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 |
Description |
Examination of the CSF on the final LP revealed yeast forms on the gram stain consistent with Cryptococcal neoformans. Cryptococcal antigen titre was 1: 512. Amphotericin B and Flucytosine were started IV with high dose IV steroids. With treatment, acuity, color vision and fields all returned to normal. Cryptococcal antigen of the CSF from the initial LP was negative. However, 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. It remained unclear as to whether the primary diagnosis was cryptococcal meningitis in an immunocompetent host, or if instead, a fungal meningitis complicated a case of neurosarcoidosis in the context of immunosuppression secondary to IV steroids. The speciation of the yeast was Cryptococcus neoformans var grubii, commonly found in eucalyptus plant, wood and pigeon feces and found around the world. Inoculation may have occurred via trans-sphenoidal biopsy, as Cryptococcus is usually acquired via inhalation of spores. Biopsy could have introduced fungal elements into the CNS. HIV was negative (tested twice), but CD4 counts were decreased. In culture growth occurs in 3-7 days with identification in 3-4 days; however occasionally growth is slower and incubation should be continued for 4-6 weeks. Cryptoccocus neoformans is a saprophytic fungus that may cause disease in normal hosts as well as the immunocompromised. In the immunocompetent individuals Cryptococcus neoformans var. gattii is implicated more often compared to the immunocompromised with Cryptococcus neoformans var neoformans. In those who are immunocompromised with defective cell mediated immunity, such as those being treated with corticosteroids, HIV, lymphoma and leukemic patients, infections can be more serious with rapid progression and dissemination. Infections are more often pulmonary than meningoencephalitis. In the immunocompetent infections are usually of the basal leptomeninges. |
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 |
Format Creation |
Microsoft PowerPoint |
Type |
Text |
Source |
45th Annual Frank Walsh Society Meeting |
Relation is Part of |
NANOS Annual Meeting 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/s6sb73bg |
Setname |
ehsl_novel_fbw |
ID |
179176 |
Reference URL |
https://collections.lib.utah.edu/ark:/87278/s6sb73bg |