Fatal Familial Insomnia

A Wired article (Sleep Disorders Traced to Genes) reminded me of Fatal Familial Insomnia (FFI) and introduced me to a sleep disorder, Delayed Sleep Phase Syndrome:

Patients typically are unable to fall asleep before 2 a.m. and have extreme difficulty waking early (e.g., by 7 a.m.). People with DSPS are sometimes called "night owls" or are described as "not being morning people." If they are able to sleep a full 7 to 8 hours (e.g., until 10 a.m.), they feel rested and function normally.

I'm typically unable to get to bed before 2 a.m., but I don't think that qualifies without the accompanying insomnia.

So back to FFI. It's exciting because it's a prion disease like the transmissible spongiform encephalopathies, but it mainly affects the thalamus, leading to insomnia-related death before the rest of the brain is seriously involved. Here's an overview of FFI by Ann M. Akroush.

I also found a couple of interesting journal articles available on-line: Fatal familial insomnia- clinical, neuropathological, and genetic description of a Spanish family -- Tabernero et al. 68 (6)- 774 -- Journal of Neurology, Neurosurgery, and Psychiatry (full text), and an abstract of Fatal insomnia in a case of familial Creutzfeldt-Jakob disease with the codon 200(Lys) mutation -- Chapman et al. 46 (3)- 758 -- Neurology.