NOVEL - Frank B. Walsh Collection

A White Matter Riddle Encased in Mystery, Coiled Inside an Enigma

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Identifier walsh_2024_s3_c5
Title A White Matter Riddle Encased in Mystery, Coiled Inside an Enigma
Creator Nathan Tagg; Elizabeth Rooks; Vanessa Smith; Thomas Cummings
Affiliation (NT) (ER) (TC) Duke University; (ER) University of Hawaii
Subject Homonymous Hemianopsia; Magnetic Resonance Imaging (MRI); Aneurysm
Description A 59 y/o woman who developed the gradual onset of left homonymous hemianopsia, hemiparesis, hemisensory loss four months after undergoing endovascular stent-assisted coiling of a R MCA aneurysm. MRI imaging showed a progressive leukoencephalopathy with features of inflammation limited to the right hemisphere. Symptoms partially responded to steroids but the steroid sparing agents mycophenolate mofetil and rituximab failed to control progression over a period of 3 years. A brain biopsy showed granulomatous foreign body giant cells centered on non-polarizable filamentous material.
History A 59-year-old woman was admitted for evaluation of progressive paranoia and was treated for bipolar disorder. An MRI during evaluation revealed a 10 x 7 x 7 mm right MCA aneurysm. She underwent stent-assisted coil embolization nine months later without immediate complications. Four months post-procedure she developed gradual onset left-sided weakness and a focal aware seizure involving the left side. An MRI without contrast revealed extensive right hemispheric vasogenic edema. She continued to progress over the ensuing months and eventually developed a left homonymous hemianopsia. Repeat MRI imaging showed multilobar right hemispheric T2 changes and nodular leptomeningeal and parenchymal enhancement with no significant findings in the left hemisphere (Figures 1, 2). Extensive serum and CSF evaluation was negative. She was treated empirically with high dose steroids with improvement in symptoms. Her symptoms recurred with each attempted steroid taper, necessitating repeat admissions and steroid courses. She transitioned to mycophenolate mofetil (MMF) in combination with steroids. Further diagnostic studies were deferred at patients request and her disease progressed requiring readmission and addition of rituximab. Additional imaging revealed non-small cell lung cancer and she was treated with localized radiation therapy. Three years after symptom onset she was referred to our service and reported fluctuating symptoms responsive to steroids superimposed on a course of gradual worsening. Exam revealed a left homonymous hemianopia, left hemi-motor and sensory deficit. Extensive serum and CSF testing was remarkable only for one IgG band unique to CSF. A paraneoplastic panel was negative. PET scan was negative for new malignancy. MR vessel wall imaging was negative for vasculitis. MRI brain revealed right hemispheric encephalomalacia with residual patchy nodular enhancement (Figures 3,4). With no clear diagnosis for her unremitting leukoencephalopathy and enhancing lesions, a diagnostic brain biopsy was performed.
Disease/Diagnosis Final Diagnosis: Delayed onset leukoencephalopathy secondary to foreign body from vascular intracerebral stent or coil. A stereotactic parenchymal biopsy demonstrated multinucleated foreign body giant cells centered on nonpolarizable filamentous structures. Histological examination was most consistent with a foreign body granulomatous type reaction following intravascular stent & coil. This rare entity is though to be a consequence of either a hypersensitivity / allergic reaction to endovascular hardware or a foreign body reaction to hardware coating material (i.e. polymer).
Date 2024-03
References Ridwan S, et al. Delayed Leukoencephalopathy and Foreign Body Reaction After Endovascular Treatment in Patients With Intracranial Aneurysms and Aneurysmal Subarachnoid Hemorrhage-A Systematic Review of the Literature. Front Surg. 2021 Dec 23 Ikemura A, et al. Leukoencephalopathy: A Rare Complication after Coiling of Cerebral Aneurysms. AJNR Am J Neuroradiol. 2020 Feb;41(2):286-292 Miyamoto S, et al. Reversible and multiphasic parenchymal changes in MRI after coil embolization for a ruptured cerebral aneurysm. Surg Neurol Int. 2023 Apr 21;14:147 Bakola E, et al. Delayed recurrent enhancing white matter lesions complicating coiling of intracranial aneurysm. Eur J Neurol. 2021 Jul;28(7):2388-2391 Bakola E, et al. Delayed Leukoencephalopathy as a Complication after Endovascular Therapy of Intracranial Aneurysms-A Case Series. J Clin Med. 2023 Jan 7;12(2):496
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/s6az5q8k
Setname ehsl_novel_fbw
ID 2715711
OCR Text Show
Reference URL https://collections.lib.utah.edu/ark:/87278/s6az5q8k