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Ozge Sarac, Mehtap Caglayan, Pinar Kosekahya, Murad Bavbek, Nurullah Cagil
A 32 year old female patient with CM 1 diagnosis was referred for the management of papilledema. Ophthalmologic examination revealed normal visual acuity (20/20 in both eyes), normal optic nerve function tests and normal slit-lamp biomicroscopic findings. Fundoscopy revealed bilateral irregular optic nerve heads with blurred margins. B scan ultrasonography (USG) and Spectral domain optical coherence tomography were performed and bilateral optic nerve heads were diagnosed as ODD. We concluded that the pseudopapilledema must be taken into account before making papilledema diagnosis in patients with CM 1 to protect the patients from redundant interventional procedures...
August 2018: Neuro-ophthalmology
Azusa Yamagishi, Toshiyuki Oshitari, Ayako Tawada, Takayuki Baba, Shuichi Yamamoto
A 61-year-old man with hyperthyroidism had exophthalmos with dilated conjunctival vessels in both eyes. Magnetic resonance imaging showed that the superior ophthalmic veins had a funicular-like appearance. Cerebral angiography showed no blood flow from both internal carotid arteries to the cavernous sinus, thus excluding a carotid-cavernous fistula. Blood tests showed an elevation of IgG4 (281 mg/dl), and a IgG4-related ophthalmic disease was considered. Steroid pulse therapy was performed, and all of the abnormal findings were improved...
August 2018: Neuro-ophthalmology
Marie Takeishi, Toshiyuki Oshitari, Satomi Ota, Takayuki Baba, Shuichi Yamamoto
A 76-year-old woman was diagnosed with thyroid-associated orbitopathy (TAO) associated with hypothyroidism at another clinic. Magnetic resonance imaging showed enlargements of the extraocular muscles. Because her best-corrected visual acuity (BCVA) was 0.01OD, she was referred to the Chiba University Hospital. She had an elevation of serum IgG4. She was diagnosed with possible IgG4-related ophthalmic disease (IgG4-ROD) accompanied by optic nerve compression with the mass. She underwent methylprednisolone pulse therapy, and, two months later, her BCVA and visual field defect were greatly improved...
August 2018: Neuro-ophthalmology
Christelle Blanc, Samuel Bidot, Françoise Héran, Émilie Tournaire-Marques, Catherine Vignal-Clermont
We report the case of a woman who developed right third nerve dysfunction with synkinesis and ocular neuromyotonia secondary to a compressive arterial aneurysm. Surprisingly, our examination showed a downward drift of the right eye in sustained up-gaze resulting in transient hypotropia, suggesting either fatigability of the superior rectus or contraction of the inferior rectus. We believe this ocular motility pattern is secondary to a co-contraction of the inferior rectus in up-gaze caused by synkinesis (explaining the downward drift), followed by failure of the inferior rectus to relax upon return to primary position caused by ocular neuromyotonia (explaining the hypotropia)...
August 2018: Neuro-ophthalmology
Oana M Dumitrascu, Erin M Okazaki, Steven H Cobb, Matthew A Zarka, Stephen A De Souza, Gyanendra Kumar, Cumara B O'Carroll
Amyloid beta-related angiitis (ABRA) is a subtype of cerebral amyloid angiopathy-related inflammation, with distinctive pathology and prognosis compared with cerebral amyloid angiopathy (CAA). On a spectrum of increasing severity, ABRA is considered to be in-between the less aggressive inflammatory-CAA and the more severe primary central nervous system (CNS) angiitis. Whereas retinal pathological changes were described in subjects with primary or secondary CNS angiitis, and non-inflammatory CAA, bilateral posterior pole superficial and peripapillary retinal hemorrhages have not been reported as initial signs in patients with pathology-confirmed ABRA, accompanying neurological spells and characteristic neuroimaging findings...
August 2018: Neuro-ophthalmology
Chike Ilorah, Brandon Bond, Jorge C Kattah, Bahareh Hassanzadeh
Ecchordosis physaliphora (EP) is a rare non-malignant mass that originates from remains of the notochord and is typically asymptomatic. A 42-year-old man presented with sudden onset of painless horizontal diplopia and his neurological exam showed sixth cranial nerve palsy. Magnetic resonance imaging (MRI) identified a non-enhanced retroclival mass (EP) with increased signal intensity on T2 and decreased signal intensity on T1-weighted sequences. He was treated with methylprednisolone, completely recovered in four weeks and has remained symptom free...
August 2018: Neuro-ophthalmology
Sonya T Blizzard, Megan E Collins, Neil R Miller
Cavernous angiomas of the cranial nerves are extremely rare, and those of the oculomotor (third) cranial nerve are rarer still, with no previous cases having been reported in the literature. We report herein the first case of a presumed cavernous angioma involving the subarachnoid portion of the left third nerve presenting as an acute left third nerve palsy in an infant. The child was followed without intervention and the palsy completely resolved. Given the poor functional results with attempted surgical excision and the potential for spontaneous improvement in oculomotor nerve function, it is reasonable to follow patients with these lesions without intervention...
August 2018: Neuro-ophthalmology
Pinar Kosekahya, Mehtap Caglayan, Ozlem Unal, Sema Yuzbasioglu, Mustafa Koc, Cemile Ucgul Atilgan, Fatma Yulek
In this prospective study, the biomechanical properties of optic nerve head (ONH) and cornea in both eyes of patients with non-arteritic anterior ischaemic optic neuropathy and healthy control eyes were investigated. ONH elastometry was measured with real-time elastography, and corneal elastometry was measured with ocular response analyser. Elastometry of cornea and ONH was lower in both eyes of patients with unilateral non-arteritic ischaemic optic neuropathy than in healthy control eyes. The role of these biomechanical differences in the pathogenesis of non-arteritic ischaemic optic neuropathy should be investigated further...
August 2018: Neuro-ophthalmology
Margaret Wang, Ama Sadaka, Thomas Prager, Andrew G Lee, Francesco Pellegrini, Daniele Cirone, Luca De Simone, Luca Cimino
Acute idiopathic blind spot enlargement (AIBSE) is often caused by Acute Zonal Occult Outer Retinopathy (AZOOR), an outer retinal disease. We report two illustrative cases of AZOOR. The first one was a 21-year-old white female who presented with a scotoma and "shimmering lights" in her left eye. In the second case, a 73-year-old white female was referred for evaluation of a "bitemporal hemianopsia" that started years prior, with no clinical significant photopsias. To our knowledge, case two is the longest documented duration of bilateral, progressive, and chronic, idiopathic, enlargement of the blind spot (CIBSE) documented in the English language ophthalmic literature...
August 2018: Neuro-ophthalmology
Rafat Ghabrial, Arjun Ananda, Sebastiaan J van Hal, Elizabeth O Thompson, Stephen R Larsen, Peter Heydon, Ruta Gupta, Svetlana Cherepanoff, Michael Rodriguez, Gabor Michael Halmagyi
Invasive fungal sinusitis causes painful orbital apex syndrome with ophthalmoplegia and visual loss; the mechanism is unclear. We report an immunocompromised patient with invasive fungal sinusitis in whom the visual loss was due to posterior ischaemic optic neuropathy, shown on diffusion-weighted MRI, presumably from fungal invasion of small meningeal-based arteries at the orbital apex. After intensive antifungal drugs, orbital exenteration and immune reconstitution, the patient survived, but we were uncertain if the exenteration helped...
August 2018: Neuro-ophthalmology
Aastha Takkar, Manoj Kumar Goyal, Reema Bansal, Vivek Lal
Despite the potential of Idiopathic Intracranial Hypertension (IIH) to cause visual morbidity, limited literature is available focussing on predictors of visual outcome in IIH. This study was planned to assess visual morbidity in patients of IIH in terms of clinical and neuro-ophthalmo- logical parameters. In this prospective study of 40 patients of IIH, neuro-ophthalmological parameters were noted in the form of visual acuity, visual field, contrast sensitivity, Retinal Nerve Fibre Layer thickness, and visual evoked potential...
August 2018: Neuro-ophthalmology
Omar Alsinaidi, Aasef G Shaikh
The authors describe diffusion-weighted magnetic resonance imaging (DWI) as an accessible way to screen for acute retinal pathology in emergency setting. They describe three proof-of-principle patients who had abrupt onset of vision loss from acute retinal pathology where DWI was diagnostic. The deficits included central retinal artery occlusion and acute retinitis with retinal necrosis. DWI can be non-specific for aetiology, but it can rapidly localise the retinal pathology and initiate early therapeutic interventions in the emergency room...
June 2018: Neuro-ophthalmology
Neil M D'souza, Huy D Nguyen, Stacy V Smith, Priyadharsini Nagarajan, Adi Diab, Richard C Allen, Dan S Gombos, Andrew G Lee
A 23-year-old man with a history of metastatic melanoma developed painful vision loss to counting fingers with enhancement of optic nerve on contrast-enhanced magnetic resonance imaging (MRI) and received a diagnosis of optic neuritis from an outside hospital. Despite empiric corticosteroid therapy, the patient worsened and developed secondary central retinal vein occlusion with further deterioration of vision. Repeat MRI demonstrated optic nerve sheath (ONS) involvement suggestive of optic perineuritis (OPN) and an ONS biopsy confirmed a rare case of isolated metastatic melanoma...
June 2018: Neuro-ophthalmology
Isabel Pascual-Camps, Clara Martínez-Rubio, Roberto Gallego-Pinazo, Enrique España-Gregori
Arteriovenous fistulas (AVFs) are abnormal shunts between the arterial and venous vascular systems. These usually produce ocular pain, increased intraocular pressure (IOP), and diplopia. Less frequently, they may cause retinal changes with visual impairment. Our purpose is to illustrate different retinal manifestations of AVF. We report the multimodal imaging study of three cases with retinal changes due to AVF, showing neurosensory retinal detachment, macular oedema, and macular ischemia. In conclusion, AVF may appear with different ophthalmic alterations...
June 2018: Neuro-ophthalmology
Bahareh Hassanzadeh, Jeffrey DeSanto, Jorge C Kattah
Ipilimumab is a novel anti-melanoma agent known to infrequently cause multi-organ autoimmunity. We report a case of pituitary hypophysitis and orbital inflammation followed by an orbital apex syndrome. A 64-year-old woman with a history of skin melanoma, receiving ipilimumab treatment, was seen for near total loss of vision in the right eye and proptosis. Headache of 3-month duration preceded the onset of diplopia followed by severe loss of vision in the right eye. Neuro-ophthalmologic examination was consistent with an orbital apex syndrome...
June 2018: Neuro-ophthalmology
Jason T Chisholm, Michelle M Abou-Jaoude, Amy B Hessler, Padmaja Sudhakar
A 24-year-old non-obese, but slightly overweight, female presented with a two-week history of progressive severe headache associated with two days of blurry vision. Clinical exam was significant for bilateral papilledema and an enlarged blind spot on visual field testing. Contrast enhanced MRI head revealed no space occupying lesion. A lumbar puncture revealed an elevated opening pressure of 38 cm H2 O with normal cerebrospinal fluid composition leading to a diagnosis of pseudotumor cerebri syndrome (PTCS)...
June 2018: Neuro-ophthalmology
Daniah Alshowaeir, Abdulrazag Ajlan, Sajjad Hussain, Adel Alsuhaibani
The outcome of two patients with visual loss from osteopetrosis who underwent an optic nerve sheath fenestration (ONSF) is reported. A 20-year-old male and 26-year-old female with osteopetrosis had optic nerve edema. Computed tomography and magnetic resonance imaging demonstrated optic canals stenosis. Both patients underwent unilateral ONSF. After ONSF, the patients experienced improvement in visual acuity and optic nerve appearance. Therefore, when papilledema is recognized in osteopetrosis patients, it may be reasonable to start with an ONSF even if the optic canal seems to be stenotic because of the lower morbidity that is associated with this procedure compared with other surgical options...
June 2018: Neuro-ophthalmology
Krishnan Ravindran, Philip Schmalz, Nurhan Torun, Michael Ronthal, Yu-Ming Chang, Ajith J Thomas
The Tolosa-Hunt syndrome is a rare clinical condition characterized by painful opthalmoparesis associated with idiopathic granulomatous inflammation of the orbital apex and cavernous sinus. Historically, this condition was thought to result from arteritic changes in the internal carotid artery and cavernous sinus. Modern digital angiographic techniques were unavailable when THS was initially described, and few reports exist on its high-resolution angiographic findings. Painful ophthalmoparesis, especially of the oculomotor nerve, warrants vascular imaging because of the concern for an underlying aneurysm...
June 2018: Neuro-ophthalmology
Tyler Moore, Kenn Freedman
Basal encephaloceles are uncommon malformations that are usually congenital in nature, although, occasionally, they can be traumatic. When they are congenital, they are associated with skull and facial midline defects, central nervous system abnormalities, and possible ocular and visual problems. The authors present a case of an adult male with a basal encephalocele who presented with a bitemporal hemianopsia. Ocular abnormalities, visual loss, and visual field deficits have previously been reported in patients with basal encephalocele; however, there does not appear to be any reported cases with formal visual field testing to demonstrate specific defects such as a bitemporal hemianopsia...
June 2018: Neuro-ophthalmology
Siegfried K Wagner, Nabil Uddin, Saurabh Jain
Miller Fisher syndrome is an acute demyelinating polyneuropathy classically presenting with ataxia, areflexia, and ophthalmoplegia. The authors report the case of a 27-year-old female, who presented with limb weakness and double vision following a prodromal pharyngitis. Ophthalmic examination revealed fluctuant ophthalmoplegia eventually consistent with bilateral sixth cranial nerve palsies, prompting investigation for anti-ganglioside antibodies, which returned positive. Due to disabling diplopia, the patient was treated with botulinum toxin, with a resulting favourable reduction in the size of strabismus...
June 2018: Neuro-ophthalmology
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