insomnie fatale familiale

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