ENCEPHALITIS LETHARGICA

My Mum's mother Helena Tivey nee Clarke died of encephalitis lethargica about 10 days after my mum was born. (Mum was named Helena after her but was always known as Ena - last 3 letters of Helena)

Encephalitis lethargica (EL) or von Economo disease is an atypical form of encephalitis. Also known as "sleepy sickness" or as "sleeping sickness" (though different from the sleeping sickness transmitted by the tsetse fly, EL is a devastating illness that swept the world in the 1920s and then vanished as quickly as it had appeared. EL attacks the brain, leaving some victims in a statue-like condition, speechless and motionless. Between 1915 and 1926, an epidemic of encephalitis lethargica spread around the world; no recurrence of the epidemic has since been reported, though isolated cases continue to occur.//

Symptoms
Encephalitis lethargica is characterized by high fever, sore throat, headache, double vision, delayed physical and mental response, sleep inversion, catatonia and lethargy. In acute cases, patients may enter a coma-like state (akinetic mutism). Patients may also experience abnormal eye movements ("oculogyric crises"), parkinsonism, upper body weakness, muscular pains, tremors, neck rigidity, and behavioral changes including psychosis.

Cause
The cause of encephalitis lethargica is not known for certain.
Research in 2004 suggested that the disease is due to an immune reaction. In this study, many of the people with encephalitis lethargica had experienced recent pharyngitis. They hypothesised that encephalitis lethargica, sydenham's chorea and PANDAS (paediatric autoimmune neuropsychiatric disorders associated with streptococcal infections) are mediated by variations of the post-streptococcal immune response. There is also some evidence of an autoimmune origin with antibodies (IgG) from patients with EL binding to neurons in the basal ganglia and mid brain.

Treatment
Treatment for encephalitis lethargica in the early stages is patient stabilization, which may be very difficult. There is little evidence so far of a consistent effective treatment for the initial stages, though some patients given steroids have seen improvement. Other patients have been less fortunate, and the disease then becomes progressive, with evidence of brain damage similar to parkinson's disease. Treatment is then symptomatic. The course of encephalitis lethargica varies depending upon complications or accompanying disorders.

The discovery that L-DOPA could relieve some symptoms was described in the book Awakenings by Oliver Sacks in 1973.

I remember my great aunt (Elsie Tivey) talking about Helena's illness - she believed it was caused by the tse tse fly in some bananas that she'd eaten. She said Helena would be talking and just fall asleep mid sentence.