Subacute sclerosing panencephalitis (SSPE) is a rare and chronic form of progressive brain inflammation caused by a persistent infection with measles virus (which can be a result of a mutation of the virus itself).

• No cure for SSPE exists and the condition is often fatal

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Clinical features

History of primary measles infection usually before the age of 2 years, followed by several asymptomatic years (6–15 on average)

Gradual, progressive psychoneurological deterioration:

• Personality change
• Seizures
• Myoclonus
• Ataxia
• Photosensitivity
• Ocular abnormalities
• Spasticity
• Coma

Stage 1

• Behaviour becomes abnormal and erratic:
  • Irritable and personality alterations, often accompanied by dementia
• Lose control of movement, person develops myoclonic spasms/jerks

Stage 2

• ↑ Intensity of spasms and the mental deterioration
• Loss of the ability to walk
• Speech impairment and increasingly deteriorated comprehension
• Difficulty swallowing

Advanced stages

• Steady decline in body function with increased intensity of stage 2 symptoms/signs
• Blindness

Final stage

• Mute patient, in a vegetative state, and/or comatose

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Diagnosis

Clinical diagnosis:

• Changes in personality
• Gradual onset of mental deterioration
• Myoclonia

Serology:

• ↑ Anti-measles antibody (IgG) (serum & CSF)

Electroencephalogram (EEG):

• Characteristic periodic activity (Rademecker complex)
• Widespread cortical dysfunction

MRI:

Tissue biopsy:

• White matter of both the hemispheres and brainstem affected, as well as the cerebral cortex
• Eosinophilic inclusion bodies are present in the nuclei of neurons (gray matter) and oligodendrocytes (white matter)

Differential diagnosis:

• Acute disseminated encephalomyelitis

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Management

Stage 1:

• Oral inosine pranobex (oral isoprinosine) + intrathecal/intraventricular interferon alpha
• Oral inosine pranobex (oral isoprinosine) + interferon beta
• Intrathecal interferon alpha + IV ribavirin

Stage 2 or beyond

Incurable