Tödliche familiäre Schlaflosigkeit

Also known as Insomnia familial fatal, Familial fatal insomnia, FFI, Insomnia, Fatal Familial, Fatal insomnia

Krankheit

Key facts

Other names: Thalamic Creutzfeldt-Jakob disease
Specialty: Neurology , psychiatry , sleep medicine , neuropathology
Symptoms: Progressive insomnia , ataxia , double vision , weight loss , high blood pressure , excessive sweating , fever , agrypnia excitata , panic attacks , dementia , delusions , phobia's , tachycardia , vivid dreams
Complications: Permanent state of hypnagogia later in the illness, heart attack
Usual onset: 45–50 years old
Duration: 18 months (average)
Types: Fatal familial insomnia, sporadic fatal insomnia
Causes: PrP type 2 ( Genetic mutation (fCJD D178N-129MM/MV), sporadic form (sCJDMM2-T, very rare))
Risk factors: Family history
Diagnostic method: Suspected based on symptoms, supported by sleep study , PET scan and genetic testing (if familial form is suspected)
Differential diagnosis

Article · Deutsch

Bei der tödlichen familiären Schlaflosigkeit (auch letale familiäre Insomnie, engl. Fatal Familial Insomnia, kurz FFI) handelt es sich um eine erbliche, sehr seltene und im Verlauf von Monaten bis Jahren stets tödlich endende übertragbare spongiforme Enzephalopathie (transmissible spongiform encephalopathy, TSE).

Abstract from DBpedia / Wikipedia · CC BY-SA