Episodes Of Visual Loss Research Articles

Encephalopathy with a reversible lesion in the splenium of the corpus callosum in adults

Background & Objectives: Mild encephalitis/encephalopathy with a reversible splenial lesion (MERS) is a clinical and radiological syndrome with a lesion in splenium of the corpus callosum. This lesion is typically detected in the corpus callosum with magnetic resonance imaging (MRI) and usually disappears within a few weeks. The clinical and radiological features of MERS were evaluated for this study. Methods: Twelve patients were included in the study. They were treated between 2018 and 2021 years. The patients’ demographic and clinical features, laboratory data, treatment and prognoses were documented. Results: There were 7 male and 5 female patients in the series. The mean age was 37.92 ± 15.32 (19–62) years. There was history of upper respiratory tract infection in 7 patients, visual loss episodes in 3 patients, epileptic seizure in 2 patients, arthroplasty in 1 patient, cardiac arrhythmia in 1 patient, and COVID-19 (SARS-CoV-2) infection in 2 patients. Focal lesion was detected in the splenium of the corpus callosum with diffusion-weighted MRI which resolved with repeat diffusion MRI and clinical symptoms improved. Conclusions: Axial diffusion-weighted MRI images show typical splenium ovoid lesion of corpus callosum in patients with MERS. Patients with MERS have a good prognosis. It is important to consider MERS as one of the differential diagnoses in adult patients with fever and cognitive impairment.

Paracentral Acute Middle Maculopathy After COVID-19 Disease: Multimodal Evaluation.

To report the case and multimodal imaging findings of a healthy young woman who developed paracentral acute middle maculopathy (PAMM) 9 weeks after COVID-19 disease. Case report. Ultra-widefield fundus photography, macular spectral-domain optical coherence tomography (SD-OCT), fluorescein angiography (FA), and OCT-angiography (OCT-A) were performed. A 36-year-old woman who developed PAMM 9 weeks after SARS-CoV-2 infection. A 36-year-old woman went to the emergency department (ED) with sudden, painless, left eye (LE) vision loss. The only relevant past medical history was COVID-19 disease 9 weeks before. Best corrected visual acuity (BCVA) was 20/200, a LE relative afferent pupillary defect (RAPD) was present and superficial hemorrhages throughout the macular area and peripheral retina were found. Nearly four hours after admission, LE BCVA recovered to 20/20 without RAPD. Five days after presentation in the ED, the patient returned with recurrent LE vision loss, with spontaneous recovery within 12 hours. Macular SD-OCT revealed hyperreflectivity of the inner plexiform and inner nuclear layers and the diagnosis of PAMM was established. The patient started oral acetylsalicylic acid and oral prednisolone. The patient did not report any new episodes of vision loss and there was a progressive resolution of abnormal fundus findings. SARS-CoV-2 infection increases the risk of vascular thrombotic events with possible involvement of the retinal circulation, and PAMM may present as a possible complication. Ophthalmologists should be able to recognize it promptly through multimodal imaging findings.

OA06 Behçet's associated myositis: an unusual cause of exercise-induced leg pain

Abstract Introduction This case describes a 29-year-old gentleman of Cypriot heritage who presented with exercise-induced bilateral lower leg pain and swelling which made it difficult for him to cycle. Symptoms progressed over months to calf pain at rest accompanied by fatigue. He subsequently presented with central visual loss in the right eye, followed by the left. The second episode of visual loss coincided with development of oro-genital ulceration and, following rheumatology review, a diagnosis of Behçet’s disease was made. The case is significant in terms of the chronicity and nature of presenting symptoms which is not typically described in this disease. Case description A 29-year-old gentleman presented with exercise-induced bilateral lower leg pain and swelling for which he was reviewed in emergency care on four occasions. He received treatment for presumed cellulitis and underwent US-doppler studies, excluding thromboembolic disease. MRI of the lower legs was requested given lack of diagnosis with ongoing symptoms. This demonstrated bilateral muscle oedema in the antero-lateral compartments and lateral gastrocnemius heads, prompting rheumatology referral. Prior to attending rheumatology, the patient developed bilateral sequential visual loss. This was attributed to right acute macular neuroretinopathy, followed by significant left posterior pole choroidal ischaemia 2 months later. Each presentation was associated with transient low-grade anterior uveitis, vitritis, and the presence of a pre-papillary vitreous infiltrate. Initial treatment included a six-week oral prednisolone taper from 60mg daily. He reported a progressive leg pain which initially occurred during cycling but later affecting his walking. He was of Cypriot heritage, with no family history of autoimmune disease. A focused history revealed oral ulceration and an acne-like pustular rash over the back. His gait was antalgic with minimal toe-off phase. Investigations demonstrated raised ESR and CRP, negative autoimmune immunology, negative myositis ENA, normal CK, and positive HLA-B51. Given these features, a provisional diagnosis of Behçet’s was made. Nerve conduction studies were requested to consider whether changes could be secondary to denervation. They displayed non-specific S1 neurogenic changes and small tibial motor amplitudes. Subsequent MRI lumbar spine excluded radiculopathy. Repeat CK remained within normal limits. Muscle biopsy was sought and performed with a clinical suspicion of atypical myositis which has previously been reported in Behçet’s. At the second episode of visual loss, he was admitted under ophthalmology and treated with intravenous methylprednisolone and azathioprine. The case was discussed with our regional centre of excellence for Behçet’s and infliximab at 5mg/kg was commenced. Discussion This case was notable for the complexity and atypical presenting features with calf pain induced by exercise likely due to atypical myositis (biopsy report still awaited at time of submission). More classical Behçet’s disease symptoms including oro-genital ulceration were significantly later. The case highlights the importance of multidisciplinary working and involving specialist centres especially when rapid escalation to biologic treatment is required. Leg symptoms are persistent but improved despite ongoing treatment with prednisolone and azathioprine with the recent addition of infliximab. Visual symptoms have stabilised and skin involvement has settled. Management Rationale At the time of first review in rheumatology clinic, high-dose steroid treatment had already been initiated; therefore, expediting the investigative aspect of his management felt most appropriate, particularly given that myositis seemed to be the predominant feature at that time. In joint consultation with neurophysiology and neurology, putting the EMG and MRI findings in context, MRI of the lumbar spine was felt necessary to exclude discogenic disease causing denervation oedema. Unfortunately, visual symptoms progressed rapidly after this, requiring admission, which precluded an earlier opportunity for biopsy but leading to quick authorisation and organisation of treatment with infliximab. Key learning points This case highlights an atypical presentation of Behçet’s disease including associated likely atypical myositis. Causes of exercise-induced leg pain in a young, fit patient need to be considered: The need for multidisciplinary discussion both across specialties and with regional centres is key in order to progress with diagnostics and treatment In sharing this case for discussion, we wish to highlight possible causes of exercise-induced calf pain and a pathway for investigation of this. Our patient had atypical myositis as the cause for his symptoms in the context of multi-system disease. Both his myositis and ophthalmology presentations were somewhat atypical but multidisciplinary working, along with the development of some further symptoms, were helpful in making the diagnosis.

Defective complex III mitochondrial respiratory chain due to a novel variant in CYC1 gene masquerades acute demyelinating syndrome or Leber hereditary optic neuropathy

Complex III (CIII) is the third out of five mitochondrial respiratory chain complexes residing at the mitochondrial inner membrane. The assembly of 10 subunits encoded by nuclear DNA and one by mitochondrial DNA result in the functional CIII which transfers electrons from ubiquinol to cytochrome c. Deficiencies of CIII are among the least investigated mitochondrial disorders and thus clinical spectrum of patients with mutations in CIII is not well defined. We report on a 10-year-old girl born to consanguineous Iranian parents presenting with recurrent visual loss episodes and optic nerve contrast enhancement in brain imaging reminiscent of an acquired demyelination syndrome (i.e. optic neuritis or multiple sclerosis), who was ultimately confirmed to have a novel homozygous missense variant of unknown significance, c.949C > T; p.(Arg317Trp) in the CYC1 gene, a nuclear DNA subunit of complex III of the mitochondrial chain. Sanger sequencing confirmed the segregation of this variant with disease in the family. The effect of this variant on the protein structure was shown in-silico. Our findings, not only expand the clinical spectrum due to defects in CYC1 gene but also highlight that mitochondrial respiratory chain disorders could be considered as a potential differential diagnosis in children who present with unusual patterns of acquired demyelination syndromes (ADS). In addition, our results support the hypothesis that mitochondrial disorders might have an overlapping presentation with ADS.

Neuromyelitis optica – A masquerade of multiple sclerosis

We describe a case of neuromyelitis optica - a Masquerade of multiple sclerosis. Neuromyelitis optica should be differentiated from multiple sclerosis, as treatment of multiple sclerosis can worsen symptoms of neuromyelitis optica. Both visual and neurological prognosis in neuromyelitis optica are poorer than in multiple sclerosis. Episodes of visual loss in neuromyelitis optica are recurrent leading to severe visual impairment. Immunosuppressive agents such as corticosteroids remain the mainstay of therapy for acute episodes. Keywords: Aquaporin-4, Azathioprine, Multiple sclerosis, MRI, Neuromyelitis optica.

Giant Cell Arteritis in Ophthalmology Practice: a Case Report

Introduction. Giant cell (temporal) arteritis refers to a group of chronic and acute systemic vasculitis mainly affecting the extracranial and intracranial arteries of large and medium caliber. Loss of vision due to anterior ischemic optic neuropathy (AION) or occlusion of the central retinal artery is one of the most severe and most common complications of giant cell arteritis. This case report describes a patient with giant cell arteritis, the outcome of it was a total vision loss in the right eye. The patient repeatedly visited the ophthalmologists in the outpatient clinics with complaints of intermittent episodes of vision loss.Purpose. To present methods of diagnosis and treatment, through which the doctor at the initial reception will be able to suspect the disease and start treatment timely.Conclusions. The anamnestic criteria for the GCA diagnosis in the practice of ophthalmologist are: female, age over 50 years, headaches with paresthesia, intermittent lameness of the mandible, short-term episodes of vision loss. The necessary laboratory methods of research include: clinical blood test with determination of erythrocyte sedimentation rate, determination of C-reactive protein level.

Sequential bilateral retrobulbar optic neuritis in a young female

Optic neuritis (ON) is the demyelinating inflammation of the optic nerve head usually associated with multiple sclerosis. It is usually associated with sudden onset loss of vision with dyschromatopsia and retro-orbital pain. Atypical features for a demyelinating ON include the absence of pain, severe visual loss, progression of visual loss, pain for more than 2 weeks, and lack of recovery after 3 weeks. Atypical features in fundus examination include marked swelling of the nerve with retinal exudates, peripapillary hemorrhages and bilateral presentation, which may occur either simultaneously or sequentially. Atypical ON requires careful consideration and differentiation from demyelinating ON and ischemic optic neuropathy since the treatment is different. We describe a patient who presented with recurrent, sequential episode of visual loss over 2 years, without evidence of any additional neurological deficits, sarcoidosis, or systemic autoimmune disease. A diagnosis of chronic relapsing inflammatory optic neuropathy was made, and she was given intravenous steroids for 3 days, which improved the vision from hand movement close to face to 5/60 in the right eye. She was given immunosuppressants for 3 months, and oral steroids were gradually tapered. Her present vision is the right eye is 6/6 with normal intraocular pressure and color vision.

Influence of baseline demographic and clinical characteristics in the visual outcome of intermediate uveitis: a survival analysis

Background/aimsTo describe in patients diagnosed with intermediate uveitis (IU) the incidence rate (IR) of visual loss and newly diagnosed clinical complications during follow-up. Also, to analyse the influence of baseline...

Longitudinal Change of Circumpapillary Retinal Nerve Fiber Layer Thickness in Children With Optic Pathway Gliomas.

To evaluate longitudinal changes in circumpapillary retinal nerve fiber layer (RNFL) thickness, as measured by spectral-domain optical coherence tomography (SD OCT), in children with optic pathway gliomas. Longitudinal cohort study. Global and quadrant-specific circumpapillary RNFL thickness measures were acquired using either a hand-held SD OCT during sedation or a table-top SD OCT in children old enough to cooperate. Vision loss was defined as either a 0.2 logMAR decline in visual acuity or progression of visual field. Percent change in circumpapillary RNFL thickness in eyes experiencing vision loss was compared to eyes with stable vision. Fifty-five eyes completed 250 study visits. Ten eyes (18%) from 7 patients experienced a new episode of vision loss during the study and 45 eyes (82%) from 39 patients demonstrated stable vision across study visits. Percent decline of RNFL thickness between the baseline visit and first event of vision loss event was greatest in the superior (-14%) and inferior (-10%) quadrants as well as global average (-13%). Using a threshold of ≥10% decline in RNFL, the positive and negative predictive value for vision loss when 2 or more anatomic sectors were affected was 100% and 94%, respectively. Children experiencing vision loss from their optic pathway gliomas frequently demonstrate a ≥10% decline of RNFL thickness in 1 or more anatomic sectors. Global average and the inferior quadrant demonstrated the best positive and negative predictive values. Circumpapillary RNFL is a surrogate marker of vision and could be helpful in making treatment decisions for children with optic pathway gliomas.

Vision loss with bending over

A 66-year-old African American man presented with episodic transient visual loss triggered by bending forward. The initial examination did not suggest intraocular pathology and the patient was nearly sent for vascular evaluation given his cardiovascular risk factors. Fundus photographs taken during an episode of visual loss suggested an intraocular process, however. Gonioscopy revealed a microhyphema causing a “snow globe” effect in the anterior chamber, most likely related to recent bleb manipulation in the affected eye.

Clinical features of MS associated with Leber hereditary optic neuropathy mtDNA mutations

Objective:To determine whether the association between multiple sclerosis (MS) and Leber hereditary optic neuropathy (LHON) (known as “Harding disease”) is a chance finding, or the 2 disorders are mechanistically linked.Methods:We performed a United Kingdom–wide prospective cohort study of prevalent cases of MS with LHON mitochondrial DNA (mtDNA) mutations. The new cases were compared with published cases, enabling a comprehensive clinical description. We also performed a meta-analysis of studies screening patients with MS for LHON mtDNA mutations to find evidence of a genetic association.Results:Twelve new patients were identified from 11 pedigrees, and 44 cases were identified in the literature. The combined cohort had the following characteristics: multiple episodes of visual loss, predominance for women, and lengthy time interval before the fellow eye is affected (average 1.66 years), which is very atypical of LHON; conversely, most patients presented without eye pain and had a poor visual prognosis, which is unusual for optic neuritis associated with MS. The number of UK cases of LHON-MS fell well within the range predicted by the chance occurrence of MS and the mtDNA mutations known to cause LHON. There was no association between LHON mtDNA mutations and MS in a meta-analysis of the published data.Conclusions:Although the co-occurrence of MS and LHON mtDNA mutations is likely to be due to chance, the resulting disorder has a distinct phenotype, implicating a mechanistic interaction. Patients with LHON-MS have a more aggressive course, and prognostication and treatment should be guarded.

Early factors associated with axonal loss after optic neuritis

Acute optic neuritis due to an inflammatory demyelinating lesion of the optic nerve is often seen in association with multiple sclerosis. Although functional recovery usually follows the acute episode of visual loss, persistent visual deficits are common and are probably due to axonal loss. The mechanisms of axonal loss and early features that predict it are not well defined. We investigated clinical, electrophysiological, and imaging measures at presentation and after 3 months as potential markers of axonal loss following optic neuritis. We followed 21 patients after their first attack of acute unilateral optic neuritis for up to 18 months. Axonal loss was inferred from optical coherence tomography measures of retinal nerve fiber layer (RNFL) thickness at least 6 months following the episode. Visual function, visual evoked potential, and optic nerve magnetic resonance imaging measures obtained during the acute episode and 3 months later were investigated for their association with later axonal loss. After multivariate analysis, prolonged visual evoked potential latency and impaired color vision, at baseline and after 3 months, were significantly and independently associated with RNFL thinning. Low-contrast acuity measures exhibited significant univariate associations with RNFL thinning. The association of RNFL loss with a prolonged visual evoked potential (VEP) latency suggests that acute and persistent demyelination is associated with increased vulnerability of axons. VEP latency and visual function tests that capture optic nerve function, such as color and contrast, may help identify subjects with a higher risk for axonal loss who are thus more suitable for experimental neuroprotection trials.

Psychiatric Presentation in Possible Susac's Syndrome

Back to table of contents Previous article Next article LettersFull AccessPsychiatric Presentation in Possible Susac's SyndromeStephanie Assuras, M.D., and Maureen Lacy, M.D.Stephanie AssurasSearch for more papers by this author, M.D., and Maureen LacySearch for more papers by this author, M.D.Published Online:1 Jul 2011AboutSectionsPDF/EPUB ToolsAdd to favoritesDownload CitationsTrack Citations ShareShare onFacebookTwitterLinked InEmail To the Editor: Susac's syndrome is a rare neurologic microangiopathy characterized by acute encephalopathy, branch retinal artery occlusion, and sensorineural hearing loss. Case studies have reported variable clinical presentations, which have included epileptic seizures and transient inverted vision. Although advances in neuroradiology suggest that MRI may aid in differential diagnosis, the variability in the presentation of encephalopathy continues to make diagnosis challenging.Here, we present a patient with possible Susac's syndrome and a history of psychiatric symptoms who was referred for a neuropsychological evaluation after complaints of cognitive changes.Case ReportWe present a 38-year-old, right-handed, single Caucasian woman, who, at the age of 19, developed sudden onset of partial loss of sight bilaterally, which resolved without neurologic evaluation or intervention. Her episode of vision loss was immediately followed by symptoms of depression, including general malaise, sadness, and suicidal ideation. Over the following year, the patient continued to experience depressive episodes and occasional periods of grandiosity and paranoia. She was diagnosed with bipolar disorder at age 21 and successfully treated with the lowest doses of aripiprazole and sertraline. Of note, she described a family history of significant psychiatric illness, including depression and behavioral disturbances. Her psychiatric symptoms remained relatively stable until age 30, when she attempted suicide. Several months after her suicide attempt, she experienced a new onset of physiologic and neurologic symptoms, including dizziness, mild hearing loss, dysarthria, and ataxia, as well as cognitive symptoms, including difficulty with navigation, reduced concentration, and impaired reading comprehension. She was referred to a neurologist, and neuroimaging revealed white-matter changes, prominent lateral ventricles, and cerebellum volume loss. The patient's condition was ultimately characterized as a Susac-like syndrome manifesting as current spastic ataxia and previous episodes of retinal vein occlusion and vertigo.Upon neuropsychological examination at age 38, the patient reported that she continues to have motor and cognitive symptoms, although they have not progressed for the past 5 years. She also denied any recurrence of significant psychiatric symptoms since her suicide attempt at age 30. Neuropsychological assessment revealed moderate impairments in basic and complex attention, nonverbal learning, and aspects of executive functioning, with language and memory representing significant strengths of the cognitive profile. Emotionally, she endorsed minimal symptoms of depression and anxiety.DiscussionThis case summary provides an example of a woman diagnosed with possible Susac's syndrome manifested as ataxic gait, dysarthria, and vision and hearing changes, with associated cognitive decline. Although the patient's significant psychiatric family history and age at psychiatric symptom onset is consistent with a diagnosis of bipolar disorder, her psychiatric symptoms should be considered a possible manifestation of encephalopathy related to Susac's syndrome, as the exacerbation of her psychiatric symptoms occurred in conjunction with her ophthalmologic and neurologic episodes. This case highlights the complicated nature of Susac-associated encephalopathy and how proper assessment and evaluation of the onset and course of the symptom profile may assist in earlier diagnosis of Susac's syndrome. Also, differentiation of psychiatric versus neurologic symptoms is imperative to treatment outcome, because treatment for Susac's syndrome is often via corticosteroids, which may exacerbate psychiatric symptoms, and treatment of psychiatric-presenting encephalopathic symptoms with psychotropic medications may delay proper treatment of Susac's syndrome.Dept. of Psychiatry and Behavioral Neuroscience, University of Chicago Medical Center, Chicago, ILCorrespondence: [email protected]com1. Susac JO , Hardman JM , Selhorst JB . Microangiopathy of the brain and retina. Neurology 1979; 29:313–316Crossref, Medline, Google Scholar2. Marie II , Guegan-Massardier E , Levesque H , et al.: Susac's syndrome or retinocochleocerebral vasculopathy: a misdiagnosed and overlooked disorder. Eur J Intern Med 2000; 11:108–111Crossref, Medline, Google Scholar FiguresReferencesCited byDetailsCited byBipolar disorder and Susac syndrome: a case report15 July 2021 | International Clinical Psychopharmacology, Vol. 36, No. 6Long-Term Neuropsychological and Psychiatric Outcomes in Susac’s SyndromeCorey Bolton, M.A., Maureen Lacy, Ph.D.23 April 2019 | The Journal of Neuropsychiatry and Clinical Neurosciences, Vol. 31, No. 2Short Follow-up Bias Confounds Estimates of the “Typical” Clinical Course of Susac SyndromeJournal of Neuro-Ophthalmology, Vol. 37, No. 2The Clinical Neuropsychologist, Vol. 29, No. 5Nature Reviews Neurology, Vol. 9, No. 6 Volume 23Issue 3 Summer 2011Pages E30-E30 Metrics PDF download History Published online 1 July 2011 Published in print 1 July 2011

Not Again!

A 58-year-old white man presented with a second episode of vision loss in the same eye. Diagnostic evaluations conducted at each episode were unrevealing for an underlying etiology, and a presumptive diagnosis of recurrent non-arteritic anterior ischemic optic neuropathy (NAION) was made. The evaluation of recurrent NAION is discussed.

Optic neuritis presenting with amaurosis fugax

Optic neuritis usually presents with rapid and gradual loss of vision that is either complete or incomplete, and typically associated with retro-orbital pain. To our knowledge there have been no documented reports of optic neuritis presenting with multiple episodes of amaurosis fugax, the sudden and transient loss of vision lasting seconds to minutes. We report here the case of a young woman with a possible diagnosis of demyelinating left optic neuritis that presented solely with multiple episodes of brief and transient sudden loss of vision. Ophthalmological exams were normal between episodes of vision loss. The patient's magnetic resonance imaging of the brain showed a subtle enhancement of the left optic nerve along with multiple periventricular lesions, highly suggestive of a demyelinating disease. The frequent episodes of visual loss resolved completely with high dose parenteral steroids. Neurologists and other clinicians should be aware of this unusual presentation of optic neuritis as treatment modalities differ greatly from other causes of amaurosis fugax.

Intracranial hypertension in a slim young male

A 25 year old male engineering student reported to the neurological emergency department with two weeks' history of exercise-exacerbated loss of vision. At the beginning, he had noticed sudden darkening of vision when playing soccer. Episodes of vision loss had gradually increased in frequency, and now occurred even with mild physical exercise or change of posture many times per day. He did not complain of headaches at any point, and there was no double vision. Clinical examination revealed bilateral visual acuity of 0.5 c.c. Ophthalmoscopy showed marked bilateral papillary edema. Examination of this slim (BMI 17.2) patient was unremarkable otherwise. Because of moderate acne, he had been on oral tetracycline for four months.

Retinal Arteriolar Spasm During Transient Monocular Visual Loss in Eosinophilic Vasculitis

A patient with eosinophilic vasculitis and acquired immunodeficiency syndrome (AIDS) developed episodic transient monocular visual loss. During or immediately after two visual loss episodes, we demonstrated narrowed retinal arterioles, delayed arterial filling time, and segmented retinal venous flow in the affected eye on fundus photography and fluorescein angiography (FA). Such findings have only rarely been reported in patients with transient monocular visual loss in other conditions, probably because the episodes have ended before fundus photography and FA could be performed. This is the first report to capture retinal vascular changes associated with transient monocular visual loss in a patient with eosinophilic vasculitis.

Expert Opinion: Retinal Migraine: Migraine Associated With Monocular Visual Symptoms (CME)

Expert Opinion: Retinal Migraine: Migraine Associated With Monocular Visual Symptoms (CME)

Steroid Responsive Acute Recurrent Visual Loss: An Unusual Presentation Of Fibrous Dysplasia

We report a case of orbitofrontal fibrous dysplasia that presented with three episodes of acute recurrent visual loss. All the episodes of vision loss were reversed by corticosteroid treatment. Though surgical intervention is generally advocated when fibrous dysplasia is associated with visual loss, corticosteroid therapy may be tried when waiting for surgery or in patients contraindicated for surgery. This is second case reported in literature for successful treatment of fibrous dysplasia associated visual loss with steroid.

Giant arachnoid granulations

A 43-year-old woman presented with new-onset right temporal headache associated with episodes of visual loss in her right eye for 3 months. Initial MRI showed a filling defect in the left transverse sinus, and subsequent contrast-enhanced MR venography (MRV) confirmed a …