| Identifier |
walsh_2014_s3_c2-2 |
| Title |
Is it a Crime to be Blind? I plead the 4th! |
| Creator |
Jasmine Gopwani, Edward Margolin, Tran Le, Wayne T. Cornblath, MD, Clinical Professor, Ophthalmology and Visual Sciences, Professor, Department of Neurology, University of Michigan, Rasmus Kiehl |
| Subject |
Pachimeningitis; IgG-4 |
| History |
A 30 year old previously healthy man from Sudan presented to a community hospital with a seizure. MRI revealed a 'right frontal lobe tumor'. Brain biopsy was performed but the pathology findings were inconclusive. Initial interpretation was "brain tissue with increased cellularity consistent with gliosis but no evidence of malignancy", however second opinion was of "gliomatosis cerebri with the growth pattern of exceptionally extensive infiltration". Yet another pathologist read it as "mild lymphocytic meningitis with no definite neoplasia". CSF analysis demonstrated mild lymphocytosis but normal cytology and flow cytometry. The patient was treated conservatively until nine months later when he was admitted for a psychotic episode with hallucinations after attempting to stab the floor nurse with a knife. Neuro-imaging revealed new a left frontal lobe lesion. Second brain biopsy was suggestive of chronic encephalitis and meningitis. Extensive infectious work-up was negative. Neuroimaging was unchanged. Six months later vision on the right deteriorated over two weeks to no light perception, vision on the left was 20/20. The right optic nerve was very pale. MRI, in addition to previous surgical changes, showed enhancement of the right optic nerve. A course of high dose intravenous steroids was given; however, the vision remained unchanged on the right, and two months later vision in the left eye started to deteriorate and measured 20/70. Another course of high dose intravenous steroids was administered. Extensive work up including CT chest/abdomen/pelvis, repeated CSF analysis, neuroimaging, and multiple serologies was non-contributory. One month later vision in the right eye improved to light perception and dramatically declined on the left to no light perception. Another course of high dose steroids was administered. MRI demonstrated leptomeningeal enhancement and involvement of the cavernous sinus. A gold quantiferon test returned positive and presumptive diagnosis of tuberculous meningoencelphalitis was made. Triple anti-tuberculous therapy was initiated. A diagnostic procedure was performed. |
| Pathology |
Immunoglobulin G4-related Hypertrophic Pachymeningitis |
| Disease/Diagnosis |
Immunoglobulin G4-related hypertrophic pachymeningitis involving cerebral parenchyma and causing severe bilateral optic neuropathy. |
| Presenting Symptom |
A 30 year old previously healthy man from Sudan presented to a community hospital with a seizure. |
| Neuroimaging |
Magnetic Resonance Imaging |
| Date |
2014-03 |
| References |
1. Goyal M, Malik A, Mishra NK, Gaikwad SB. Idiopathic hypertrophic cranial pachymeningitis: Spectrum of disease. Neuroradiology. 1997; 39:619-23. 2. Harada T, Ohasi T, Ohki K et al. Optic neuropathy associated with hypertrophic cranial pachymeningitis. Br J Ophthalmology1996; 80:574-575. 3. Wallace ZS, Carruthers MN, Khosroshahi A, Carruthers R, Shinagare S, Stemmer-Rachamimov A, Deshpande V, Stone JH. IgG4-related disease and hypertrophic pachymeningitis. Medicine. 2003; 92 (4):206. 4. Kim EH, Kim SH, Cho JM, Ahn JY, Chang JH. Immunoglobulin G4-related hypertrophic pachymeningitis involving cerebral parenchyma. J Neurosurg. 2001; 115 (6):1242. |
| Language |
eng |
| Format |
video/mp4 |
| Type |
Image/MovingImage |
| Source |
46th Annual Frank Walsh Society Meeting |
| Relation is Part of |
Case presented at the NANOS 2014 Walsh Session, March 2 |
| Collection |
Neuro-Ophthalmology Virtual Education Library - Walsh Session Annual Meeting Archives https://novel.utah.edu/Walsh/index3.html |
| Publisher |
Spencer S. Eccles Health Sciences Library, University of Utah |
| Holding Institution |
Spencer S. Eccles Health Sciences Library, University of Utah, 10 N 1900 E SLC, UT 84112-6265 |
| Rights Management |
Copyright 2014. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright |
| ARK |
ark:/87278/s6bz93nf |
| Setname |
ehsl_novel_fbw |
| ID |
179218 |
| Reference URL |
https://collections.lib.utah.edu/ark:/87278/s6bz93nf |
