Talk:Childhood dementia

On the Management / Treatment section

According to Childhood Dementia: A Collective Clinical Approach to Advance Therapeutic Development and Care, which I used in the draft:

"(...) less than 5% of the conditions having disease-modifying therapies, (...)".

From my understanding this should be the percentage of the conditions that are currently treatable.

With that in mind, I chose a Management section instead of Treatment for now. But should both be included? Or should treatable and untreatable Childhood dementia be made into subtopics / child articles? Or should a different solution be taken?

Let me know your thoughts! Irina Rainbow (talk) 20:41, 5 September 2024 (UTC)

About my removal of the sentence in History

@Ozzie10aaaa

I removed this sentence as it seemed incorrect, but the source may still be useful (it just seems it was misinterpreted in the sentence).

Feel free to work on 'History' though! Just make sure what you add meets WP:Verifiability! Irina Rainbow (talk) 17:33, 24 September 2024 (UTC)

ok, no problem, thank you--Ozzie10aaaa (talk) 18:40, 24 September 2024 (UTC)

On the position of 'Prognosis'

Hey @Ozzie10aaaa, I noticed you moved the Prognosis section back down.

You mentioned the medical manual of style, just as I did, which does list Prognosis beneath Treatment.

However, you may have missed this sentence from the MOS:

"If a disease is incurable, then the Prognosis section can be moved up, and a section called Management is more appropriate than Treatment."

From the sources, it seems only a relatively small amount of conditions considered part of Childhood Dementia are treatable.

Due to this, I think Prognosis should be the first section, especially as Childhood Dementia is a devastating group of diseases with very significant impacts on both life expectancy and quality.

I feel this is the most consequential aspect of the group, and so, also the first thing the article should give to the reader (after the lead ofc).

I'd like to hear your thoughts (and anyone else reading this), so we can hopefully all reach a consensus that's best for the article. Irina Rainbow (talk) 18:41, 25 September 2024 (UTC)

there are many diseases/disorders that unfortunately have a poor prognosis however (again ) per MEDMOS[1] it is best kept after 'treatment',Ozzie--Ozzie10aaaa (talk) 19:04, 25 September 2024 (UTC)

I'm sorry but I did specifically quote the MEDMOS.

..I reccomend checking the sentence (if you haven't)

p.s: Even if it wasn't stated by MEDMOS, the sources currently used in the article clearly show:

A) the vast majority of childhood dementias are currently incurable

B) the prognosis of these conditions tends to be quite poor, not only in terms of mortality rate but also the tremendous effect these conditions have on quality of life.

Isn't this one of the most important things for the average reader of the article? If yes, wouldn't having Prognosis as one of the first sections, instead of the 5th, improve the article? Irina Rainbow (talk) 15:18, 30 September 2024 (UTC)

Hello @Ozzie10aaaa

I wanted to let you know that I plan to move the Prognosis back up since I've gotten no further reply on this topic and I believe it would be a positive change to the article.

I'll give you 2-3 more days just in case! (For you and anyone else who wants to weigh in) Irina Rainbow (talk) 16:46, 8 October 2024 (UTC)

p.s:

I searched the current sources using the keyword 'treat'. I'm compiling here what I could find that mentions the treatable conditions, which does seem to show they're a small portion of the total:

1)

The collective burden of childhood dementia: a scoping review:

"Of these, 25 were analysed separately as treatable conditions."

2) Childhooddementia.org:

"There are also more than 25 treatable disorders with close to normal life expectancy assuming early diagnosis and stringent treatment not listed here."

Note: This appears to be sourced from the scoping review

3) The Conversation:

"Childhood dementia treatments currently under evaluation or approved are for a very limited number of disorders, and are only available in some parts of the world."

Irina Rainbow (talk) 18:48, 25 September 2024 (UTC)

you should always use MEDRS Wikipedia:Why MEDRS?--Ozzie10aaaa (talk) 19:06, 25 September 2024 (UTC)

Clarification on the last edit

I didn't actually re-add Incidence.

I tried to see what having Incidence and Prevalence as subsections would look like, but after checking featured medical articles, I don't think it's the norm.

So I left that idea out of the edit, but forgot I had started writing it in the edit summary.

I'll try to slow down when finishing an edit, since that seems to be the root of my edit summary issues. Irina Rainbow (talk) 13:57, 2 October 2024 (UTC)

A lot of the time we use the term "Epidemiology" as a subsection to talk about prevelance, incidence, gender differences etc. IntentionallyDense (talk) 14:52, 2 October 2024 (UTC)

Ah I see. I'm more or less aware of that, since I've read WP:MEDSECTIONS. But thank you for trying to make sure I know what the Epidemiology section is supposed to contain, since I have been editing it.

(p.s: Did you really mean "Epidemiology" as a subsection or as a section? If it was a mistake np, just wanted to be sure I understand)

p.p.s: Love your userpage btw! Even found some essays I hadn't seen, so tysm! Irina Rainbow (talk) 17:55, 2 October 2024 (UTC)

I meant section sorry about that. And thank you! I spent probably too much time looking at user essays for that! IntentionallyDense (talk) 18:47, 3 October 2024 (UTC)

Are NCLs the most common subtype of conditions in Childhood Dementia?

I've been unsure about whether this was really the case since seeing it added to the article.

After a few days failing to log in to the Childhood Dementia Iniative's 'knowledgebase', I was now able to check its statistics.

For those who haven't encountered the knowledgebase, I reccomend taking the time to see its description in childhooddementia.org, and judge it for yourself.

Going to 'Diseases', and filtering by highest INCIDENCE PER 100,000 BIRTHS, you can currently find that NCLs or Batten Disease are listed as the 11th highest in this category.

The knowledgebase requires a login to access (though to my knowledge, it seems anyone can register). So here is a small compilation of the 11 conditions/subtypes with the highest incidence of the 182 listed:

DISEASE NAME	INCIDENCE PER 100,000 BIRTHS
Organic acidurias	33.33
Amino acidopathies	11.80
Phenylketonuria (including variants)	9.90
Mitochondrial disorders	7.00
Rett Syndrome	5.56
Urea cycle disorders	2.90
Leigh Syndrome	2.50
Nonketotic hyperglycinemia (including variants)	1.32
Vanishing white matter disease	1.25
MPS III (Sanfilippo syndrome)	1.20
Neuronal Ceroid Lipofuscinoses (NCLs or Batten Disease)	1.19

Note: The knowledgebase seems to only contain the Australian prevalence of subtypes. But for what it's worth, NCLs are still far from the highest among these. Irina Rainbow (talk) 20:07, 2 October 2024 (UTC)

On the other hand, the source used in the article (NCL Disorders: Frequent Causes of Childhood Dementia, 2013) states that "Dementia in children or young adults is most frequently caused by neuronal ceroidlipofuscinoses (NCL), a group of incurable lysosomal storage disorders linked by (...)"

So it seems these sources disagree on this.

Of note, the above source dates to 2013, whereas the knowledgebase appears to be much more recent and kept up to date. Irina Rainbow (talk) 20:16, 2 October 2024 (UTC)

After scouring all other sources I know of, I found only one source very weakly supporting the sentence currently in the article:

Behaviours and psychological symptoms of childhood dementia: two cases of psychosocial interventions, which cites the original 2013 source.

While it mentions Batten's Disease frequently, the only relevant passages I could find were:

1. "Batten disease, or neuronal ceroid lipofuscinosis, and Sanfilippo syndrome, or mucopolysaccharidosis type III, are two of the more common forms of childhood dementia disorders worldwide." (highlighted relevant part)
2. "Batten disease and Sanfilippo syndrome are two of the more common forms of CD.1,3–6

p.s: I'm going to ask at the teahouse what I should do for now, as noone has replied yet to this discussion. Irina Rainbow (talk) 14:23, 3 October 2024 (UTC)

Your note at Wikipedia talk:WikiProject Medicine was a better choice. The Teahouse is best if you have basic questions about how to edit, but they don't have any specific subject-matter knowledge. WhatamIdoing (talk) 20:35, 3 October 2024 (UTC)

@Irina Rainbow I'm a little confused by what you are trying to say here. Organic acidurias are more common but that doesn't mean they are a more common cause of Childhood dementia. IntentionallyDense (talk) 18:58, 3 October 2024 (UTC)

Huh. I was assuming that since Childhood Dementia is the group, then the most common subtypes of that group are..well the most common of that group.

This does depend on the definition of Childhood Dementia, which varies..

I'm using the definition: "a unifying taxonomy representing a heterogeneous collective of childhood neurodegenerative disorders." from The collective burden of childhood dementia: a scoping review (one of the main sources for the article) since it seems like the most *useful* one, as these seem to be conditions causing dementia that presents from childhood to young adulthood.

Note: Since they're definitions, I don't think there's really a "right" or "wrong" one in this case? From what I've seen there doesn't seem to be a medical consensus yet)

I hope this helps you understand what I meant? The issue then is whether NCLs are its most common subtype or not. I think it's probably not, but not for sure and I could use some help. Irina Rainbow (talk) 19:59, 3 October 2024 (UTC)

The table that you've created here (thank you!) is a little unclear. As presented, it could mean that .0000033% kids have organic acidurias (but only a fraction of them have childhood dementia); it could also mean that .0000033% kids have childhood dementia as a result of an organic aciduria (and some additional/omitted percentage of kids have an organic aciduria without childhood dementia).

The source that says Batten and Sanfilippo "are two of the more common forms" is the kind of statement that's easier to understand. The source cited in the lead begins with the statement that "Dementia in children or young adults is most frequently caused by neuronal ceroidlipofuscinoses (NCL)", which is unambiguous.

One question to ask is: given that the data is limited (e.g., only looking at Australian kids), should we be trying to rank the conditions? Maybe it would be more pointful to provide a relatively complete list of conditions than to say that X causes more causes of childhood dementia than Y.

One reason that I question the ranking is because you could have an extremely rare disease that results in 100% CD, and a larger disease that results in 1% CD, and the larger disease would be more common, even though the relative risk is much lower. A policy maker might care about raw numbers (we have 100 kids with ____, so we need Y nurses trained), but I think parents are going to care about the chance of developing this complication. WhatamIdoing (talk) 20:48, 3 October 2024 (UTC)

I see. It seems you're using a different definition of Childhood Dementia.

For example in "you could have an extremely rare disease that results in 100% CD, and a larger disease that results in 1% CD"

If CD is treated as a group of diseases, then you can't cause Childhood Dementia, but a condition can cause dementia in children. A bit confusing, I know.

Childhood Dementia seems to be treated either as a group of diseases, as a complication, or both.

I'll list here the links to all the definitions I can find in sources that seem at least potentially reliable.

Note: I'll include whether they see it as a group, complication, or condition. Also the dates of publication that I can find (take with a grain of salt).

(group) The collective burden of childhood dementia: a scoping review (Introduction)20 July 2023, published in the Brain in November 2023

(both complication and group) childhooddementia.org

(group) Childhood Dementia: A Collective Clinical Approach to Advance Therapeutic Development and Care, from Pediatric Neurology Volume 139, February 2023, Pages 76-85

(condition) Dementia.org.au Last updated 24 September 2024

(ambiguous?) DZNE (German Center for Neurodegenerative Diseases) 22.11.2021

(complication?) NCL Disorders: Frequent Causes of Childhood Dementia 2013 Winter

(group) The psychosocial impact of childhood dementia on children and their parents: a systematic review 2023 Sep 7

(condition) The Conversation April 26, 2024

(I'm not sure) The Australian Childhood Dementia Study 11 April 2002

This begs the question:

Fundamentally, is this an article about the group of Childhood Dementia(s), or about dementia in children? I started the article as a draft, and I think even now it's currently about the group.

I have more important context but this is already very long. Feel free to ask. Sorry for the essay. You can probably tell I really care about this. Irina Rainbow (talk) 10:58, 4 October 2024 (UTC)

I'm glad you're writing about this. I doubt that we would get a well-developed article otherwise.

I clicked on the post at The Conversation (website). It says "Broadly speaking, childhood dementia is caused by any one of more than 100 rare genetic disorders...Yet this devastating condition has lacked awareness, and importantly, the research attention needed to work towards treatments and a cure."

The first of these two sentences seems to consider CD to be the result of other diseases. The second calls it a "condition". I'm not sure exactly what the perfect word is: a complication? I think you can have a condition that results from other conditions. Consider, e.g., pneumonia, which is a medical condition but which also is a complication of other medical conditions. WhatamIdoing (talk) 17:21, 4 October 2024 (UTC)

The pneumonia parallel seems pretty accurate. Good one.

The conditions considered by the Childhood Initiative (by proxy also the burden of review, partly made by members of the Initiative) and by other sources, are probably all conditions that cause dementia in children at least some of the time.

To clarify things, I see 2 kinds of definitions:

• "childhood dementia as dementia in childhood"
• "Childhood Dementia as a group of conditions that cause dementia in childhood"

We can have both as articles if *both* have notability.

But this reflects *much* more the latter. And this was also my intent when I made it*

*Ofc it's not *my* article. It's wikipedia's. My interest is along the lines of stewardship, not ownership.

So my suggestion is leaving this article for the group of Childhood Dementia(s); and we could make a new article for dementia in childhood (probably as a sub-article to Dementia or another).

It's late for me so I'll mention another important problem at a later time. I try not to do this kind of thing when sleepy, but I thought it could be good for us to clear this up sooner than later. So we can go back to working on improving the article (Trying to get it to a B and hopefully GA status. A or Featured would be amazing but we'll see.)

Anyhow, if I made any mistake, please do let me know. Thank you so much for your help guys! Irina Rainbow (talk) 21:20, 4 October 2024 (UTC)

I'm inclined to make the article more general, i.e., including every kid with any type dementia, no matter what the cause is.

I don't know much about this, so I think the most useful question I could ask is: What's an example of dementia in a child, that isn't Childhood Dementia™?

For example, with Chronic fatigue syndrome (ME/CFS), there's ME/CFS itself, and then there are people who are tired all the time. We can say that someone with insomnia has "a syndrome" that causes "fatigue", and it happens "chronically", but that's not "chronic fatigue syndrome" itself; it's just being tired all the time. WhatamIdoing (talk) 01:03, 2 November 2024 (UTC)

Based on the wording in "NCL Disorders: Frequent Causes of Childhood Dementia" I think the authors are trying to say that neuronal ceroidlipofuscinoses are the most common cause of childhood dementia and not that neuronal ceroidlipofuscinoses are the most common disorder than could cause childhood dementia. The wording used in the original article is "Dementia in children or young adults is most frequently caused by neuronal ceroidlipofuscinoses" while the wording in the wikipedia article is "Neuronal ceroid lipofuscinoses, a group of lysosomal storage disorders, are thought to be its most common subtype." which I don't think is what the authors were trying to say. I have changed the wording to what I think is a more accurate portrayal of what the authors wrote. IntentionallyDense (talk) 20:48, 3 October 2024 (UTC)

This seems like the kind of thing that would be worth seeking additional sources on. A trip to OMIM maybe, or perhaps we could find a decent textbook somewhere? Epidemiology is a pretty typical subject for textbooks, because of the horses and zebras problem. WhatamIdoing (talk) 21:47, 3 October 2024 (UTC)

I’ll see what I can find. IntentionallyDense (talk) 02:35, 4 October 2024 (UTC)

OMIM is not likely to have frequency data on "the most common cause of childhood dementia". OMIM has a few tidbits about incidence of specific subtypes in specific populations, e.g., "CLN3 is especially enriched in Finland with an incidence of 1:21,000 live births and a carrier frequency of 1 in 70" & "Moore et al. (2008) estimated that NCL in Newfoundland has an incidence of 1 in 7,353 live births. The incidence of CLN2 was 9.0 per 100,000, or 1 in 11,161 live births, the highest reported in the world.". A textbook might have a more general epidemiological approximation, if indeed there is a textbook on the subject, but a good textbook might avoid making such a generalization and just mention that incidence varies a lot by population and subtype and mention some of the specific studies like in OMIM. The current Iran J Child Neural 2013 reference is probably OK. Jaredroach (talk) 21:33, 8 October 2024 (UTC)

Found a mention of OMIM while adding references to the article:

"At present, Human Phenotype Ontology (HPO) and Online Mendelian Inheritance in Man (OMIM) do not catalogue search terms to identify childhood dementia conditions."

Source: The psychosocial impact of childhood dementia on children and their parents: a systematic review Irina Rainbow (talk) 15:07, 9 October 2024 (UTC)

Proposal to rename the article for disambiguation purposes

Reasoning:

Currently, there appear to be 2 definitions used for 'Childhood Dementia':

a) dementia during childhood;

b) a group of conditions that cause dementia during childhood.

If you see the current article, and its past revisions, it should be evident that it reflects much more the group of childhood dementia conditions than dementia in childhood. Less importantly, it was also the intended scope of the article when I started its draft.

The current article's name 'Childhood dementia' is ambiguous in regards to the 2 above definitions, it is the name used by most if not all sources, whether they are referring to the group of childhood dementias, dementia in childhood, or both.

List of definitions in sources, made in the previous Talk topic.

This has already caused confusion among the article's editors.

Proposal:

Rename / move the current Childhood dementia article to 'Childhood dementias' or another title that is unambiguous.

(Potential) Create a new article for dementia during / in childhood.

I think this could be a sub-article under Dementia, but I'm not sure, so feedback would be appreciated.

Suggestions for the tnew article's title: 'Childhood dementia' or 'Dementia in childhood'. Infantile dementia is another possibility, but I'm not sure it would be as good.

Irina Rainbow (talk) 09:43, 6 October 2024 (UTC)

I don't see any problem with the current article and title. It is pretty clearly about childhood dementia, not about "disorders that might cause childhood dementia". An article about "disorders that might cause childhood dementia" probably would not be a standalone article in Wikipedia. It might qualify as a list. If one wanted to list all disorders that cause childhood dementia, the best (and most usual WIkipedia way) would be to put a "Causes" section into the article about the symptom (i.e., the current article). See Coffee ground vomiting  (section Causes) for an example. Indeed, there is such a section in the current article. It could be expanded if desired, perhaps into a bulleted list with OMIM links for each genetic cause. But the current freeform text is arguably just fine for an encyclopedia entry. Jaredroach (talk) 19:17, 9 October 2024 (UTC)

Ah, I'm not sure you understood what I meant.

Myself and other editors have disagreed on whether the article is about dementia that presents itself during childhood, or the group of disorders that cause dementia during childhood. These are 2 very related, but still different things. Like how COVID-19 can cause pneumonia, but the pneumonia and COVID-19 are not the same thing.

You said that the current article is pretty clearly about childhood dementia, but I urge you to look deeper, into its past revisions before my recent edits, even if you aren't new to the article. It should be fairly quick.

I'd go and find the examples myself, and I did, but for the sake of reducing my own bias, I trust you to do so. But if you want me to do it, you only need to let me know. Irina Rainbow (talk) 15:32, 10 October 2024 (UTC)

It is not relevant to look at past revisions; I am judging the current article. Jaredroach (talk) 21:57, 10 October 2024 (UTC)

I'll follow that, @Jaredroach.

I'll place here the current text of the first three sections of the article (so I don't flood this page) and then mention the rest.

For more convenience, I underlined the parts I think are relevant.

Lead:

" Childhood dementia is an umbrella group consisting of over 100 rare neurodegenerative conditions. These are typically genetic, that cause progressive cognitive decline and the loss of previously acquired skills (e.g: talking, walking, reading/writing, etc). The group of conditions has a very poor prognosis on average, as the progression of cognitive decline and other symptoms/complications have a very significant impact on the life expectancy and quality of those affected. Neuronal ceroid lipofuscinoses, a group of lysosomal storage disorders, are the most common cause of childhood dementia. "

Classification and terminology:

" Childhood dementias are a heterogenous group of genetic neurodegenerative disorders, that present symptoms before the age of 18. They are typically monogenic (caused by mutations of a single gene).

Their main characteristics are chronic and widespread cognitive decline; loss of previously acquired developmental skills after a period of typical development; and behaviours and psychological symptoms of dementia (BPSD).

Childhood dementias are distinct from sources of intellectual disability in childhood that are non-progressive (e.g traumatic brain injury) or acquired (e.g nutritional deficiencies or encephalitis). "

Signs and symptoms;

" By its definition, childhood dementias always cause a global decline typically begins after a period of seemingly normal development.

Note: Noticed I made some typographical errors here. Will fix rn.^

(...)

Other symptoms and complications can occur depending on the subtype.

Other symptoms:

• Behavioral changes: Personality shifts^{[citation needed]}, aggression, and hyperactivity.
• Motor decline: Loss of coordination^{[citation needed]}, balance^{[citation needed]}, and movement abilities.
• Seizures: Frequent in many forms of childhood dementia.

Other complications:

• Loss of vision or hearing.
• Cardiovascular, respiratory, and/or digestive problems
• Bone or joint problems.

Out of the remaining sections, 5 out of 6 clearly talk about the group of childhood dementia conditions. Diagnosis is the only exception.

In conclusion, 7 of the 8 sections of this article clearly refer to the group of childhood dementia conditions. If after reading this you still believe this isn't the case, I'd be eager to hear your reasoning. Irina Rainbow (talk) 17:21, 11 October 2024 (UTC)

Agree with all of this. I don’t think a name change is needed here. IntentionallyDense (talk) 00:08, 11 October 2024 (UTC)