Fatal insomnia is a typically hereditary prion disorder causing difficulty sleeping, motor dysfunction, and death.
FI, a very rare disease, usually results from an autosomal dominant mutation, but several sporadic cases have been identified. Average age at onset is 40 yr (ranging from the late 30s to the early 60s).
Common early symptoms include difficulty falling asleep and intermittent motor dysfunction (eg, myoclonus, spastic paresis). This stage can last for months but eventually progresses to severe insomnia, myoclonus, sympathetic hyperactivity (eg, hypertension, tachycardia, hyperthermia, sweating), and dementia. Death occurs in an average of 13 mo.
FI should be considered in patients with motor dysfunction, sleep disturbances, and a family history. Genetic testing can confirm the diagnosis.
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