Admissions Ad Nauseum: A Cryptic Case of Chiasmopathy

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Identifier walsh_2013_s4_c3
Title Admissions Ad Nauseum: A Cryptic Case of Chiasmopathy
Creator Sara Ann Simpson; Alex Merkler; Ehud Lavi; Joseph Safedieh; Alan Segal; Marc Dinkin
Affiliation Weill Cornell Medical College, New York, NY
Subject Cryptococcal Meningitis; Leptomeningeal disease; Chiasmopathy
History A 38 year old healthy female presented with blurred vision. Her ophthalmologist found acuities of 20/200 OD and 20/50 OS, a central scotoma OD and a temporal hemianopia OS. Neurological examination was normal. MRI revealed patchy leptomeningeal enhancement. VEP, SSEP, BAEP were all unrevealing. ANA was elevated at 1:320. Lumbar puncture (LP) had elevated opening pressure of 31 cm of water, WBC 34, protein of 231, glucose <10 and positive oligoclonal bands. Fungal, bacterial, mycobacterial cultures, cytology and flow cytometry were negative. A second LP was similar. Our neuro-ophthalmology service found acuities of 20/200 OD and 20/30 OS, dyschromatopsia OD, a right relative afferent pupillary defect, trace temporal disc pallor OU and a generally depressed field OD and temporal hemianopsia OS consistent with a junctional scotoma. MRI demonstrated diffuse leptomeningeal enhancement within the brainstem, optic chiasm, infundibulum, pituitary gland, prepontine cistern and cranial nerves VI-VIII bilaterally. Chest CT was negative. PET scan showed increased uptake in a supraclavicular node, which was normal on biopsy. RPR, Lyme, HIV, ANA, dsDNA and ACE were negative. Bone marrow biopsy showed no malignancy. A trans-sphenoidal biopsy showed normal pituitary tissue and dura. She was treated with IV methylprednisolone for 3 days and vision improved to 20/30 OU then discharged on 60mg prednisone daily only to returned two days later with headache and visual acuity of light perception OD and 20/400 OS with visual field constriction OD>OS. Secondary to hyponatremia she experienced a generalized tonic clonic seizure. After 1 gram of IV methylprednisolone daily and sodium repletion, for three days, acuities returned to 20/50 OD and 20/30 OS. Vision declined two more times with oral prednisone taper, and improved with 1 gram IV methylprednisolone bolus. MRI showed persistent peri-chiasmatic and perineural enhancement and new hydrocephalus, a third LP was performed.
Pathology A mucicarmine stain of the pituitary biopsy was positive, consistent with the presence of mucopolysaccharides on the capsule of cryptococcal yeast at the time of the biopsy. The speciation of the yeast was Cryptococcus neoformans var grubii, commonly found in eucalyptus plant, wood and pigeon feces and found around the world.
Disease/Diagnosis Cryptococcal neoformans var grubii meningitis
Clinical Visual acuity of 20/200 OD and 20/50 OS, a central scotoma OD and a temporal hemianopia OS. VEP, SSEP, BAEP were all unrevealing. ANA was elevated at 1:320. Lumbar puncture (LP) had elevated opening pressure of 31 cm of water, WBC 34, protein of 231, glucose <10 and positive oligoclonal bands.
Presenting Symptom A 38 year old healthy female presented with blurred vision.
Neuroimaging MRI; PET Scan; CT Scan
Treatment Methylprednisolone
Date 2013-02
References 1. Betts, Robert F; SW Chapman and RL Penn. Reese and Betts' A Practical Approach to Infectious Diseases (2002) Lippincott Williams & Wilkins. 2. Cremer PD, IH Johnson and GM Halmagyi. Pseudotumour cerebri syndrome due to cryptococcal meningitis. Journal of Neurology Neurosurgery and Psychiatry (1997) 62: 96-98. 3. Hoang, LMN et al. Cryptococcus neoformans infections at Vancouver Hospital and Health Sciences Centre (1997-2002): epidemiology, microbiology and histopathology. Journal of Medical Microbiology (2004) 53, 935-490. 4. Levy, R et al. Late presentation of Cryptococcus gattii meningitis in a traveller to Vancouver Island: A case report. Canadian Journal of Infectious Disease and Medical Microbiology (2007) 18 (3): 197-199. 5. Seaton, RA et al. Visual loss in immunocompetent patients with Cryptococcus neoformans var gatii meningitis. Transactions of the Royal Society of Tropical Medicine and Hygiene (1997) 91; 44-49. 6. Seaton, RA et al. The effect of corticosteroids on visual loss in Cryptococcus neoformans var gatii meningitis. Transactions of the Royal Society of Tropical Medicine and Hygiene (1997) 91; 44-49.
Language eng
Format application/pdf
Type Text
Source 45th Annual Frank Walsh Society Meeting
Relation is Part of NANOS Annual Meeting Frank B. Walsh Sessions; 2013
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 2013. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright
ARK ark:/87278/s69c9v0v
Setname ehsl_novel_fbw
ID 179172
Reference URL https://collections.lib.utah.edu/ark:/87278/s69c9v0v
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