Identification and diagnosis of ultra-rapid progressive dementia: evidence from a prospective cohort study and systematic literature review.

Background And Objectives: The term rapid progressive dementia (RPD) may be applied to patients who develop dementia within 1 year or complete incapacitation within 2 years of the first symptom of impairment. However, in select cases, cognitive impairment may emerge abruptly, with symptoms evolving across hours or days. We sought to determine the frequency, etiologies, and factors that associated with ultra-RPD.

FDG-PET patterns associate with survival in patients with prion disease.

Objective: Prion disease classically presents with rapidly progressive dementia, leading to death within months of diagnosis. Advances in diagnostic testing have improved recognition of patients with atypical presentations and protracted disease courses, raising key questions surrounding the relationship between patterns of neurodegeneration and survival. We assessed the contribution of fluorodeoxyglucose (FDG-PET) imaging for this purpose.

Effect of GBA1 Mutations and APOE Polymorphisms on Survival and Progression Among Ashkenazi Jews with Dementia with Lewy Bodies.

Background: Glucocerebrosidase 1 (GBA1) mutations are associated with reduced survival in Parkinson's disease but their effect on survival in dementia with Lewy bodies (DLB) is unclear.

Objective: To assess the impact of GBA1 mutations on survival among Ashkenazi Jews with DLB, while controlling for APOE status.

Methods: One hundred and forty participants from Tel Aviv Medical Center, Israel were genotyped for GBA1 mutations and APOE polymorphisms.

Long-term cognitive outcomes in Susac syndrome: A case series.

Susac syndrome (SuS) presents with encephalopathy, visual disturbances, and hearing loss from immune-mediated microvascular occlusion. While acute SuS is well-described, long-term cognitive outcomes with current treatments are underknown. We assessed ten SuS patients treated in accordance with evidence-based guidelines using immunotherapies targeting humoral and cell-mediated pathways.

Smartphone-based gait assessment for multiple sclerosis.

Introduction: Multiple Sclerosis causes gait alteration, even in the early stages of the disease. Traditional methods to quantify gait impairment, such as performance-based measures, lab-based motion analyses, and self-report, have limited ecological relevance. The Mon4t® app is a digital tool that uses sensors embedded in standard smartphones to measure various gait parameters.