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Pediatric neurosurgeon

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Fatal familial insomnia

Alexey Kryvenko, medical expert
Last reviewed: 07.07.2025

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Epidemiology

Symptoms
Diagnostics
Who to contact?

Fatal familial insomnia is a common inherited prion disorder that causes sleep disturbances, movement disorders, and death.

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Epidemiology

Fatal familial insomnia is a very rare (sporadic) autosomal dominant disorder, with no more than 40 cases reported worldwide to date. The average age of onset is about 40 years (30 to 60).

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Symptoms fatal familial insomnia

In the early stages, fatal familial insomnia manifests itself as sleep-onset disorder and intermittent movement disorders (myoclonic seizures, spastic paresis). This stage can last for several months, but eventually worsens with the development of severe insomnia, myoclonus, sympathetic hyperreactivity (high blood pressure, tachycardia, hyperthermia, increased sweating) and dementia. Death occurs on average after 13 months.

Suspicion of fatal familial insomnia should arise if the patient has movement disorders, sleep disorders, and a family history.

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Diagnostics fatal familial insomnia

Genetic testing is being conducted which can confirm the diagnosis.

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Who to contact?

Neurologist