Rare and unusual dementias

Gupta, Susham; Fiertag, Olivia; Warner, John C.

doi:10.1192/apt.bp.107.003558

Cited by 8 publications

(7 citation statements)

References 30 publications

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“…From age at diagnosis, we defined whether patients had early-onset (aged under 65 years) or late-onset dementia (aged 65 + years). People with early-onset dementia are more likely to have rarer forms of dementia than in late-onset (DementiaUK, 2022 ; Gupta et al, 2009 ), which can present additional symptomatology (Giebel et al, 2020 ). Together with the need for greater support with day-to-day activities, such as washing or preparing food, rare dementias can present varied symptoms that can have a greater impact on health and cognition (Gerritsen et al, 2019 ; Koedam et al, 2010 ; Smits et al, 2015 ).…”

Section: Methodsmentioning

confidence: 99%

Social and spatial inequalities in healthcare use among people living with dementia in England (2002–2016)

Watson

Green

Giebel

et al. 2022

Aging & Mental Health

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Objectives Healthcare services for people living with dementia (PLWD) are stretched, and government promises of increased funding remain undelivered. With the UK dementia population to surpass 1 million by 2024, and dementia care costs predicted to almost treble by 2040, it is essential we understand differences in healthcare use among PLWD. This study aimed to explore social and spatial variations in healthcare use among people diagnosed with dementia (2002–2016). Methods Data were derived from Electronic Health Records of Clinical Practice Research Datalink GP patients in England ( n = 142,302). To standardise healthcare contacts, rates of healthcare contacts per year were calculated for three primary (GP observations and medications) and three secondary healthcare types [Accident & Emergency (A&E) attendances and, emergency and elective hospital admissions]. Fully-adjusted generalised linear regression models were used to identify healthcare use variation by social and spatial groups. Twelve models were generated, one for each healthcare type in early- and late-onset populations separately. Results This study highlights numerous social and spatial variations in healthcare use among PLWD. Among PLWD, several groups tended to have healthcare service use more closely associated with negative outcomes, including a greater likelihood of A&E attendances and emergency and elective hospital admissions. These groups include: men, people from White ethnicity groups and people from more deprived and rural areas. Conclusions Systemic and social measures are needed to reduce variations in healthcare use inequalities in PWLD. These include greater healthcare continuity, health checks and medicines reviews, culturally appropriate services, better and more accessible treatment and improved infrastructure.

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Section: Methodsmentioning

confidence: 99%

Social and spatial inequalities in healthcare use among people living with dementia in England (2002–2016)

Watson

Green

Giebel

et al. 2022

Aging & Mental Health

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“…Though often described in children, pellagra can affect adults leading to hyperpigmentation and other skin changes, stomatitis, gastrointestinal disturbances, and progressive dementia with confusional states, ataxias and seizures [4]. It also can be found in alcoholics, patients with Hartnup's disease (congenital defect in tryptophan absorption), and carcinoid syndrome (increased conversion of hydroxytryptophan to serotonin) [3]. Low levels of urinary metabolites 2-methylnicotinamide and 2-pyridone can help diagnosis; treatment is with oral niacin (Tables 17.2 and 17.3).…”

Section: Vitamin-related Deficienciesmentioning

confidence: 98%

“…Other forms of rare dementias are PPA (non-fluent), semantic dementia, progressive supranuclear palsy, corticobasal degeneration, multiple system atrophy/Shy-Drager syndrome, amytrophic lateral sclerosis, motor neurone disease, spinocerebellar or Friedreichs ataxia, polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy (NasuHakola disease), etc. [3]. Treatment for all these disorders is mostly non-specific, and maintaining nutritional support as part of a care package is vital to prevent more rapid deterioration and quality of life, as patients are likely to present with dysphagia and appetite as illness progresses.…”

Section: Degenerative Causesmentioning

confidence: 98%

“…Even prediabetic people with impaired glucose tolerance are also at similar risk. Hypertension, dyslipidemia, and obesity are also associated with diabetes, and this increases the risk of developing dementia [3]. Diabetes-related disorders lead to micro-and macro-cerebrovascular disease, which can lead to early cognitive deficits and contribute to the development of subsequent dementia.…”

Section: Endocrinal and Metabolic Causesmentioning

confidence: 99%

“…Symptoms depend on underlying cerebral and neurological pathology, which includes diffuse meningovascular and parenchymal inflammation, infarcts leading to focal neurological deficits and tabes dorsalis, a degeneration of the ascending fibers of the dorsal root ganglia affecting the posterior columns of the spinal cord. Treatment with high doses of intramuscular penicillin, doxycycline, or ceftriaxone can prevent deterioration [3].…”

Section: Infectious Causesmentioning

confidence: 99%

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Rare and Unusual Dementias

Gupta¹,

Nomani²

2015

Diet and Nutrition in Dementia and Cognitive Decline

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Understanding Creutzfeldt-Jakob disease in Iran: a systematic review of case reports

Mohebbi,

Motamedaria,

Naderi

et al. 2024

J Int Med Res

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Objective To systematically review the reported cases of Creutzfeldt-Jakob disease (CJD) in Iran. Methods A comprehensive literature review of CJD cases in Iran was undertaken using the PubMed®, Scopus® and Google Scholar databases. In addition, the Iranian database MagIran was searched for Persian language reports. Case selection used the following criteria: (i) patients of Iranian origin; (ii) publication in peer-reviewed journals or reputable medical databases; (iii) a definitive diagnosis of CJD based on established diagnostic criteria. Results Thirteen cases from twelve reports were included in this systematic review. The majority of the cases were female (11 of 13; 84.6%). The mean ± SD age of patients at hospital admission was 59.38 ± 7.44 years. The findings of the case review suggested that the prevalence of CJD in Iran is not fully established. CJD may be misdiagnosed alongside other clinical signs. The most prevalent early indications of the disease were psychiatric and neurological in nature. A considerable delay in diagnosis was observed in some cases and there was a shortage of brain autopsy records. Conclusion Efforts to improve diagnostic capabilities, promote awareness and establish monitoring systems are necessary for managing the challenges of providing an early diagnosis of CJD in Iran.

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Rare and unusual dementias

Cited by 8 publications

References 30 publications

Social and spatial inequalities in healthcare use among people living with dementia in England (2002–2016)

Social and spatial inequalities in healthcare use among people living with dementia in England (2002–2016)

Rare and Unusual Dementias

Understanding Creutzfeldt-Jakob disease in Iran: a systematic review of case reports

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