Matthew R. Gifford, OD Primary Care Resident Indiana University School of

Pseudo-Foster Kennedy Syndrome: A Manifestation of Cryptococcal Meningitis

Abstract​: A 29 year-old male with recent diagnoses of cryptococcal meningitis and AIDS exhibits OD and progressive optic atrophy OS over a four-month course in the absence of compressive lesion confirmed via neuroimaging studies.

● Optometric Case History A 29 year-old male presented with complaint of distance and near blur with left since suffering fungal meningitis one month prior. The patient was hospitalized for two weeks and concurrently diagnosed with AIDS. His blurred vision had improved but not returned to previous level of clarity. No ocular or visual complaints relating to the right eye. The patient had never worn refractive correction. Visit 1: At his initial optometric appointment, uncorrected DVA was 20/20 OD, 20/25 OS (with no improvement following refraction). The patient reported diplopia with left gaze upon EOM testing. FDT exhibited an inferotemporal defect OD and diffuse generalized depression OS. Anterior segment examination was unremarkable. Posterior segment examination revealed swelling of the with mild peripapillary hemorrhages, tortuous vessels and C/D ratio of 0.20 round OD, possible mild temporal pallor without edema and C/D ratio of 0.25 round OS. There was a single blot hemorrhage at 10:00 near the ora serrata OD. photography was performed OU. ONH OCT showed RNFL thickening (OD>OS) but within normal limits OU. Visit 2: The patient returned to the clinic two days later for baseline HVF 30-2 testing, which illustrated a mild arcuate defect extending from the blind spot (superior>inferior) OD, generalized depression with an arcuate defect extending from the blindspot (inferior>superior) OS; testing was reliable. The patient also exhibited a 2+ left APD; patient subjectively reported that OS perceived 70% brightness compared to OD with red cap test. Visit 3: The patient presented for follow-up testing one month later and reported improvement of vision OS but not complete resolution. Diplopia had resolved upon EOM testing. Uncorrected DVA remained at 20/20 OD, 20/25 OS. The patient continued to exhibit a left APD and subjectively reported OS perceived 80% brightness compared to OD with red cap test. Posterior segment examination revealed papilledema with superior and inferior splinter hemorrhages OD, temporal atrophy OS. Fundus photography was performed OU. ONH OCT showed increased RNFL thickness OD, new temporal atrophy OS. HVF 30-2 testing illustrated improvement without enlarged blindspot OD, improvement of density of defects OS. Visit 4: The patient presented for follow-up testing one month later and reported stability of vision with persistent mild blur OS. No significant changes to preliminary testing were noted. Posterior segment examination revealed papilledema with 1+ trace peripapillary hemorrhages OD, 1+ temporal pallor OS. Fundus photography was performed OU. ONH OCT showed decreased RNFL thickness OD, progressive loss of RNFL 360 degrees with retinal ganglion cell loss in posterior pole OS. HVF 30-2 testing illustrated an essentially full field OD, generalized reduction in sensitivity with enlarged blindspot extending into arcuate defect but with mildly improved sensitivity OS Visit 5: The patient presented for follow-up testing two months later and reported stability of vision with persistent mild blur OS. HVF 30-2 testing illustrated stability OU. Visit 6: The patient presented for follow-up testing one week later and reported stability of vision. No significant changes to preliminary testing were noted. Posterior segment examination revealed mild nasal papilledema OD, temporal pallor with increased C/D ratio of 0.25H/0.30V OS. Fundus photography was performed OU. ONH OCT showed RNFL thickness within normal limits OD, significant RNFL thinning 360 degrees (greatest superotemporal to inferonasal) due to retrograde axonal loss OS. The patient was advised to return to clinic within four months for follow-up evaluation or sooner if any ocular or visual complaints arise in the interim, but the patient was lost to follow-up.

Gifford

● Pertinent Medical Findings At the time of AIDS diagnosis, CD4 count was 41 cells/mm​3;​ viral load was 216,000 copies/mL. Upon admission to the hospital, the patient exhibited left lateral gaze diplopia. His medical records from hospitalization include MR venogram with diffuse leptomeningeal enhancement indicative of meningitis; MRI of brain exhibited left posterior ethmoid sinusitis but no other pathology. Two lumbar punctures were performed (before and after initiation of treatment). Opening pressure decreased from 270 to 230 over the course of eleven days of treatment, which included low dose Diamox. The patient had previously presented as an outpatient for sinusitis, flu-like illness, dehydration, hyponatremia and severe, debilitating headaches. The patient clinically improved throughout hospitalization; he was prescribed a two-week course of amphotericin B and oral flucytosine and began Atripla treatment. Septra DS and Zithromax were prescribed as prophylactic treatment for pneumocystis pneumonia and mycobacterium avium-intracellulare, respectively, in addition to fluconazole. At the time of discharge, the left lateral gaze diplopia had improved, but a left lateral gaze nystagmus was noted. Approximately 1.5 months following discharge, CD4 count was 77 cells/mm​3;​ viral load was 20 copies/mL. A spinal tap was performed and showed no fungus within the culture.

● Differential Diagnoses Differential diagnoses consist of true Foster Kennedy Syndrome and other causes of Pseudo-Foster Kennedy Syndrome, which include anterior ischemic optic neuropathy (arteritic and non-arteritic), sequential optic neuritis, chronic unilateral optic neuropathy and pachymeningitis.

● Discussion Pseudo-Foster Kennedy is unique in that it exhibits one-sided optic atrophy with contralateral papilledema in the absence of a compressive lesion. It is important to closely monitor a patient long-term in the event of a sequential optic neuritis. A supposedly resolving papilledema (as monitored via ONH OCT) may, in fact, be the progression of papillitis to optic atrophy.

● Treatment & Management Optometric treatment in this case consisted of close and careful observation for progression of signs and symptoms. Regular ONH OCT and HVF 30-2 are vital to assess both structure and function. Co-management with the medical providers treating the cryptococcal meningitis is of the utmost importance.

● Conclusion Cryptococcal meningitis (CM) is one of the most common life-threatening, opportunistic infections to be associated with AIDS; approximately 6-10% develop cryptococcal meningitis, of which 40% are the AIDS-defining event that prompts testing and subsequent diagnosis. Patients are generally symptomatic with fever, headache and malaise (as was the case with this patient) for several weeks prior to presentation. Prompt referral for new, unusual persistent headaches is essential. Additionally, initiation of antiretroviral therapy may be delayed if CM is present at the time of HIV/AIDS diagnosis to avoid immune reconstitution inflammatory response, which is a paradoxical inflammatory response once an immunosuppressed patient regains the ability to mount an immune response; however, this would be at the discretion of the medical doctor.

[All clinical imaging, photography and visual fields available for poster. Bibliography with expanded literature review to follow if selected.]