The Role of Optic Nerve Diffusion Restriction on Visual Prognosis in Methanol Poisoning.

The aim of this study was to investigate the role of optic nerve diffusion status on cranio-orbital magnetic resonance imaging (MRI) in predicting visual prognosis in cases of methanol intoxication. Diffusion-weighted imaging (DWI) from 16 eyes of eight patients who were admitted to our clinic due to methanol intoxication was analysed retrospectively. The relationship between clinical and laboratory findings, treatment regimen, visual prognosis, and imaging findings was investigated. Diffusion restriction (DR) of the optic nerve on DWI was observed in seven (43%) eyes. Regardless of the clinical and laboratory characteristics and treatment regimen, visual acuity (VA) improved in eyes in which restricted diffusion regressed over the follow-up period. DWI of the optic nerve during the acute phase of methanol poisoning may provide prognostically important data. Improvement of DR during follow-up may be an indicator of an increase in VA.

Adult Spinal Primary Leptomeningeal Medulloblastoma Presenting as Pseudotumour Cerebri Syndrome.

A previously well 34-year-old man presented with severe pseudotumour cerebri. Imaging showed that he had a cauda equina tumour which proved to be a medulloblastoma. There was no tumour mass in the posterior fossa so we assume that this was a primary leptomeningeal medulloblastoma. In patients with somewhat atypical pseudotumour, spinal imaging should always be considered.

Capillary malformation-arteriovenous malformation syndrome with spinal involvement.

Capillary malformation-arteriovenous malformation (CM-AVM) is a recently identified autosomal dominant disorder. Arteriovenous lesions have been reported in the brain, limbs, and face. We report a 7-year-old patient with CM-AVM with spinal AVM, which is a rarely reported association.

Factors associated with early improvement after intravenous thrombolytic treatment in acute ischemic stroke.

OBJECTIVE: : The aim of this study was to determine the factors associated with early neurological improvement (ENI) in patients who experienced acute ischemic stroke and were treated with intravenous recombinant tissue plasminogen activator (IV rt-PA), and determine the relationship with the outcome at the first control. METHOD: : This study included 377 patients who were treated with IV rt-PA in Izmir Dokuz Eylül University Hospital between January 2010 and October 2018. ENI was defined as a 4 or more improvement in the National Institutes of Health Stroke Scale (NIHSS) score in the first hour, the twenty-fourth hour and the seventh day when compared to the pretreatment phase. The modified Rankin Scale (mRS) 0-1 score was defined as 'very good outcome'. RESULTS: : The basal NIHSS (p=0.003, p=0.003, p=0.022) was high in the first hour, twenty-fourth hour, and seventh day ENI groups. Blood urea nitrogen (BUN) level was low in the first- and twenty-fourth-hour ENI groups (p=0.007, p=0.020). Furthermore, admission glucose was low at the twenty-fourth hour and on the seventh day ENI groups (p=0.005, p=0.048). A high infarct volume was observed on magnetic resonance imaging (MRI) at the twenty-fourth hour and on the seventh day non-ENI groups (p= <0.001, p= <0.001). CONCLUSION: : Management of factors associated with ENI and determination of treatment strategies accordingly are important for obtaining a better clinical outcome. It can help quickly select patients, who, even though they will not respond to rt-PA, may be appropriate candidates for bridging therapy.

Fibromuscular dysplasia as a cause of stroke in a 9-year-old girl.

Fibromuscular dysplasia is a rare, idiopathic and nonatheromatous disease. It is rarely encountered as a cause of stroke in children. We report a nine-year-old girl with stroke in whom extensive fibromuscular dysplasia of intracranial vessels was established. She also had familial combined hyperlipidemia as an additional risk factor. This case suggests that additional risk factors like hyperlipidemia in cases with fibromuscular dystrophy may facilitate the occurrence of stroke at early ages.

Unilateral Purtscher-like retinopathy as the presenting feature of a case with spontaneous carotid artery dissection.

Carotid artery dissection should be considered in the differential diagnosis when a clinician faces with a case presenting with a unilateral Purtscher-like retinopathy.

Challenges in the Diagnosis of Intraocular Lymphoma

Intraocular lymphomas are among the rare malignancies that present with a wide variety of clinical manifestations. Differential diagnosis can be very troublesome due to its mimicking nature, insidious disease onset, and partial treatment response to steroids. The most important step in diagnosis is a high index of suspicion. Signs of the disease are now easier to detect using multimodal imaging techniques. In this case series, we reviewed the clinical characteristics of two women aged 70 and 71 years and a 72-year-old man with intraocular lymphoma and described their multimodal imaging findings in detail. Bilateral eye involvement was present in all three cases at our first ophthalmological examination. While the disease first presented with ocular involvement in two of the three cases, ocular involvement was detected seven years after initial heart involvement in one patient. All three patients had diffuse large B-cell lymphomas (one diagnosed with retinal biopsy, one with conjunctival biopsy, and the remaining with stereotactic brain biopsy). Intraocular lymphoma should be diagnosed and treated using a multidisciplinary approach, and we share our experience in this case series.

Optic nerve sheath decompression saves sight in severe papilloedema: results from 81 eyes in 56 patients with pseudotumor cerebri.

PURPOSE: To report the outcome of optic nerve sheath decompression (ONSD) for papilloedema in a teaching hospital in western Turkey. METHODS: The charts of 56 patients who had ONSD surgery between April 2007 and September 2019 were collated; and a total of 81 operated and 31 fellow eyes were included. Pre- and postoperative ophthalmologic examination including best-corrected visual acuity (BCVA), colour vision (CV), visual field (VF) analysis, fundoscopic examination and demographic and medical characteristics of the patients were noted and outcomes after surgery were investigated. RESULTS: Of all study eyes, 49 (43.7%) eyes had BCVA 0.2 or less and 62 (55.3%) eyes had mean deviation (MD) below - 20.0 dB. 62 (55.3%) eyes had Frisen grade 4 or 5 papilloedema. Almost half of the eyes had severe vision loss. After ONSD, BCVA, CV and MD in both operated and fellow non-operated eyes improved significantly (p < 0.001, p = 0.009 and p < 0.001 for operated, p < 0.001, p = 0.007 and p < 0.001 for fellow eyes, respectively). Earlier surgery and higher cerebrospinal fluid opening pressure were related to better outcomes. None of the patients had major operative complications. CONCLUSION: Optic nerve sheath decompression can safely improve vision not only of the operated but also of the non-operated eye, even in cases with severe vision loss from severe bilateral papilloedema. Regardless of initial VA and VF, patients may benefit from ONSD; the earlier it is done the more likely the better outcome.

Multimodal imaging features of a spontaneously resolved unilateral congenital macrovessel-related macular edema in a 13-year-old boy.

Purpose: Congenital retinal macrovessels (CRM) are large aberrant blood vessels that cross the horizontal raphe. Visual acuity may be negatively affected in cases afflicted with CRM due to macular serous detachment, hemorrhage, exudation, foveolar cyst formation and retinal artery occlusion. Even the presence of an anomalous vessel itself running through the foveal avascular zone may compromise the visual acuity. In this case report, we discuss the multimodal imaging characteristics of a case of symptomatic unilateral CRM-related cystoid macular edema and serous macular detachment in a 13-year-old boy. Observations: Optical coherence tomography angiography (OCTA) imaging of the superficial retina revealed the aberrant vessel and anomalous arteriovenous communication between the aberrant vessel and the retinal artery. The foveal avascular zone also appeared partly distorted. Conclusions: The present case reports the second-youngest patient afflicted with unilateral CRM and associated macular edema. There was spontaneous resolution of macular edema within six weeks. In light of the observations in this case and the literature review, the need to refrain from rapid therapeutic intervention in the eyes of patients affected with CRM and macular edema must be emphasized.

Chronic recurrent multifocal osteomyelitis in a patient with selective immunoglobulin M deficiency.

Chronic recurrent multifocal osteomyelitis is an unusual inflammatory process of unknown origin involving multiple osseous sites, often recurrently. Selective immunoglobulin M (IgM) deficiency is a rare primary immunodeficiency disease, which can be associated with autoimmune diseases such as systemic lupus erythematosus, Hashimoto's disease, or hemolytic anemia. Here we report a case of a chronic recurrent multifocal osteomyelitis coexisting with selective IgM deficiency.

Unilateral orbital emphysema after sneezing.

We report a case who developed unilateral orbital emphysema after sneezing in a 30-year-old man presented with left crepitant eyelid swelling and progressive ptosis. Sneezing may cause orbital emphysema in cases with a history of minor periorbital trauma. While periodical followup without any medication is considered satisfactory, needle decompression may provide early cure.

Vertebral artery dissection in a patient with Wildervanck syndrome.

Vertebral artery dissection as a cause of stroke is rarely reported in children. The association between vertebral artery dissection and Klippel-Feil syndrome is also very rare. We report on a case of vertebral artery dissection with posterior circulation involvement in a child with Klippel-Feil syndrome after a hard physical-training lesson. She was also diagnosed with Wildervanck syndrome, with additional clinical findings. Vertebral artery dissection should be considered in patients with Klippel-Feil syndrome who present with acute-onset neurologic signs. Movements such as hyperextension with rotation of the neck should be avoided in these cases.

Misdiagnosis due to the hook effect in prolactin assay.

OBJECTIVES: To describe a patient who was misdiagnosed as having a nonfunctional pituitary tumor due to the hook effect on prolactin measurements. CLINICAL PRESENTATION AND INTERVENTION: A 45-year-old female was admitted with visual disturbances, panhypopituitarism and central diabetes insipidus due to pituitary tumor recurrence. She had been operated 4 times earlier and received cranial irradiation for a suspected nonfunctional pituitary adenoma. Serum prolactin was moderately elevated (164.5 ng/ml), but increased markedly after 1:100 dilution to 14,640 ng/ml. Diagnosis of a giant macroprolactinoma was made and cabergoline was started. Prolactin level normalized and a mild shrinkage of the tumor was achieved after 12 months of therapy. CONCLUSION: The hook effect must be kept in mind while evaluating a giant pituitary adenoma with moderately elevated prolactin levels. This way unnecessary surgical procedures or irradiation may be avoided.

Unilateral acute retinal necrosis initially presenting with painful orbitopathy.

Painful orbital inflammation and scleritis-like clinical appearance can be the initial manifestation of acute retinal necrosis. We report a 25-year-old woman who first presented with painful orbitopathy that eventually developed into unilateral acute retinal necrosis with typical fundus appearance. Our case illustrates the importance of careful fundus examination in cases presenting with painful orbital inflammation.

Orbital Apex Syndrome Secondary to Herpes Zoster Ophthalmicus.

Orbital apex syndrome is a rare complication of herpes zoster ophthalmicus. A patient being followed in our clinic for herpes zoster ophthalmicus developed orbital apex syndrome in the second week of treatment. Clinical diagnosis was supported by magnetic resonance imaging. Treatment with systemic steroid and antiviral therapy resulted in total regression of ophthalmoplegia at 2 months. However, optic neuropathy-induced vision loss was permanent. This case report examines orbital apex syndrome secondary to herpes zoster ophthalmicus, which has rarely been documented in the ophthalmic literature.

Basilar artery thrombosis in a child heterozygous for prothrombin gene G20210A mutation.

Prothrombin G20210A mutation is an important prothrombotic condition for venous thrombosis. Recently, some studies have also considered it to be a risk factor for arterial ischemic stroke in children. A 10-year-old boy with basilar artery thrombosis who was heterozygous for prothrombin G20210A mutation is described. In concordance with the previous literature, the present case suggests that prothrombin G20210A mutation may be a risk factor for arterial ischemic stroke in childhood.

Convergence spasm due to aquaporin-positive neuromyelitis optica spectrum disorder.

A female 27 presented with nausea and diplopia for 1 week. On examination she had normal vertical gaze but would develop convergence with miosis whenever she made horizontal saccades. Pupils were 6 mm and unreactive to light. MRI showed extensive hyperintensity in the dorsal midbrain and thalamus. Spinal MRI and CSF were both normal. Serum aquaporin-4-antibody was positive. She was treated with steroids and plasmapheresis and after 3 months convergence spasm resolved but pupils remained unreactive. Neuromyelitis optica often presents with brainstem signs, rarely a dorsal midbrain syndrome. Convergence spasm is occasionally of organic neurologic origin.

Bilateral Severe Sterile Inflammation with Hypopyon after Simultaneous Intravitreal Triamcinolone Acetonide and Aflibercept Injection in a Patient with Bilateral Marked Rubeosis Associated with Ocular Ischemic Syndrome.

We report the clinical course of a diabetic patient with bilateral cataract and rubeosis in association with ocular ischemic syndrome and initially treated him with simultaneous intravitreal 2 mg aflibercept and 2 mg triamcinolone acetonide injection at the same setting prior to planned cataract surgery and further photocoagulation. However, sterile anterior segment inflammation characterized by hypopyon occurred four days apart in OU. Right eye developed the sterile inflammation at the third postinjection day and the left eye developed the sterile inflammation at the seventh postinjection day (two days after the uneventful cataract surgery with intraocular lens implantation) without any pain or significant redness. Vitreous biopsy taken during the right phacovitrectomy was negative for any microbial contamination. Both eyes were treated successfully with intensive topical prednisolone acetate with a relatively good visual outcome. It is likely that underlying ocular ischemic syndrome might have facilitated the formation of sterile inflammation as blood-aqueous barrier disruption and flare have already been present.

Unilateral Adult Xanthogranulomatous Infiltration of the Conjunctiva, Limbus and Sclera Leading to a Combined Ophthalmic Artery and Central Retinal Vein Occlusion.

OBJECTIVE: To describe the features of a female patient with a biopsy-proven xanthogranulomatous infiltration of the conjunctiva, limbus and sclera who had an exudative retinal detachment, combined ophthalmic artery and central retinal vein occlusion unilaterally. METHOD: A-53-year old otherwise healthy woman presenting with a painful visual loss in her right eye underwent an ophthalmic examination, meticulous systemic work-up and histopathologic assessment. RESULTS: Ophthalmic examination revealed multiple subconjunctival masses, upper limbal infiltrations, trace cells in the anterior chamber, pale looking posterior fundus, 360 degree scattered retinal hemorrhages and marked exudative retinal detachment in her right eye. Left eye was completely normal.A biopsy taken from one of the subconjunctival masses demonstrated a diffuse infiltration of the histiocytes and this was interpreted as a xanthogranulomatous infiltration with the help of immunohistochemical staining techniques. CONCLUSION: Present case is the only reported adult case with xanthogranulomatous-like infiltration of the eyeball featuring both anterior and posterior segment involvement without any concomitant major systemic disturbances.