Subacute sclerosing panencephalitis history and symptoms

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Characterized by a history of primary measles infection before the age of 2 years, followed by several asymptomatic years (6–15 on average), and then gradual, progressive psychoneurological deterioration, consisting of personality change, seizures, myoclonus, ataxia, photosensitivity, ocular abnormalities, spasticity, and coma.

History and Symptoms

Symptoms include

• Bizarre behavior
• Dementia (loss of cognitive (thought), emotional, and social abilities)
• Coma
• Gradual behavioral changes
• Myoclonic jerking (quick muscle jerking or spasms)
• School problems
• Seizures
• Unsteady gait
• Very tense muscles or muscles that lack tone, with weakness in both legs

Progression of Symptoms

• The progression of symptoms begins with stage 1 — in this stage the behaviour of person become more abnormal and erratic, the person can be irritable and personality alterations can occur. This is often accompanied by memory loss and mental deterioration characterised by intellectual difficulty. As the nervous system begins to lose control of movement, the person develops myoclonic spasms/jerks (these being involuntary motions and spasms in extremities). The myoclonic spasm is a key warning sign of SSPE as these spasms are only normally seen as the body drifts off to sleep — breathing rate decreases, heart rate slows and body temperature lowers causing the brain to think the body is dying and so a myoclonic spasm ensues to try and rouse the body — and so if myclonic spasms are seen when the person isn't tired or during the day then this is indicative of a potentially serious problem (such as SSPE).

• As the disease progresses towards stage 2, the intensity of the spasms and the mental deterioration increases. The spasms can grow to such an extent that loss of the ability to walk can be a common sign. Also, the person will suffer speech impairment and increasingly deteriorated comprehension coupled with dysphagia. At this point the infection is at stage 2.

• The final, advanced stages of SSPE include the steady decline in body function with increased intensity of the stage 2 symptoms/signs and also blindness. At the end of the final stages the person is likely to be mute and/or comatose.