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. 2023 Jan-Feb;26(1):77-80.
doi: 10.4103/aian.aian_537_22. Epub 2023 Jan 17.

Obstructive Hydrocephalus as the Solitary Manifestation of Young Onset Erdheim-Chester Disease

Shreyashi Jha  1 , Dipti Baskar  1 , B N Nandeesh  1 , Seena Vengalil  1 , Ravindranadh Chaudhary  1 , Atchayaram Nalini  1 , Ravi Yadav  1 , Dwarkanath Srinivas  2
Affiliations

Obstructive Hydrocephalus as the Solitary Manifestation of Young Onset Erdheim-Chester Disease

Shreyashi Jha et al. Ann Indian Acad Neurol. 2023 Jan-Feb.
No abstract available

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Conflict of interest statement

There are no conflicts of interest.

Figures

Figure 1
Figure 1
(a) Axial T2-weighted image shows patchy hyperintensities with expansion involving the pontomedullary junction and bilateral cerebellar hemispheres. (Solid white arrows). (b) Axial T1-weighted image shows subtle hypointensity of the lesion in the left cerebellar hemisphere (solid white arrow). (c) Axial FLAIR image shows dilated lateral and third ventricles (solid white arrows) with subependymal CSF seepage, leading to FLAIR periventricular hyperintensities. (d) Right parasagittal delayed T2 sequence showing obstructive hydrocephalus with hyperintensity in the cerebellar hemisphere with mass effect on the fourth ventricle (solid white arrow). (e) Axial 3D T1 gradient post-contrast image shows patchy, feathery enhancement in bilateral cerebellar hemispheres and pons with a homogenously enhancing rounded lesion in the left cerebellar hemisphere (solid white arrow). (f) Axial chest CT lung window shows multiple cystic lesions (solid white arrows) in the anterior segments of bilateral upper lobes
Figure 2
Figure 2
(a-d) Microphotograph showing neuroparenchyma with stromal rarefaction, scattered histiocytic infiltration (arrows) along with a few lymphocytes. (H & E stain; A, B – ×100; C, D – ×200). (e and f) Microphotograph showing histiocytes (arrows) stained by CD68 (CD68, IHC ×200). (g) Microphotograph showing the absence of staining for CD1a. (CD1a, IHCX 100). (H and I) Microphotograph showing histiocytes stained with BRAFV600E IHC; (H – ×100, I – ×200)
See this image and copyright information in PMC

References

    1. Boyd LC, O'Brien KJ, Ozkaya N, Lehky T, Meoded A, Gochico BR, et al. Neurological manifestations of Erdheim–Chester Disease. Ann Clin Transl Neurol. 2020;7:497–506. - PMC - PubMed
    1. De Macêdo LP, Ferreira US, Ferreira YD, de Carvalho Júnior EV, Faquini IV, Almeida NS, et al. Erdheim-Chester disease with intracranial involvement causing hydrocephalus: Case report. Interdiscip Neurosur. 2020;21:100722.
    1. Cohen Aubart F, Idbaih A, Galanaud D, Law-Ye B, Emile J-F, Charlotte F, et al. Central nervous system involvement in Erdheim-Chester disease: An observational cohort study. Neurology. 2020;95:e2746–54. - PubMed
    1. Lachenal F, Cotton F, Desmurs-Clavel H, Haroche J, Taillia H, Magy N, et al. Neurological manifestations and neuroradiological presentation of Erdheim-Chester disease: Report of 6 cases and systematic review of the literature. J Neurol. 2006;253:1267–77. - PubMed
    1. Mazor RD, Manevich-Mazor M, Shoenfeld Y. Erdheim-Chester disease: A comprehensive review of the literature. Orphanet J Rare Dis. 2013;8:1–3. - PMC - PubMed