External beam radiation for localized periocular amyloidosis: a case series

Copperman, Thomas S; Truong, Minh Tam; Berk, John L.

doi:10.1080/01676830.2018.1483407

Cited by 15 publications

(11 citation statements)

References 21 publications

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“…A positive effect of LD-RT on amyloid load was previously reported in patients presenting peripheral amyloidosis [30][31][32][33][34][35][36] and recently in three AD mouse models [15][16][17]. Nevertheless, this reduction of the number of amyloid plaques was not observed in our rat model.…”

Section: Discussionsupporting

confidence: 42%

Treatment by Low-Dose Brain Radiation Therapy Improves Memory Performances without Changes of the Amyloid Load in the TgF344-AD Rat Model.

Ceyzériat¹,

Zilli²,

Fall³

et al. 2020

Preprint

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Background. Alzheimer’s disease (AD) is a neurodegenerative condition affecting memory performance. This pathology is characterized by intracerebral amyloid plaques and tau tangles coupled with neuroinflammation. During the last century, numerous therapeutic trials, targeting amyloid, tau or neuroinflammation, unfortunately failed. Development of new therapeutic approaches for AD is eagerly awaited. In this study, we propose to test the use of low-dose brain radiotherapy (LD-RT). LD-RT showed efficacy to reduce amyloid load in patients with peripheral pathologies and also presents anti-inflammatory effects in patients with systemic inflammatory diseases. Furthermore, previous evidence showed an effect on amyloid load, memory performance and modulation of the immune response in AD mice. Methods. The hemi-brain of 14-15 months old TgF344-AD rats was irradiated with two LD-RT schedules (5 fractions of 2 Gy, delivered weekly or daily). Rats underwent behavioral tests to assess memory and general locomotion, before and/or 4 months post treatment. LD-RT impact on amyloid and neuroinflammation was measured using immunohistology.Results. An improvement of spatial working memory was observed in the daily treated group but not in animals treated weekly. On the other hand, both LD-RT schedules were unable to impact amyloid load. An increase of astrocyte reactivity was observed in TgAD groups compared to wild-type animals but LD-RT did not impact it. The weekly treatment increased CD68+ microglial density in the molecular layer of the hippocampus but no effect was observed with the daily treatment. One major limitation of this study was the already well-established amyloid pathology, which was probably too advanced to observe a potential decrease. Nevertheless, we observed a memory improvement, the main cognitive symptoms of AD, after daily treatment, offering encouraging data to further evaluate the LD-RT therapeutic potential. Conclusions. This study showed a biological effect of LD-RT in an AD rat model on memory performance (after daily treatment) and microglial density (after weekly treatment). The lack of effect of both regimens on amyloid pathology, unexpected, was possibly due to the advanced pathology of our animals. The positive effect on cognition encourages to further evaluate the LD-RT therapeutic potential at an earlier AD stage.

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Section: Discussionsupporting

confidence: 42%

Treatment by Low-Dose Brain Radiation Therapy Improves Memory Performances without Changes of the Amyloid Load in the TgF344-AD Rat Model.

Ceyzériat¹,

Zilli²,

Fall³

et al. 2020

Preprint

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“…We confirm the effectiveness of radiotherapy, particularly in combination with surgical debulking, in selected cases. [17][18][19] However, in general we observed no differences in outcome according to different treatment regimens.…”

Section: Discussionmentioning

confidence: 53%

“…MC and/or abFLCR (34) LC isotype of MC and/or abFLCR matching with aLC (18) Clonal infiltrate at amyloid deposition site (n = 154) involvement (29% vs 6%; P < .001), respectively. Patients with lung locAL were more frequently asymptomatic (71% vs 23%; P < .001).…”

Section: Statistical Analysesmentioning

confidence: 99%

Localized immunoglobulin light chain amyloidosis: Novel insights including prognostic factors for local progression

et al. 2020

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In localized light chain amyloidosis (locAL), amyloidogenic light chains (aLC) are produced and deposited locally by a B‐cell clone. We present 293 patients with immunohistochemically confirmed locAL. Lung (nodular pulmonary) with 63 patients was the most involved organ. The aLC was λ in 217 cases (κ:λ ratio 1:3). A local B‐cell clone was identified in 30% of cases. Sixty‐one (21%) had a concomitant autoimmune disorder (cAD). A monoclonal component (MC) were present in 101 (34%) patients and were more frequent in subjects with cAD (51% vs 34%; P = .03). Cigarette smoking was more prevalent in lung locAL (54% vs 37%; P = .018). After a median follow‐up of 44 months, 16 patients died and 5‐ and 10‐years locAL progression‐free survival (PFS) were 62% and 44%. Interestingly, locAL‐PFS was shorter among patients with an identified clonal infiltrate at amyloid deposition site (40 vs 109 months; P = .02) and multinuclear giant cells and/or an inflammatory infiltrate resulted in longer locAL‐PFS in lung involvement (65 vs 42 months; P = .01). However, no differences in locAL PFS were observed in patients with cAD, a MC and involved organ site. Treatment was administered in 163 (54%) patients and was surgical in 135 (46%). Median locAL‐PFS after first treatment was 56 months. Responders had longer locAL‐PFS (78 vs 17 months; P < .001). Three patients with lung locAL and a MC were diagnosed as systemic AL amyloidosis at follow‐up. In summary, locAL pathogenesis seems to be heterogeneous and the clonal infiltrate leads local progression.

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“…However, the efficacy of treatment for this patient is currently indeterminate given the short follow-up time. Copperman et al 43 reported a series of localized orbital amyloidosis in which external beam radiation therapy was used as an adjunct to surgery. At 1-year follow-up, there were no signs of progression, but after more than 2 years of follow-up, 2 out of 4 patients experienced progressive amyloid deposition, of which one required further surgical debulking.…”

Section: Discussionmentioning

confidence: 99%

Clinical and Radiological Features of Intramuscular Orbital Amyloidosis: A Case Series and Literature Review

Tong

Juniat

McKelvie

et al. 2021

Ophthalmic Plastic &Amp; Reconstructive Surgery

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Purpose: Orbital amyloidosis of the extraocular muscles (EOMs) is a rare condition, and its clinicoradiological features are not well elucidated. This study describes the characteristic clinical signs, MRI features, and potential treatment options. Methods: Retrospective multicenter case series and literature review of EOM amyloidosis. Results: Five cases were identified for inclusion. Common clinical findings were diplopia, ophthalmoplegia, and proptosis. Systemic amyloidosis was more likely to present with multiple muscle involvement, but no particular pattern was observed with localized disease. On MRI, amyloid deposition was characterized as a heterogeneous intramuscular mass with T2 hypointensity and post contrast enhancement. Management is dependent on the extent of disease and functional impairment; options include surgical debulking and radiation therapy. Conclusion: EOM amyloidosis is uncommon. The combination of clinical and radiologic findings described in this study should lead to its clinical suspicion.

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External beam radiation for localized periocular amyloidosis: a case series

Cited by 15 publications

References 21 publications

Treatment by Low-Dose Brain Radiation Therapy Improves Memory Performances without Changes of the Amyloid Load in the TgF344-AD Rat Model.

Treatment by Low-Dose Brain Radiation Therapy Improves Memory Performances without Changes of the Amyloid Load in the TgF344-AD Rat Model.

Localized immunoglobulin light chain amyloidosis: Novel insights including prognostic factors for local progression

Clinical and Radiological Features of Intramuscular Orbital Amyloidosis: A Case Series and Literature Review

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