Відео

I used google translate to read your message: My condolences for your daughter. There are many diseases that are labelled rare, without a cure and that is very difficult for patients and loved ones. I hope that explaining the mechanisms of all diseases, common and rare, increases the "general" knowledge of how the brain works. I hope that by lifting our level of knowledge the rare diseases reciprocally benefit as well. So that people with rare diseases feel less unseen. Wishing you all the best.

Thanks for watching it till the end and noticing. You are right there is a tiny bit missing. I record the videos in one take and do not edit them. It feels more natural, but therefor my videos are not flawless. I shorten the videos to stop just before I exit presentation mode on my computer (last seconds). And this time I cut out too early. The final sentence is: "We are going to talk about hemorrhagic stroke in the next Brain Bit by Bit, so I do hope you will stay tuned".

Thanks. The swiss cheese - with non-enhancing holes in the enhancing cheese - is a qualitative description of the enhancement pattern . And a description of the enhancement pattern is always a bit subjective. On histology in radiation necrosis the non-enhancing dots or "circles" on imaging are necrotic tissue surrounded by enhancing reactive gliosis. In pseudoprogression the underlying pathology is leaky vessels and edema and enhancement in the brain parenchyma, but typically not dot-like necrotic tissue in it, so no holes in a cheese. Strictly/radio-pathological "swiss cheese" or soap bubbles fits radiation necrosis and not pseudoprogression. But from a practical point of view, I have seen cases of pseudo progression with a pattern reminding me of swiss cheese (and not only before lunch when I was hungry).

@@brain-bitbybit2009 thank you for the explanation. From a medical perspective; what is the relevance of differentiating between pseudoprogression and radionecrosis?

@@baybars_0 Well, pseudoprogression is a good thing (looking bad), because it means there is a immune response: it has a more favourable outcome and survival compared to patients without a lot of visible changes after therapy. And radio necrosis is a bad thing, because it means the tissue has died. From a practical point of view you cannot always tell the difference. So I "cheat" by looking at the timing after therapy of the changes and take that into account, in my report I communicate doubt if I have it and we discuss the imaging findings in a multidisplinary team. Depending on the clinical information, often the therapy is continued and we do follow up scan to see what is was/is.

It is a very good question. As a radiologist I only know what I heard from clinicians, at conferences or from literature. Memory problems and dementia like symptoms have been described in patient with (atypical, unusual presentation of) intracranial hypotension. The exact mechanism is unclear to me. When I have given it some thought I might discuss it in the future topics with the glymphatic system and CSF dynamics.

Involvement of the corticospinal tract is not a predominant feature of MLD. As shown in the cases MLD affects first the deep cerebral white matter. But the internal capsule and the brain stem's cortico spinal tract can be abnormal as well. There is no mechanism for the corticospinal tract to be spared from sulfatide-storage-damage/demyelination.

@@brain-bitbybit2009 thanks. It can affect subcortical white matter also?

​@@brain-bitbybit2009can you please tell, MLD can affect subcortical white matter also?

@@ananthkumar.m1755 you have a lot of interest in MLD - For a special reason? I myself am intrigued by the different patterns of WM involvement in leukodystrophies because they reflect the metabolism and local brain differences. I have seen only a few cases of MLD, because I don't work in a center with a metabolic focus. As far as I know the subcortical U-fibers are spared in the beginning/in typical cases, but as the disease has become more advanced the U-fibers can be involved as well.

@@brain-bitbybit2009 recently my relative got diagnosed with lueckodystrophy. So I am doing some research on this. However thanks for your details. Subcortical u fiber and subcortical white matter both are same?

The tigroid pattern happens in a few leukodystrophies. It is caused by relative sparing of the myelin in the perivenular regions. The tigroid pattern was initially described in Metachromatic leukodystrophy and Pelizaeus-Merzbacher. Later also cases of Alexander and other leukodystrophies with a striped pattern were described. Probably with the increased spatial resolution of MRI we see this pattern more often nowadays and also in more leukodystrophies. Another disease that can have a tigroid pattern is Krabbe's disease. In Krabbe the underlying cause of the stripes is not only preserved myelin on histology but also some globoid cell accumulation.

👋Thanks for watching. Google translate told me your language is Korean, otherwise I would not have been able to understand your comment.

أتمنى الصحة والعافية لابنتك أنا كذلك أم لطفلة تعاني نفس المرض اريد ان أسألك هل لديك أطفال اخرين سالمين انا حامل وخائفة أن يتكرر مع هذا الحمل

Hello, Well noticed! The ganglionic eminence and germinal matrix are kind of interchangeable at the end of pregnancy, so then you are correct. But in the first embryonic (roughly first half) period the germinal matrix is much more than the ganglionic eminence. The germinal matrix is where the precursors from the neurons and glial cells divide. So early in pregnancy is surrounds the entire ventricle. The germinal matrix is all the dark pink in the slide from an 8 week old embryo (beginning video), which is pretty thick surrounding the 3rd and 4th ventricle. The germinal matrix is the dark purple on the image from 15 weeks in the image from Del Biglio et al at (in the video about 1 minute). The germinal matrix regresses during pregnancy and you can see on the 2nd and 3rd column of the image from Del Biglio/from 20 and 26 weeks that it remains at the ganglionic eminence (labelled GE). Hope this clarifies. Christianne

@@brain-bitbybit2009 I guess I understood what you mean, but my English is not good enough. So I want to confirm what i got. In image of Del Biglio, the first row illustrate GE and the second row illustrate the germinal matrix ? so can i consider that germinal matrix is an structure that superimpose GE ( or belong to GE. For example GE like a house and germinal matrix is a thing inside it ) ?

@@longvo4072 I consider the GE to be a part of the germinal matrix. The 5 images in the upper row, the purple ones, are H&E staining at different timepoints and the 5 blueish images in the lower row are stained for proliferation (Ki67). The germinal matrix = "GE plus tissue surrounding ventricles" in the left images and the germinal matrix = only caudathalamic groove in the images on the right.

@@brain-bitbybit2009 thanks for your explanation.

@@brain-bitbybit2009 thanks very much for your explanation.

Thanks for watching. Leigh is a heterogenous disease, so prognosis differs and treatment should be tailored to the underlying mutation/defect. If this is a personal question, your own doctor or specialist in the region where you live can help, explain and inform you. In general this website is useful for MDs and patients: www.ninds.nih.gov/ with a section on Leigh syndrome and treatment. For MDs: There was an interesting article in Neuropediatrics 2014 with review of long term survivors DOI: 10.1055/s-0034-1383823

The amount of information in the vlogs and way it is brought can be difficult to frame if you do not have a medical background. (BBbB is intended for medical professionals) The vlogs are informative and for specific questions you should ask your own doctor.

Hello, Thank you for your comment. I am not sure what you mean. On the image from Michel SJ and Given CA in Radiology 2006; 241(1): 310-314 the carotids are visible and normal. On the image from Yalcinkaya C, Benbir G, Salomons GS et al from Neuropediatrics 2005; 36(5): 336-9 the carotid is visible bilateral in the cavernous sinus with normal caliber and there is a flow void from CSF/pulsation surrounding the basilar artery. I have no indication for vascular problems on these images. To answer the first part of your question: I am not aware of elastin issues in Canavan. Did you find a description or case of this on the internet? Elastin and Canavan (ASPA) is not a relation I can explain pathophysiologically with my knowledge and therefore I do not think is very likely. Have a nice day and let me know if something else triggers a question, C

@@brain-bitbybit2009 My apologies, I was looking at other cases of this disease with sites like Radiopaedia, it can be seen with the Niemann-Pick (Type C) cases too if you check, which is also prevalent with Ashkenazi Jewish cases, they show a very close Carotid Sheath space, the flow voids are very slim for example, should be much wider to allow for all the structures in the Carotid Sheath, the fascia wrapping around all the structures in the cervical spine, so much more than just the Carotids as you mention, what I saw was a lot of compression in the C2 area, as with trauma or connective tissue disorders. As for the Elastin reference, if you google that with the term "Ashkenazi Jewish" you will find the article I was reading that suggested integrity issues of the pelvic area so that lack of soft tissue (ligament?) integrity this article which I was referring to. Message me if you want me to share some of those images if you don't find them on Radiopaedia. article.imrpress.com/journal/CEOG/47/1/10.31083/j.ceog.2020.01.5100/1581669631803-861037243.pdf

You are welcome, hope the video was helpful. To answer your question: Everybody has their own way of reporting/describing the MRI. I would state the obvious first (there is an asymmetry), identify the abnormal hemisphere (the larger one in this case) and list the abnormalities - blurred GW boundary etc - and then conclude it is HME.

Hi, how is your son doing now?

I am sorry, but I do not understand your question. Can you rephrase it?

It doesn't go away it's ur brain.

Happy that this video was helpful for you. A video on MOGHE and the new ILAE classification has been published last month: ua-cam.com/video/XrUjcasGMpg/v-deo.html

I hope the video did not confuse you and your daughter is doing well. The amount of information in the vlogs and way it is brought can be difficult to frame if you do not have a medical background.