This article requires a subscription to view the full text. If you have a subscription you may use the login form below to view the article. Access to this article can also be purchased.
Fatal familial insomnia (FFI), a rare autosomal dominant disease exhibiting the PRNP D178N/129 M mutation, is the third most common hereditary prion disease.1 The onset of FFI is in middle to late adulthood. Features of this disease are progressive insomnia, dysautonomia, motor signs, and cognitive disturbances leading to death within 18 months on average. We report a patient with an atypical presentation characterized by an early age and psychiatric symptoms with psychotic and catatonic features.
Case report.
An 18-year-old man was hospitalized for psychotic mood disturbances with catatonic features. From age 13, he regularly used cannabis. He was the first child of three. His sisters, ages 17 and 12 years, and his mother, age 41, were healthy. His father died at age 46 and his paternal grandfather at age 72, both from ruptures of cerebral aneurysm.
Four months before admission, the patient had transient diplopia. Two months later, he developed depressive symptoms with insomnia and nocturnal sweating. He was first treated as an outpatient with tianeptine, loprazolam, and cyamemazine, together with a cannabis withdrawal. He initially felt better, and insomnia resolved. Two weeks before admission, he abruptly exhibited psychomotor excitement with threatening hallucinations, extreme anxiety, and total insomnia. On admission, psychiatric examination showed psychotic symptoms (persecutory …
AAN Members
We have changed the login procedure to improve access between AAN.com and the Neurology journals. If you are experiencing issues, please log out of AAN.com and clear history and cookies. (For instructions by browser, please click the instruction pages below). After clearing, choose preferred Journal and select login for AAN Members. You will be redirected to a login page where you can log in with your AAN ID number and password. When you are returned to the Journal, your name should appear at the top right of the page.
AAN Non-Member Subscribers
Purchase access
Letters: Rapid online correspondence
REQUIREMENTS
You must ensure that your Disclosures have been updated within the previous six months. Please go to our Submission Site to add or update your Disclosure information.
Your co-authors must send a completed Publishing Agreement Form to Neurology Staff (not necessary for the lead/corresponding author as the form below will suffice) before you upload your comment.
If you are responding to a comment that was written about an article you originally authored:
You (and co-authors) do not need to fill out forms or check disclosures as author forms are still valid
and apply to letter.
Submission specifications:
- Submissions must be < 200 words with < 5 references. Reference 1 must be the article on which you are commenting.
- Submissions should not have more than 5 authors. (Exception: original author replies can include all original authors of the article)
- Submit only on articles published within 6 months of issue date.
- Do not be redundant. Read any comments already posted on the article prior to submission.
- Submitted comments are subject to editing and editor review prior to posting.
