Brain edema with clasmatodendrosis complicating ataxia telangiectasia

Shimoda, Konomi; Mimaki, Masakazu; Fujino, Shuhei; Takeuchi, Masato; Hino, Rumi; Uozaki, Hiroshi; Hayashi, Masaharu; Oka, Akira; Mizuguchi, Masashi

doi:10.1016/j.braindev.2017.02.007

Cited by 4 publications

(6 citation statements)

References 7 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…In contemporary clinical and experimental reports, clasmatodendrosis has been observed in white matter disease with or without dementia [7,8,9,10,11], models of interaction between astrocytes and beta-amyloid plaques [12,13], in hippocampal models and cell cultures [14,15,16,17], head trauma [18,19,20,21], models of cerebral ischemia [22,23,24,25,26], models of status epilepticus [27,28,29,30,31], in toxic exposures [32,33,34,35], demyelinating diseases [36,37,38], in osmotic-induced demyelination and an inherited condition with cerebral edema [39,40], in association with autophagy and/or apoptosis [24,27,41,42,43,44], encephalitides or infection-associated encephalopathies [45,46,47,48], and in chimeric antigen receptor (CAR) T-cell therapy [49,50]…”

Section: Introductionmentioning

confidence: 99%

The phenomenon of clasmatodendrosis

Balaban

Miyawaki

Bhattacharyya

et al. 2021

Heliyon

View full text Add to dashboard Cite

Clasmatodendrosis derives from the Greek for fragment (klasma), tree (dendron), and condition (-osis). Cajal first used the term in 1913: he observed disintegration of the distal cell processes of astrocytes, along with a fragmentation or beading of proximal processes closer to the astrocyte cell body. In contemporary clinical and experimental reports, clasmatodendrosis has been observed in models of cerebral ischemia and seizures (including status epilepticus), in elderly brains, in white matter disease, in hippocampal models and cell cultures associated with amyloid plaques, in head trauma, toxic exposures, demyelinating diseases, encephalitides and infection-associated encephalopathies, and in the treatment of cancer using immune effector cells. We examine evidence to support a claim that clasmatodendrotic astrocyte cell processes overtly bead (truncate) as a morphological sign of ongoing damage premortem. In grey and white matter and often in relationship to vascular lumina, beading becomes apparent with immunohistochemical staining of glial fibrillary acidic protein when specimens are examined at reasonably high magnification, but demonstration of distal astrocytic loss of processes may require additional marker study and imaging. Proposed mechanisms for clasmatodendrotic change have examined hypoxic-ischemic, osmotic-demyelinating, and autophagic models. In these models as well as in neuropathological reports, parenchymal swelling, vessel-wall leakage, or disturbed clearance of toxins can occur in association with clasmatodendrosis. Clasmatodendrotic features may serve as a marker for gliovascular dysregulation either acutely or chronically. We review correlative evidence for blood-brain barrier (BBB) dysfunction associated with astrocytic structural change, with attention to interactions between endothelial cells, pericytes, and astrocytic endfeet.

show abstract

Section: Introductionmentioning

confidence: 99%

The phenomenon of clasmatodendrosis

Balaban

Miyawaki

Bhattacharyya

et al. 2021

Heliyon

View full text Add to dashboard Cite

show abstract

“…50 cases (13 studies) reported pneumothorax. 2 cases (2 studies [ 91 , 240 ]) reported bilateral pneumothoraces. A further 5 cases (1 study [ 175 ]) reported that they had 2 pneumothoraces, but it could not be discerned if it was bilateral or two separate events.…”

Section: Resultsmentioning

confidence: 99%

The natural history of ataxia-telangiectasia (A-T): A systematic review

et al. 2022

View full text Add to dashboard Cite

Background Ataxia-telangiectasia is an autosomal recessive, multi-system, and life-shortening disease caused by mutations in the ataxia-telangiectasia mutated gene. Although widely reported, there are no studies that give a comprehensive picture of this intriguing condition. Objectives Understand the natural history of ataxia-telangiectasia (A-T), as reported in scientific literature. Search methods 107 search terms were identified and divided into 17 searches. Each search was performed in PubMed, Ovid SP (MEDLINE) 1946-present, OVID EMBASE 1980 –present, Web of Science core collection, Elsevier Scopus, and Cochrane Library. Selection criteria All human studies that report any aspect of A-T. Data collection and analysis Search results were de-duplicated, data extracted (including author, publication year, country of origin, study design, population, participant characteristics, and clinical features). Quality of case-control and cohort studies was assessed by the Newcastle-Ottawa tool. Findings are reported descriptively and where possible data collated to report median (interquartile range, range) of outcomes of interest. Main results 1314 cases reported 2134 presenting symptoms. The most common presenting symptom was abnormal gait (1160 cases; 188 studies) followed by recurrent infections in classical ataxia-telangiectasia and movement disorders in variant ataxia-telangiectasia. 687 cases reported 752 causes of death among which malignancy was the most frequently reported cause. Median (IQR, range) age of death (n = 294) was 14 years 0 months (10 years 0 months to 23 years 3 months, 1 year 3 months to 76 years 0 months). Conclusions This review demonstrates the multi-system involvement in A-T, confirms that neurological symptoms are the most frequent presenting features in classical A-T but variants have diverse manifestations. We found that most individuals with A-T have life limited to teenage or early adulthood. Predominance of case reports, and case series demonstrate the lack of robust evidence to determine the natural history of A-T. We recommend population-based studies to fill this evidence gap.

show abstract

“…2,6,8,9 A notable finding of our study is that the LPS100 þ HT group exhibited astrocytic clasmatodendrosis and putative phagocytosis of degenerating astrocytes by activated microglia. Astrocytic clasmatodendrosis is characterized by swollen cell cytoplasm and vacuolated cell bodies with beaded and fragmented processes and is associated with various degenerative disorders in human patients, including Alzheimer's disease, poststroke dementia, mixed dementia, and Ataxia telangiectasia [47][48][49][50][51][52] as well as in experimental conditions. 53,54 In fact, the postmortem immunohistochemical analysis of the brains of all patients with ANE and HSES has confirmed the presence of clasmatodendritic astrocytes.…”

Section: Discussionmentioning

confidence: 99%

Developing a mouse model of acute encephalopathy using low-dose lipopolysaccharide injection and hyperthermia treatment

Kurata

Saito

Kawashima

et al. 2019

Exp Biol Med (Maywood)

View full text Add to dashboard Cite

Acute encephalopathy (AE) is mainly reported in East Asia and, in most cases, results from pediatric viral infections, leading to fever, seizure, and loss of consciousness. Cerebral edema is the most important pathological symptom of AE. At present, AE is classified into four categories based on clinical and pathophysiological features, and cytokine storm-induced AE is the severest among them. The pathogenesis of AE is currently unclear; this can be attributed to the lack of a simple and convenient animal model for research. Here, we hypothesized that the induction of systemic inflammation using lipopolysaccharide (LPS) injection followed by hyperthermia (HT) treatment can be used to develop an animal model of cytokine storm-induced AE. Postnatal eight-day-old mouse pups were intraperitoneally injected with low-dose LPS (50 or 100 mg/kg) followed by HT treatment (41.5 C, 30 min). Histological analysis of their brains was subsequently performed.Fluorescein isothiocyanate assay combined with immunohistochemistry was used to elucidate blood-brain barrier (BBB) disruption. LPS (100 mg/kg) injection followed by HT treatment increased BBB permeability in the cerebral cortex and induced microglial activation. Astrocytic clasmatodendrosis was also evident. The brains of some pups exhibited small ischemic lesions, particularly in the cerebral cortex. Our results indicate that a low-dose LPS injection followed by HT treatment can produce symptoms of cytokine storm-induced AE, which is observed in diseases, such as acute necrotizing encephalopathy and hemorrhagic shock and encephalopathy syndrome. Thus, this mouse model can help to elucidate the pathogenetic mechanisms underlying AE.

show abstract

Brain edema with clasmatodendrosis complicating ataxia telangiectasia

Cited by 4 publications

References 7 publications

The phenomenon of clasmatodendrosis

The phenomenon of clasmatodendrosis

The natural history of ataxia-telangiectasia (A-T): A systematic review

Developing a mouse model of acute encephalopathy using low-dose lipopolysaccharide injection and hyperthermia treatment

Contact Info

Product

Resources

About