Contents
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
Clinical features
History of primary measles infection usually before the age of 2 years, followed by several asymptomatic years (6â15 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
- â 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
Diagnosis
Clinical diagnosis:
- Changes in personality
- Gradual onset of mental deterioration
- Myoclonia
Serology:
- â 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
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