A 62 year old female presents with labile affect and depression. She exhibits changes in personality and behavior which are rapidly progressive. In the next two months she has hallucinations, memory loss, and develops myoclonus. By the fourth month from the onset of the disease, she shows rigidity of all the extremities and she becomes demented. The following tests are done:

MRI shows no demyelination or tumors.

CSF reveals: protein - 96mg/dl, glucose - 60 mg/dL, cell count < 5cells/ul, albumin 22 mg/dL.

The EEG show triphasic waves with sharpened outlines that occur once every second.

By the seventh month the patient dies.

1-Microscopically there is:

A-Loss of pigment of the substantia nigra and locus ceruleus

B-Presence of neuritic plaques and tangles

C-Spongiform change of the neuropil, neuronal loss, and gliosis

D-All of the above

E-None of the above

2-This disease can be transmitted by:

A-Corneal transplantation

B-Deep implantation of contaminated EEG electrodes

C-Contaminated preparations of human growth hormone

D-None of the above

E-All of the above

3-The following is/are true about this disease:

A-90% of the cases are sporadic

B-10% of the cases are familial

C-There are very rare iatrogenic cases

D- It is invariably fatal

E-All of the above

4-The treatment for this disease is:

A-Dopaminergic agents

B-No treatment is currently available

C-Surgical excision of the hippocampus

D-Acetylcholinesterase inhibitors

E-None of the above

5. The prognosis of this disease is:

A-It is a chronic disease

B-All the patients die within one year after onset of the symptoms

C-If treated promptly, the patient can survive

D-None of the above

6 -For the diagnosis of this disease:

A-EEG pattern is typical, but nonspecific

B-CSF protein can be slightly elevated

C-Definitive diagnosis is by detection of PrPsc in brain tissue

D-In familial cases, mutant forms of PrPc can be found in lymphocytes

E-All of the above are true