Ischemic Neuropathy Research Articles

Differential diagnosis of optic neuritis and anterior ischemic optic neuropathy

Background. Acute anterior ischemic neuropathy and optic neuritis are among the most common diseases that are the most frequent cause of both reversible and persistent visual acuity reduction. There is a sudden loss of vision, the clinical picture is often similar, and the restoration of visual functions and the prevention of complications in the form of optic nerve atrophy and disease relapses depend on a correct diagnosis and timely prescribed therapy. A significant level of medico-social and economic losses for society in all regions of the world is related to complications, namely the occurrence of optic nerve atrophy due to both optic nerve inflammation (neuritis) and ischemic optic neuropathy. The purpose was to determine the differential signs of optic neuritis and anterior ischemic optic neuropathy. Materials and methods. Eighty-five patients were examined, 52 (61.2 %) women and 33 (38.8 %) men. Seventy-one (83.5 %) participants were employed. The patients were divided into two groups: the first one — 38 patients with optic neuritis (papillitis); the second one — 47 people with ischemic optic neuropathy. General ophthalmological examinations were carried out. Results. Computed perimetry showed the presence of scotoma and quadrantanopia, both in anterior optic neuropathy (typical altitudinal scotomas) and optic neuritis (peripheral and central scotomas). The difference in the indicators of the electrical activity of the visual analyzer according to phosphene between the groups was 15.8 % (p < 0.05), which indicates a more significant increase in the functional activity of the visual analyzer in the group with anterior optic vascular neuropathy. Thinning of the peripapillar nerve fiber layer and partial atrophy of the optic nerve were noted 2 times less often in patients with anterior vascular ischemic neuropathy — 7 (16.3 %) vs. 14 (33.3 %) in those with partial atrophy of the optic nerve (Pearson’s χ2 = 22.59, p = 0.00001). Conclusions. The issue of early differential diagnosis of anterior optic ischemic neuropathy and optic neuritis is relevant, which determines the prognosis of the disease, allows increasing the effectiveness of treatment and preventing irreversible consequences. The therapeutic effect of treating the consequences of damage to the optic nerve is not stable and is weak, and success is not always achieved. First of all, the treatment of the underlying disease is indicated. Computed perimetry showed the presence of scotoma and quadrantanopia in both anterior optic neuropathy (typical altitudinal scotomas) and optic neuritis (peripheral and central scotomas).

Differences in ocular adverse events associated with phosphodiesterase-5 inhibitors: a real-world pharmacovigilance study

ABSTRACT Objective Our study aims to characterize the ocular safety profiles of phosphodiesterase type 5 (PDE5) inhibitors and explore the differences among different PDE5 inhibitors. Methods We analyzed reports on ocular adverse events associated with sildenafil, vardenafil and tadalafil submitted to the FDA Adverse Event Reporting System (FAERS) database from the first quarter of 2004 to the first quarter of 2023. Disproportionality analysis was conducted to evaluate reporting risk profiles. Results Among 61,211 reports qualifying for analysis, 5,127 involved sildenafil, 832 vardenafil, and 3,733 tadalafil. All PDE5 inhibitors showed increased reporting odds ratios (ROR) for ocular adverse events, with vardenafil highest (ROR 4.47) followed by sildenafil and tadalafil. Key ocular adverse events included cyanopsia, optic ischemic neuropathy, visual field defects, unilateral blindness and blindness. Sildenafil showed the highest disproportionality for cyanopsia (ROR 1148.11) while vardenafil and tadalafil showed the highest disproportionality for optic ischemic neuropathy. Time-to-onset analysis also revealed significant differences, with sildenafil having a later median time-to-onset compared to vardenafil and tadalafil. Conclusions This comprehensive pharmacovigilance study reveals distinct patterns of ocular adverse events associated with PDE5 inhibitors. These findings contribute to a better understanding of the safety profiles of PDE5 inhibitors and may guide healthcare professionals in clinical decision-making.

Micro- and Macrovascular Complications of Type 2 Diabetes in Southern Iran

Objectives: Type 2 diabetes mellitus (T2DM) is one of the most common diseases worldwide, imposing a great burden on the community by its complications. This study evaluates micro-and macrovascular complications of T2DM and the associated factors in the south of Iran. Methods: This cross-sectional study was conducted on T2DM patients referred to the Diabetes Clinic of Shahid Mohammadi Hospital in Bandar Abbas City, Iran, in 2018. Patients’ age, sex, marital status, smoking status, diabetes medications, cardiovascular disease, and duration of T2DM were recorded. Weight, height, and blood pressure were measured and body mass index was calculated for every participant. Also, all patients were evaluated regarding diabetes complications, including retinopathy, nephropathy, and neuropathy. Serum fasting plasma glucose (FPG), lipid profile, glycated hemoglobin, creatinine levels as well as urinary albumin were also measured in the patients. Results: Of the 263 T2DM patients included in the current study with a mean age of 55.32±13.09 years, 109 (41.4%) were male. The most common T2DM complications were chronic kidney disease (CKD) (49.8%) and nephropathy (36.5%), followed by neuropathy (30.8%), retinopathy (19%) and ischemic heart disease (8.7%). Elevated systolic blood pressure was significantly correlated with the presence of all complications. Patients with ischemic heart disease, CKD, neuropathy, and retinopathy were significantly older than subjects without such complications. Diabetes was significantly more prolonged in patients with ischemic heart disease and CKD. Elevated FPG was only correlated with CKD (P=0.019) and nephropathy (P=0.003), while increased glycated hemoglobin and decreased high-density lipoprotein were significantly associated with retinopathy (P=0.050 and P=0.001, respectively). Moreover, a significant relationship was observed between elevated urinary albumin with retinopathy and neuropathy. Discussion: Diabetic kidney disease was the most common complication of T2DM in the current study. Increased systolic blood pressure is the most important risk factor for the development of diabetic complications.

Frequency and characteristics of severe relapse in giant cell arteritis.

To assess the frequency and characteristics of severe relapse in patients with giant cell arteritis (GCA) in a real-life setting. In a monocentric database of 530 patients, we retrospectively analysed patients who experienced at least one relapse and distinguished severe from nonsevere relapses. Severe relapse was defined by the occurrence of an ischaemic event (ophthalmologic, neurologic, digestive, limb ischaemia), the occurrence of an aortic complication (i.e. new or worsening of aortic dilation, aortic dissection), or new or worsening of vascular stenosis. From the cohort of 530 patients, 242 (45.7%) patients experienced relapse at least once, including 13 (2.5% of the cohort) who experienced severe relapse. Among the 464 recorded relapses, 14 (3% of all relapses) were severe. Severe relapse corresponded to the following vascular events: a peripheral limb ischaemia in 6 patients, a visual event in 3 patients (including 2 acute anterior ischaemic anterior neuropathies), an aortic complication in 3 patients, a mesenteric ischaemia in one patient and an ischaemic stroke in one patient.When compared with the 229 patients who experienced nonsevere relapses, severe relapse patients were younger at diagnosis (p= 0.02), more frequently showed limb claudication at baseline (p< 0.0001) and fewer GCA-related cranial signs (p< 0.0001). At diagnosis, more large-vessel vasculitis on imaging (82% vs 36%, p= 0.002) were observed in patients with severe relapse. The death rate did not differ between patients with severe and nonsevere relapses. In a real-life setting, relapse affects nearly half of GCA patients, but severe relapse is rare.

Association between Bell's Palsy and Cardiometabolic Risks: An Age- and Sex-Matched Case-Control Study.

Bell's palsy is possibly an ischemic cranial neuropathy, although reactivation of herpes virus infection has been proposed. This was an age-and sex-matched and 1:2 case-control study enrolling Bell's palsy patients during 2011-2021 in a university hospital to investigate the significant associations of cardiometabolic risks (CMRs) with Bell's palsy. We analyzed the differences in waist circumference (WC), body mass index (BMI), systolic and diastolic blood pressures (SBP and DBP), fasting blood sugar (FBS), and lipid levels at 12 weeks post-Bell's palsy with those of the controls by descriptive statistics (p < 0.05). The differences in means or medians of individual CMR values across the consecutive 10-year age intervals were analyzed by ANOVA F-tests and Kruskal-Wallis tests (p < 0.05). A total of 140 cases and 280 controls were enrolled. Bell's palsy patients had significantly higher WC, BMI, SBP, DBP, FBS, and triglyceride but lower high-density lipoprotein cholesterol (HDL-C) and low-density lipoprotein cholesterol (LDL-C). Moreover, high WC, elevated FBS (≥100 mg/dL), SBP (≥130 mmHg), and total cholesterol were significantly associated with Bell's palsy cases by multivariable analysis. Only FBS in Bell's palsy patients significantly elevated across consecutive 10-year age intervals. Screening and monitoring for CMRs, especially hyperglycemia, in every patient presenting with Bell's palsy is essential despite initial normoglycemia, particularly in older-onset cases.

POST-COVID SYNDROME. OPTIC NERVE LESIONS (Literature Review)

Coronavirus infection COVID19 is an acute respiratory viral disease, which in most cases is severe and leads to the development of various complications known as post-COVID syndrome. Post-covid syndrome or long-covid syndrome is a consequence of coronavirus infection (COVID-19), while 20% of those who have been ill suffer from long-term symptoms, the duration of which varies greatly and is more than 12 weeks. According to the WHO, the post-covid syndrome includes 33 symptoms, while in younger people who have been ill, the incidence of this symptom complex is much higher. According to the literature, post-COVID syndrome may reappear 3-6 months after clinical recovery. This condition is characterized by various non-specific symptoms, which can be manifested by dysfunction of individual organs, skin vasculitis, neurological symptoms up to the development of mental disorders. Summing up, it should be noted that during the pandemic period, certain experience has been accumulated regarding ophthalmic problems. The data obtained are sometimes contradictory, but it is already clear that ophthalmic disorders in COVID-19 can be leading along with damage to other organs. Perhaps a new disease leads to an exacerbation of an existing or manifestation of dormant ophthalmopathology. Regardless of what is the cause, it is necessary to remain vigilant about the state of the organ of vision both in the immediate and long-term period after a new coronavirus infection. KEYWORDS. Postcovid syndrome, optic neuritis, optic vasculitis, anterior ischemic neuropathy

Synergistic Protection of Retinal Ganglion Cells (RGCs) by SARM1 Inactivation with CNTF in a Rodent Model of Nonarteritic Anterior Ischemic Optic Neuropathy.

We evaluated whether inhibiting sterile alpha and (Toll/interleukin receptor (TIR)) motif-containing 1 (SARM1) activity protects retinal ganglion cells (RGCs) following ischemic axonopathy (rodent nonarteritic anterior ischemic optic neuropathy: rNAION) by itself and combined with ciliary neurotrophic factor (CNTF). Genetically modified SARM1(-) rats were rNAION-induced in one eye and compared against equivalently induced wild-type animals of the same background. Optic nerve (ON) diameters were quantified using optical coherence tomography (SD-OCT). RGCs were quantified 30 d post-induction using retinal stereology for Brn3a(+) nuclei. ON sections were analyzed by TEM and immunohistochemistry. SARM1(-)(-) and WT animals were then bilaterally sequentially rNAION-induced. One eye received intravitreal vehicle injection following induction; the contralateral side received CNTF and was analyzed 30 d post-induction. Inhibiting SARM1 activity suppressed axonal collapse following ischemic axonopathy. SARM1(-) animals significantly reduced RGC loss, compared with WT animals (49.4 ± 6.8% RGC loss in SARM1(-) vs. 63.6 ± 3.2% sem RGC loss in WT; Mann-Whitney one-tailed U-test, (p = 0.049)). IVT-CNTF treatment vs. IVT-vehicle in SARM1(-) animals further reduced RGC loss by 24% at 30 d post-induction, but CNTF did not, by itself, improve long-term RGC survival in WT animals compared with vehicle (Mann-Whitney one-tailed t-test; p = 0.033). While inhibiting SARM1 activity is itself neuroprotective, combining SARM1 inhibition and CNTF treatment generated a long-term, synergistic neuroprotective effect in ischemic neuropathy. Combinatorial treatments for NAION utilizing independent neuroprotective mechanisms may thus provide a greater effect than individual treatment modalities.

Histopathological Study of Peripheral Neuropathies on Nerve Biopsy: A Cross-sectional Study

Introduction: Peripheral neuropathy is common in clinical practice, with a reported prevalence of 2.4% in the general population. There are numerous aetiologies for peripheral neuropathy like diabetes, ischaemia, vasculitis, inflammatory demyelinating disorders, nutritional deficiencies, paraproteinemic disorders, paraneoplastic syndromes, toxic exposures, and hereditary neuropathies. An exhaustive haematological, biochemical, and serological work-up, cerebrospinal fluid evaluation, electrodiagnostic tests, and nerve biopsy are required when overlapping clinical features present a diagnostic challenge. Aim: To analyse the histopathological characteristics of nerve biopsies in individuals with peripheral neuropathy. Materials and methods: This cross-sectional study was conducted in the Department of Pathology at Kasturba Medical College from January 2011 to December 2016. Nerve biopsies received in the Department of Pathology, Kasturba Medical College and Hospitals in the Ambedkar Circle and Attavar area, as well as at Government Wenlock Hospital, Mangaluru, Karnataka, India were studied. Clinical and laboratory data were collected from biopsy requisition forms and patient case records from the aforementioned hospitals. Results were presented in numbers and percentages. Results: A total of 134 nerve biopsies were included in the study. The age range of all cases studied was 7-86 years, with a mean age of 51.8 years. The study population consisted of 63.4% males and 36.6% females, resulting in a male-to- female ratio of 1.7:1. Vasculitic Neuropathy (VN) accounted for 38.1% cases, followed by chronic inflammatory neuropathies (21.7%) and Diabetic Neuropathy (DN) (12.6%). Other diagnosis included ischaemic neuropathy (6.7%), Hereditary Motor and Sensory Neuropathy (HMSN) (3.7%), subacute inflammatory demyelinating neuropathy (3.0%), as well as two cases each of Hansen’s neuritis, amyloid neuropathy, and acute inflammatory demyelinating neuropathy (1.5% each). One case of toxic neuropathy (0.7%) was identified, while 9.0% of cases displayed histological features that were either non specific or not characteristic of any specific diagnosis. Conclusion: Vasculitic neuropathy was found to be the most common aetiology of peripheral neuropathy, followed by Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) and DN. The histopathological examination of nerve biopsies is a useful tool in distinguishing between diseases with overlapping clinical features, confirming or excluding vasculitis, and diagnosing hereditary conditions in settings where genetic testing is unavailable or unaffordable.

A RARE CAUSE OF TRIGEMINAL THROPHIC SYNDROME: ISCHEMIC CRANIAL NEUROPATHY

Trigeminal trophic syndrome (TTS) is a rare disease characterized by trigeminal anesthesia, facial paresthesia, and ulcerated lesions on the face as a result of damage to the trigeminal nerve due to different causes. Ulceration is most commonly seen as a single lesion on the nasal wing. Multiple ulcerated lesions are rarely encountered in the regions at trigeminal nerve dermatome. Here, a case of TTS presenting with multiple facial ulcerations after ischemic cranial neuropathy is reported.

Multiparametric ultrasound for upper extremity dialysis access evaluation.

The evolution of ultrasound (US) techniques has greatly improved the evaluation of many parameters in dialysis vascular access, which is typically achieved through an arteriovenous fistula (AVF) or graft (AVG). These techniques include grayscale B-mode, color Doppler, power Doppler, spectral Doppler, non-Doppler US flow imaging techniques, contrast-enhanced US, and elastography. In conjunction with a patient's medical history and physical examination, US provides crucial information about the native vascular bed prior to the surgical creation of an arteriovenous anastomosis. It also tracks the maturation progress of the newly created AVF or AVG and aids in diagnosing potential complications of the vascular access. These complications include thrombosis, steal syndrome, aneurysms, pseudoaneurysms, hematomas, infection, ischemic neuropathy, exacerbation of preexisting congestive heart failure, and stenosis.

A Case of Ischemic Monomelic Neuropathy following Endovascular Arteriovenous Fistula Creation and Assisted Maturation.

Dialysis access steal syndrome (DASS) is a phenomenon known to occur following creation of an arteriovenous fistula or arteriovenous graft. The clinical presentation of DASS is characterized by symptoms of limb ischemia due to diversion of arterial flow from the distal extremity. Ischemic monomelic neuropathy (IMN) is a rare subtype of DASS classically described as an acute, isolated presentation of peripheral neuropathy following dialysis access creation. Although the underlying pathophysiology is not entirely understood, the clinical manifestation of IMN is often described as severe distal limb pain that progresses to motor and sensory defects. The onset of IMN may occur immediately following dialysis access creation or intervention. Here, we present a case of IMN following assisted maturation of an endovascular fistula.

Оценка влияния хирургической реконструкции внутренней сонной артерии на гемодинамику глаза и морфофункциональные показатели зрительного анализатора у пациентов с хронической ишемической нейрооптикопатией, ассоциированной с гемодинамически значимым атеросклеротическим стенозом внутренней сонной артерии

Purpose. To evaluate the effect of carotid endarterectomy (CEA) on eye hemodynamics and morphofunctional parameters of the visual analyzer in patients with chronic ischemic neuropathy (CIN) associated with hemodynamically significant atherosclerotic stenosis of the internal carotid artery (ICA). Material and methods. The study included 50 patients (50 eyes) with unilateral manifestations of CIN. All patients underwent optical coherence tomography (OCT), Doppler ultrasound of the ophthalmic artery (OA), short posterior ciliary arteries (SPCA), central retinal artery (CRA) before and after surgical reconstruction of the ICA. Results. 24 months after CEA of the ICA, in all cases there were significant improvements in blood flow in the OA by 52.8 %, in the SPCA by 26 %, in the CRA by 30.2 %, a decrease in the thickness of the peripapillary layer of retinal nerve fibers by 9.5 % and the layer of ganglionic cells by 8 % relative to baseline (p &lt; 0.05). Conclusion. The causes of CIN progression after ICA CEA can be considered as the consequences of induced reperfusion of the optic nerve structures and the brain. Keywords: chronic ischemic neuroopticopathy, ocular ischemic syndrome, internal carotid artery, carotid endarterectomy

Биомаркеры ишемии органа зрения как предикторы ишемического инсульта головного мозга

Purpose. To study biomarkers of ischemia of the organ of vision in patients with acute disorders of the arterial circulation of the retina and optic nerve. Material and methods. We observed 38 patients (38 eyes). The patients were divided into two groups, the first group consisted of 26 patients (26 eyes) with acute circulatory disorders of the optic nerve (anterior ischemic neuropathy), the second group consisted of 12 patients (12 eyes) with acute circulatory disorders of the retina (occlusion of the central artery retina and its branches). The control group consisted of 12 practically healthy individuals. Results. In a biochemical study in patients with anterior ischemic neuropathy (1st group), the level of NSE in the lacrimal fluid was increased compared to the control group and averaged 32.3 ± 0.85 ng/ml, although in the blood serum it remained within the normal range , which reflects the beginning of destructive processes in the nervous tissue. In patients with acute circulatory disorders of the retina (2nd group), the content of NSE in the lacrimal fluid was increased by 1.7 times compared with the control group. Conclusion. The NSE study can be used in a comprehensive examination of patients with acute circulatory disorders of the optic disc and retina as an additional marker of the neurodegenerative process. Since data from traditional methods of research do not always show the degree of damage to the visual pathways, the study of the level of NSE helps to make assumptions about the severity of the disease and predict the outcome. Keywords: visual organ ischemia, occlusion, anterior ischemic neuropathy, central retinal artery, diagnostics, NSE protein

No Association Between Non-arteritic Anterior Ischemic Optic Neuropathy and COVID-19 Vaccination (P2-9.002)

<h3>Objective:</h3> To investigate the association between Non-arteritic anterior ischemic optic neuropathy (NAION) and COVID-19 vaccination. <h3>Background:</h3> NAION has been reported after COVID-19 vaccinations. <h3>Design/Methods:</h3> Reports filed under “optic ischaemic neuropathy” were assessed on the likelihood of being NAION or of unspecified etiology. NAION diagnosis was determined by adverse event description analysis. The reporting rate of NAION cases after COVID-19 vaccination was compared to the reporting rate of NAION after influenza vaccinations in two time periods: the COVID-19 vaccine period (December 2020–July 2021) and the pre-COVID-19 period (February 2019–September 2019). Self-controlled case series and case-centered analyses were used. Six weeks after vaccination was defined as the risk period of probable association. <h3>Results:</h3> 43 cases with “optic ischaemic neuropathy” after COVID-19 vaccination and 0 cases after any other vaccinations were reported during COVID-19 vaccination period. 18 of these cases were likely to be NAION. 25 of these cases were assessed and the diagnostic certainty of NAION was unclear or of other etiology. 3 cases were reported in the pre-COVID-19 period after the zoster, influenza, and hepatitis A vaccines. Excluding unclear cases, the reporting rate of NAION cases after COVID-19 vaccination is not significantly different compared to after other vaccinations in comparable time periods (1.64 vs 1.57 per 10 million vaccination p= 0.942. However, this is within the incidence range reported in the general population. Using self-controlled and case centered analyses, there is a significant difference in the reporting rate of NAION after COVID-19 vaccination between the risk and control period (80% vs 6%, p&lt; 0.0001). <h3>Conclusions:</h3> There is no association between NAION and COVID-19 vaccination and the reporting rate of NAION after COVID-19 vaccinations and other vaccinations is comparable. Although, NAION was reported significantly higher in the risk period compared to the control period, it is within the expected incidence range. <b>Disclosure:</b> Dr. Bellacicco has nothing to disclose. Mr. Jaffry has nothing to disclose. Mr. Benalcazar has nothing to disclose. Mr. Mandava has nothing to disclose. Mr. Mostafa has nothing to disclose. Dr. Khan has nothing to disclose. Dr. Souayah has received publishing royalties from a publication relating to health care.

When Two is More Than Too Much: Carotid Stenosis and Giant Cell Arteritis (P1-9.002)

<h3>Objective:</h3> NA <h3>Background:</h3> NA <h3>Design/Methods:</h3> Case Report <h3>Results:</h3> A 72-year-old male was seen at the neuro-ophthalmology clinic for a right oculomotor palsy and right central retinal artery occlusion (CRAO) occurring within three days of each other. Stroke work up at an outside institution revealed the significant finding of a severe, right internal carotid stenosis for which a carotid endarterectomy was planned. The patient however developed a right CRAO before the endarterectomy could be performed. Though the patient reported experiencing headaches, unintentional weight loss, and jaw claudication as well as having elevated sedimentation rate, C-reactive protein, and platelet count at the time of initial presentation of the oculomotor palsy, the diagnosis of giant cell arteritis (GCA) was dismissed in favor of the carotid stenosis as the more likely etiology for the patient’s presentation. It was not until a couple of months after the patient’s initial diagnoses of right oculomotor palsy and CRAO that the diagnosis of GCA was made and appropriate treatment with steroids started. <h3>Conclusions:</h3> Though carotid stenosis has been reported as a cause of ischemic oculomotor palsies, this is much less common compared to GCA which has been known to cause diplopia from other extraocular cranial motor neuropathies as well as visual loss from arteritic ischemic optic neuropathy and CRAO. Though headaches and periocular pain could accompany ischemic extraocular motor neuropathies unrelated to GCA, clinicians should be more astute in recognizing other symptoms like jaw and tongue claudication, unintentional weight loss, anorexia, etc. that might indicate an alternative etiology–in this case, GCA–other than what is readily obvious, e.g., carotid stenosis. With both GCA and carotid stenosis having the potential to cause serious permanent disability, it is imperative to acknowledge the possibility of their coexistence and address each in the most appropriate and timely manner. <b>Disclosure:</b> An immediate family member of Dr. Santos has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Horizon Therapeutics. An immediate family member of Dr. Santos has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Cochrane. Dr. Santos has stock in Gilead Sciences. Dr. Santos has stock in Novocure. Dr. Santos has stock in Shockwave Medical. Dr. Santos has stock in Teva. An immediate family member of Dr. Santos has received research support from American Academy of Neurology. The institution of an immediate family member of Dr. Santos has received research support from Trinity Health Saint Mary’s Foundation. An immediate family member of Dr. Santos has a non-compensated relationship as a Delegate with AAN Neurology on the Hill that is relevant to AAN interests or activities. An immediate family member of Dr. Santos has a non-compensated relationship as a Participant with AAN Palatucci Advocacy Leadership Forum that is relevant to AAN interests or activities. An immediate family member of Dr. Santos has a non-compensated relationship as a Scholar with AAN Health Equity Symposium Scholarship that is relevant to AAN interests or activities. An immediate family member of Dr. Santos has a non-compensated relationship as a Participant with AAN Emerging Leaders Program that is relevant to AAN interests or activities.

Clinical Images: Optic perineuritis in relapsing granulomatosis with polyangiitis.

The patient, a 76-year-old man who was previously diagnosed as having granulomatosis with polyangiitis (GPA) and was receiving rituximab maintenance therapy every 6 months, presented with malaise, night sweat, transient lockjaw, and right-sided facial pain and swelling following dental extraction 2 weeks earlier. He subsequently developed retrobulbar pain, conjunctival injection, chemosis, and fluctuating visual impairment. T1-weighted contrast magnetic resonance imaging (MRI) of the orbit revealed contrast enhancement of the right optic nerve sheath sparing the optic nerve itself (“doughnut sign”) consistent with optic perineuritis (coronal image in A; axial image in B). Laboratory evaluation showed elevated C-reactive protein (276 mg/liter) and leukocytosis (13,430/μl) (Supplementary Table 1, http://onlinelibrary.wiley.com/doi/10.1002/art.42426). Blood cultures and cerebrospinal fluid analysis including mycobacterial and fungal polymerase chain reaction showed no evidence of infection. Moreover, empirical antibiotic therapy did not result in clinical improvement. In GPA, optic neuropathy accompanied by retrobulbar pain and/or oculomotor nerve palsy usually occurs as a result of compression by inflammatory orbital masses (1, 2). However, it may rarely be encountered due to posterior ischemic neuropathy, optic neuritis, and/or perineuritis without inflammatory pseudotumor (1). Imaging may reveal signs of pachymeningitis, often subtle, and additional orbital apex infiltration (1-3). In some patients, high levels of inflammation markers can be detected (1, 2). Optic perineuritis has a poor prognosis with frequent recurrence of symptoms following reduction of glucocorticoid dose, thus requiring aggressive immunosuppressive treatment with cyclophosphamide and/or rituximab (1, 2). In our patient, immunosuppressive therapy was switched to combined rituximab and pulse cyclophosphamide treatment with the addition of a glucocorticoid for the re-induction of remission, resulting in protracted resolution of clinical symptoms and MRI findings (Supplementary Figure 1, http://onlinelibrary.wiley.com/doi/10.1002/art.42426). Disclosure Form Supplementary Figure 1: MRI follow-up showing resolution of contrast enhancement of the right optic nerve sheath following re-induction of remission with combined rituximab and pulse cyclophosphamide treatment and addition of glucocorticoid (A coronal image, B axial image). Supplementary Table 1: Laboratory values Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.

Successful Management of a Spontaneous Retrobulbar Hematoma After Heart Transplantation. A Case Report

We present a case of a 65-year-old patient who underwent heart transplantation. After the surgery, left proptosis, conjunctival chemosis, and ipsilateral palpebral ecchymosis were found while he was still intubated. A retrobulbar hematoma was suspected, confirmed by a computed tomography scan. Initially, expectant management was considered, but with the appearance of an afferent pupillary defect, the patient underwent orbital decompression and posterior collection drainage, which prevented visual impairment. Spontaneous retrobulbar hematoma after heart transplantation is a rare condition that risks vision. We intend to discuss the importance of postoperative ophthalmologic examination after heart transplantation in intubated patients for early diagnosis and rapid treatment. Spontaneous retrobulbar hematoma (SRH) after heart transplantation is an exceptional condition that risks vision. Bleeding in the retrobulbar space provokes an anterior ocular displacement, extending the vessels and the optic nerve, which can generate ischemic neuropathy and, finally, a loss of vision [1]. A retrobulbar hematoma is usually associated with trauma or eye surgery. Though, in non-traumatic cases, the underlying cause is not evident. An adequate ophthalmologic examination is usually not performed in complex surgeries like heart transplantation. However, this simple measure can prevent permanent vision loss. Non-traumatic risk factors should also be considered, which include vascular malformations, bleeding disorders, use of anticoagulants, and increased central venous pressure usually triggered by a Valsalva maneuver [2]. The clinical presentation of SRH consists of ocular pain, decreased visual acuity, conjunctival chemosis, proptosis, abnormal extraocular movements, and elevated intraocular pressure (IOP). Its diagnosis is often clinical; however, it can be confirmed with computed tomography or magnetic resonance imaging. Treatment aims to reduce IOP with surgical decompression or pharmacologic measures [2]. In the reviewed literature, less than 5 spontaneous ocular hemorrhages related to cardiac surgery have been reported [3-6], of which only one is related to heart transplantation [3]. A clinical challenge of an SRH after heart transplantation is presented below. Surgical management was performed with a favorable result.

Systemic lupus erythematosus with acute ischemic optic neuropathy complicated with neuromyelitis optica: a case report

BackgroundNeuromyelitis optica is a relapsing–remitting disease characterized by a recurrent attack of optic neuritis and transverse myelitis; sometimes associated with acute brainstem syndrome. Systemic lupus erythematosus is an autoimmune multisystem disorder in which ocular involvement such as acute ischemic optic neuropathy is a rare manifestation. However, neuromyelitis optica can be associated with systemic lupus erythematosus.Case presentationA 24-year-old Bangladeshi woman was admitted to the hospital with complaints of sudden, progressive, painless vision loss in both eyes, and progressive weakness in both lower limbs for 48 hours. She also gave a history of arthralgia, a photosensitive skin rash, intermittent fever, oral ulcerations, and alopecia for the last 2 months. On examination, the fundus was suggestive of bilateral acute ischemic neuropathy, and examinations of the lower limb revealed spastic paraparesis with sensory abnormality. Laboratory investigations revealed the presence of positive anti-aquaporin 4 antibody, strongly positive antinuclear antibody, and anti-ds DNA with the longitudinally extensive lesion on magnetic resonance imaging of the spinal cord. She was treated with methylprednisolone, hydroxychloroquine, and mycophenolate, and was discharged with improvement of her paraparesis. However, her vision did not improve substantially.ConclusionThe importance of this report is to shed some light on the occurrence of two devastating complications that is, bilateral acute ischemic optic neuropathy in systemic lupus erythematosus complicated by neuromyelitis optica, as well as evidence of rare presentations for systemic lupus erythematosus and treatment modalities of ischemic optic neuropathy with systemic lupus erythematosus.

Ішемічні порушення кінцівки при АВФ для хронічного гемодіалізу

Ischemic steal syndrome (ISS) develops from 2 to 20% of cases in patients on chronic hemodialysis with arteriovenous fistula (AVF) or arteriovenous prosthesis (AVP). Moreover, there is a different frequency of ISS development in patients with distal (radiocephalic) AVF and proximal AVF. For radiocephalic fistulas, this index is about 2%. With proximal fistulas, ISO can reach up to 20-25%. The purpose of the study: To conduct an analysis of the clinical course of ischemic syndrome distal to permanent vascular access for people suffering from Stage 5 of Chronic kidney disease with efferent renal replacement therapy. Research materials and methods. The terms of the development of ischemic disorders after the formation of permanent vascular access (PVA) in patients with AVF are from an hour to a week ("acute"), but can appear after a month or more ("chronic") and even after several years. Despite the fact that the severity of "chronic" ischemia is usually moderate, long-term complaints of hypoperfusion can lead to trophic disorders, up to necrosis and gangrene (about 1% of patients). It is also noted that up to 25% of patients have temporary ischemic complications after the formation of AVF and AVP. There is currently no generally accepted ISS classification. In the foreign literature, there are several attempts by different researchers to develop a classification that would be of practical importance for the diagnosis and treatment of ISS. Research results and their discussion. Most of the authors base their practice primarily on the clinical diagnosis of ISS. Instrumental diagnostics has a clarifying character. Taking an anamnesis can reveal risk factors for the development of ISS. To identify complaints, some authors proposed a questionnaire based on the visual analog scale (VAS), in which points are obtained by multiplying the subjective severity of symptoms (from 0 to 10) by their frequency of occurrence (0 – never, 10 – always). A decrease in tactile and temperature sensitivity may be detected during a neurological examination. One of the most severe variants of acute ischemic syndrome is the development of so-called unilateral ischemic neuropathy. Trophic disorders occurring on the limbs containing arteriovenous access (AVA) can also be the result of venous hypertension, which is accompanied by edema. NKF/DOQI 2006 recommendations require the immediate elimination of access when an UIN is detected. However, even in cases where this complication is detected in the shortest possible time, and the fistula is ligated, full regression of symptoms does not occur in all patients. Delay in surgical tactics significantly worsens the prognosis. Conclusions. The most serious complaint that complicates full-fledged hemodialysis is pain due to ischemia of all three nerves of the forearm, which appears or worsens during the replacement therapy procedure. Weakening of the pulse distal to the AVF is characteristic of most patients, both with proximal and distal AVF.

Optic Neuropathy Induced by Sildenafil: When Blinded by Pleasure: Case Report and Literature Review)

Sildenafil citrate is phosphodiesterase 5 inhibitor (PDE5i) that is widely used to treat male erectile dysfunction. However, this molecule has been associated with many visual side effects which most are transient but some can lead to persistent vision loss. Non arteritic anterior ischemic neuropathy (NAION) has been reported in some cases as a very rare, but possible, Sildenafil citrate’s visual adverse effect. We present the case of young patient under erectile dysfunction medication who developed an induced toxic neuropathy, while reviewing the other reported cases in medical literature.