Chapter 45. Neuroimmunology

Question 1

Question 45-1:
Multiple sclerosis is clearly due to inappropriate immune attack. Which of the following statements are true regarding immune response in MS?
A. There is not identified autoantigen
B. MS is immune mediated but not necessarily autoimmune
C. T -helper cells are thought to mediate the disease
D. There is believed to be activation of myelin-specific T cells
E. All are true

Answer 1

Answer 45-1: E.
All of the Statements are thought to be true
regarding immunopathogenesis of MS. The
differentiation of immune-mediated from
autoimmune is that in the latter there is an
identified autoantigen. While there is
activation of myelin-specific T cells, there is
no identified autoantigen, which would make
this a prototypic autoinunune disease. (p823)

Question 2

Question 45-2:
Which of the following have been shown to be beneficial for patients with MS?
I. Glatiramer acetate
2. Interferon β- 1a
3. Interferon β- 1b
4. Interferon y
Select: A: 1,2,3. B: 1,3. C =2,4. D: 4 only. E = All

Answer 2

Answer 45-2: A.
Glatiramer acetate (copolymer-1, Copaxone),
interferon β-1a (Avonex, Rebif), and
interferon β-1b (Betaseron) have all been
shown to be effective in treatment of MS, and
all are approved in the US. Interferon y is a
Thl cytokine which in clinical trial worsened
MS. (p823-824)

Question 3

Question 45-3: 
Which type of immune response characterizes myasthenia gravis? 
A.  B-cell mediated 
B.  T-cell mediated 
C.  Both 
D.  Neither

Answer 3

Answer 45-3: C.
Myasthenia gravis is mediated by a
combination of T-cell and B-celt immunity.
Identification of an autoantigen is easier in B cell
mediated diseases where the antigen is
against the entire protein. In contrast, T-cell mediated diseases are mediated against small
peptides, which are harder to identify. (p821)

Question 4

Question 45-4:
Which of the following statements are true regarding the inununology and pathology of AIDP and CIDP?
A. AIDP and CIDP are both immune mediated
B. There is endoneurial1ymphocytic infiltlate
C. Axonal damage can occur
D. Specific autoantibodies have been found for AIDP but not CIDP
E. All are true

Answer 4

Answer 45-4: E.
All of these are true. AIDP and CIDP are both
inunune-mediated polyneuropathies with
lymphocytic infiltrate in the peripheral nerves.
Axonal damage can occur, probably because
of bystander damage. Specific autoantibodies
have been found for AIDP but not CIDP, even
though the conditions are closely related.
(825-826)

Question 5

Question 45-5; 
High-dose corticosteroids are used for which of the following? 
A.  AIDP 
B.  CIDP 
C.  Both 
D.  Neither

Answer 5

Answer 45-5: B.
Corticosteroids have not been shown to be
effective for patients with AlDP, but they
appear to be useful for patients with CIDP.
and are one modality of treatment. (p826)

Question 6

Question 45-6: 
IVIg is used for treatment of which of the following? 
A.  AIDP 
B.  CIDP 
C.  Both 
D.  Neither

Answer 6

Answer 45-6: C.
IV Ig has been used successfully for treatment
of both AIDP and ClDP. Plasma exchange has
also been used in both conditions, and is
approximatelyequaIly effective to IV lg.
(p826)

Question 7

Question 45-7:
Which of the following statements are true regarding thymectomy for patients with myasthenia gravis?
1. Thymectomy is best perfomedprior to puberty
2. Thymectomy is most indicated for elderly patients
3. Thymectomy has short-term benefits which have to be followed by long-term immunosuppressants
4. Thymectomy has a remission rate of at least 80%
Select: A = 1.2.3. B = 1. 3.C = 2.4. D= 4 only. E = All

Answer 7

Answer 45-7: D.
Thymectomy is an option for patients with
myasthenia, especially relatively young .
patients, under the age of 65. However,
thymectomy should not be performed prior to
puberty. Remission rates after thymectomy in
appropriate subjects are 80-90%. The benefit
of thymectomy is long tenn, and there is often
no early improvement. (p826)

Question 8

Question 45-8:
A patient with proximal weakness had a muscle biopsy which shows perifascicular atrophy with degenerating fibers at the periphery of the fascicles, and capillary damage with microinfarcts.
Immunofluorescence shows immune complex deposition within the endothelium. Which is the most likely diagnosis?
A. Polymyositis
B. Dermatomyositis
C. Inclusion body myositis
D. None of these

Answer 8

Answer 45-8: B.
The perifascicular atrophy and immune
complex deposition are more characteristic of
dermatomyositis than polymyositis.
Dermatomyositis has a greater association
with cancer than other inflanunatory
myopathies, so screening for cancer is
appropriate. (p827)

Question 9

Question 45-9: 
A patient with symptoms of encephalitis is found to have anti-Hu antibodies. Which is the most likely underlying cause? 
A.  Breast cancer 
B.  Ovarian cancer 
C.  Small cell lung cancer 
D.  Non-small cell lung cancer

Answer 9

Answer 45-9: C.
Anti-Hu can be associated with encephalitis or
peripheral neuropathy and is most likely
associated with small cell lung cancer. (p828)

Question 10

Question 45-10: 
A patient presents with cerebellar ataxia and is found to have anti-Yo antibodies. Which is the most likely diagnosis? 
A.  Breast cancer 
B.  Melanoma 
C.  Small cell lung cancer 
D.  Non-small cell lung cancer

Answer 10

Answer 45-10: A.
Breast and ovarian cancer are the disorders
most associated with cerebellar ataxia and
anti-Yo antibodies. (p828)