Subacute sclerosing panencephalitis

(SSPE)
• is a chronic complication of measles with a delayed
onset and an outcome that is nearly always fatal

• persistent infection with an altered measles virus that

is harbored intracellularly in the central nervous system
for several years

• After 7-10 yr the virus apparently regains virulence and

attacks the cells in the CNS that offered the virus
protection  inflammation and cell death 
neurodegenerative process
 Subacute sclerosing panencephalitis
(SSPE)
• Measles at an early age favors the
development of SSPE:
– 50% of patients with SSPE had primary measles
before 2 yr of age
– 75% had measles before 4 yr of age
• Males are affected twice as often as females
• More cases reported from rural than urban
populations.
 Subacute sclerosing panencephalitis
(SSPE)
Clinical manifestations of SSPE begin insidiously
7-13 yr after primary measles infection.

Stage 1
• Subtle changes in behavior or school
performance appear, including irritability,
reduced attention span, and temper
outbursts. Fever, headache, and other signs of
encephalitis are absent.
 Subacute sclerosing panencephalitis
(SSPE)
Stage 2
• The hallmark is massive myoclonus, which
coincides with extension of the inflammatory
process site to deeper structures in the brain,
including the basal ganglia.
• Involuntary movements and repetitive myoclonic
jerks begin in single muscle groups but give way
to massive spasms and jerks involving both axial
and appendicular muscles.
• Consciousness is maintained.
 Subacute sclerosing panencephalitis
(SSPE)
Stage 3
• Involuntary movements disappear and are replaced by
choreoathetosis, immobility, dystonia, and lead pipe rigidity
that result from destruction of deeper centers in the basal
ganglia.
• The sensorium deteriorates into dementia, stupor, and then
coma.

Stage 4
• Characterized by loss of critical centers that support
breathing, heart rate, and blood pressure.
• Death soon ensues.
 Subacute sclerosing panencephalitis
(SSPE)
Diagnosis
A. documentation of a compatible clinical course
B. at least 1 of the following supporting findings:
(1) measles antibody detected in cerebrospinal fluid
(2) characteristic electroencephalographic findings
“suppression burst episodes”
(3) typical histologic findings in and/or isolation of virus
or viral antigen from brain tissue obtained by biopsy or
postmortem examination

Cerebrospinal fluid analysis reveals normal cells but elevated

IgG and IgM antibody titers in dilutions >1 : 8.
 Subacute sclerosing panencephalitis
(SSPE)
• Management is supportive
– carbamazepine to control of myoclonic jerks in the
early stages of the illness
 Subacute sclerosing panencephalitis
(SSPE)
• Most die within 1-3 yr of onset from infection
or loss of autonomic control mechanisms.
• SSPE has been described in patients who have
no history of measles infection and only
exposure to the vaccine virus.
– wild-type virus (subclinical measles previously)