Rapidly progressive young-onset dementia.
OBJECTIVE
To characterize a cohort of individuals who have experienced rapidly progressive dementia with onset before age 45.


BACKGROUND
Very little data regarding the clinical features or clinical spectrum of rapidly progressive young-onset dementia (RP-YOD) is available, primarily consisting of case reports or small series.


METHODS
A search of the Mayo Clinic medical record was employed to identify patients who had onset before age 45 of rapidly progressive dementia. All available medical records, laboratory data, neuroimaging studies, and pathologic data were reviewed.


RESULTS
Twenty-two patients met the predefined inclusion and exclusion criteria. Behavioral and affective disorders, cerebellar dysfunction, and visual and/or oculomotor dysfunction were common early clinical features within the cohort, as were clinical features often associated with Creutzfeldt-Jakob disease. Diagnostic testing identified an etiology in most patients.


CONCLUSIONS
Presentations of RP-YOD result from a variety of etiologies and significant overlap in clinical features is observed. Clinical features often associated with Creutzfeldt-Jakob disease seem to be common within the entire cohort of RP-YOD patients. Diagnostic studies aided in establishing a diagnosis in most patients, however 5 had uncertain diagnoses despite exhaustive evaluation.
Kelley
Brendan J
BJ
Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA.
Boeve
Bradley F
BF
Josephs
Keith A
KA
D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D001706:Biopsy; D001921:Brain / Q000473:pathology; D019411:Clinical Laboratory Techniques; D007562:Creutzfeldt-Jakob Syndrome / Q000473:pathology / Q000523:psychology; D003704:Dementia / Q000473:pathology / Q000523:psychology*; D018450:Disease Progression; D004569:Electroencephalography; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D001523:Mental Disorders / Q000209:etiology / Q000523:psychology; D009483:Neuropsychological Tests; D055815:Young Adult