Identifier |
walsh_2024_s2_c2 |
Title |
Headache and Visual Deficits: Ironing Out the Details |
Creator |
Amalie Chen; Leigh Rettenmaier; Andres Santos; Mariel Kozberg; Sashank Prasad |
Affiliation |
(AC) Mass Eye & Ear; Brigham & Women's Hospital; (LR) Massachusetts General Hospital; (AS) Brigham & Women's Hospital; (MK) Mass General Hospital; (SP) Perelman School of Medicine, University of Pennsylvania |
Subject |
Cerebral Amyloid Angiopathy; Vision Field Loss; Balint Syndrome; Seizure |
Description |
A 67-year-old woman with hypertension, hyperlipidemia, and diabetes developed posterior-predominant, non-positional headaches associated with nausea and vomiting. Two weeks after headache onset, she developed significant visual hallucinations. She described seeing "graphics," consisting of formed and unformed images and words coming and going throughout her field of vision. She noticed that faces looked distorted, but she did not have difficulty recognizing familiar people. She could not read the time on a clock or use her phone despite being able to see both. She had difficulty navigating unfamiliar settings, needing to hold onto walls or furniture to get around. She denied frank vision loss or double vision. She denied recent fever, rash, insect bites, unintentional weight loss, night sweats, or fatigue. Examination demonstrated normal level of arousal, comprehension, fluency of speech, repetition, reading, and writing. Visual acuity and color vision were normal. Visual field testing demonstrated a dense, congruous, left homonymous hemianopsia. She had impaired visual search within the intact field, but naming of visually presented objects was normal. On the Navon figure, she described the local elements but not the global form. She did not demonstrate left-sided neglect for tactile stimuli or with line cancellation testing. Pupils were equal and briskly reactive, with no relative afferent pupillary defect. Slit lamp and funduscopic exam were unremarkable. Efferent exam was normal. Cranial nerve, motor, sensory, cerebellar and gait exams were normal. Reflexes were 2+ and symmetric throughout, except the left plantar response was extensor. |
History |
MRI brain with gadolinium contrast revealed diffuse leptomeningeal enhancement, most prominently in the bilateral temporal parieto-occipital and left frontal sulci (Fig.1), with associated T2/FLAIR sulcal hyperintensity (Fig.2). T2-weighted images showed enlarged perivascular spaces (i.e. Virchow-Robin spaces) in the centrum semiovale (Fig.3). There were no other significant parenchymal T2-FLAIR abnormalities. Susceptibility-weighted imaging (SWI) lacked cortical superficial siderosis or microhemorrhages (Fig.4). CSF showed RBC 0 cells/mL, 4 WBC cells/mL, protein 78 mg/dL, glucose 89 mg/dL, and was negative for xanthochromia, malignant cells, infection, or autoimmune antibodies. Body PET/CT was normal. ESR and CRP were normal. ANA was negative and other serum autoimmune markers including ACE were normal. The patient had a witnessed generalized tonic-clonic seizure captured on EEG, which demonstrated a right temporal-onset seizure with generalization, as well as independent left temporal spike-wave discharges. She was initiated on levetiracetam and had no further seizures. Before the initiation of corticosteroids or other empiric therapies, she underwent a right occipital craniotomy with open biopsy that revealed thickened small and medium vessels within the leptomeninges and superficial neocortex with amorphous eosinophilic deposits in the vessel wall (Fig.5). Congo red stain of these deposits demonstrated apple-green birefringence under polarized light (Fig.6). Beta-amyloid immunostaining showed extensive amyloid deposition in the leptomeningeal and superficial cortical vessels, with scattered intraparenchymal amyloid plaques (Fig.7). Tau immunohistochemistry was negative. Additionally, a prominent perivascular lymphoid infiltrate was identified in the leptomeninges (Fig.8) without significant parenchymal inflammation. There were no microhemorrhages or hemosiderin deposits noted. Leptomeningeal-predominant cerebral amyloid angiopathy - related inflammation (CAA-ri) was diagnosed. She was treated with IV methylprednisolone with prompt significant improvement in her headaches and visual deficits. She has been continued on a prednisone taper and transitioned to mycophenolate mofetil immunotherapy without further relapse to date. |
Disease/Diagnosis |
Leptomeningeal-predominant cerebral amyloid angiopathy-related inflammation causing headache, homonymous hemianopia, visual processing deficits, and seizures. |
Date |
2024-03 |
References |
1. Auriel E, Charidimou A, Gurol ME, et al. Validation of Clinicoradiological Criteria for the Diagnosis of Cerebral Amyloid Angiopathy-Related Inflammation. JAMA Neurol. 2016;73(2):197-202. doi:10.1001/jamaneurol.2015.4078 2. Salvarani C, Morris JM, Giannini C, Brown RD, Christianson T, Hunder GG. Imaging Findings of Cerebral Amyloid Angiopathy, Aβ-Related Angiitis (ABRA), and Cerebral Amyloid Angiopathy-Related Inflammation. Medicine (Baltimore). 2016;95(20):e3613. doi:10.1097/MD.0000000000003613 3. van Veluw SJ, Scherlek AA, Freeze WM, et al. Different microvascular alterations underlie microbleeds and microinfarcts. Ann Neurol. 2019;86(2):279-292. doi:10.1002/ana.25512 4. Moussaddy A, Levy A, Strbian D, Sundararajan S, Berthelet F, Lanthier S. Inflammatory Cerebral Amyloid Angiopathy, Amyloid-β-Related Angiitis, and Primary Angiitis of the Central Nervous System. Stroke. 2015;46(9):e210-e213. doi:10.1161/STROKEAHA.115.010024 5. Kozberg MG, Yi I, Freeze WM, et al. Blood-brain barrier leakage and perivascular inflammation in cerebral amyloid angiopathy. Brain Commun. 2022;4(5):fcac245. doi:10.1093/braincomms/fcac245 6. Piazza F, Greenberg SM, Savoiardo M, et al. Anti-amyloid β autoantibodies in cerebral amyloid angiopathy-related inflammation: Implications for amyloid-modifying therapies. Ann Neurol. 2013;73(4):449-458. doi:10.1002/ana.23857 7. Charidimou A, Boulouis G, Frosch MP, et al. The Boston Criteria v2.0 for cerebral amyloid angiopathy: A multicentre MRI-neuropathology diagnostic accuracy study. Lancet Neurol. 2022;21(8):714-725. doi:10.1016/S1474-4422(22)00208-3 |
Language |
eng |
Format |
application/pdf |
Type |
Text |
Source |
2024 North American Neuro-Ophthalmology Society Annual Meeting |
Relation is Part of |
NANOS Annual Meeting 2024 |
Collection |
Neuro-Ophthalmology Virtual Education Library: Walsh Session Annual Meeting Archives: https://novel.utah.edu/Walsh/ |
Publisher |
North American Neuro-Ophthalmology Society |
Holding Institution |
Spencer S. Eccles Health Sciences Library, University of Utah |
Rights Management |
Copyright 2024. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright |
ARK |
ark:/87278/s6sa80mz |
Setname |
ehsl_novel_fbw |
ID |
2715703 |
Reference URL |
https://collections.lib.utah.edu/ark:/87278/s6sa80mz |