Questions marked with * are more appropriate for residents.
1. Rheumatoid arthritis can cause:
3. CK of 4500 is most likely seen in:
4. A 61 year-old man developed hoarseness and subsequently weakness of the grip of one hand, then the other. Weakness progressed over 3 months. Sensation is normal and CK is normal. A quadriceps muscle biopsy is shown. The most likely cause of the illness is:


5. Patients with congenital muscular dystrophy frequently also have:
6. The most common cause of neuropathy in adults over 50 years is:
7. Severe hypotonia in the neonatal period can be caused by:
8. Severe hypotonia in the neonatal period is caused by a mutation or abnormality involving all of the following except:
9. A 3 year-old boy with weakness, seizures, and psychomotor retardation had the muscle biopsy shown below.
Abnormal laboratory studies include all of the following except:
Abnormal laboratory studies include all of the following except:
10. The pathological changes in muscle shown below occur in patients with mitochondrial myopathies due to:
11. A 60 year-old woman has proximal weakness and a CK of 1800. The most likely cause is:
12. Which of the following is a trinucleotide repeat disorder?
13. Dystrophin is located:
14. Severe limb girdle muscle dystrophy may result from mutations of:
15. A baby boy who was severely hypotonic since birth died of respiratory insufficiency at 4 months of age. The muscle biopsy (ATPase stain) is shown below.

The risk of subsequent siblings developing the same condition is:

The risk of subsequent siblings developing the same condition is:
16. Which of the following is caused by T-cells attacking myofibers:
17. The illustrated pathology occursin all of the following neuropathies except:
18. If a transected nerve is promptly and precisely reconnected:
19. The highest CSF protein is seen in:
20. The pathology illustrated in this nerve biopsy (thick epoxy section, toluidin blue stain) is caused by:
21.The slowest nerve conduction velocity is seen in:
22. Which of the following shows beta amyloid and tau deposits in muscle?
23. The pathology in this muscle biopsy is from:


A. A 54 year-old man with proximal weakness and a CK of 2700
B. A 4 year-old boy with weakness, GI bleed, and CK of 2600
C. A 49 year-old woman with longstanding rheumatoid arthritis, neuropathy, and multiorgan involvement.
D. An 83 year-old woman with proximal and distal weakness for 8 years and a CK of 600.
B. A 4 year-old boy with weakness, GI bleed, and CK of 2600
C. A 49 year-old woman with longstanding rheumatoid arthritis, neuropathy, and multiorgan involvement.
D. An 83 year-old woman with proximal and distal weakness for 8 years and a CK of 600.
24. The illustrated findings are most consistent with:
25. CTG expansion in a neonate is manifested by:







