic factors within diabetic nerves (NGF, VEGF< erythropoietin)
D) None (all are true)

A

A) Ischemic 2/2 vasculopathy of the VASA NERVORUM

80
Q

This syndrome presents as searing and pulling sensations involving small cutaneous areas, that are evoked by extending or stretching the limb, as happens when reaching for an object, kneeling, or pointing with the foot

A

Migratory Sensory Neuritis

Wartenberg Syndrome

81
Q

The following are associated with polyneuropathy + paraproteinemia EXCEPT:

a) Multiple Myeloma
B) Plasmacytoma
C) Waldenstrom macroglobulinemia
D) None of the above (all are a/w)

A

D) None of the above

82
Q

What is the most frequent paraprotein in adults?

A polyneuropathy is associated with which paraprotein more commonly?

A

IgG - most frequent pp in adults

IgM - pp + polyneuropathy

83
Q

Re myelomatous neuropathy:

Abnormal monoclonal globulin with the ____ chain component is found in:

Multiple Myeloma
Osteosclerotic Myeloma

A

Multiple Myeloma - kappa light chain

Osteosclerotic Myeloma - lambda light chain

84
Q

Waldenstrom macroglobulinemia is associated with a monoclonal increase in?

A) IgG plasma fraction
B) IgM plasma fraction
C) IgA plasma fraction
D) None of the above

A

B) IgM plasma fraction

1/2 will have specific anti-MAG antibodies

85
Q

A few patients with Waldenström hyperproteinemia have a hyperviscosity state manifest by diffuse slowing of the retinal and cerebral circulations, giving rise to episodic confusion, coma, impairment of vision, and sometimes strokes. What do you call this syndrome?

A

Bing-Neel syndrome

86
Q

Which of the following is TRUE of CIDP?

A) Symmetric, involving the lower extremities
B) Associated with c. jejuni infections
C) HLA antigens occur with greater frequency in pts w/ CIDP
D) As in GBS, corticosteroids are also not effective

A

True: C) HLA antigens occur with greater frequency in pts w/ CIDP

  • Asymmetric, involving the UPPER extremities
  • NOT associated with infections
  • Corticosteroids are EFFECTIVE
87
Q

NCS characteristics of CIDP?

A

Typical findings: multifocal conduction block, prolonged distal latencies

Absence of denervation changes early

88
Q

Multifocal motor neuropathy presents as?

It is associated with which IgM antibody?

A

Motor nerve conduction blocks
MC in Men
Painless
(+) Atrophy

anti-GM1

89
Q

What is the characteristic feature of leprous polyneuritis?

A

Temperature-dependent pattern (cooler areas)

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