Reversible splenial lesion syndrome

可逆性脾病变综合征
  • 文章类型: Journal Article
    可以观察到各种疾病继发的RESLES(可逆性脾病变综合征),淀粉样蛋白内水肿可能在SCC(call体脾)的发病机理中起关键作用。一些研究表明,缺氧缺血性脑病可能是SCC病变的危险因素。然而,高海拔环境对SCC的潜在影响,尤其是在慢性暴露期间,保持模糊。
    我们的研究包括19名在高海拔地区符合RESLES诊断标准的患者。包括十名患有RESLES的低海拔患者作为对照。所有参与者都接受了两次MRI(磁共振成像)扫描。常规血液检查,肝脏,肾脏和甲状腺功能,凝血功能,在住院期间和出院前检测电解质和维生素.此外,患者于2023年5月获得随访.
    高海拔的低氧环境可能会增加RESLES的风险。两组均表现出不同的临床症状。高海拔患者的CRP水平明显高于低海拔患者。高海拔患者的病变大小与SaO2水平呈正相关。然而,低海拔的患者病变大小与几种炎症标志物呈正相关趋势(WBC,NEU和CRP)。所有患者的预后良好,可能不受醋酸泼尼松的影响。
    高海拔地区的低氧环境可能在RESLES的病因中起作用。此外,RESLES是一种可逆的疾病,糖皮质激素的给药对于其治疗可能是不必要的。
    UNASSIGNED: RESLES (Reversible splenial lesion syndrome) can be observed secondary to various diseases, and intramyelinic edema may play a crucial role in the pathogenesis of SCC (Splenium of the corpus callosum). Some studies have suggested that hypoxic-ischaemic encephalopathy may constitute a risk factor for SCC lesions. However, the potential impact of high-altitude environments on SCC, especially during chronic exposure, remain obscure.
    UNASSIGNED: Our study included 19 patients who satisfied the diagnostic criteria of RESLES at high altitudes. Ten low-altitude patients with RESLES were included as controls. All participants received MRI (Magnetic resonance imaging) scans twice. Routine blood tests, liver, kidney and thyroid function, coagulation function, electrolytes and vitamins were detected during hospitalization and before discharge. In addition, the patients were followed up in May 2023.
    UNASSIGNED: Hypoxic environments at high altitudes may increase the risk of RESLES. The two groups showed different clinical symptoms. High-altitude patients had significantly higher CRP levels than low-altitude patients. The lesion size in high-altitude patients showed a positive correlation with SaO2 levels. However, the patients at low altitudes had positive correlation trends between lesion size and several inflammatory markers (WBC, NEU and CRP). All patients had a benign prognosis that may not be affected by the use of prednisone acetate.
    UNASSIGNED: Hypoxic environments at high altitudes may play a role in the aetiology of RESLES. Additionally, RESLES is a reversible disease and the administration of glucocorticoids may be dispensable for its treatment.
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  • 文章类型: Case Reports
    登革热病毒感染可呈现多种临床表现,范围从无症状或轻度疾病到严重的出血性休克。在这份报告中,我们为一名25岁的女性患者提供发烧投诉,头痛,呕吐,两天来都有令人毛骨悚然的感觉.在进一步检查中,脑膜炎的检查结果是阴性的,患者的登革热IgM抗体检测呈阳性。MRI大脑显示局限性中央病变,累及call体的脾,有利于call体的细胞毒性病变和脾的短暂病变。根据核磁共振,确诊为可逆性脾病变综合征(RESLES).开始了支持性治疗,病人完全康复,没有神经缺陷。一个月后对大脑进行了重复MRI检查,显示脾病变完全消退。如果登革热得到有效治疗,它经常有一个良好的预后与缓解罕见的,不同的放射学关联。
    Infection with the dengue virus can present with a variety of clinical manifestations that can range from asymptomatic or mild disease to severe hemorrhagic shock. In this report, we present a 25-year-old female patient with complaints of fever, headache, vomiting, and a reeling sensation for two days. On further examination, the workup for meningitis was negative, and the patient tested positive for dengue IgM antibodies. The MRI brain showed a restricted central lesion involving the splenium of the corpus callosum in favor of a cytotoxic lesion of the corpus callosum and a transient lesion of the splenium. Based on the MRI, a diagnosis of reversible splenial lesion syndrome (RESLES) was confirmed. Supportive treatment was initiated, and the patient made a complete recovery with no neurological deficits. A repeat MRI of the brain was done one month later, and it revealed complete resolution of the splenial lesion. If dengue fever is treated effectively, it frequently has a favorable prognosis with remission of uncommon, distinct radiological associations.
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  • 文章类型: Journal Article
    可逆性脾病变综合征(RESLES)是一种新的临床放射学综合征。我们回顾性分析了130例中国儿童RESLES的临床特征,这是文献中最大的案例系列。
    对2017-2023年江西省儿童医院确诊为RESLES患儿的临床资料进行回顾性分析。130例患者分为≤3岁组(A组)83例和>3岁组(B组)47例。使用卡方检验或Fisher检验来评估数据。
    绝大多数患者(127/130例,97.7%)有感染的前驱症状。A组发生胃肠道前感染(60/83,72.3%)较B组(11/47,23.4%)差异有统计学意义(P<0.05)。B组患者发生呼吸道感染的比例(33/47,70.2%)高于A组(17/83,20.5%),差异有统计学意义(P<0.05)。A组癫痫发作率(82/83,98.8%)高于B组(24/47,51.1%),差异有统计学意义(P<0.05)。B组意识障碍和头痛/头晕(27/47,57.4%;37/47,78.7%)较A组(3/83,3.6%;1/83,1.2%)有统计学意义,分别为(P<0.05)。抽搐伴轻度胃肠炎(CwG)患者A组(50/83,60.2%)较B组(8/47,17.0%)差异有统计学意义(P<0.05)。然而,B组脑炎/脑病(20/47,42.6%)较A组(10/83,12.0%)差异有统计学意义(P<0.05)。MRI显示典型部位的细胞毒性水肿(RESLES1型仅限于call体的脾,RESLES2型扩散到整个call体,邻近的白质,或两者)。初次检查后3天至50天,所有病例的MRI病变均完全恢复。所有儿童均表现出正常的神经发育。
    感染是RESLES的最常见原因。胃肠道感染常见于≤3岁的儿童,而呼吸道感染在>3岁的儿童中很常见。年轻的病人更容易出现抽搐,年龄较大的儿童更容易出现意识障碍和头痛/头晕的症状。RESLES有特征性的MRI表现,预后良好。
    UNASSIGNED: Reversible splenial lesion syndrome (RESLES) is a new clinico-radiological syndrome. We retrospectively analyzed the clinical features of 130 children with RESLES in China, which is the largest case series available in the literature.
    UNASSIGNED: The clinical data of children diagnosed as RESLES in Jiangxi Provincial Children\'s Hospital between 2017 and 2023 were retrospectively analyzed. The 130 cases were divided into two groups: ≤ 3 years old group (group A) (n = 83) and > 3 years old group (group B) (n = 47). The chi-squared test or Fisher\'s test was used to evaluate the data.
    UNASSIGNED: The vast majority of patients (127/130 cases, 97.7%) had prodromal symptoms of infection. Preceding infections of the gastrointestinal tract were statistically more significant in group A (60/83, 72.3%) than in group B (11/47, 23.4%) (P < 0.05). Preceding infections of the respiratory tract were statistically more significant in group B (33/47, 70.2%) than in group A (17/83, 20.5%) (P < 0.05). Seizures were statistically more significant in group A (82/83, 98.8%) than in group B (24/47,51.1%) (P < 0.05). The disturbance of consciousness and headache/dizziness were statistically more significant in group B (27/47, 57.4%; 37/47, 78.7%) than in group A (3/83, 3.6%; 1/83, 1.2%), respectively (P < 0.05). Convulsions with mild gastroenteritis (CwG) were statistically more significant in group A (50/83, 60.2%) than in group B (8/47, 17.0%) (P < 0.05). However, encephalitis/encephalopathy was statistically more significant in group B (20/47, 42.6%) than in group A (10/83, 12.0%) (P < 0.05). MRI showed cytotoxic edema in typical locations (RESLES type-1 limited to the splenium of the corpus callosum and RESLES type-2 spread to the entire corpus callosum, adjacent white matter, or both). There was full recovery of the lesions of MRI in all cases from 3 days to 50 days after the initial examinations. All the children showed normal neurodevelopment.
    UNASSIGNED: Infection was the most common cause of RESLES. Infections of the gastrointestinal tract are common in ≤ 3 years old children, while infections of the respiratory tract are common in >3 years old children. Younger patients are more likely to develop convulsions, and older children were more likely to have symptoms with disturbance of consciousness and headache/dizziness. RESLES has characteristic MRI manifestations and a good prognosis.
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  • 文章类型: Case Reports
    Diquat(DQ),化学上称为1,1'-乙烯-2,2'-联吡啶,是一种用于叶去除和干燥的非选择性除草剂。它对中枢神经系统细胞有毒性作用,和毒性神经损伤包括轴突变性和脑桥髓质溶解。同时,DQ还可以经由过程氧化应激影响多巴胺能神经细胞的活性,导致变性和减少多巴胺的摄取。随着DQ在农业生产中的应用越来越多,急性DQ中毒引起的神经毒性的临床报道也越来越多。目前,DQ快相相关性中毒性脑病主要累及脑桥,中脑,基底神经节,丘脑和其他大脑区域。然而,这种情况是不寻常的,因为病变主要累及call体的脾。这也是第一次被报道。
    Diquat (DQ), chemically known as 1,1 \'-ethylene-2,2\' -bipyridine, is a non-selective herbicide for leaf removal and drying. It has toxic effects on central nervous system cells, and toxic neurological lesions include axonal degeneration and pontine myelolysis. At the same time, DQ can also affect the activity of dopaminergic nerve cells through oxidative stress, causing degeneration and reducing dopamine uptake. With the increasing application of DQ in agricultural production, the clinical reports of neurotoxicity caused by acute DQ poisoning are also increasing. At present, DQ rapid-phase-related toxic encephalopathy mainly involves the pons, midbrain, basal ganglia, thalamus and other brain regions. However, this case is unusual in that the lesion mainly involved the splenium of the corpus callosum. It is also the first time to be reported.
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  • 文章类型: Case Reports
    可逆性脾病变综合征(RESLES)是一种罕见的神经系统疾病,其特征是call体的脾暂时异常,这在精神障碍中已经有报道。以前对双相情感障碍(BD)的研究主要集中在大脑结构和功能等方面,神经化学变化,和遗传学。然而,在2型双相情感障碍(BD-II)患者中,尚无研究报告该综合征在轻躁狂发作期间的发生和在缓解期的消失.
    我们提供了一例30岁女性BD-II患者的病例报告,该患者在轻度躁狂发作期间表现出RESLES症状。病人,有12年精神病史,最初经历过反复的抑郁发作,第一次轻躁狂发作发生在8年前。在此期间,该患者多次到门诊就诊,因多次自杀未遂而调整药物治疗。这一次,由于怀孕期间停药,她因第二次轻度躁狂发作而入院。在她的大脑磁共振图像上观察到了RESLES,用碳酸锂和喹硫平治疗后缓解,直至缓解。
    我们提出了关于识别BD-II伴轻躁狂发作的RESLES的第一份报告,随后在缓解阶段消失。我们的病例报告强调了BD和RESLES之间的潜在关联,强调未来研究的必要性,以更深入地探索将这两个条件联系起来的潜在机制。
    UNASSIGNED: Reversible splenial lesion syndrome (RESLES) is a rare neurological condition characterized by temporary abnormalities in the splenium of the corpus callosum, which has been reported in mental disorders. Previous studies on bipolar disorder (BD) primarily focused on aspects such as brain structure and function, neurochemical changes, and genetics. However, there have been no studies reporting the occurrence of this syndrome during hypomanic episodes and its disappearance during the remission phase in bipolar disorder type 2 (BD-II).
    UNASSIGNED: We present a case report of a 30 years-old female patient with BD-II who exhibited symptoms of RESLES during a hypomanic episode. The patient, with a 12 years psychiatric history, has experienced recurrent depressive episodes initially, with the first hypomanic episode occurring 8 years ago. During this period, this patient made several visits to the outpatient clinic to have her medications adjusted due to repeated suicide attempts. This time, she was admitted to our hospital with a second hypomanic episode due to drug withdrawal during pregnancy. The RESLES was observed on her brain magnetic resonance image, and it was alleviated after treatment with lithium carbonate and quetiapine until achieving remission.
    UNASSIGNED: We present the first report of identifying RESLES in BD-II with hypomanic episodes, which subsequently disappears during the remission phase. Our case report highlights a potential association between BD and RESLES, emphasizing the need for future studies to explore the underlying mechanisms connecting these two conditions in greater depth.
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  • 文章类型: Case Reports
    背景:自身免疫性胶质纤维酸性蛋白(GFAP)星形细胞病通常表现为脑膜脑脊髓炎。许多患者在神经系统症状之前出现流感样症状。call体(SCC)的可逆性病变是继发于多种病因的临床和放射学综合征,包括感染,这被称为RESLES。
    方法:我们报告了一例不规则高热的病例,颞部疼痛,我们神经科收治的下肢疲劳伴尿频。CSF检测显示GFAP-IgG阳性,白细胞计数和蛋白质升高,低葡萄糖和氯,而MRI显示SCC有可逆性病变,引导我们诊断自身免疫性GFAP自身细胞病伴有RESLES。该男孩在抗病毒和类固醇治疗后明显改善。
    结论:自身免疫性GFAP自身细胞病伴有RESLES很少见,其共存的发病机制尚未明确。自身免疫性GFAP自身细胞病和RESLES均与病毒感染有关。我们的病例包括感染症状,抗病毒治疗后有所改善,提示病毒感染可能在发病机制中起关键作用。
    BACKGROUND: Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy usually presents as meningoencephalomyelitis. Many patients developed flu-like symptoms preceding the neurologic symptoms. Reversible lesion in the splenium of the corpus callosum (SCC) is a clinical and radiological syndrome secondary to many kinds of etiologies, including infections, which is termed RESLES.
    METHODS: we reported a case developing irregularly high fever, both temporal pain, low limbs fatigue with frequent urination admitted to our neurology department. CSF test showed GFAP-IgG positive, elevated WBC counts and protein, with low glucose and chlorine, while MRI showed a reversible lesion on SCC, leading us to diagnose autoimmune GFAP autocytopathy accompanied with RESLES. The boy had significantly improved after anti-virus and steroids therapy.
    CONCLUSIONS: Autoimmune GFAP autocytopathy accompanied with RESLES is rarely seen, and pathogenesis for the co-existence has not been clarified. Autoimmune GFAP autocytopathy and RESLES are both related to viral infection. Our case covered infectious symptoms and improved after antiviral treatment, suggesting virus infection may perform a key role in pathogenesis.
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  • 文章类型: Case Reports
    轻度脑炎/脑病伴可逆性脾病变(MERS)在老年人中较少见,大多数都有一些后遗症。然而,即使在老年人中,MERS可能预后良好,并不总是需要特定的治疗。
    Mild encephalitis/encephalopathy with a reversible splenial lesion (MERS) is less common in the elderly, and most have some sequelae. However, even in the elderly, MERS may have a good prognosis, and a specific treatment is not always required.
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  • 文章类型: Case Reports
    未经证实:可逆性脾病变综合征(RESLES)是一种放射学特征为由多种因素引起的可逆性病变的疾病,主要涉及call体(SCC)的脾。RESLES最常见的原因包括感染,抗癫痫药物的使用和戒断,和严重的代谢紊乱.然而,自身免疫性脑炎(AE)的病例并不常见。
    UNASSIGNED:一名26岁的女性计算机编程工程师,由于3天的易怒发作,没有病史或精神病史,向精神病医院报告,胡言乱语,肢体僵硬,梦游,幻觉,和阵发性躁狂症.头颅MRI显示SCC信号异常。进行腰椎穿刺并在CSF和血清中进行自身抗体的进一步测试。患者的CSF抗NMDAR(滴度为1:3.2)抗体阳性,血清抗NMDAR(滴度为1:32)和mGluR5(滴度为1:10)抗体也呈阳性。骨盆增强CT显示盆腔肿大;评估双侧卵巢畸胎瘤(成熟畸胎瘤和未成熟畸胎瘤)。经腹左附件切除术后经病理证实,右卵巢活检,卵巢囊肿切除术.静脉注射类固醇后,患者得到了很大改善,免疫球蛋白,口服泼尼松,手术治疗,和化疗。随访MRI显示病变完全解决。在3个月的随访中,患者症状完全缓解,没有任何复发和肿瘤的迹象。抗NMDAR抗体的滴度在血清中降低至1:10。
    未经批准:此处,我们报告了一个罕见的AE与重叠的自身抗体,以及RESLES和双侧卵巢畸胎瘤。目前的情况提供了mGluR5抗体在抗NMDAR脑炎中并发的可能性。然而,潜在的机制仍然难以捉摸。此外,我们提供了更多的证据,表明重叠的抗体相关病理可能是引起RESLES的众多原因之一.尽管如此,在解释观察时应谨慎,考虑到这是一个单一的案例研究。
    Reversible splenial lesion syndrome (RESLES) is a spectrum of disease radiologically characterized by reversible lesions caused by multiple factors, primarily involving the splenium of the corpus callosum (SCC). The most common causes of RESLES include infection, antiepileptic drug use and withdrawal, and severe metabolic disorders. Nevertheless, cases of autoimmune encephalitis (AE) are uncommon.
    A 26-year-old female computer programming engineer with no previous medical or psychiatric history reported to the psychiatric hospital due to a 3-day episode of irritability, babbling, limb stiffness, sleepwalking, hallucinations, and paroxysmal mania. Brain MRI revealed abnormal signals of the SCC. Lumbar puncture was performed and further testing for auto-antibodies was conducted in both the CSF and serum. CSF of the patient was positive for anti-NMDAR (titer of 1:3.2) antibodies, and serum was also positive for anti-NMDAR (titer of 1:32) as well as mGluR5 (titer of 1:10) antibodies. Enhanced CT of the pelvis showed an enlarged pelvic mass; bilateral ovarian teratomas (mature teratoma and immature teratoma) were evaluated, which were pathologically confirmed after transabdominal left adnexal resection, right ovarian biopsy, and ovarian cystectomy. The patient considerably improved after intravenous administration of steroids, immunoglobulin, oral prednisone, surgical treatment, and chemotherapy. A follow-up MRI revealed completely resolved lesions. During a 3-month follow-up, the patient experienced complete resolution of symptoms without any sign of recurrence and tumors. The titer of the anti-NMDAR antibody decreased to 1:10 in serum.
    Herein, we report a rare case of AE with overlapping auto-antibodies, along with RESLES and bilateral ovarian teratomas. The current case provides the possibility of the concurrence of mGluR5 antibodies in anti-NMDAR encephalitis. However, the underlying mechanism remains elusive. Furthermore, we provide additional evidence that overlapping antibodies-related pathology may be one of the many causes of RESLES. Nonetheless, caution should be observed in interpreting the observation, considering that this is a single-case study.
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  • 文章类型: Case Reports
    背景:严重急性呼吸综合征冠状病毒2(SARS-CoV-2)疫苗接种已在全球范围内进行,偶尔有相关神经系统并发症的报告。具体来说,疫苗接种对X连锁Charcot-Marie-Tooth病1型(CMTX1)患者的影响尚不清楚.在磁共振成像(MRI)上,CMTX1患者可以出现中风样发作并伴有可逆性后部脑病综合征,虽然这是罕见的。
    方法:一名39岁男性因发作性失语和吞咽困难入院2d,入院前39d接受SARS-CoV-2疫苗接种。体格检查显示pes腔和肌腱反射降低。脑部MRI显示双侧,对称,限制扩散与T2超信号在大脑半球。神经传导研究显示周围神经损伤。他被诊断出患有Charcot-Marie-Tooth病,以及X染色体上GJB1基因的半合子突变,已知是CMTX1的致病性。最初,我们怀疑短暂性脑缺血发作或脱髓鞘性白质脑病。我们开始了抗血栓治疗和免疫疗法的治疗。出院后1.5个月,脑部MRI显示病变完全消退,没有复发。
    结论:SARS-CoV-2疫苗接种可能是CMTX1的诱发因素,并引发突然出现。
    BACKGROUND: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccinations have been administered worldwide, with occasional reports of associated neurological complications. Specifically, the impact of vaccinations on individuals with X-linked Charcot-Marie-Tooth disease type 1 (CMTX1) is unclear. Patients with CMTX1 can have stroke-like episodes with posterior reversible encephalopathy syndrome on magnetic resonance imaging (MRI), although this is rare.
    METHODS: A 39-year-old man was admitted with episodic aphasia and dysphagia for 2 d. He received SARS-CoV-2 vaccination 39 d before admission. Physical examination showed pes cavus and reduced tendon reflexes. Brain MRI showed bilateral, symmetrical, restricted diffusion with T2 hyperintensities in the cerebral hemispheres. Nerve conduction studies revealed peripheral nerve damage. He was diagnosed with Charcot-Marie-Tooth disease, and a hemizygous mutation in the GJB1 gene on the X chromosome, known to be pathogenic for CMTX1, was identified. Initially, we suspected transient ischemic attack or demyelinating leukoencephalopathy. We initiated treatment with antithrombotic therapy and immunotherapy. At 1.5 mo after discharge, brain MRI showed complete resolution of lesions, with no recurrence.
    CONCLUSIONS: SARS-CoV-2 vaccination could be a predisposing factor for CMTX1 and trigger a sudden presentation.
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  • 文章类型: Case Reports
    call体在脑功能中起着至关重要的作用。特别是,在call体的躯干中,在各种疾病的过程中,可能是暂时的,可逆性改变(可逆性脾病变综合征(RESLES)),以及部分可逆和不可逆的变化。本文讨论了RESLES和其他条件随call体叶片变化的分化,以及伴随的临床症状。此外,本文报道了在核磁共振(NMR)图像中出现上述变化的患者的病例报告.一名诊断为Ehlers-Danlos综合征VI型的20岁患者因精神运动性激动而被紧急送往精神科病房,拒绝接受食物和液体,带有信息的妄想声明,宏伟,和幻听.在进行的大脑磁共振成像(MRI)中,T2加权图像中无特征性的call体显示出高度密集的区域,在弥散磁共振(DWI)序列和表观扩散系数(ADC)序列中,信号强度降低且无出血迹象。提出了call体亚急性缺血性中风的假设。在大脑的控制MRI中,call体的变化完全回归,因此排除了缺血性病因,有利于诊断RESLES。住院期间,患者经历了严重的精神状态波动,具有典型的偏执综合征的主要症状,表现为思维和感知的过程和结构紊乱,长效肌内注射奥氮平后得到明显稳定的改善。考虑到临床和放射学图片,这一集的年龄,疾病发展的速度,在脑核磁共振图像的放射学变化的撤回后,其临床症状的持续,以及引入抗精神病药物后临床状况的显着改善,最终诊断为精神分裂症。
    The corpus callosum plays a vital role in brain function. In particular, in the trunk of the corpus callosum, in the course of various diseases, there may be temporary, reversible changes (reversible splenial lesion syndrome (RESLES)), as well as partially reversible and irreversible changes. This article discusses the differentiation of RESLES and other conditions with changes in the corpus callosum lobe, as well as the accompanying clinical symptoms. Moreover, a case report of a patient in whom the above changes appeared in the nuclear magnetic resonance (NMR) image is presented. A 20-year-old patient with the diagnosis of Ehlers-Danlos syndrome type VI was admitted to the psychiatric ward in an emergency because of psychomotor agitation, refusal to take food and fluids, delusional statements with a message, grandeur, and auditory hallucinations. In the performed magnetic resonance imaging (MRI) of the brain, the corpus callosum non-characteristic in T2-weighted images revealed a hyperintensive area, which was significantly hyperintensive in diffusion magnetic resonance (DWI) sequences and in apparent diffusion coefficient (ADC) sequences with reduced signal intensity and no signs of bleeding. The hypothesis of subacute ischemic stroke of the corpus callosum was presented. In the control MRI of the brain, changes in the corpus callosum completely regressed, thus excluding an ischemic etiology and favoring the diagnosis of RESLES. During hospitalization, the patient experienced significant fluctuations in mental status, with the dominant symptoms typical of the paranoid syndrome in the form of disturbances in the course and structure of thinking and perception, and a clear and stable improvement was obtained after the administration of long-acting intramuscular olanzapine. Taking into account the clinical and radiological picture, the age of the episode, the rapidity of the disease development, the persistence of its clinical symptoms after the withdrawal of radiological changes in the brain NMR image, as well as the significant improvement in the clinical condition after the introduction of antipsychotic drugs, the final diagnosis was made of schizophrenia.
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