# Insônia fatal esporádica

> Página oficial: https://raras.org/doenca/insonia-fatal-esporadica
> Fonte: Raras.org — Banco de Dados de Doenças Raras em Português (CC BY-NC-SA 4.0)
> Última atualização: 2026-05-07

## Identificadores

- **ORPHA**: 586130 — https://www.orpha.net/en/disease/detail/586130
- **CID-10**: A81.9

## Descrição clínica

A rare sporadic human prion disease characterized by adult onset of progredient neurodegeneration presenting as a combination of psychiatric, sleep, and oculomotor disturbances, with development of progressive cognitive impairment (the predominantly affected cognitive domains being memory, temporal and/or spatial orientation, language, executive functions, and attention), postural instability, and sometimes additional motor abnormalities and autonomic hyperactivity, in the course of the disease. Bilateral thalamic hypometabolism on FDG-PET imaging and positive prion seeding activity in the cerebrospinal fluid are present in many cases. The disease is fatal within typically two to three years.

## Epidemiologia e herança

- **Prevalência**: <1 / 1 000 000
- **Casos conhecidos**: 27

## Ensaios clínicos ativos (1)

- **NCT00183092** [COMPLETED]: CJD (Creutzfeldt-Jakob Disease) Quinacrine Study — https://clinicaltrials.gov/study/NCT00183092

## Importante

O Raras **não diagnostica e não prescreve**. Esta página é educativa e informativa.
Pacientes devem consultar profissionais de saúde qualificados para decisões clínicas.

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**Citação sugerida**: Raras.org — Insônia fatal esporádica. Disponível em: https://raras.org/doenca/insonia-fatal-esporadica
**Formato HTML**: https://raras.org/doenca/insonia-fatal-esporadica
**Formato RDF/Turtle**: https://raras.org/api/rdf?orpha=586130