Angelina Espino Barros Palau

Neuro-ophthalmic presentations and treatment of Cryptococcal meningitis-related increased intracranial pressure

OBJECTIVE To illustrate three different ophthalmic presentations of cryptococcal meningitis (CM). INTRODUCTION CM is the most common manifestation of extra-pulmonary cryptococcosis. Intracranial hypertension occurs in up to 75% of patients with CM and is associated with increased mortality. CM can present to the ophthalmologist as vision loss, papilledema, abducens palsy, and/or other cranial neuropathies. PARTICIPANTS AND METHODS We report three cases, two C. neoformans and one C. gattii, highlighting the various CM presentations. The first was a woman immunosuppressed following kidney transplantation in whom idiopathic intracranial hypertension (IIH) was initially suspected. The second was a man immunocompromised by previously undiagnosed HIV/AIDS who presented with signs and symptoms of increased intracranial pressure. The third case is an immunocompetent man with bilateral disc edema and an incomplete macular star diagnosed with presumed neuroretinitis. Further evaluation revealed positive CSF cryptococcal antigen with culture positive for C. gattii. CONCLUSIONS Ophthalmologists should be aware that cryptococcosis can mimic more benign etiologies including IIH and neuroretinitis. Additionally, C. gattii, an emerging organism, can infect immunocompetent patients. In contrast to the typical treatment of increased ICP, serial lumbar punctures are recommended while acetazolamide and surgical CSF shunting may be harmful.

Suprasellar Primitive Neuroectodermal Tumor in an Adult

Primitive neuroectodermal tumors (PNET) of the central nervous system (CNS) are a heterogeneous group of embryonal malignancies that are composed of undifferentiated or poorly differentiated neuroepithelial cells. Supratentorial PNET is the second most common CNS embryonal malignancy in children, but it is rare in adults. We report the case of a 31-year-old woman with bilateral vision loss and a bitemporal hemianopia. Neuroimaging revealed a suprasellar mass, and pathology was consistent with PNET. After surgical debulking of the tumor followed by radiation therapy and chemotherapy, the patient had significant visual recovery and remained stable over 14 months of follow-up.

Neuro-behćet disease presenting with oculopalatal tremor

A 39-year-old woman with a history of Behçet disease presented for evaluation of oscillopsia that began postpartum. Examination showed oculopalatal tremor (OPT), documented videographically. Brain magnetic resonance imaging revealed bilateral pseudohypertrophy of the inferior olivary nuclei. Treatment with gabapentin was initiated for OPT presumed secondary to neuro-Behçet disease.

Neuro-Ophthalmology Annual Review.

AbstractThe aim of this study was to update the practicing ophthalmologist on the English-language neuro-ophthalmology literature from the prior year. This study is a review of English-language literature from August 1, 2012, to August 1, 2013. The authors searched PubMed articles published from August 1, 2012, to August 1, 2013, limited to English-language publications including original articles, review articles, and case reports and excluding letters to the editor, unpublished work, and abstracts. We researched the following topics: pupillary abnormalities, eye movement dysfunction, neuromuscular diseases, optic neuropathies, optic neuritis and demyelinating disease including multiple sclerosis, lesions of the chiasm and posterior primary visual pathways, elevated intracranial pressure, tumors and aneurysms affecting the visual pathways, vascular diseases, higher visual functions, and neuroimaging advances. We intend to share clinically relevant literature of the past year with the practicing ophthalmologist. We aimed to highlight remarkable and interesting literature rather than exhaustively including all new neuro-ophthalmological publications of the year. We reviewed literature in the past year with a focus on relevance and novelty. This review updates the comprehensive ophthalmologist on neuro-ophthalmic topics.

Metastatic Papillary Thyroid Carcinoma Presenting as Abducens Palsy Complicated by Ocular Neuromyotonia

ABSTRACT Papillary thyroid carcinoma (PTC) is a type of well-differentiated thyroid cancer that accounts for the majority of thyroid malignancies. The prognosis of PTC is very good and distant metastases are rare, especially to the skull base. The authors report the case of a 47-year-old woman with biopsy-proven PTC treated with surgery and radiation therapy who presented with headache and diplopia after 5 years and was found to have clivus and cavernous sinus metastasis. Following radiation therapy for her skull base and cavernous sinus lesion, she subsequently developed sixth nerve ocular neuromyotonia. Possible causes and treatments are reviewed.

Anti-asialo-GM1 and GD1a variant of miller fisher variant of Guillain-Barré syndrome

We present a woman with acute onset of bilateral ophthalmoparesis and ataxia occurring 4 weeks after gastroenteritis. Serum antibody titers against asialo-GM1 and GD1a, typically associated with inflammatory axonal neuropathies, were elevated but titers against anti-GQ1b, the most commonly found antibody found in the Miller Fisher variant of Guillain-Barre syndrome were not. No other etiology for ophthalmoparesis was found despite extensive patient evaluation. Intravenous immunoglobulin was administered, and the patient gradually improved over subsequent months. This case is unique for its antiganglioside antibody profile associated with Miller Fisher syndrome.

Bilateral optic atrophy in endemic typhus

Murine typhus is an infectious disease caused by Rickettsia typhus. The disease is transmitted to humans by rat fleas through direct inoculation. Southeast Texas has one of the highest incidence rates of endemic typhus in the United States. Ophthalmic manifestations of murine typhus include vitritis, retinal lesions, and retinal vascular leakage. Optic neuropathy in the setting of murine typhus has been rarely described and only in the setting of disc edema or optic neuritis. We present a rare case of bilateral optic atrophy as the presenting ophthalmic sign of R. typhus infection. A 55-year-old white male presented with a 1-month history of painless, bilateral progressive vision loss and floaters. Medical history was significant for well-controlled diabetes mellitus type 2 and systemic hypertension. Ocular history was significant for remote right-sided ocular trauma from injury with an arrow. The patient smoked cigarettes, 1 pack per day. He denied any recent travel or sick exposures. He denied exposure to chemicals, toxins, or poisons. He had a dog and a cat. He denied any recent tick bite. His symptoms started 1 month before presentation with fever and malaise. The constitutional symptoms were followed by blurred vision, floaters, and dyschromatopsia. His medical doctor diagnosed infectious mononucleosis. However, he subsequently experienced development of a cutaneous vesicular eruption on his right torso and was diagnosed with herpes zoster. He was treated with oral famciclovir. Around the same time, he was evaluated by an outside ophthalmologist. His acuity was 20/20 OU, but he had rare cells and vitreous haze OU. He was prescribed topical steroid drops in addition to the oral antiviral treatment. His vision worsened, and he was administered an intravitreal steroid injection in the right eye. His vision, however, further deteriorated. A fluorescein angiogram (FA) showed no optic disc leakage or vasculitis. Magnetic resonance imaging of the brain and orbits with and without contrast and fat suppression was unremarkable. Testing for HIV, syphilis, sarcoidosis including serum angiotensin-converting enzyme and lysozyme, antinuclear antibody, tuberculosis QuantiFERON-TB testing, and Lyme titer were all negative. Toxoplasma and Bartonella antibodies were also negative. A vitreous tap revealed only a few scattered lymphocytes and a few mononuclear cells. Special stains for fungus, acid-fast bacilli, and molecular testing were all negative. He was then referred to our neuro-ophthalmology clinic. On examination, his vision was 20/100 OD and 20/50 OS. He had no rash or history of insect/animal bite. Both pupils were poorly reactive, but he had no relative afferent pupillary defect. Slit-lamp examination showed iris atrophy in the inferior segment of his right eye, presumed secondary to the old foreign body (arrow) injury. However, there were also posterior synechiae at anterior lens capsule. There was no active anterior uveitis. Humphrey 24-4 visual field testing of the right eye showed a dense inferior altitudinal and superior arcuate defects with a mean deviation (MD) of –19.19. The left eye visual field had an inferior altitudinal and superior arcuate defect with a MD of –20.56. Ophthalmoscopy showed normal macula, vessels, and periphery in each eye. Both optic nerves, however, were pale with a cup-to-disc ratio of 0.3. OCT of the optic nerves confirmed the bilateral optic atrophy in the papillomacular bundle (Fig. 1). Lumbar puncture showed a normal opening pressure of 15 cm H2O. Cerebrospinal (CSF) protein was elevated at 105 mg/dL, but CSF cell count and glucose were within normal limits. CSF herpes simplex virus, varicella zoster virus, and Epstein–Barr virus (EBV) polymerase chain reaction were negative. Gram stain showed no infectious organism, and cultures were negative. Additional laboratory tests were sent including a complete blood cell count, EBV viral capsid antigen antibody (VCA IgG), EBV nuclear antigen antibody, rapid plasma reagin, West Nile virus antibodies, Histoplasma urine antigen, neuromyelitis optica antibody, and R. typhi antibodies. R. typhi antibodies came back positive with both IgM and IgG titers positive at greater than 1:256. In consultation with an infectious disease specialist, the patient received a 14-day course of doxycycline. His vision, unfortunately, did not improve, and he experienced development of optic atrophy. Murine typhus is a rickettsial disease characterized by high fever, constitutional symptoms, and typically a maculopapular rash. The rash, however, can be vesicular and may mimic herpes zoster. R. typhi is usually a mild and self-limited disease, although 10% of hospitalized patients require intensive care because of respiratory distress, renal failure, or severe neurologic involvement. Ophthalmic involvement is rare and only a few reports on ophthalmic murine typhus can be found in the literature. The largest cases series of murine typhus–associated ophthalmic findings was reported by Khairallah et al. The most common ophthalmic findings, present in 50% of their patients, were white retinal lesions located in the inner retina associated with mild vitritis. These lesions were thought to represent areas of retinitis from either rickettsial multiplication or inflammatory cell accumulation. Similar retinal lesions have also been described by Hudson et al. and by Lu et al. Angiographically confirmed retinal vasculitis (38.9%) and retinal hemorrhages (22.2%) were also common in Khairallah et al.’s series. Optic neuropathy has also been described in murine typhus. The exact mechanism of this typhus-associated optic neuropathy is unknown, but it probably represents an inflammatory reaction. In Khairallah et al.’s series, 66.6% of eyes had staining on FA, 11.1% had disc edema, and

Downbeat down south

A 59-year-old woman with late-onset diabetes mellitus presented with a 4-week history of oscillopsia and vertigo. Physical examination revealed downbeating nystagmus in the primary position that worsened on right gaze and left gaze. Magnetic resonance imaging of the brain and orbits showed enhancement and signal abnormality in the right temporal lobe without evidence of a cervicomedullary junction lesion. Serum anti-glutamic acid decarboxylase 65 (GAD65) antibody titer was markedly elevated. Given these findings, her vertigo and downbeat nystagmus were likely secondary to elevated anti-GAD antibodies.

Anatomy of the Optic Nerve and Visual Pathway

Visual information is transduced in the retinas and transmitted via the optic nerves. At the optic chiasm some fibers decussate while others remain ipsilateral to facilitate contralateral visual field representation in post-chiasmatic structures. The anatomy and physiology facilitate localization of lesions. In turn, anatomic localization narrows the differential diagnosis of disease facilitating focused laboratory and radiographic investigation. In turn, treatments are directed based on localization and diagnosis.

Dermatomyositis-Related Nonischemic Central Retinal Vein Occlusion.

A 25-year-old woman with dermatomyositis suffered a right central retinal vein occlusion (CRVO) with visual acuity of 20/40. Examination of the right eye showed vitreous cells, suggesting inflammation of the central retinal vein leading to a CRVO as the presumed mechanism. She was admitted to hospital, and extensive evaluation was negative. She was maintained on corticosteroids to manage her dermatomyositis. One month later, she had macular edema and elevated intraocular pressure. Both resolved with dorzolamide, timolol, and intravitreal bevacizumab, and vision returned to 20/20 in the right eye.