Behcet syndrome is a systemic vasculitis characterized by relapsing uveitis, oral aphthous, and genital ulcers. We present a rare case of a 14-year-old male with juvenile Behcet syndrome (JBS) presenting as bilateral optic neuritis with oral aphthous. Initial treatment with methylprednisolone did not improve the patient's condition. Serum plasmapheresis was performed, resulting in improved visual acuity and papillitis. The patient was discharged with azathioprine, which led to symptom regression. This case highlights the atypical neurologic presentation of JBS and the potential efficacy of plasma exchange in refractory cases.

OBJECTIVES:It is estimated that up to 65 % of pwMS (people with multiple sclerosis) experience varying degrees of cognitive impairment, the most commonly affected domain being Information Processing Speed (IPS). As sleep disturbance is a predictor of detriments in IPS, the authors aimed to study the association between the severity of Restless Legs Syndrome (RLS) and Obstructive Sleep Apnea (OSA) symptoms with IPS in pwMS. METHODS:In a cross-sectional study, the authors enrolled people with relapsing-remitting and secondary progressive MS referred to the comprehensive MS center of Kashani Hospital in Isfahan, Iran. The authors used Berlin and STOP-Bang questionnaires for assessing OSA symptoms, and the International Restless Legs Syndrome Study Group (IRLSSG) scale for the presence and severity of symptoms of RLS. The authors used the Integrated Cognitive Assessment (ICA) test, a language and education-independent tool, to assess visual processing speed. RESULTS:The authors included 211 pwMS, with a mean age of 36.73 ± 8.9 (81.9 % female). PwMS with higher RLS scores showed lower IPS, with ICA indexes of 0.66 ± 0.09 vs. 0.61 ± 0.12 in low- and high-risk RLS groups, respectively (p < 0.01). There were no significant associations between IPS as measured by the ICA index and OSA symptom severity. CONCLUSION:The authors found impairments in IPS in pwMS to be linked with the severity of RLS symptoms, but not with OSA. Considering the high prevalence and underdiagnosis of RLS in pwMS, and the profound impact of IPS on quality of life, this association highlights the importance of screening and treating RLS in this population.

Background Appropriate treatment reduces the severity and duration of relapses in demyelinating diseases of Central Nervous System (CNS). If high-dose corticosteroids treatment fails, therapeutic plasma exchange (TPE) is considered as a rescue treatment. Objectives This study aimed to investigate early clinical response and complications of TPE and prognostic factors in CNS demyelinating relapses. Design This prospective observational study was designed in a tertiary center during one year. Methods All adult patients diagnosed corticosteroid-resistant Multiple Sclerosis (MS), NeuroMyelitis Optica Spectrum Disorder (NMOSD), idiotypic Transverse Myelitis or Clinical Isolated Syndrome relapses, were eligible. Clinical response is defined based on Expanded Disability Status Scale (EDSS) at discharge. Clinical and laboratory complications recorded. Results Seventy-two patients were analyzed which 58.3% patients were female. MS was diagnosed for 61.1% of cases. Thirty-five patients (48.6%) responded and the mean differences of EDSS significantly decreased 0.60 score (CI95%:0.44-.77). Electrolyte imbalances and thrombocytopenia occurred in 80.6% and 55.6% of cases respectively and 40.3% of patients had systemic reactions. However, 26.4% patients experienced moderate to severe complications. In patients with moderate to severe disability, responders were younger (MD: 8.42 years, CI95%: 1.67-15.17) and had lower EDSS score at admission (median:6, IQR: 5.5-6 against 7.5 IQR: 6.5-8). The risk of failure was higher in active progressive MS patients compared with RRMS patients (OR: 6.06, CI 95%:1.37-26.76). Patients with thrombocytopenia were hospitalized more than others (MD: 1.5 days, CI 95%: 0-3). Females were more prone to hypokalemia and systemic reactions (OR: 3.11, CI 95%:1.17-8.24 and OR: 6.67, CI 95%:2.14-20.81 respectively). Conclusion The most common indication of TPE was corticosteroid-resistant severe MS relapses. About half of the patients presented an early clinical response. Lower disability, younger age and RRMS diagnosis are prognostic factors of better response. One out of four patients experienced moderate to severe complications, mainly electrolyte imbalances and systemic reactions. Appropriate interventions against these complications should be considered during TPE, especially in females.

Autoimmune cerebellar ataxia (ACA) is a condition characterized by progressive ataxia resulting from an immune-mediated attack on cerebellar structures. The presence of anti-Tr/DNER antibodies, strongly associated with Hodgkin lymphoma, has been identified in ACA. However, cases with no underlying malignancy are rare. We report the case of a 49-year-old woman presenting with progressive ataxia, slurred speech, and dizziness over three months. The patient exhibited significant cerebellar symptoms, including dysarthria and limb ataxia, without signs of other systemic illnesses. Comprehensive investigations, including imaging, lumbar puncture, and autoantibody testing, were performed. The cerebrospinal fluid (CSF) sample revealed positivity for Tr/DNER antibodies, leading to a diagnosis of autoimmune cerebellar ataxia. The patient underwent nine sessions of plasmapheresis, followed by six doses of intravenous immunoglobulin (IVIG), resulting in significant clinical improvement. Despite extensive cancer screening, no underlying malignancy was detected, suggesting a non-tumor origin of anti-Tr/DNER antibodies. The patient's gait improved, ataxia resolved, and cerebellar tests normalized following treatment. The patient was further managed with rituximab treatment every six months. This case represents a presentation of anti-Tr/DNER-associated autoimmune cerebellar ataxia without malignancy. The successful treatment with plasmapheresis and IVIG suggests that these interventions may be effective in managing autoimmune cerebellar ataxia associated with anti-Tr/DNER antibodies. Further research is needed to understand the underlying mechanisms of this condition and to determine the optimal treatment strategies.

Background/Objective(s) Fatigue is a common complaint in Multiple Sclerosis (MS) and Neuromyelitis Optica Spectrum Disorder (NMOSD) patients. The underlying mechanism of fatigue in these diseases is not well known. This cross-sectional study was designed to explore the association of brain magnetic resonance imaging (MRI) volumetric markers and fatigue among individuals with NMOSD (PwNMOSD) and MS (PwMS). Material(s) and Method(s) A total of 57 PwMS and 23 PwNMOSD participated in the study. The age of patients was between 18 and 55 years, and all of them had a disease duration of less than 6 years and the Expanded Disability Status Scale (EDSS) less than 6 points. Patients with psychologic disorders, systemic diseases such as anemia and hypothyroidism, or drug abuse were not included in the study. The Fatigue Severity Score (FSS) and Modified Fatigue Impact Scale (MFIS) questionnaires were employed to evaluate fatigue severity. All participants underwent brain MRI using a 1.5 Tesla device to measure brain structure volumes. We used the Vol brain online system for white matter, gray matter, and cerebellum volume measurement, then two neurologists checked the volume structure manually with ITK snap software and finally, we normalized them. Result(s) 24% of PwMS and 34% of PwNMOSD had severe fatigue. Based on univariate regression, MFIS was associated with the right thalamus (OR = 0.29, p-value = 0.035), total cerebellum (OR = 0.9, p-value = 0.038), right putamen (OR = 0.111, p-value = 0.042), and left globus pallidus (OR = 0.001, p-value = 0.043) volumes in PwMS. Also, FSS was related to total volumes of gray matter (OR = 0.96, p-value = 0.029, thalamus (OR = 0.54, p-value = 0.037), cerebellum (OR = 0.84, p-value = 0.004), putamen (OR = 0.31, p-value = 0.042), hippocampus (OR = 0.15, p-value = 0.049), and accumbens (OR = 0.001, p-value = 0.023) in PwMS. All analyses were adjusted for correlated clinical regressors. Multivariate regression confirmed that FSS was associated with gray matter (OR = 0.96, p = 0.044) and cerebellar volumes (total: OR = 0.84, p = 0.01; right: OR = 0.73, p = 0.014; left: OR = 0.69, p = 0.008) in PwMS. Conversely, no significant associations were found between imaging markers and FSS or MFIS in PwNMOSD. Conclusion(s) Gray matter and cerebellar atrophy were linked to severe fatigue in PwMS, while no correlation was observed between fatigue and brain structure volumes in PwNMOSD. Further research is necessary to clarify the distinct mechanisms of fatigue in these diseases, including the investigation of spinal cord lesions in PwNMOSD.

BACKGROUND:Multiple Sclerosis (MS) is a chronic neuroinflammatory disease that affects the central nervous system. Asymmetry is one of the finding in brain MRI of these patients, which is related to the debilitating symptoms of the disease. This study aimed to investigate and compare the thalamic asymmetry in MS patients and its relationship with other MRI and clinical findings of these patients. METHODS:This cross-sectional study conducted on 83 patients with relapse-remitting MS (RRMS), 43 patients with secondary progressive MS (SPMS), and 89 healthy controls. The volumes of total intracranial, total gray matter, total white matter, lesions, thalamus, and also the thalamic asymmetry indices were calculated. The 9-hole peg test (9-HPT) and Expanded Disability Status Scale (EDSS) were assessed as clinical findings. RESULTS:We showed that the normalized whole thalamic volume in healthy subjects was higher than MS patients (both RRMS and SPMS). Thalamic asymmetry index (TAI) was significantly different between RRMS patients and SPMS patients (p = 0.011). The absolute value of TAI was significantly lower in healthy subjects than in RRMS (p < 0.001) and SPMS patients (p < 0.001), and SPMS patients had a higher absolute TAI compared to RRMS patients (p = 0.037). CONCLUSIONS:In this cross-sectional study we showed a relationship between normalized whole thalamic volume and MS subtype. Also, we showed that the asymmetric indices of the thalamus can be related to the progression of the disease. Eventually, we showed that thalamic asymmetry can be related to the disease progression and subtype changes in MS.

Multiple sclerosis is a chronic demyelinating disease of the central nervous system that can be exacerbated by obesity-induced systemic inflammation. Previous studies have shown that weight loss can improve symptoms of MS. Bariatric surgery and non-surgical methods, like diet therapy, are two common approaches to weight loss. In this systematic review, we have reviewed the effectiveness and safety of surgical and non-surgical methods for weight loss in MS patients. This systematic review was conducted based on PRISMA guidelines. PubMed, Embase, Cochrane, Scopus, Web of Science, Science Direct databases were used. Four articles on bariatric surgery and nine articles on diet therapy were included. Both bariatric surgery and diet therapy are safe and effective methods for weight loss in MS patients as in normal population, and both of them can improve quality of life without exacerbating disease progression. No major complications were reported. However, no studies directly compared these two methods and also there is uncertainty regarding long-term effects. Therefore more studies are needed to compare bariatric surgery with other weight loss methods and also to investigate the effect of these methods on more MS-related outcomes to determine the safest and most effective weight loss method in these patients.

Background: Sleep disorders are more prevalent in people with multiple sclerosis (pwMS) than in the general population. This study aimed to examine the clinical and sociodemographic factors contributing to sleep disorders in pwMS. Method: The participants in this cross-sectional study were pwMS from the Isfahan Province in Iran. Sleep disorders were assessed using the Insomnia Severity Index (ISI), International Restless Legs Syndrome Study Group (IRLSSG), Berlin, and STOP-Bang questionnaires. A logistic regression model was applied to determine the accuracy of independent factors in predicting sleep impairment. A multivariate logistic regression analysis was conducted to examine the impact of multiple sclerosis (MS) types on sleep disorder severity predictability by independent variables. Result: A total of 796 pwMS were included in the current study, 693 with relapsing-remitting MS and 103 with secondary-progressive MS. Rest leg syndrome (RLS) and insomnia disorders were not present in 48.1% and 50.5% of the pwMS, respectively. According to STOP-Bang and Berlin, 87.3% and 88.4% of patients had a low-severity risk for obstructive sleep apnea (OSA), respectively. The logistic regression showed that age, gender, and Expanded Disability Status Scale (EDSS) were associated with the risk of OSA (p < 0.05). RLS severity was also correlated with age and EDSS (p < 0.05). The association between sleep disorder severity and independent variables was not affected by MS type in multivariate logistic regression. Conclusion: In this study, we found that sleep disorders such as RLS, insomnia, and OSA are common among pwMS in Iran. Sociodemographic factors, as well as disease characteristics, can have an impact on sleep disorders among pwMS.

Familial Mediterranean fever (FMF) is a rare autoinflammatory disorder characterized mainly by recurrent self-limited episodes of fever and polyserositis. FMF-related neurologic complication is an old debate, and the correlation between FMF and demyelinating disorders has been a matter of dispute for a long time. Few reports demonstrated a relationship between FMF and multiple sclerosis; however, the existence of a causal relationship between FMF and demyelinating disorders is still a puzzle. This report presents the first case of transverse myelitis following FMF attacks in which neurologic manifestations were resolved using colchicine treatment. Due to relapses of FMF, which were accompanied by transverse myelitis, rituximab was administered, which resulted in stabilizing disease activity. Accordingly, in the case of colchicine-resistant FMF and FMF-related demyelinating conditions, rituximab could be considered as a potential therapeutic option to alleviate both polyserositis and demyelinating manifestations.

Objectives: It is estimated that up to 65% of pwMS (people with multiple sclerosis) experience varying degrees of cognitive impairment, the most commonly affected domain being information processing speed (IPS). As sleep disturbance is a predictor of detriments in IPS, we aimed to study the association between severity of restless leg syndrome (RLS) and obstructive sleep apnea (OSA) symptoms with IPS in pwMS using a language and education independent tool. Methods: In a cross-sectional study, we enrolled pwMS referred to multiple sclerosis comprehensive center of Kashani hospital in Isfahan, Iran. We used Berlin and STOP-Bang questionnaires for assessing OSA symptom severity. The International Restless Legs Syndrome Study Group scale was utilized for determining presence and severity of symptoms of restless leg syndrome. We used Integrated Cognitive Assessment (ICA) test assess visual processing speed. Results: We included 211 pwMS, with a mean age of 36.88±8.76 (82.9% female). There were significant association between RLS symptoms severity and ICA index (p=0.00), but there is no association between ICA index and OSA symptom severity using STOP-Bang scale (ICA index of 0.61±0.14 and 0.60±0.08 in low and high-risk pwMS respectively; p=0.897) and Berlin scale (ICA index of 0.61±0.14 and 0.59±0.15 in low and high-risk pwMS, respectively; p=0.384). Conclusion: We found that the severity of RLS in patients with MS can worsen information processing speed, but sleep apnea did not show any effect on this domain of cognitive dysfunction.

Delayed radiation myelopathy (DRM) is a rare yet severe complication of radiotherapy. This condition has a progressive pattern that is often irreversible. Several therapeutic strategies have been introduced to alleviate disease complications, including corticosteroids, hyperbaric oxygen, anticoagulants, and antivascular endothelial growth factor (VEGF) agents. However, despite their beneficial effect, they have not been the definitive treatments for DRM. Here we present the case of a 55-year-old woman with a history of multiple myeloma who developed neurological complications 11 months after radiation therapy. As her radiologic findings demonstrated transverse myelitis, based on the DRM diagnostic criteria, the diagnosis of delayed radiation myelitis was reached. Therefore, methylprednisolone pulse therapy was initiated, resulting in the complete resolution of her neurological symptoms. However, on her follow-up examination, although she did not have new neurological complications, magnetic resonance imaging (MRI) demonstrated a residual enhancement in the thoracic spinal cord area. Hence, due to the possibility of myelitis progression and spinal cord atrophy, intravenous immune globulin (IVIG) was administered, resulting in the resolution of lesion enhancement. Considering this outcome and the immunomodulatory properties of IVIG, it could be regarded as a potential therapeutic option in the case of DRM activity.

Dear Editor, Cerebrovascular complications and encephalitis may both occur during or shortly after COVID-19 infection.1 Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an inherited disease of small vessels.2 Four patients with CADASIL have been reported in the literature since February 2022, with ischemic syndromes being more prevalent during COVID-19 infection.2-5 Furthermore, CADASIL has presented in two other patients for the first time as a postinfectious manifestation of COVID-19 with radiologic features mimicking encephalomyelitis.6,7 We report a novel case in which cognitive and psychologic disorders after COVID-19 infection led to a diagnosis of CADASIL. A 42-year-old smoking male presented to our neurologic clinic with forgetfulness and behavioral changes that appeared 3 weeks after symptoms of COVID-19 infection that included fever and chills. The COVID-19 diagnosis was confirmed based on SARS-CoV-2 positivity and the results of chest CT in August 2021. His family history was negative for recurrent strokes, but his father had symptoms of dementia. At the time of admission, the patient did not show signs of either a systemic infection or focal neurologic deficit. Laboratory tests only revealed mild thrombocytopenia with elevated C-reactive protein (20 mg/L), ESR at 4 mm/h, platelets at 131,000/μL, white blood cell (WBC) at 4,200/μL, and d-dimer at 520 (ng/mL), EEG showed mild diffuse encephalopathy with normal background activity. Brain MRI showed multiple periventricular hyperintensities in FLAIR and T2-weighted images, with extension to temporal subcortical and U-fiber white matter. None of the diffusion-weighted images showed restricted diffusion (Fig. 1). Analysis of the cerebrospinal fluid (CSF) showed a normal biochemical and cytologic profile: WBC at 2 cells/mm3, red blood cell at 10 cells/mm3, protein at 35 mg/dL, and glucose at 67 mg/dL. There were no oligoclonal bands or an increased IgG index. Cardiac arrhythmias, vasculitis, and thrombophilia were excluded through the standard extensive workup for ischemic stroke in young adults: antithrombin III at 75% (normal limit [NL]=70%–120%), antiphospholipid antibody at 6 U/mL (NL <12 U/mL), lupus anticoagulant antibody at 32 sec (NL <40 sec), B2 macroglobulin antibody at 1.6 μmol/mL (NL <3 μmol/mL), and homocysteine at 15 μmol/mL (NL=5–17 μmol/mL). Antibody assays of serum and CSF were negative for autoimmune encephalitis (anti-NMDA, anti-CASPR2, antiGABA-B-receptor, anti-AMPA1, anti-AMPA2, and anti-LGI1 antibodies) and the antiaquaporin antibody test. CADASIL was diagnosed based on the presence of a typical NOTCH3 mutation (variant NM_0000435:c.C328:p.R110C, heterozygote, autosomal dominant). We prescribed aspirin 80 mg once daily to prevent possible strokes. After an 18-month Zeynab Jalilian Saba Naghavi Iman Adibi Vahid Shaygannejad

BACKGROUND:Cognitive dysfunction, including reduced Information processing speed (IPS), is relatively common in multiple sclerosis(MS). IPS deficits have profound effects on several aspects of patients' life. Previous studies showed that deep gray matter atrophy is highly correlated with overall cognitive impairment in MS. However, the effect of deep gray matter atrophy on IPS deficits is not well understood. In this study, we evaluated the effects of deep gray matter volume changes on IPS in people with early relapse-remitting MS (RRMS) compared to healthy control. METHODS:In this case-control study, we enrolled 63 case with RRMS and 36 healthy controls. All patients were diagnosed within 6 years. IPS was evaluated using the Integrated Cognitive Assessment (ICA) test. We also performed a 1.5T MRI to evaluate deep gray matter structures. RESULTS:People with RRMS had lower accuracy in the ICA test (p = .01). However, the reaction time did not significantly differ between RRMS and control groups (p = .6). Thalamus volume was significantly lower in the RRMS group with impaired IPS compared to the RRMS with normal IPS and control groups (p < 10-4). Other deep gray matter structures were not significantly different between the RRMS with impaired IPS group and the RRMS with normal IPS group. CONCLUSION:Some people with MS are impaired in IPS even in the early stages of the disease. Thalamic atrophy affected IPS in these patients, however atrophy in other deep gray matter structures, including caudate, putamen, globus pallidus, hippocampus, amygdala, accumbens, and cerebellum, were not significantly correlated with IPS impairment in early RRMS.

Background: The aim of this study was to evaluate the safety of Sinofarm vaccine (BBIBP-CorV) in patients with multiple sclerosis (pwMS). Methods: This study was conducted on pwMS patients in Isfahan, Iran. All participants received two doses of BBIBP-CorV (Sinopharm vaccine). Demographic information and data on vaccine side effects were collected after each dose using questionnaires. All patients that recorded worsening of MS symptoms were evaluated and those with true relapse were treated with IV methyl prednisolone. Results: Of the 1538 patients, 1151 (74.8%) were female and the mean age was 40.45 +/- 9.74. The average disease duration was 10.38 +/- 6.81 years and 76.1% of participants had RRMS. 92.8% of the participants were using DMTs and mean EDSS was 2.06 +/- 3.16. 54.2% (833 patient) reported at least one adverse event after the first dose of vaccine and 46.8% (720 patient) after the second dose; in both cases going away in a few days. Most prevalent adverse events after both doses were injection site pain, headache, myalgia, fever and fatigue. Adverse events were more prevalent in younger and less prevalent in mildly disabled patients. There were seven cases of Covid-19 infection between the first and second vaccination dose, and eight cases during one-month follow -up after the second dose, none of whom needed mechanical ventilation. Ten patients after first dose and thirteen patients after the second dose experienced acute relapse. A patient had two relapses, one after each vaccine dose that were clinically and radiologically confirmed. The first relapse occurred seven days after the first vaccination with hemiparesis and other relapse, 14 days after the second dose with diplopia, hemiparesis and ataxia. Conclusion: Adverse events in pwMS following vaccination with Sinopharm vaccine was similar to the general population, which were more common in younger patients and less common in those with mild disability. As no increase in relapse rate after vaccination was detected, Sinopharm vaccine was safe in MS patients.

Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) could prompt various neurological complications. Abrupt visual disturbance was reported as a rare severe manifestation of post-coronavirus disease 2019 (COVID-19). Autoimmune conditions were assumed to have an undeniable role in creation of such circumstances. This report presents a 69-year-old woman with sudden bilateral blindness three weeks after recovering from a SARS-CoV-2 infection. Demyelination due to COVID-19-related autoimmune disorder of the central nervous system (CRAD-C) was considered to be the etiology of her bilateral blindness. Due to her progressive demyelination, immunosuppressive treatments were administered, which resulted in stabilizing post-COVID-19 demyelinating lesions. Accordingly, in the case of COVID-19-related neurological deficits, especially the acute and progressive symptoms, there should be great consideration of autoimmune response to prevent serious complications; hence early diagnosis, treatment, and long-term assessment of patients are necessitated.

During the coronavirus disease 2019 (COVID-19) pandemic, mass vaccination was a beneficial strategy in many countries. Nevertheless, reports of serious complications such as postvaccination neuromyelitis optica spectrum disorder (NMOSD) raised concerns about the safety of vaccines. Anamnart and colleagues explained postvaccination NMOSD following different vaccines, including COVID-19. To emphasize the message of this article, in this letter, we present a unique case of postvaccination NMOSD with a fulminant and fatal course, which may show a plausible relationship between COVID-19 vaccination and triggering anti-aquaporin-4 antibody (AQP4Ab).

Coronavirus (COVID-19) is a worldwide epidemic. Although the main target of COVID-19 is the respiratory system, it is known that the virus can cause neurological complications. Previous studies have shown that its neurological manifestations are usually seen in critically ill patients. In this study, we introduced patients who developed COVID-induced encephalitis despite their good general condition and mild symptoms. The only symptoms of encephalitis in these patients were cognitive impairment, that persisted for more than 6 months. This disorder was confirmed by Neuropsychiatry Unit Cognitive Assessment Tool (NUCOG) test results in patients. While previous studies have shown that COVID-induced cognitive impairment improves over time. Therefore, it is recommended that the diagnosis and treatment of encephalitis be considered in patients with COVID-19 who have mild cognitive and behavioral symptoms.

Background: : Despite investigations on the effect of disease modifying therapies (DMTs) used in multiple sclerosis (MS) on coronavirus disease 2019 (COVID-19); there are still controversies. Objective: We designed this study to evaluate the epidemiological features of covid-19 in a large sample of people with MS (pwMS) in Isfahan, Iran, as well as the association between DMTs, risk of COVID-19 infection and hospitalization. Methods: In an observational pwMS, we interviewed subjects on their MS and COVID-19 history. Results: 3050 subjects were included (74% female) with a mean age of 41.36. 423 (13.8%) had confirmed COVID-19 which shows that pwMS are at a higher risk of infection compared to the general population, No significant relationship was observed in COVID-19 infection when individual drugs. Dimethyl fumarate and rituximab had the lowest and the highest relative risks for hospitalization rate compared to other drugs, respectively. Conclusion: We found no evidence supporting a higher prevalence of COVID-19 in pwMS compared to the general population. However, our results show pwMS to be more prone to hospitalization compared to the general population, Therefore, it is advised to use safer treatment if possible until complete vaccination, and to postpone the use of rituximab.

Protein S deficiency is a thrombophilia associated with an increased risk of thromboembolism. Previous studies have shown its role as a predisposing factor for venous thromboembolism, but its role in recurrent arterial ischemic stroke remains uncertain. Here we report a patient with recurrent ischemic stroke due to protein S deficiency. Oral anticoagulant treatment with vitamin K antagonist (VKA) drugs is used to treat and prevent thromboembolic events caused by thrombophilia, but it has many limitations, especially in the case of recurrent thromboembolic events. Direct oral anticoagulants (DOACs) have many advantages over VKA. Previous studies have shown that they are safe in cases of thrombophilia, but they are not well studied in recurrent ischemic stroke due to protein S deficiency. In this study our patient was treated with rivaroxaban. Protein S deficiency may be a predisposing factor in recurrent ischemic stroke, and rivaroxaban can be a safe and effective treatment option. Further studies are needed to confirm our findings.

In the context of the coronavirus disease 2019 (COVID-19) pandemic, a recent report by Naudin et al1Naudin I. Long A. Michel C. Devigne B. Millon A. Della-Schiava N. Acute aortoiliac occlusion in a patient with novel coronavirus disease-2019.J Vasc Surg. 2021; 73: 18-21Abstract Full Text Full Text PDF Scopus (9) Google Scholar sought to explain arterial thrombosis as a presentation of COVID-19 infection. Their patient was admitted with acute monoparesis and sensory loss of the left lower limb as a consequence of aortoiliac thrombosis and limb ischemia during COVID-19 infection.1Naudin I. Long A. Michel C. Devigne B. Millon A. Della-Schiava N. Acute aortoiliac occlusion in a patient with novel coronavirus disease-2019.J Vasc Surg. 2021; 73: 18-21Abstract Full Text Full Text PDF Scopus (9) Google Scholar However, we want to emphasize that, in addition to limb ischemia, spinal cord ischemia should be considered in patients with acute limb paresis and COVID-19 infection. We read very interesting article from Naudin et al while we were finishing a case series including very similar cases about two male patients with paraparesis and COVID-19 infection. The first patient was admitted with sudden paraplegia, urinary retention, and a pain and temperature sensory level of T6, with intact proprioceptive senses. His cremasteric and bulbocavernosus reflexes were absent. Dorsalis pedis pulses were present. A full spinal cord magnetic resonance imaging was normal. The next day, signs of limb ischemia were developed. Color Doppler ultrasound examination and computed tomography angiography revealed an infrarenal abdominal aorta thrombosis, with extension to both common iliac arteries. The second patient, with known multiple sclerosis, was admitted with urinary incontinence and acute paraparesis. In addition, he had diminished deep tendon reflexes of the lower limbs and hypoesthesia in the right leg. Distal pulses in the lower extremities were present. The next day, he developed signs of limb ischemia. Color Doppler ultrasound examination and computed tomography angiography revealed complete thrombosis of the infrarenal abdominal aorta with extension to both common iliac arteries. The results of COVID-19 tests were positive for both patients. It is plausible that the paraparesis in both patients was a sign of spinal cord ischemia as a consequence of aortic thrombosis in the context of COVID-19 infection. Spinal cord ischemia is a rare disease with a prevalence of 1.2% of all strokes.2Yadav N. Pendharkar H. Kulkarni G.B. Spinal cord infarction: clinical and radiological features.J Stroke Cerebrovasc Dis. 2018; 27: 2810-2821Abstract Full Text Full Text PDF PubMed Scopus (49) Google Scholar In patients with COVID-19, who are more vulnerable to thromboembolic events,3Levi M. Thachil J. Iba T. Levy J.H. Coagulation abnormalities and thrombosis in patients with COVID-19.Lancet Haematol. 2020; 7: e438-e440Abstract Full Text Full Text PDF PubMed Scopus (990) Google Scholar such rare conditions may occur more frequently. Therefore, in patients with any acute neurologic deficit, different ischemic disorders, including spinal cord ischemia, as a consequence of thrombotic complication of COVID-19 should be considered and evaluated. Acute paraplegia with or without coronavirus disease 2019 infection: Decision-making algorithmJournal of Vascular SurgeryVol. 74Issue 3PreviewWe thank our colleagues for their letter. Acute paraplegia should always suggest two urgent diagnoses that require immediate specific treatment: acute aorta occlusion (AAO), favored by coagulation disorders in those with coronavirus disease 2019 (COVID-19) infection, and spinal cord compression, with COVID-19 as a coexisting condition. Full-Text PDF Open Archive