Growth hormone secreting pituitary carcinomas: Case report and review of literature.

OBJECTIVE: Pituitary carcinoma is a rare tumor, defined as a tumor of adenohypophyseal cells with systemic or craniospinal metastasis. We present a case of a growth hormone (GH)-secreting pituitary carcinoma with a review of literature to better characterize this disease. DESIGN: Case report and literature review of 25 cases of GH-secreting pituitary carcinomas RESULTS: The age of diagnosis of GH-secreting carcinomas ranged 24-69 years old with a mean age of 44.4 with 52% of cases present in females. Mean latency period between diagnosis of acromegaly and transition to pituitary carcinoma was 11.4 years with mean survival being 3.4 years. CONCLUSION: Growth hormone (GH)-secreting pituitary carcinomas are rare and hard to distinguish from aggressive pituitary adenomas. From review of literature, treatment options include debulking surgery, radiotherapy, or chemotherapy with dismal outcomes. There are no diagnostic markers or features which can predict metastatic progression of these tumors. Future studies with genomic landscapes and relevant tumor markers are needed to identify pituitary tumors most likely to metastasize.

Binocular Diplopia Caused by an Epiretinal Membrane With Foveal Displacement.

A 73-year-old woman presented with 3 years of monocular visual distortion and progressive binocular diplopia. She was found to have a comitant left hypertropia due to an epiretinal membrane causing inferior foveal drag. Displacement of the fovea from an epiretinal membrane is a likely under-recognized cause ocular cause of a comitant binocular diplopia.

Posttraumatic Emphysema of the Optic Nerve Sheath.

The authors describe the case of a 19-year-old female who suffered posttraumatic emphysema of the optic nerve sheath. She suffered massive head trauma requiring emergent neurosurgery and was incidentally found to have air in her optic nerve sheath on CT scan. At 6 weeks follow up, her visual acuity (20/25 uncorrected) and color perception in the affected eye were excellent. Her examination was notable for an afferent pupillary defect, mild disc pallor, and optic nerve atrophy on optical coherence tomography. This is a case of a patient with posttraumatic optic nerve sheath emphysema who recovered excellent visual function and received follow-up ophthalmic imaging.

Tumoral Presentation of Homonymous Hemianopia and Prosopagnosia in Cerebral Amyloid Angiopathy-Related Inflammation.

While cerebral amyloid angiopathy is a common cause of lobar hemorrhage, rarely it may be associated with an inflammatory response, thought to be incited by amyloid deposits. We report a 73-year-old woman with an extensive cancer history who presented with tumor-like lesions and symptoms of homonymous hemianopia and prosopagnosia. Found to have cerebral amyloid angiopathy-related inflammation proven by brain biopsy, she was treated successfully with immunosuppression.

Detection of third and sixth cranial nerve palsies with a novel method for eye tracking while watching a short film clip.

OBJECT: Automated eye movement tracking may provide clues to nervous system function at many levels. Spatial calibration of the eye tracking device requires the subject to have relatively intact ocular motility that implies function of cranial nerves (CNs) III (oculomotor), IV (trochlear), and VI (abducent) and their associated nuclei, along with the multiple regions of the brain imparting cognition and volition. The authors have developed a technique for eye tracking that uses temporal rather than spatial calibration, enabling detection of impaired ability to move the pupil relative to normal (neurologically healthy) control volunteers. This work was performed to demonstrate that this technique may detect CN palsies related to brain compression and to provide insight into how the technique may be of value for evaluating neuropathological conditions associated with CN palsy, such as hydrocephalus or acute mass effect. METHODS: The authors recorded subjects' eye movements by using an Eyelink 1000 eye tracker sampling at 500 Hz over 200 seconds while the subject viewed a music video playing inside an aperture on a computer monitor. The aperture moved in a rectangular pattern over a fixed time period. This technique was used to assess ocular motility in 157 neurologically healthy control subjects and 12 patients with either clinical CN III or VI palsy confirmed by neuro-ophthalmological examination, or surgically treatable pathological conditions potentially impacting these nerves. The authors compared the ratio of vertical to horizontal eye movement (height/width defined as aspect ratio) in normal and test subjects. RESULTS: In 157 normal controls, the aspect ratio (height/width) for the left eye had a mean value ± SD of 1.0117 ± 0.0706. For the right eye, the aspect ratio had a mean of 1.0077 ± 0.0679 in these 157 subjects. There was no difference between sexes or ages. A patient with known CN VI palsy had a significantly increased aspect ratio (1.39), whereas 2 patients with known CN III palsy had significantly decreased ratios of 0.19 and 0.06, respectively. Three patients with surgically treatable pathological conditions impacting CN VI, such as infratentorial mass effect or hydrocephalus, had significantly increased ratios (1.84, 1.44, and 1.34, respectively) relative to normal controls, and 6 patients with supratentorial mass effect had significantly decreased ratios (0.27, 0.53, 0.62, 0.45, 0.49, and 0.41, respectively). These alterations in eye tracking all reverted to normal ranges after surgical treatment of underlying pathological conditions in these 9 neurosurgical cases. CONCLUSIONS: This proof of concept series of cases suggests that the use of eye tracking to detect CN palsy while the patient watches television or its equivalent represents a new capacity for this technology. It may provide a new tool for the assessment of multiple CNS functions that can potentially be useful in the assessment of awake patients with elevated intracranial pressure from hydrocephalus or trauma.

Bilateral visual loss due to a giant olfactory meningioma.

Olfactory groove meningiomas can present as large and insidious masses in the anterior cranial base. Due to their location and minimal clinical symptoms, these tumors can go undetected until they have grown extremely large. We present a clinical case and discuss the surgical management of a 63-year-old man who presented for an initial eye examination with bilateral visual loss for over 2 years due to a giant olfactory meningioma encompassing his entire frontal lobe and compressing on his optic nerves.

Clinical neuro-ophthalmic findings in familial dysautonomia.

BACKGROUND: To define the clinical neuro-ophthalmic abnormalities of patients with familial dysautonomia (FD). METHODS: Sixteen patients (32 eyes) with the clinical and molecular diagnoses of FD underwent thorough neuro-ophthalmic clinical evaluation. RESULTS: Visual acuity ranged from 0.05 to 1.0 decimal units and was reduced in 15 of 16 patients. Mild to moderate corneal opacities were found in most patients but were visually significant in only 2 eyes. Red-green color vision was impaired in almost all cases. Depression of the central visual fields was present on automated visual fields in all patients, even in those with normal visual acuity. Temporal optic nerve pallor was present in all cases and was associated with retinal nerve fiber layer loss in the papillomacular region. Various ocular motility abnormalities also were observed. CONCLUSION: Patients with FD have a specific type of optic neuropathy with predominant loss of papillomacular nerve fibers, a pattern similar to other hereditary optic neuropathies caused by mutations either in nuclear or in mitochondrial DNA, affecting mitochondrial protein function. Defects of eye movements, particularly saccades, also appear to be a feature of patients with FD.

Orbital dissemination of Lemierre syndrome from gram-positive septic emboli.

A 45-year-old patient presented with bilateral orbital abscesses. He was found to have Lemierre syndrome, a condition involving septic thrombophlebitis of the internal jugular vein. The patient developed severe proptosis, sepsis, and cavernous sinus thrombosis. Despite aggressive antibiotic and anticoagulation therapy, visual loss was rapid, and the patient ultimately died. Lemierre syndrome, previously thought to be rare, is now becoming more commonly reported. Its prompt diagnosis and treatment are essential for patient survival.

Isolated lymphoma of the anterior visual pathway diagnosed by optic nerve biopsy.

A 72-year-old previously healthy man developed rapidly progressive visual loss, and brain imaging showed features suggestive of a malignant glioma of the anterior visual pathway. Biopsy of one optic nerve yielded a diagnosis of lymphoma. There was no evidence of an extracranial non-Hodgkin lymphoma, so the conclusion was that this represented a primary central nervous system lymphoma (PCNSL). PCNSL isolated to the optic chiasm has been described only once in an immunocompetent patient. Our patient is unusual in that the lymphoma involved the optic nerve, chiasm, and tract in an immunocompetent patient.

Linezolid-associated toxic optic neuropathy: a report of 2 cases.

We describe 2 cases in which the prolonged use of linezolid to treat complicated methicillin-resistant Staphylococcus aureus infections was followed by acutely developed blurred vision and progressive loss of vision and color perception during the ensuing few weeks. Both patients received a diagnosis of toxic optic neuropathy, and linezolid therapy was stopped. The patients experienced an initial rapid partial improvement and a subsequent gradual, almost complete, recovery over many months.

Optical coherence tomography findings in nicotinic acid maculopathy.

PURPOSE: To report optical coherence tomography findings in nicotinic acid maculopathy. DESIGN: Observational case report. METHOD: The patient was examined with ophthalmoscopy, fluorescein angiography, and optical coherence tomography. RESULTS: A 71-year-old man presented with bilateral decreased visual acuity with metamorphopsia. Optical coherence tomography demonstrated cystoid spaces in the outer plexiform layer and inner nuclear layer. Fluorescein angiography did not show leakage. Two weeks after stopping the nicotinic acid, the cystoid spaces resolved. CONCLUSIONS: Niacin maculopathy causes cystoid spaces in the inner nuclear and outer plexiform layers that resolve with discontinuation of the drug.

Adult wilms' tumor metastatic to the choroid of the eye.

BACKGROUND: Ocular manifestations of Wilms' tumor are rare, particularly in adults. We present the first reported case of a choroidal metastasis resulting from Wilms' tumor. DESIGN: Case report. PARTICIPANT: A 37-year-old white male with an adult-onset biopsy-proven Wilms' tumor with multiorgan metastatic disease. RESULTS: We report a unique case of uveal metastasis presumed from a Wilms' tumor. The patient's history included a primary Wilms' tumor with known disseminated metastasis, orbital pain, metamorphopsia, and decreased vision. Ophthalmoscopy revealed a unifocal choroidal tumor in the inferotemporal quadrant of the right eye. Ultrasonographic measurements of the tumor were 4.9 mm in apical height and 15 x 13 mm in basal diameter. Unusual ultrasound findings included echodense stromal opacities and retrobulbar edema. Fluorescein angiography revealed double circulation and late intratumoral fluorescence. CONCLUSIONS: This is the first reported case of a choroidal metastasis by a Wilms' tumor.