[Creutzfeldt-Jakob disease: a case that initiated with psychiatric symptoms]. / Creutzfeldt-Jakob Hastaligi: Psikiyatrik Belirtilerle Baslayan Bir Olgu.

Creutzfeldt-Jakob disease (CJD) is a rapidly progressive prion disease that causes deficits involving movement, cognition, and mental status. The clinical heterogeneity of the disease can make diagnosis difficult. Thorough neurologic, cognitive, and psychiatric examinations are necessary for observing its clinical features. In this case report we describe a 62-year-old male patient who was initially followed with a diagnosis of depression and later was diagnosed with CJD. The patient had a one-year history of anhedonia, loss of interest, social withdrawal, anxiety and decrease in speech and was given paroxetin 20 mg/day with a diagnosis of depression. During follow up, neurological symptoms including ataxia and rigidity became evident and dementia and akinetic mutism developed in a rapidly progressive course. Although electroencephalography (EEG) and magnetic resonance imaging (MRI) revealed nonspecific findings initially, typical findings for CJD were seen during the follow up. The positive 14-3-3 protein in CSF supported the diagnosis. The aim of this report is to emphasize the fact that CJD may present with different psychiatric symptoms and can be initially misdiagnosed. CJD should be considered in the differential diagnosis of patients who have focal neurological signs in addition to psychiatric symptoms. Repeated neurological examinations, EEG and cranial MRI may help in the diagnosis of these patients.

Unusual features in eyelid myoclonia with absences: a patient with mild mental retardation and background slowing on electroencephalography.

"Eyelid myoclonia with and without absences" has been incorporated into the new ILAE diagnostic scheme as a type of epileptic seizure with etiologic, therapeutic, and prognostic implications. Eyelid myoclonia with absences (EMA) is characterized by eyelid myoclonia and absences provoked mainly by eye closure and photosensivity. EMA can be a part of idiopathic, symptomatic, or probably symptomatic epileptic syndromes. EMA is the defining seizure symptom that differentiates the idiopathic reflex epileptic syndrome Jeavons syndrome from eyelid myoclonia with absences. Jeavons syndrome is characterized by unique clinical and electroencephalographic features and often genetic clustering. EMA is easily diagnosed by clinical manifestations and properly conducted electroencephalography. However, it is often misdiagnosed as tics or other types of epileptic seizures and syndromes, particularly in patients with mental retardation, behavioral disturbances, and atypical electroencephalographic findings. We describe a 19-year-old woman with EMA who remained undiagnosed for many years. She was mildly mentally retarded and her electroencephalogram showed slow background activity, which are unusual findings in Jeavons syndrome.