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This letter, published in the Journal of Neurology, discusses the expanding phenotype of psychiatric-onset prodromal dementia with Lewy bodies (DLB). DLB is the second most common neurodegenerative cause of dementia, characterized by parkinsonism, cognitive fluctuations, visual hallucinations, and rapid eye movement sleep behavior disorder. However, atypical presentations, such as psychiatric-onset prodromal DLB, can be difficult to diagnose due to symptomatic overlap with primary late-onset psychiatric disorders. The letter presents three cases that highlight the need to consider prodromal DLB as a differential diagnosis of behavioral variant frontotemporal dementia (bvFTD). The authors emphasize the importance of recognizing specific clinical features, such as REM sleep behavior disorder and autonomic dysfunction, in distinguishing DLB from other neurodegenerative dementias. Accurate diagnosis is crucial for genetic counseling and appropriate therapeutic interventions. [Extracted from the article]

Introduction: Progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS) are progressive neurodegenerative syndromes characterised by Parkinsonism with additional features including cognitive dysfunction, falls, and oculomotor abnormalities. Understanding the epidemiology of these conditions is critical to planning for future service provision. Methods: We conducted a systematic review of studies reporting incidence and prevalence of CBS and PSP. A search of the PubMed and EMBASE data bases was conducted from their date of inception to 13th July 2021. Meta-analysis of studies sharing similar methodologies was carried out to generate estimated pooled prevalence and incidence. Results: We found 32 studies meeting our criteria for inclusion. There were 20 studies with data on prevalence and 12 with incidence data of PSP. Prevalence of CBS was reported in eight studies while seven studies reported incidence. Reported estimates of prevalence for PSP ranged from 1.00 (0.9–1.1) to 18 (8–28) per 100,000 while prevalence rates for CBS ranged from 0.83 (0.1–3.0) to 25 (0–59). Incidence rates for PSP and CBS respectively ranged from 0.16 (0.07–0.39) to 2.6 per 100,000 person-years and 0.03 (0–0.18) to 0.8 (0.4–1.3) per 100,000 person-years. A random effects model meta-analysis of studies with similar methodologies yielded a pooled prevalence estimate for PSP of 6.92 (4.33–11.06, I2 = 89%, τ2 = 0.3907) and 3.91 (2.03–7.51, I2 = 72%, τ2 = 0.2573) per 100,000 for CBS. Conclusion: Studies of the epidemiology of PSP and CBS report highly heterogeneous findings. There is a need for further studies using rigorous phenotyping and the most recent diagnostic criteria to understand the true burden of these conditions. [ABSTRACT FROM AUTHOR]

Atypical parkinsonian syndromes are neurodegenerative conditions, characterised by rapid disease progression and shorter life expectancy compared to idiopathic Parkinson's disease. These conditions inflict substantial physical and psychosocial burden on patients and their families; hence, there is a clear rationale for a palliative care approach from diagnosis. An interdisciplinary care model has been shown to improve symptom burden, quality of life and engagement with advance care planning, in a heterogeneous group of neurodegenerative conditions. In this update, we summarise how the landscape for treating these patients has changed and the questions that still need to be resolved. [ABSTRACT FROM AUTHOR]