CNS Infections- SSPE

Question 1

Subacute Sclerosing Panencephalitis (SSPE) is

Answer 1

a rare, slowly progressive, inflammatory, demyelinating disease of the CNS which occurs primarily in children and young adults caused by a defective measles virus infection of the CNS.

Question 2

People at a higher risk for SSPE include

Answer 2

immigrants & internationally adopted children

Question 3

Neurons and glial cells are infected by measels virus but intact, infectious virion is never __________.

Answer 3

recovered from infected brain biopsies

Question 4

Involvement of both ___________ with changes predominantly in the posterior cerebral hemispheres. Also see _________ (lymphocytes, plasma cells) of cerebral vessels.

Answer 4

white and gray matter

perivascular cuffing

Question 5

Varying degrees of demyelination observed. Intense inflammation is seen (gliosis and microglial reaction are striking), however this is ____________.

Answer 5

NOT an autoimmune disease

Question 6

S/S

Answer 6

1. Insidious onset, behavioral changes/problems 2. lowered school performance
2. Followed by obvious intellectual decline disturbed motor functions (awkwardness, stumbling, myoclonic jerks – AKA drop attacks)
3. seizures
4. blindness (retinopathy)
5. stuporous rigid state
6. loss of cortical function, mutism, flexed posture

Question 7

Therapy

Answer 7

• No therapy exists
• Death occurs slowly in 1→2 y after onset of symptoms
• Rarely there is long-term remission (5 → 20 years) yet CSF measles virus titers remain elevated

Question 8

CSF shows

Answer 8

• Normal CSF parameters, except hyperglobulinorrachia (highly elevated measles-specific antibody titers)
• Elevated serum measles IgG titer causes increased CSF:serum measles IgG index
• *(indicates intrathecal synthesis of measles-specific IgG)**

Question 9

EEG shows

Answer 9

“Burst-suppression” pattern