https://pubmed.ncbi.nlm.nih.gov/36571866/

Childhood Dementia: A Collective Clinical Approach to Advance Therapeutic Development and Care

PMID: 36571866 DOI: 10.1016/j.pediatrneurol.2022.11.015

Abstract

Childhood dementias are a group of over 100 rare and ultra-rare pediatric conditions that are clinically characterized by chronic global neurocognitive decline.

This decline is associated with a progressive loss of skills and shortened life expectancy. With an estimated incidence of one in 2800 births and less than 5% of the conditions having disease-modifying therapies, the impact is profound for patients and their families. Traditional research, care, and advocacy efforts have focused on individual disorders, or groups classified by molecular pathogenesis, and this has established robust foundations for further progress and collaboration. This review describes the shared and disease-specific clinical changes contributing to childhood dementia and considers these as potential indicators of underlying pathophysiologic processes.

Like adult neurodegenerative syndromes, the heterogeneous phenotypes extend beyond cognitive decline and may involve changes in eating, motor function, pain, sleep, and behavior, mediated by physiological changes in neural networks. Importantly, these physiological phenotypes are associated with significant carer stress, anxiety, and challenges in care. These phenotypes are also pertinent for the development of therapeutics and optimization of best practice management. A collective approach to childhood dementia is anticipated to identify relevant biomarkers of prognosis or therapeutic efficacy, streamline the path from preclinical studies to clinical trials, increase opportunities for the development of multiple therapeutics, and refine clinical care.

Very sad, good job it’s rare.

monkey skipper said:

https://pubmed.ncbi.nlm.nih.gov/36571866/

Excellent, another pre-existing condition that means we should welcome the release and freedom of death from SARACAIDS-CoV ¡

Sorry we mean with.

> pediatric conditions that are clinically characterized by chronic global neurocognitive decline.

Sounds like Rett syndrome.

“Rett syndrome is a genetic neurological and developmental disorder that affects the way the brain develops. This disorder causes a progressive loss of motor skills and language.

“Most babies with Rett syndrome seem to develop as expected for the first six months of life. These babies then lose skills they previously had — such as the ability to crawl, walk, communicate or use their hands.

“Over time, children with Rett syndrome have increasing problems with the use of muscles that control movement, coordination and communication. Rett syndrome can also cause seizures and intellectual disabilities. Unusual hand movements, such as repetitive rubbing or clapping, replace purposeful hand use.”

mollwollfumble said:

> pediatric conditions that are clinically characterized by chronic global neurocognitive decline.

Sounds like Rett syndrome.

“Rett syndrome is a genetic neurological and developmental disorder that affects the way the brain develops. This disorder causes a progressive loss of motor skills and language.

“Most babies with Rett syndrome seem to develop as expected for the first six months of life. These babies then lose skills they previously had — such as the ability to crawl, walk, communicate or use their hands.

“Over time, children with Rett syndrome have increasing problems with the use of muscles that control movement, coordination and communication. Rett syndrome can also cause seizures and intellectual disabilities. Unusual hand movements, such as repetitive rubbing or clapping, replace purposeful hand use.”

So the plural

pediatric conditions that are clinically characterized by

Sounds like Rett syndrome

functioning as singular.

All right¡

SCIENCE said:

So the plural

pediatric conditions that are clinically characterized by

Sounds like Rett syndrome

functioning as singular.

All right¡

OK wise guy/girl/bot. :-)

There is a second one that I know about. Rett syndrome and childhood disintegrative disorder both appear in DSM, the diagnostic and statistical manual of mental disorders. Both are pervasive developmental disorders akin to that other famous pervasive developmental disorder, autism.

“Childhood disintegrative disorder (CDD), also known as Heller’s syndrome and disintegrative psychosis, is a condition characterized by late onset (>3 years of age) of developmental delays in language, social function, and motor skills. Thomas Heller, an Austrian educator, first described childhood disintegrative disorder in 1908. It is a complex disorder that affects many different areas of the child’s development. It is grouped with the pervasive developmental disorders and is related to the better known and more common disorder of autism.”

mollwollfumble said:

SCIENCE said:

So the plural

pediatric conditions that are clinically characterized by

Sounds like Rett syndrome

functioning as singular.

All right¡

OK wise guy/girl/bot. :-)

There is a second one that I know about. Rett syndrome and childhood disintegrative disorder both appear in DSM, the diagnostic and statistical manual of mental disorders. Both are pervasive developmental disorders akin to that other famous pervasive developmental disorder, autism.

“Childhood disintegrative disorder (CDD), also known as Heller’s syndrome and disintegrative psychosis, is a condition characterized by late onset (>3 years of age) of developmental delays in language, social function, and motor skills. Thomas Heller, an Austrian educator, first described childhood disintegrative disorder in 1908. It is a complex disorder that affects many different areas of the child’s development. It is grouped with the pervasive developmental disorders and is related to the better known and more common disorder of autism.”

Fair play, that’s the one we thought of.

Results from my PET scan came back a few minutes ago. Good news. I don’t have dementia.

mollwollfumble said:

Results from my PET scan came back a few minutes ago. Good news. I don’t have dementia.

Goodo, carry on.

Bubblecar said:

mollwollfumble said:

Results from my PET scan came back a few minutes ago. Good news. I don’t have dementia.

Goodo, carry on.

What was the RUDAS score then¿

mollwollfumble said:

Results from my PET scan came back a few minutes ago. Good news. I don’t have dementia.

Excellent. My FIL has it, and I have learnt a great deal. Most importantly for my wife to knock me off if I develop it.