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. 2025 Aug 24;197(28):E862-E866.
doi: 10.1503/cmaj.250313.

Neurosarcoidosis presenting as possible normal pressure hydrocephalus in a 71-year-old man

Jihad Yaqoob Ali Al Kharbooshi  1 , Cathy Meng Fei Li  2 , C Thomas Appleton  2 , Courtney S Casserly  2
Affiliations

Neurosarcoidosis presenting as possible normal pressure hydrocephalus in a 71-year-old man

Jihad Yaqoob Ali Al Kharbooshi et al. CMAJ. .
No abstract available

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Conflict of interest statement

Competing interests:: Thomas Appleton has received honoraria or consulting fees from AbbVie, Novartis, Pfizer, UCB, Fresenius, and KABi. Courtney Casserly has received honoraria or consulting fees from EMD Serono, Biogen Idec, Roche, Amgen, Novartis, Sanofi Genzyme, Teva, Alexion, and Horizon. She has served as the principal investigator for an investigator-initiated clinical trial sponsored by Biogen and has received the Western Teaching Innovation Award 2024. She is the Ontario representative at the Canadian Neurological Society and an expert consultant on the MOG Project.

Figures

Figure 1:
Figure 1:
Brain computed tomography (CT) at initial presentation of a 71-year-old man with cognitive decline and gait instability. (A) Axial noncontrast CT scan of the patient’s brain showing lateral ventricular enlargement, with an Evans index of 0.33 (normal: 0.20–0.25; borderline or early ventriculomegaly: 0.25–0.30; ventriculomegaly: > 0.30). Evans index is calculated as the ratio between the maximum width of the frontal horns (solid blue arrow) and the maximal internal diameter of the skull (dashed red arrow). (B) Coronal CT image of the head showing features consistent with disproportionately enlarged subarachnoid-space hydrocephalus, characterized by ventriculomegaly (green star), widened Sylvian fissures (dashed red circle), and high convexity crowding (solid blue circle). The callosal angle (the angle between the 2 white lines) is reduced to 78° (normal 100°–120°). Periventricular hypodensities are noted around the bilateral frontal horns, indicative of transependymal cerebrospinal fluid flow (yellow arrows in panels A and B).
Figure 2:
Figure 2:
Brain and spine magnetic resonance imaging obtained 1 week after admission. (A) Axial fluid-attenuated inversion recovery image of the brain showing substantial enlargement of the ventricular system with associated periventricular hyperintensities (solid yellow arrows), consistent with transependymal cerebrospinal fluid flow, similar to findings in Figure 1. (B) Sagittal T1-weighted postcontrast image showing an abnormal nodular leptomeningeal enhancement involving the upper cervical spinal cord, medulla, ventral pons, and the floor of the fourth ventricle (dashed orange arrows). (C) Sagittal T1-weighted postcontrast spine and (D) corresponding axial image showing faintly enhancing intramedullary nodular lesions (blue arrows) in the spinal cord at the T12–L1 level (conus medullaris). The spinal cord appears abnormally expanded at this level.
Figure 3:
Figure 3:
Histopathology findings (nonnecrotizing granulomatous inflammation). (A) The inflammatory cell infiltrate comprises mononuclear inflammatory cells (blue arrows), plasma cells (yellow arrows), and macrophages including focal multinucleated Langhans giant cells (black arrow). (B) CD68 highlights the macrophages and Langhans giant cells. (C) CD3 delineates the abundant mononuclear inflammatory cells as T-lymphocytes. (D) CD138 confirms the plasma cell component. Scale bar represents 60 μm. Immunohistochemical staining with anti-CD68, anti-CD3, and anti-CD138 antibodies was performed to identify macrophages, T-lymphocytes, and plasma cells, respectively.
Figure 4:
Figure 4:
Brain and spine magnetic resonance imaging (MRI) following shunt insertion and subsequent medical treatment. (A) Axial T2-weighted brain MRI with a fluid-attenuated inversion recovery sequence obtained 3 weeks after shunt placement showing decreased ventricular size and resolution of periventricular hyperintensities from transependymal cerebrospinal fluid flow, compared with Figure 2A. (B) Sagittal T1-weighted postcontrast brain MRI and (C) sagittal T1-weighted fat-saturated postcontrast spine MRI obtained 1 year after treatment with steroids and methotrexate showing resolution of previously noted abnormal enhancement.
See this image and copyright information in PMC

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