The corresponding estimates for motor vehicle injuries were 34%, 11%, and 10%. The proportion of unintentional fatal injuries cases that could have been prevented in the absence of difficulties initiating sleep, difficulties maintaining sleep, and having a feeling of nonrestorative sleep were 8%, 9%, and 8%, respectively. There was a dose-dependent association between the number of insomnia symptoms and risk of unintentional fatal injuries (P for trend 0.001) and fatal motor vehicle injuries (P for trend 0.023), respectively. Measurements and results: There were 277 unintentional fatal injuries, including 57 fatal motor vehicle injuries during follow-up. Participants: A total of 54,399 men and women 20-89 y of age who participated in the Nord-Trøndelag Health Study between 19. Design: Population-based prospective cohort study with a mean follow-up of 14 y, linking health survey data with information on insomnia symptoms to the National Cause of Death Registry. Study Objectives: To assess the association between insomnia symptoms and risk of fatal unintentional injuries. Insomnia Symptoms and Risk for Unintentional Fatal Injuries—The HUNT Study Conclusions In patients with suspected prion disease, a characteristic change in sleep pattern can be an important clinical clue for identifying sFI or FFI polysomnography (PSG), genetic analysis, and nuclear imaging may aid in diagnosis. Genetic tests identified no prion protein (PrP) gene mutation, but neuropathological examination and molecular study showed protease-resistant PrP (PrPres) in several brain regions and severe atrophy of the anterior-ventral and medial-dorsal thalamic nuclei similar to that described in FFI. Following death of this patient, an interview with a close family member indicated the patient's illness included a major change in her sleep pattern, corroborating the reported autopsy diagnosis of sFI. Case Presentation We report a case of a 33-year-old female who died of a prion disease for whom the diagnosis of sFI or FFI was not considered clinically. Sporadic fatal insomnia in a young woman: A diagnostic challenge: Case Reportīackground Sporadic fatal insomnia (sFI) and fatal familial insomnia (FFI) are rare human prion diseases. Brain tissue testing is necessary for disease confirmation and is particularly beneficial in cases with an unusual clinical presentation. Although rare, a prion disease diagnosis should not be discounted in adolescents exhibiting neurologic signs. There was no evidence of an environmental source of infection, and this patient represents the youngest documented case of sporadic prion disease. Genetic, neuropathologic, and biochemical analyses of the patient’s autopsy brain tissue were consistent with sporadic fatal insomnia, a type of sporadic prion disease. A prion disease was confirmed in an adolescent with disease onset at 13 years of age. The occurrence of sporadic prion disease among adolescents is extremely rare.
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