Brainscape's Knowledge GenomeTM

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


See full index

Neurology Articles > MGUS PN > Flashcards

MGUS PN Flashcards

1
Q

What percentage of people over 70 have MGUS?

A

5.3%

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

What percentage of patients with MG-IgM develop peripheral neuropathy?

A

50% of patients with MG-IgM develop peripheral neuropathy.

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

What is the role of protein electrophoresis in evaluating M-protein?

A

It quantitatively evaluates the M-protein when detectable.

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

What information does immunofixation provide about M-protein?

A

Subtype of M-protein (also very sensitive for M protein)

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

Which size fibers are mainly affected in DADS?

A

Large sensory fibers.

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

What symptoms are usually presented in DADS?

A

Distal hypoesthesia, sensory ataxia, and minimal or no weakness.

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

What percentage of DADS patients have no detectable IgM MG?

A

One-third of DADS patients have no detectable IgM MG.

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

What is the CIDP variant called when DADS patients lack IgM MG?

A

It is referred to as distal CIDP.

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

What percentage of DADS cases with IgM MG present anti-MAG antibodies?

A

67% of DADS cases with IgM MG present anti-MAG antibodies.

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

What MAG antibody BTU titer is less likely to be related to neuropathy?

A

Titers smaller than 8000 BTU are less likely related to neuropathy.

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

Nerve conduction studies show what key findings in anti-MAG-associated neuropathies?

A

Disproportionally prolonged distal motor latency and reduced conduction velocity.

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

What terminal latency index (TLI) value increases likelihood of anti-MAG-associated neuropathies?

A

TLI < 0.26 increases likelihood.

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

Are conduction block and temporal dispersion common in anti-MAG-associated neuropathies?

A

No, they are rarely seen.

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

What is the most common syndrome associated with anti-MAG antibodies?

A

DADS is the most common syndrome associated with anti-MAG antibodies.

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

What is the response to corticosteroids or immunoglobulin in IgM-MGUS with anti-MAG neuropathy?

A

Little to no response.

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

What is the first-line drug for IgM-MGUS with anti-MAG neuropathy?

A

Rituximab.

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

What percentage of patients treated with rituximab achieve a good clinical response?
Need to clarify context of this question)

A

Less than 50% of cases.

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

What was the clinical outcome for patients treated with ibrutinib in a small case series for IgM anti MAG demyelinating PN?

A

Early and persistent clinical response.

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

What are the hallmark features of the chronic ataxia syndromes caused by IgM disialosyl antibodies (CANOMAD and CANDA)?

A

Pure sensory ataxia, areflexia, minimal motor involvement, with or without ophthalmoplegia.

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

What does the complete presentation of CANOMAD include?

A

Chronic ataxic neuropathy, ophthalmoplegia, IgM paraprotein, cold agglutinins, disialosyl antibodies.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What is absent in the incomplete form CANDA compared to CANOMAD?

A

External ocular muscles weakness and cold agglutinins.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

What type of antibodies are frequently detected in the CANOMAD/CANDA?

A

Anti-ganglioside antibodies, especially against disialosyl-type epitopes (GD1b, GD3, GT1b, GQ1b).

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Slowed CV are seen in most CANOMAD/CANDA but what percentage of patients may show normal motor conduction velocities with reduced amplitudes?

A

Up to 27% of patients.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

What is considered first-line therapies for CANOMAD/CANDA?

A

IVIG, Rituximab. Steroids produce no clinical benefit.

25
Q

When is rituximab used in treatment?
Need to clarify context—-

A

Rituximab is used for patients refractory to immunoglobulin or with early symptom recurrence.

26
Q

What is the typical type of monoclonal gammopathy light chains in POEMS?

A

Almost always lambda.

27
Q

What acronym describes some systemic symptoms in POEMS?

A

PEST: Papilledema, Edema, Sclerotic bone lesions, Thrombocytosis.

28
Q

What phenotype of neuropathy is associated with POEMS syndrome?

A

Sensorimotor, length-dependent, and demyelinating.

I guess this is similar to DADs except perhaps more motor involvement.

29
Q

What are the NCS differences in neuropathy between POEMS syndrome and typical CIDP?

A

Less temporal dispersion, fewer conduction blocks in POEMS compared to typical CIDP.

30
Q

What is the most frequent endocrinopathy in POEMS syndrome?

A

Hypogonadism is the most frequent endocrinopathy in POEMS syndrome.

31
Q

What is Castleman disease?

A

A lymphoproliferative disorder found in 11%-25% of POEMS patients.

32
Q

What percentage of POEMS syndrome patients may have meningeal thickening?

A

70%-90% - so MRI brain can be helpful.

33
Q

What percentage of POEMS syndrome cases have an elevated VEGF?

A

98%.

34
Q

What is the treatment for POEMS patients with three or fewer bone lesions and no plasma cells on iliac crest biopsy?

A

Radiotherapy of the lesions. This leads to survival rate of >70% over 10 years and can lead to improvement in PN after several months.

35
Q

What systemic treatment is necessary for POEMS patients with more than three bone lesions or plasma cells on bone marrow biopsy?

A

Autologous hematopoietic stem cell transplantation (HSCT). This can achieve 94% 5 yr survival but can take up to 3 years for max neurologic improvement.

36
Q

What percentage of AL amyloidosis cases is the lambda light chain responsible for?

A

75%-80% of cases.

37
Q

What percentage of AL-amyloidosis cases experience neuropathy?

A

One-third of cases experience neuropathy.

38
Q

What percentage of AL-amyloidosis cases involve bilateral carpal tunnel syndrome?

A

Up to 21% of cases.

39
Q

What type of neuropathy is associated with AL-amyloidosis?

A

Axonal, length-dependent neuropathy with sensory predominance.

40
Q

In rare cases, what type of neuropathy can AL-amyloidosis manifest as?

A

Demyelinating neuropathy, similar to CIDP.

41
Q

What are the 2 main red flags for AL-amyloidosis as the etiology to a CIDP like presentation?

A

Refractoriness to usual treatment and evident dysautonomia (dysautonomia is very rare in CIDP).

42
Q

What are the main nerve fibers involved initially at the onset of peripheral neuropathy in AL-amyloidosis?

A

Involvement of small, unmyelinated fibers causing burning pain, thermo-algesic hypoesthesia, and/or dysautonomia. Large fiber involvement comes later.

43
Q

What are less common patterns of peripheral neuropathy in AL-amyloidosis (less than PN) ?

A

1) Cranial nerve involvement
2) Mononeuritis multiplex
3) Lower limb radiculoplexopathy due to amyloidoma.
4) CIDP

44
Q

What percentage of patients with wild-type ATTR cardiac amyloidosis have monoclonal gammopathy?

A

20% of patients with wild-type ATTR cardiac amyloidosis have monoclonal gammopathy (which is sometimes AL amyloidosis!).

45
Q

Main biopsy sites for amyloid deposition?

A

Abdominal fat, bone marrow, salivary glands, and/or lips.

46
Q

What are determining factors for favorable outcomes in AL-amyloidosis?

A

Early diagnosis and treatment. For this reason it is considered a ‘therapeutic emergency.’

47
Q

What is neurolymphomatosis?

A

Invasion of malignant lymphoid cells in peripheral or central nervous systems.

48
Q

What condition can myelomas lead to, affecting the spinal nerves?

A

Leptomeningeal myelomatosis, causing radiculopathy or cauda equina syndrome.

49
Q

What is the most common pattern of neuropathy distribution in neurolymphomatosis?

A

Multiple mononeuropathies.

50
Q

What other forms of neurolymphomatous neuropathy presentation are described besides multiple mononeuropathies?

A

Brachial Plexopathy
LS plexopathy
Cranial Neuropathy
Radiculopathy

51
Q

What is the eponym of the condition where there is neoplastic infiltration of the nervous system in Waldenstrom’s macroglobulinemia?

A

Bing-Neel syndrome.

This commonly affects the CNS and can affect proximal nerve roots/cranial nerves.

52
Q

What is the confirmatory diagnosis method for neurolymphomatosis?

A

Nerve biopsy - but be careful bc it can be patchy.

53
Q

How can biopsy accuracy for neurolymphomatosis be improved?

A

Guided by MRI or PET-CT.

54
Q

What diagnostic method helps identify clonal lymphocytes in cerebrospinal fluid in neurolymphomatosis patients?

A

Flow cytometry.

55
Q

What mutation in CSF cytometry is highly suggestive of neurolymphomatosis?

A

MYD88 mutation. This is also common in Bing-Neel syndrome.

56
Q

What should raise suspicion for neurolymphomatosis?

A

Presence of M-protein in patients with multiple mononeuropathy, plexopathy, or radiculopathy.

57
Q

What type of neuropathy is associated with peripheral nerve involvement in cryoglobulinemia?

A

Distal sensory-motor neuropathy, symmetrical or mononeuritis multiplex pattern.

58
Q

What symptoms are significant in peripheral nerve involvement in cryoglobulinemia?

A

Sensory symptoms and significant pain in distal limbs.

Neurology Articles (9 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions