Left Visual Loss Research Articles

Endovascular treatment strategy and clinical outcome of tentorial dural arteriovenous fistula.

To evaluate treatment strategies and clinical outcomes following endovascular embolization of tentorial dural arteriovenous fistulas. We retrospectively analyzed 19 patients with tentorial dural arteriovenous fistulas admitted to the Department of Neurosurgery at Jiangsu Provincial People's Hospital between October 2015 and May 2022, all treated with endovascular therapy. To collect and analyze patients' clinical presentation, imaging data, postoperative complications, and prognosis and to analyze the safety and clinical outcomes of endovascular treatment of tentorial dural arteriovenous fistulas. Imaging cure was achieved in 18 patients, with the arterial route chosen for embolization in 17 patients and the venous route in one patient; one patient received partial embolization. Staged embolization was performed in four patients. At postoperative follow-up of 9-83 months (37.8 ± 21.2), all 19 patients had recovered well (mRS score ≤ 2). Three patients experienced perioperative complications: intraoperative Onyx reflux into the middle cerebral artery in one patient; postoperative permanent limited left visual field loss and deafness in the left ear in one patient; and transient diplopia, vertigo, and decreased pain and temperature sensation of the left limb in one patient, with no abnormalities on post-procedure magnetic resonance examinations. A total of 17 patients completed a postoperative digital subtraction angiography review during follow-up, and one patient had a recurrence of an arteriovenous fistula. Endovascular treatment of tentorial dural arteriovenous fistulas is safe and effective. Reduction of the Borden or Cognard classification via eliminating cortical venous reflux through multi-staged embolization or combined open surgery is a reasonable goal of treatment where complete obliteration of the fistula is not achievable.

Invasive Optic Nerve Aspergillosis with Intracranial Extension in an Immunocompetent Patient (P10-10.004)

<h3>Objective:</h3> To report on an uncommon case of Invasive Optic Nerve Aspergillosis in an Immunocompetent Patient <h3>Background:</h3> Orbital aspergillosis is an uncommon and life-threatening infection. It often affects immunocompromised individuals but can rarely be seen in immunocompetent patients. We present a case of invasive optic nerve aspergillosis with intracranial extension in an immunocompetent male. <h3>Design/Methods:</h3> Case Report <h3>Results:</h3> 59-year-old man presented with a 6-week history of complete left vision loss and left sided facial pain. He was initially diagnosed with optic neuritis and treated with oral prednisone without resolution of symptoms. Initial work including computed tomography (CT) of the head, magnetic resonance imaging (MRI) of the brain, digital subtraction angiography of the head, fluorescence angiography of the left eye, and temporal artery biopsy were all unremarkable. Optical coherence tomography (OCT) demonstrated mild left optic nerve edema. Due to persistent symptoms, an MRI of the orbits was obtained and revealed left optic nerve diffusion restriction with enhancement along the optic tract, suggesting an abscess. Interval OCT revealed worsening left optic nerve layer thinning. The patient was treated initially treated with ceftriaxone, metronidazole, and vancomycin. The optic nerve abscess was surgically decompressed. Cultures grew Aspergillus fumigatus. He was transitioned to oral isavuconazole for a total of 6 months of antifungal therapy. Repeat head CT one month later revealed skull base osteomyelitis with intracranial extension requiring further surgical debridement. The patient was kept on antifungal therapy. Repeat neuroimaging has been stable but there has been no improvement in his vision. <h3>Conclusions:</h3> Orbital aspergillus is a rare entity and as such is a diagnostic challenge. Multiple surgical debridements in addition to appropriate antifungal therapy may be required as recurrent infections are common. Close interval imaging should be obtained to aid in the initial diagnosis and monitoring of orbital fungal infections. <b>Disclosure:</b> Dr. Gill has nothing to disclose. Dr. Whiting has nothing to disclose. Dr. Malik has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genetech . Dr. Malik has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Jansen . Dr. Malik has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for EMD serono. The institution of Dr. Malik has received research support from National MS Society .

Teaching NeuroImages: Retinal migraine in action

A 56-year-old man with migraine headache reported a 2-day history of 5 episodes of left eye visual loss, each lasting 5 minutes. He described seeing a curtain moving in nasal to temporal direction, disappearing gradually, followed by a left periorbital pulsatile headache lasting 10 minutes.

Rhino-Sino-Cerebral Mucormycosis Associated with Long-Term Rituximab Therapy in Multiple Sclerosis: A Case Report and Review of the Literature

Introduction: Rituximab is a CD20 depleting agent, widely used as an off-label diseasemodifying treatment (DMT) in treating multiple sclerosis (MS). The present study aimed to report the first case of rhino-sino-cerebral mucormycosis in a patient with secondary progressive MS (SPMS), treated with rituximab. Case Report: The patient was a 38-year-old man with a history of SPMS treated with rituximab, who developed subacute left vision loss and impaired ocular movement. He also mentioned a concomitant severe headache and cheek swelling. Based on the brain and orbital magnetic resonance imaging (MRI) findings, rhino-sino-cerebral mucormycosis was suspected. Subsequent endoscopic examination confirmed the diagnosis. He underwent medical treatment with amphotericin B liposomal and surgical debridement leading to a significant clinical recovery. He was eventually discharged home with a solid recommendation to discontinue rituximab. Conclusion: The present report indicates a case of SPMS treated with rituximab, who developed rhino-sino-cerebral mucormycosis.

Skull Base Chordoma: The Thumb Sign

A 57 year old woman with no past medical history presented with headaches and progressive left vision loss. Subsequently, a contrast enhanced brain MRI was performed showing a large well defined skull base mass located laterally in the left petro clival fissure, exhibiting high T2 and FLAIR signal intensity (Figure A), with areas of low signal intensity on susceptibility weighted images consistent with calcifications and a heterogenous enhancement after injection. This tumor was responsible for mass effect on the orbital apex and optic nerve anteriorly and the mesio temporal lobe laterally. Most importantly, on the sagittal planes we can see the tumor projection indenting the pons which is displaced posteriorly. This finding is known as the thumb sign (Figure B and C) characteristic of chordomas. Given these MRI features and after a stereotactic biopsy the diagnosis of chordoma of the skull base was confirmed. Because of the major local extension, the patient underwent radiotherapy rather than surgical resection. Chordomas are rare skull base tumors arising from embryonal rest cells [1]. The major differential being chondrosarcomas with similar appearance but higher ADC values. The thumb sign is relatively specific of chordomas [2] although itcan be described in chondromas of the skull base and can also be seen in chondrosarcomas.

Masquerade versus masquerade

This is an atypical uniocular presentation of systemic diffuse large B-cell lymphoma. An 80-year-old Caucasian female presented with acute left visual loss, anorexia, headache, and jaw pain with disk swelling. Systemic steroids for giant cell arteritis were started despite negative temporal artery biopsy. Later, an inferior exudative retinal detachment developed. Lytic skull lesions were eventually seen on magnetic resonance imaging. Diffuse large B-cell lymphoma was diagnosed postmortem. This is the second documented case of unilateral serous retinal detachment with no uveitis/pseudouveitis or choroidal infiltrate due to lymphoma. It demonstrates pitfalls of pattern recognition when pieces of the jigsaw do not fit.

A Case of Pituitary Germinoma With Complete Recovery of Visual Field

Abstract Central nervous system germ cell tumours (GCTs) are rare, accounting for 0.1-3.4% of all primary brain malignancies. Intracranial GCTs (ICGCTs) usually arise within the pineal recess (50-65%), sellar-suprasellar region (25-35%) and rarely in the basal ganglia-thalamus (5-10%). A peakonset occurs during the second decade of life with a median age at diagnosis between10-12 years and a male predominance. Histologically, ICGCTs are classified into germinomas accounting for two thirds of cases and non-germinomatous GCTs. ICGCTs present with clinical features dependent on their location and tumour size. Symptoms most often arise from compressed optic structures, increased intracranial pressure and central endocrine abnormalities, particularly diabetes insipidus (DI). Clinical findings combined with elevated tumour marker levels within the serum and/or cerebrospinal fluid (CSF) can be diagnostic. Case: A 30-year-old lady who survived a childhood leukaemia presented as an emergency with a two-month history of headaches, dizziness and near-complete visual loss. Cranial MRI identified a large sellar mass with suprasellar extension compressing the optic chiasma and hypothalamus. Biochemical and endocrine profiles confirmed diabetes insipidus, hypocortisolaemia and hypothyroidism all requiring hormone replacement. Visual field examination revealed total left visual loss and a right temporal hemianopia. Cerebrospinal fluid analysis identified an elevated human chorionic gonadotropin (HCG) level at 16 IU/L (normal &amp;lt;2), with a normal alpha-fetoprotein (AFP) level. Both tumour markers were normal in the serum. Emergency Etoposide-Cisplatin (Em-EP) chemotherapy was initiated for a suprasellar GCT followed by the EPOMB-ITMTX regimen [Etoposide-Cisplatinwith intrathecal Methotrexate (ITMTX) alternating with Vincristine-Methotrexate-Bleomycin]. After four cycles EPOMB-ITMTX, the vision was fully restored, brain MRI demonstrated an excellent radiological response and the CSF HCG normalised. Our patient received volumetric-modulated arc cranial radiotherapy (VMAT) with a total 54 Gy in 16 fractions. She remains well without disease recurrence for 14 months. Tumour markers are normal with stable MRI brain and whole-spine. Endocrine follow-up confirms recovery in all pituitary axes except the gonadotrophins for which she remains on hormone replacement therapy. Conclusion: This case demonstrates an excellent outcome for a young adult diagnosed with a suprasellar GCT treated with chemotherapy followed by stereotactic radiotherapy. It highlights the importance in keeping a high clinical index of suspicion in young adults presenting with a midline intracranial tumour, visual dysfunction and an endocrinopathy. CSF analysis identified an elevated tumour marker level, which bypasses the need for a histological diagnosis.

Granulomatosis with polyangiitis in a patient treated with dabrafenib and trametinib for BRAF V600E positive lung adenocarcinoma

BackgroundDabrafenib and trametinib combination therapy is approved for the treatment of patients with BRAF V600E positive tumors including melanoma and lung cancer. The effect of BRAF and MEK inhibitors on the immune system is not fully understood although a number of case reports indicate autoimmune side effects related to the use of these drugs. Here, we discuss a case of a patient diagnosed with granulomatosis with polyangiitis (GPA) shortly after starting treatment with dabrafenib and trametinib for BRAF V600E positive metastatic lung adenocarcinoma.Case presentationA 57 years old female patient was diagnosed with recurrent lung adenocarcinoma following initial lobectomy for early stage disease. A BRAF V600E mutation was identified at the time of recurrence and she received combination dabrafenib and trametinib therapy. Shortly after commencement of treatment, she developed persistent fevers necessitating withholding both drugs. Pyrexia continued and was followed by left vision loss and acute kidney injury. Further rheumatological workup led to the unifying diagnosis of GPA. The patient was then treated with rituximab for GPA to the present date while all antineoplastic drugs were held. Lung cancer oligoprogression was addressed with radiation therapy and has not required further systemic treatment whereas GPA has been controlled to-date with rituximab.ConclusionsThis case report raises awareness among clinicians treating patients with lung cancer for the possibility of triggering a flare of autoimmune diseases like GPA in patients with BRAF V600E positive lung cancer receiving treatment with BRAF directed therapy.

Neurofibromatosis Type 1 Vasculopathy Presenting as Impending Central Retinal Artery Occlusion

Neurofibromatosis Type 1 Vasculopathy Presenting as Impending Central Retinal Artery Occlusion

Teaching NeuroImages: Retinal migraine in action

A 56-year-old man with migraine headache reported a 2-day history of 5 episodes of left eye visual loss, each lasting 5 minutes. He described seeing a curtain moving in nasal to temporal direction, disappearing gradually, followed by a left periorbital pulsatile headache lasting 10 minutes.

A Successful Management of 29 year old Female with Left Central Retinal Artery Occlusion due to Manifestation of Primary Antiphospholipid Syndrome

Ocular involvement in Anti Phospolipid Syndrome (APS) includes a broad spectrum of manifestations from the anterior and posterior segment or the presence of neuro-ophthalmologic features. A female, 29 years old, came to ER handled by ophthalmology department, with chief complaint left visual loss suddenly since 4 hours before admission. Investigations revealed stable vital signs, VOD 20/20, VOS 1/300, funduscopy showedpale and cherry red spot on left retina, OCT revealed hyperreflective of left inner retinal layer, IgG aCL 51.7 U/mL (50.8 U/mL in OPD 3 months later), and the other examinations were within normal limit. Patient was diagnosed with Central Retinal Artery Occlusion due toPrimary Antiphospolipid syndrome. She was performed occular massage and anterior chamber paracintesis procedure, and given O2 6-8 lpm NRBM, Timolol 0.5% eye drop left eye bid, acetazolamide 250 mg bid, Kalium Slow Release 1 tab qd, Levofloxacine eye drop 1 drop/hour post surgery. After the result of IgM aCL available, we added warfarin 2 mg qd and aspirin 320 mg qd. Patient was discharged 2 days later as visual acuity improved with VOD 20/20 and VOS 0.5/60. Key words: Central retina artery occlusion, primary anti phospolipid syndrome, anti cardiolipin antibody

Visual loss resulting from siderosis bulbi caused by a missed intraocular foreign body

Introduction: Intraocular foreign bodies (IOFB) represent an important cause of visual impairment, especially within the group of working age population. Case report: A 38-year-old male presented with severe left eye pain, accompanied by left headache, visual loss, redness, photophobia, tearing and nausea, 30 months following a minor left eye trauma when hammering metal on metal. Eye ultrasound identified a small intraocular foreign body as well as the crystalline lens luxated into the vitreous cavity. Pars plana vitrectomy was carried out with the extraction of the foreign body and of the lens. Pain was relieved, but vision was not regained. Conclusion: The poor outcome of this case follows from a combination of unusual factors: ophthalmologists’ failure to identify an occult IOFB and the lack of patient’s concern about his eye condition, until the moment when he experienced excessive pain and vision was already lost.

Visual loss due to optic nerve infarction and central retinal artery occlusion after spine surgery in the prone position: A case report.

Rationale:Visual loss after spine surgery in the prone position is a serious complication. Several cases of central retinal artery occlusion with ophthalmoplegia after spine surgery have been reported in patients with ophthalmic arteries fed by the internal carotid artery (ICA) in a normal manner.Patient concerns:A 74-year-old man developed visual loss after undergoing a spinal decompression and fusion operation in the prone position that lasted approximately 5 hours.Diagnoses:We detected an extremely rare case of visual loss due to optic nerve infarction and central retinal artery occlusion through fundoscopic examination, fluorescein angiogram, brain magnetic resonance imaging, and magnetic resonance angiography. The patient's visual loss may have been caused by compromised retrograde collateral circulation of the ophthalmic artery from branches of the external carotid artery in the presence of proximal ICA occlusion after a spinal operation in the prone position.Interventions:To recover movement of the left extraocular muscles, the patient received intravenous injections of methylprednisolone for 3 days and then oral prednisolone for 6 days.Outcomes:Twenty days after the treatment, the motion of the left extraocular muscles was significantly improved. However, recovery from the left visual loss did not occur until 4 months after the operation.Lessons:In high-risk patients with retrograde collateral circulation of the ophthalmic artery from the external carotid artery due to proximal ICA occlusion, various measures, including the use of a head fixator to provide a position completely free of direct compression of the head and face, should be considered to decrease the risk of postoperative visual loss.

Anti-MOG positive pediatric cases with diverse clinical spectrum in demyelinating disorders

Myelin oligodendrocyte glycoprotein (MOG) is a protein expressed on the outer surface of oligodendrocytes and myelin sheath in the central nervous system (CNS). It has been identified as an candidate autoantibody target in inflammatory demyelination disease. Autoantibodies against MOG predominantly in children are related to multiple sclerosis(MS), acute disseminated encephalomyelitis(ADEM), neuromyelitis optica spectrum disorder(NMOSD) and relapsing optic neuritis(ON) Here we report three cases who had anti-MOG antibody positive with distinctive clinical features. Case 1 was a 6-year- old girl who was presented with similiar attacks of encephalopathy, gait disturbance.She was diagnosed as multiphasic ADEM with bilateral patchy T2–flair hyperintensity on cranial magnetic resonance imaging( MRI). She responded to the combination of low-dose steroid and AZA treatment ; the number of attacks was significantly decreased. Case 2 was a 13-year-old boy who was presented with left visual loss. His neurological examination was normal except pale left optic disc. Orbital MRI revealed T2 hyperintensity and gadolinium enhancement of left optic nerve. AQP-4 antibody was negative, he was recovered completely with the steroid treatment. After the two years he was admitted with blurred vision on the other eye. He was diagnosed as recurrent ON as his orbita MRI showed ON. His anti-MOG antibody which analyzed at the second attack was positive. Case 3 was a 5-year-old girl who was presented with ascendent weakness. She was diagnosed as pediatric acute myelitis with T2 hyperintensity from medulla oblongata to sixth thoracal level on her MRI. Two months later, she was presented with back pain while steroid treatment was tapering. MRI showed T2 hyperintensity between T8-T12 levels. She was diagnosed with NMOSD. She was treated with the azathioprine after the second attack. We intend to emphasize that MOG antibody should be searched in pediatric demyelinating disorders and its positivity may reflect diverse clinical spectrums.

Central retinal artery occlusion in the case of traumatic orbital cellulitis

Vision can be lost after orbital infection as a result of optic nerve atrophy, central retinal artery occlusion (CRAO). We report such a case. We present a case report CRAO secondary to periorbital cellulitis. A 52-years-old male patient attended our clinic with left upper eyelid edema, hyperaemia, pain and visual loss. History revealed that he had an ocular surface trauma with cement to his left eye 7 days before. Fundus examination showed patchy filling of the branches of the central retinal artery, retinal opacification at the posterior pole and cherry red spot at the macula. His left visual acuity was declined to no perception of light. There was no clinical improvement after 7 days teatment. Orbital cellulitis did not respond to all treatment and magnetik resonance imaging demonstrated subperiosteal abscess at the posterior-superior orbital wall. We report a case of orbital cellulitis following an ocular surface trauma resulting in the rare complication of central retinal artery occlusion.

Direct percutaneous transorbital puncture under fluoroscopic guidance with a 3D skull reconstruction overlay for embolisation of intraorbital and cavernous sinus dural arteriovenous fistulas.

To describe the direct percutaneous transorbital puncture technique for embolisation of the selected intraorbital and cavernous sinus dural arteriovenous fistula, which failed to be treated by conventional endovascular techniques. One case of intraorbital and five cases of cavernous sinus dural arteriovenous fistula were embolised through direct percutaneous transorbital puncture in 2012, and the clinical data were reviewed. Under fluoroscopic guidance with a three-dimensional (3D) skull reconstruction overlay, the cavernous sinus or ophthalmic vein was punctured via the superior orbital fissure. Then a microcatheter was inserted via the needle, and Onyx was injected to embolise the fistula with or without a combination of coils. Complete obliteration of the fistula was achieved in all six patients. After operation all the patients experienced transient swelling of the punctured orbit persisting for three to five days. No other complications occurred. Follow-up of six patients at three to six months showed resolution of their initial neuro-ophthalmological symptoms in five and left visual loss in one did not recover. Six months follow-up angiogram showed no recurrence of these fistulas. Direct percutaneous transorbital puncture provides an option for the intraorbital and cavernous sinus dural arteriovenous fistulas, particularly when the conventional transvenous routes are inaccessible. Overlay of the 3D skull reconstruction can facilitate the precise puncture of the superior orbital fissure.

Polyneuritis and refractory headache overlap with synovitis, acne, pustulosis, hyperostosis and osteitis syndrome

The primary clinical manifestations of synovitis, acne, pustulosis, hyperostosis and osteitis syndrome are skin lesions and osteitis. We describe a 53-year-old woman patient suffering from overlapping refractory headache and polyneuritis (cranial nerves I, II and VII). She complained of headaches, which recurred every month, followed by bilateral anosmia and left vision loss approximately 1 year later. Prednisolone therapy improved her symptoms; however, her refractory headache, pain in the right shoulder and left hip joint, and rash recurred 1 year after prednisolone withdrawal. Also, the patient presented with left sensorineural deafness and extensive acne on her body, and bone scintigraphy and bone biopsy showed synovitis, acne, pustulosis, hyperostosis and osteitis syndrome. After treatment, her symptoms improved without recurrence. Although synovitis, acne, pustulosis, hyperostosis and osteitis syndrome induced by polyneuritis is rare, it should be considered when clinicians encounter patients presenting with headache and polyneuritis of unknown cause.

Carotid artery dissection treated with stenting after anticoagulation failure

Carotid artery dissection may occur in normal healthy vessels, as well as vessels weakened by primary arteriopathy, but is most common after trauma.1 The American Heart Association/American Stroke Association guidelines...

Sphenoid wing Metastasis from Occult Prostatic Carcinoma

Prostatic carcinoma commonly metastasizes to skeletal bone or vertebral bodies, forming osteosclerotic bone metastases, also it metastasizes to the head and neck region. This is the case of prostatic carcinoma presenting with an osteosclerotic metastasis to the sphenoid wing. A 64-year-old man complained of progressive left visual loss and exophthalmos. Computed tomography (CT) revealed a clear soft-density mass in the left sphenoid wing and fluorodeoxyglucose-positron emission tomography (FDG-PET) showed almost no uptake of FDG in this lesion. Biopsy from the sphenoid wing was obtained with the canine fossa approach. Pathological diagnosis was prostate adenocarcinoma. This case serves as a reminder that prostate carcinoma should be considered whenever osteosclerotic bone changes are observed on CT. FDGPET proved to be less sensitive than scintigraphy for the diagnosis of osteosclerotic metastasis from prostate carcinoma.

A case of atypical Takayasu arteritis initially presenting with peripheral artery disease

A 28-year-old woman developed Raynaud’s phenomenon and sudden left visual loss due to hypertensive retinopathy. One and a half years later, she noticed peripheral coldness and numbness of right foot. Enhanced computed tomography showed remarkable stenosis of left radial and bilateral tibial arteries. Initially, the diagnosis of idiopathic peripheral arterial occlusive disease (PAD) was made. PAD gradually progressed despite the medication with antiplatelet drug and vasodilator, and coronary angiography revealed a left coronary artery aneurysm. Then, she developed the same ischemic symptom of right hand and left visual loss caused by papilledema. Although 18 F-fluorodeoxyglucose positron-emission tomography showed no abnormalities, and both HLA-B52 and HLA-B39 were negative, she was diagnosed with atypical Takayasu arteritis (TA) by exclusion of other possible diseases. To the best of our knowledge, this is the first case of TA with coronary aneurysm and PAD. In TA patients, the involvement of medium-sized vascular lesion such as peripheral extremity arteries should be considered.