Initial Manic Presentation in Creutzfeldt-Jakob Disease: A Case Report

Area of Science:

• Neurology
• Prion Diseases
• Psychiatry

Background:

• Creutzfeldt-Jakob disease (CJD) is a rare, fatal neurodegenerative disorder caused by misfolded prion proteins.
• CJD typically presents with rapidly progressive dementia and neurological decline.
• Psychiatric symptoms are common early signs, but mania as a primary presentation is highly unusual.

Purpose of the Study:

• To report a rare case of Creutzfeldt-Jakob disease (CJD) presenting initially with manic symptoms.
• To highlight the diagnostic challenges posed by atypical initial presentations of CJD.
• To emphasize the importance of considering CJD in the differential diagnosis of new-onset psychiatric symptoms in older adults.

Main Methods:

• Case report of a 65-year-old woman with initial manic symptoms.
• Diagnostic investigations included brain MRI, EEG, and cerebrospinal fluid analysis.
• Cerebrospinal fluid analysis utilized real-time quaking-induced conversion (RT-QuIC) for prion protein detection.

Main Results:

• The patient initially presented with symptoms of mania, including elevated mood and insomnia.
• Progressive neurological deterioration occurred, with hallucinations and motor decline.
• Characteristic CJD findings on MRI (cortical ribboning, basal ganglia changes) and EEG (periodic sharp-wave complexes) were observed.
• RT-QuIC confirmed the presence of prion protein, leading to a diagnosis of sporadic CJD (sCJD).

Conclusions:

• Mania can be an atypical early manifestation of Creutzfeldt-Jakob disease.
• This case underscores the diagnostic difficulties when psychiatric symptoms dominate the initial presentation of CJD.
• Early consideration of CJD is crucial for accurate diagnosis, especially in older adults with rapid cognitive or motor decline alongside psychiatric symptoms.