Influence of Tumor Characteristics on Visual Field Outcomes After Pituitary Adenoma Surgery.

BACKGROUND: There were few reports about the influence of tumor characteristics on the postoperative visual field outcomes after transsphenoidal surgery for pituitary adenoma. The purpose of this study was to explore the tumor characteristics that influenced perioperative visual field changes. METHODS: Patients who underwent transsphenoidal surgery under a diagnosis of pituitary adenoma at the Kyoto University Hospital between April 2012 and December 2018 were retrospectively enrolled. Correlations among circumpapillary retinal nerve fiber layer thickness, preoperative and postoperative mean deviation (MD) of visual field, MD change after the surgery, and maximum tumor diameter were evaluated by measuring Pearson correlation coefficient. We evaluated the influences on postoperative MD using a generalized estimating equation for univariate and multivariate regression analyses. We also compared the characteristics of cystic and solid tumors. RESULTS: Thirty-two eyes of 18 patients were included in this study (9 male and 9 female patients). Postoperative MD positively correlated with maximum tumor diameter only in multivariate regression {ß = 0.22 (95% confidence interval [CI], 0.004-0.43), P = 0.046}, although maximum tumor diameter negatively correlated with postoperative MD in univariate regression (ß = -0.16 [95% CI, -0.58 to 0.26], P = 0.46). In the investigation of perioperative MD changes, eyes with cystic tumors showed significantly better improvement those with solid tumors (8.93 ± 7.85 vs 0.18 ± 6.56 dB, P = 0.007). CONCLUSIONS: Cystic and solid tumors show different characteristics of visual loss and visual field defects. The MD in eyes with cystic tumors improved significantly more than that in eyes with solid tumors.

Bilateral Orbital Inflammation as a Manifestation of Paraneoplastic Syndrome.

Paraneoplastic neurologic syndromes (PNS) constitute a rare group of disorders whose optimal treatment is yet to be established. We report a patient with bilateral orbital inflammation associated with PNS, who responded well to surgical resection of the primary tumor. An 83-year-old woman was referred to our department for treatment of a progressive reduction in visual acuity and palpebral swelling in both eyes for the past 2 months. She was scheduled to undergo thoracic surgery for lung cancer. The best-corrected visual acuity (BCVA) in the right and left eye had worsened from 0.3 to 0.5 one month before she was referred to our department to 0.03 and 0.07, respectively. A slit-lamp examination revealed edema in both eyelids. Goldmann perimetry revealed several paracentral scotomas with constriction of the peripheral visual fields of both eyes, along with central absolute scotomas in V-4e isopter in the right eye. Magnetic resonance imaging revealed swelling of the bilateral extraocular muscles, which compressed the bilateral optic nerves at the orbital apex. Seven days after the resection of the lung cancer, the BCVA improved to 0.07 and 0.15 in the right and left eyes, respectively, without concomitant immunotherapy. Intravenous methylprednisolone (500 mg/day) was administered for 3 days to treat the residual orbital inflammation. Fourteen days after surgery, the BCVA further improved to 0.4 and 0.5 in the right and left eyes, respectively. Swelling of the bilateral extraocular muscles and the visual field abnormalities improved dramatically. Early diagnosis is crucial for the management of PNS.

Analysis of Retinal Nerve Fibre Thickening in Progressive and Non-progressive Non-arteritic Anterior Ischaemic Optic Neuropathy Using Optical Coherence Tomography.

The study aims to investigate the longitudinal changes in the circumpapillary retinal nerve fibre layer thickness (cpRNFLT) in progressive and non-progressive non-arteritic anterior ischaemic optic neuropathy (NAION). This retrospective observational case series study analysed 17 eyes with NAION. Patients sustaining any additional visual loss (additional decrease in visual acuity (VA) ≥0.2 logMAR) within two months after initial onset of symptoms were classified as having progressive NAION. Of the 17 eyes with NAION, 13 (76.5%) were diagnosed as non-progressive and 4 (23.5%) were diagnosed as progressive. Compared with control eyes, eyes with non-progressive NAION showed greater cpRNFLT in all four optic disc quadrants at the initial visit (temporal and superior: P < .001; nasal and inferior: P = .002). In contrast, compared with control eyes, eyes with progressive NAION showed greater cpRNFLT in the superior and nasal quadrants (P = .004 and 0.028, respectively), but not in the temporal and inferior quadrants. During progression, eyes with progressive NAION showed a significant increase in cpRNFLT in the inferior quadrants; furthermore, there was significant increase in cpRNFLT in the nasal sector before visual loss developed after the initial visit. Progressive NAION showed development of the disc swelling from the superior to inferior portion of optic disc via the nasal swelling, suggesting that swollen axons in one ischaemic part may lead to secondary vascular infarction in another part of the optic disc. This enlargement could constitute the earliest sign of progressive NAION.

IgG4-related disease: a neuro-ophthalmological perspective.

Immunoglobulin G4-related disease (IgG4-RD) is a multifocal inflammatory disorder that causes tumefactive lesions with a dense lymphoplasmacytic infiltrate rich in IgG4 plasma cells and storiform-pattern fibrosis. The clinical symptoms are relatively mild, and the condition is usually recognized by organ swelling and damage. When referring to the ophthalmic manifestations of IgG4-RD, the term IgG4-related ophthalmic disease (IgG4-ROD) is used. IgG4-ROD is characterized by bilateral lacrimal gland enlargement accompanied by 3 distinctive features: infraorbital nerve enlargement, extraocular myositis, and compressive optic neuropathy. IgG4 implies an underlying systemic disease process requiring evaluation to detect other systemic involvement. This includes hypophysitis and hypertrophic pachymeningitis, entities of neuro-ophthalmic interest. IgG4-ROD usually responds favorably to systemic corticosteroids but may be complicated by relapse during steroid taper. Rituximab has been shown to be effective for controlling steroid-refractory IgG4-RD. In contrast to IgG4-RD, an increasing number of cases of extranodal marginal B-cell lymphoma (MALT type) associated with IgG4-ROD have been described. IgG4 may be a risk factor for later emergence of low-grade B-cell lymphoma.

[A case of non-atherothrombotic cerebroretinal small vessel disease].

BACKGROUND: There is an increasing interest in the relation between retinal artery abnormalities and cerebral small-vessel diseases (SVD), because retinal vessels share common properties with cerebral small vessels. We report a case of juvenile cerebrovascular disease presenting retinal vessel abnormalities, which clinically resembled cerebral autosomal dominant arteriopathy with stroke and ischemic leukoencephalopathy (CADASIL) but in which Notch3 gene mutations were not detected. CASE: A 42-year old woman was hospitalized at the department of Neurology in our hospital, complaining of headache and dysarthria. MRI showed bilateral spotted white matter lesions in the paraventricular area and the temporal lobe, and an ovoid lesion in the right corona radiata. Despite steroid pulse therapy, she developed right incomplete hemiparesis and new lesions were detected in the anterior temporal pole and external capsule. Her genetic analysis showed no mutations in the Notch 3 gene. Ophthalmological examination revealed arterial sheathing in the peripapillary region. Fluorescein angiography showed narrowing of the retinal arterioles and distinguished a peripheral vascular network. CONCLUSION: In this case, ophthalmological examination revealed retinal vessel abnormalities in a relatively young woman with no risk factors such as hypertention or artheriosclerosis, presenting recurrent subcortical strokes. This actual case indicates the association between retinal vessel abnormalities and cerebral SVDs.

Cause and prognosis of neurologically isolated third, fourth, or sixth cranial nerve dysfunction in cases of oculomotor palsy.

PURPOSE: To determine the cause and prognosis of neurologically isolated third, fourth, or sixth cranial nerve dysfunction in cases of oculomotor palsy, and to determine the best imaging methods to make a correct diagnosis. METHODS: The medical records of 221 consecutive patients with oculomotor palsy caused by neurologically isolated cranial nerve dysfunction were reviewed. There were 63 cases of third, 41 of fourth, and 117 of sixth cranial nerve dysfunction. The patients were examined at the Neuro-ophthalmology Clinic of Kyoto University Hospital between 1993 and 2001. RESULTS: Vascular disorders accounted for 34.9% of the third nerve dysfunction, and 90% of these recovered completely in 6 months. Ninety percent of the patients with an isolated third nerve dysfunction that was caused by an aneurysm also had anisocoria, and 68% of the patients with a third nerve dysfunction caused by a vascular disorder had anisocoria. In all of the vascular cases with anisocoria, the difference in the pupillary diameter was <1.0 mm. The presence of ptosis did not play an important role in making a diagnosis of third nerve dysfunction. Ninety percent of the patients with fourth nerve dysfunction and 60% of the patients with sixth nerve dysfunction recovered within 9 months. CONCLUSIONS: The age of the patient, signs of an improvement, and associated alterations are important diagnostic markers to determine the best type of imaging methods for evaluating neurologically isolated third, fourth, and sixth cranial nerve dysfunction.

[Clinical neuro-ophthalmology of vision].

To diagnose a patient with a failing visual system, it is necessary to localize the site of the lesion in the system, and identify the etiology that has produced it. Physicians do not see diseases but just their manifestations. Clinical neuro-ophthalmology provides the basic principles on how to progress from manifestations to the diseases they indicate. The Frank B. Walsh Neuro-Ophthalmology Society (the Walsh Society) that originated in 1969 in the United States has been the center of clinical neuro-ophthalmology case studies throughout the world. In Japan, the Ronald M. Burde Clinical Neuro-Ophthalmology Study Group (the RMB Society) was organized in 2001 to establish and promote a clinicopathologic conference in the style of the Walsh Society. On this occasion, Prof. Burde was invited to the Annual Japanese Ophthalmological Society meeting. Based on some illustrative cases presented at the annual meetings of the RMB society, this review was carried out to present the current knowledge of clinical neuro-ophthalmology.

Photopsia as a manifestation of digitalis toxicity.

CASE REPORT: To present a case of photopsia resulting from digoxin intoxication brought about by dehydration in a 72-year-old woman. COMMENTS: Ophthalmologists may be the first clinicians to notice the symptoms of digitalis intoxication, which is potentially a life-threatening condition.

Orbital pseudotumor as an initial manifestation of multicentric Castleman's disease.

BACKGROUND: To report on a patient with multicentric Castleman's disease (MCD) who showed an orbital pseudotumor as an initial manifestation. CASE: A Japanese male patient, 65 years old, initially exhibited swelling of the left eyelid. Computed tomography and magnetic resonance imaging showed an infiltrating orbital mass around the left eye. OBSERVATIONS: The patient was treated several times with short courses of oral corticosteroids without significant response. Eight years after the first examination, he was referred to our neuroophthalmology clinic owing to persistent eyelid swelling. He exhibited an accelerated erythrosedimentation rate, polyclonal hypergammaglobulinemia, and multiple cervical and thoracic mediastinal lymphadenopathies. Histopathological examination revealed mixed-type MCD from a cervical lymph node and chronic inflammatory cell infiltration from eyelid swelling. Four months after his first visit, the patient's condition was complicated by fever, general fatigue, and hypoxemia. A cell marker study of the swollen lymph node showed monoclonality, indicating the development of non-Hodgkin's lymphoma. CONCLUSIONS: The clinical course in this case suggests that an inflammatory reaction in the orbital tissue was initially present, before MCD, and that non-Hodgkin's lymphoma subsequently developed.

Dynamic MR imaging in Tolosa-Hunt syndrome.

OBJECTIVE: To evaluate the cavernous sinuses with dynamic magnetic resonance (MR) imaging in patients with Tolosa-Hunt syndrome (THS). METHODS: The sellar and parasellar regions of five patients with THS and 12 control subjects were examined with dynamic MR (1.5 T) imaging in the coronal plane. Dynamic images were obtained with spin-echo (SE) sequences in three patients, and with fast spin-echo (FSE) sequences in two patients and control subjects. Conventional MR images of the cranium including sellar and parasellar regions were also obtained on T1-weighted pre- and post-contrast SE, and T2-weighted FSE sequences in the coronal plane. RESULTS: MR images revealed affected cavernous sinus with bulged convex lateral wall in three patients and concave lateral wall in two patients. In all control subjects, cavernous sinuses were observed with concave lateral wall. The signal intensity on T1- and T2-weighted images and contrast enhancement on post-contrast images of the affected cavernous sinuses in patients were similar to those of the unaffected cavernous sinuses in patients and control subjects. The dynamic images in all patients disclosed small areas adjacent to the cranial nerve filling-defects within the enhanced venous spaces of the affected cavernous sinus, which showed slow and gradual enhancement from the early to the late dynamic images. No such gradually enhancing area was observed in control subjects except one. The follow-up dynamic MR images after corticosteroid therapy revealed complete resolution of the gradually enhancing areas in the previously affected cavernous sinus. CONCLUSION: Dynamic MR imaging may facilitate the diagnosis of THS.