Clinical analysis of 23 cases of adult reversible splenial lesion syndrome

Objective To report 2 cases of reversible splenial lesion syndrome (RESLES) and to summarize some key points in diagnosis and treatment of this disease. Methods The pathogenesis, clinical presentations, neuroimaging features and outcomes of 2 cases with RESLES were retrospectively analyzed and related literature was reviewed. Results Two patients, including a 24-year-old female and an 18-year-old male, presented with sudden-onset neurologic symptoms, which became prominent after the initiation of an upper respiratory infection. Both patients were previously healthy and had no history of seizure, usage of antiepileptic drugs, or any type of vaccination during the last 2 years. Case 1 just presented with non-vertiginous dizziness. However, Case 2 presented symptoms such as dysphrenia, fever, rigidity, tremor, leukocytosis and creatine kinase (CK) elevation. Their clinical presentations were nonspecific, without evidence of callosal disconnection syndrome. They were treated with corticosteroids. Clinically, both patients were greatly improved after 14 and 21 days. Cranial MRI showed an isolated lesion centered in the splenium of the corpus callosum. The lesions were round-shaped without contrast enhancement, and disappeared after complete remission of the underlying disease. Conclusions To our knowledge and according to previous reports, RESLES is a distinct clinicoradiological syndrome of varied etiology. In addition to sudden withdrawal of antiepileptic drugs and infection, RESLES can be caused by neuroleptic malignant syndrome (NMS). The outcome of the above mentioned cases supports the opinion that prognosis depends on the underlying disorder, but not on the presence or absence of the splenial lesion. doi: 10.3969/j.issn.1672-6731.2014.07.007

This report describes a case of malignant catatonia associated with reversible MRI changes in the splenium of the corpus callosum.We admitted a 22 year-old man mainly treated with olanzapine for...

BackgroundDengue virus infection in humans can lead to a wide range of clinical manifestations, from mild fever to potentially fatal dengue shock syndrome. The incidence of dengue fever is on the rise in tropical countries. Due to the increasing incidence of dengue fever worldwide, atypical manifestations of the disease are increasingly reported. In this article we report a patient with dengue haemorrhagic fever who presented with reversible splenial lesion syndrome.Case presentationA 24-year-old Sri Lankan man who presented with fever and confusion was eventually diagnosed to have reversible splenial lesion syndrome based on brain imaging. Clinical, serological and haematological parameters confirmed a diagnosis of dengue haemorrhagic fever. His presentation, assessment, and management are described in this case report.ConclusionReversible splenial lesion syndrome is a condition which is radiologically characterized by reversible lesion in the splenium of the corpus callosum. It is associated with infectious and non-infectious aetiologies. This case report highlights the occurrence of reversible splenial lesion syndrome as a presenting feature of the expanding list of unusual neurological manifestations of dengue infection.

BackgroundReversible splenial lesion syndrome (RESLES) is a clinico-radiological syndrome characterized by the presence of reversible lesions specifically involving the splenium of the corpus callosum (SCC). The cause of RESLES is unknown. However, infectious-related mild encephalitis/encephalopathy (MERS) with a reversible splenial lesion remains the most common cause of reversible splenial lesions. Acute intermittent porphyria (AIP) is an autosomal dominant disorder caused by a partial deficiency of porphobilinogen deaminase (PBGD), the third enzyme in the heme biosynthetic pathway. It can affect the autonomic, peripheral, and central nervous system.ResultIn this study, we report a 20-year-old woman with AIP who presented with MRI manifestations suggestive of RESLES, she had a novel HMBS nonsense mutation, a G to A mutation in base 594, which changed tryptophan to a stop codon (W198*). Conclusion: To the best of our knowledge, this is only one published case of RELES associated with AIP.

Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) may affect the central nervous system and peripheral nervous system. Major central nervous system manifestations of SARS-CoV-2 infection include seizures, meningoencephalitis, ischemic stroke, anosmia, and hypogeusia. The reversible splenial lesion syndrome was first described in 2004. Although reversible splenial lesion syndrome was initially recognized as a benign phenomenon, a second type of reversible splenial lesion syndrome was identified in later years, which has a poorer prognosis and potentially serious sequela. Reversible splenial lesion syndrome can be caused by numerous etiologies including viruses. In this report, we present a rare case of COVID-19 with reversible splenial lesion, who presented with ataxia and dizziness.

Predictive factors of de novo epilepsy in reversible splenial lesion syndrome.

In this report, we present a rare case of reversible splenial lesion syndrome which was etiologically associated with sleep deprivation. Up to our knowledge, there is no previous research reporting sleep deprivation as an inducer agent of RESLES. To elucidate our observation, future reports of larger case series are surely warranted. The clarification of the possible association between sleep deprivation and the reversible splenial lesion may also provide substantial perspectives regarding the mechanisms and health impact of sleep deprivation; the functional and pathophysiological role of the splenial corpus callosum in sleep physiology. Besides, we think that the awareness of this entity should increase among clinicians to avoid unnecessary investigations as the case in our patient.

BackgroundPosterior reversible encephalopathy syndrome (PRES) is a rare clinic-radiological entity characterized by headache, an altered mental status, visual disturbances, and seizures. Reversible splenial lesion syndrome (RESLES) is a new clinic-radiological syndrome characterized by the presence of reversible lesions with transiently restricted diffusion (cytotoxic edema) in the splenium of the corpus callosum (SCC) on magnetic resonance (MR) images. Here we report a rare case involving a 23-year-old pregnant woman with eclampsia who sequentially developed PRES and RESLES.Case presentationThe patient, a 23-year-old pregnant woman, presented with sudden-onset headache, dizziness, and severe hypertension (blood pressure, 170/110 mmHg). Brain MR imaging (MRI) revealed T2 hyperintense lesions in the posterior circulation territories. Immediate cesarean section was performed, and the patient received intravenous infusion of mannitol (125 ml, q8h) for 8 days for the treatment of PRES. Ten days later, or 1 day after the discontinuation of mannitol, T2-weighted MRI showed that the hyperintense lesions (vasogenic edema) had disappeared. However, diffusion-weighted imaging (DWI) and apparent diffusion coefficient (ADC) mapping revealed an isolated lesion in the splenium of the corpus callosum (SCC) that was accompanied by restricted diffusion (cytotoxic edema); these findings indicated reversible splenial lesion syndrome (RESLES). Five days after the discontinuation of mannitol, she had no abnormal symptoms and was discharged from our hospital. Brain MRI performed 29 days after the clinical onset of symptoms showed no abnormalities.ConclusionThe sequential occurrence of the two reversible diseases in our patient prompted us to propose a novel pathogenesis for RESLES. Specifically, we believe that the vasogenic edema in PRES was reduced with mannitol treatment, which increased the hyperosmotic stress and opened the blood–brain barrier; meanwhile, upregulation of aquaporin-4 expression secondary to the increased osmotic pressure resulted in cytotoxic edema in the astrocytes in SCC (RESLES). Further research is necessary to confirm this possible pathogenesis.

Background:Reversible splenial lesion syndrome (RESLES) is a rare clinico-radiological disorder with unclear pathophysiology. Clinically, RESLES is defined as reversible isolated splenial lesions in the corpus callosum, which can be readily identified by magnetic resonance imaging (MRI) and usually resolve completely over a period of time. RESLES could be typically triggered by infection, antiepileptic drugs (AEDs), poisoning, etc. More factors are increasingly recognized.Methods and results:We reported herein an 18-year-old female patient with lobar pneumonia who developed mental abnormalities during hospitalization. An isolated splenial lesion in the corpus callosum was found by head MRI and the lesion disappeared 15 days later. Based on her clinical manifestations and radiological findings, she was diagnosed with lobar pneumonia associated RESLES. We further summarize the up-to-date knowledge about the etiology, possible pathogenesis, clinical manifestations, radiological features, treatment, and prognosis of RESLES.Conclusion:This report contributes to the clinical understanding of RESLES which may present with mental abnormalities after infection. The characteristic imaging of reversible isolated splenial lesions in the corpus callosum was confirmed in this report. The clinical manifestations and lesions on MRI could disappear naturally after 1 month without special treatment.

A 50-year-old male presented with complaints of fatigue, confusion, and memory problems. Neurological evaluation revealed altered cognition, unsteady gait, ataxia, dysmetria, and weakness. MRI of the brain was initially unremarkable. Over several days, the patient experienced improvement of symptoms and a follow-up MRI revealed a small lesion in the splenium of the corpus callosum seen on diffusion weighted and T2 sequences. The patient was discovered to have elevated anti-voltage gated potassium channel serum autoantibodies. Follow-up MRI revealed resolution of the splenial lesion. The patient was treated with intravenous immune globulin, and improved back to his pre-treatment baseline. We believe this to be the first case of a reversible splenial lesion syndrome as a manifestation of the anti-voltage gated potassium channel autoantibody syndrome, and propose a pathophysiologic mechanism.

Metabotropic glutamate receptor 5 (mGluR5)-related autoimmune encephalitis (AE) has been rarely reported; however, there are no reports on mGluR5-related AE with reversible splenial lesion syndrome following vaccination against severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). A 29-year-old man was admitted with a history of headache and fever for 9 days and 6 days, respectively. He was initially diagnosed with an intracranial infection, however the final diagnosis was corrected as anti-mGluR5-related AE with reversible splenial lesion syndrome. He had received an inactivated SARS-CoV-2 vaccine 3 weeks prior to the examination and was initially diagnosed with an intracranial infection. Physical examination revealed bilateral horizontal nystagmus, ataxia, and neck rigidity. Antiinfective therapy was minimally helpful. An analysis of the cerebrospinal fluid did not reveal pathogens for sequencing. Magnetic resonance imaging displayed abnormal signals in the splenium of the corpus callosum. We identified mGluR5 antibodies in the cerebrospinal fluid and serum. Subsequently, intravenous methylprednisolone pulse and gamma-globulin pulse therapies were administered, which substantially improved the symptoms. Follow-up did not reveal abnormal neurological symptoms, and the lesion in the corpus callosum had resolved. AE with mGluR5 antibodies could arise from SARS-CoV-2 vaccination, which warrants the awareness of healthcare workers. Reversible splenial lesion syndrome may accompany mGluR5-related AE and mimic intracranial infection. Thus, early treatment can prevent serious residual signs and symptoms.

Clinical characteristics and outcomes of patients with reversible Splenial lesion syndrome (RESLES)

The reversible splenial lesion syndrome is frequently associated with systemic and central nervous system infections. Whether an infection associated with the occurrence of the reversible splenial lesion syndrome could play a role in the later development of multiple sclerosis is unknown. Case Report. A 27-year-old woman developed an infection-related reversible splenial lesion syndrome. Diagnostic findings did not establish a specific type of infection, but revealed evidence for a potential disposition towards autoimmunity. 32 months after the initial presentation, new clinical and radiological manifestations developed that led to a diagnosis of multiple sclerosis. In susceptible individuals, infectious disease processes involving the central nervous system, such as described in this case, might be a factor in the pathogenesis of multiple sclerosis. More research on the prodromal stage of multiple sclerosis is needed to better understand the relationship between infections and autoimmunity.

Transient splenial oedema, also known as reversible splenial lesion syndrome (RESLES), is a rare magnetic resonance imaging (MRI) finding that presents as a round or ovoid focal oedema in the posterior corpus callosum, and is associated with a wide range of clinical conditions. The aetiology of RESLES is not fully clear. We aimed to investigate conflicting pathophysiological hypotheses by measuring local glucose metabolism in patients with RESLES. We retrospectively analysed patients with RESLES after reductions in antiseizure medications during in-hospital video electroencephalography monitoring. We measured local glucose uptake using positron emission tomography/computed tomography and compared matched cohorts of patients with and without MRI evidence of RESLES using nonparametric tests. Local glucose metabolism in the splenium of seven patients with RESLES was not significantly different from the glucose metabolism of the seven patients in the matched cohort. This was true using both regular and normalized standardized glucose uptake value calculation methods (p=0.902 and p=0.535, respectively). We found no evidence of local glucose hypometabolism in RESLES, which supports previous pathophysiological considerations that suggest that RESLES is an intercellular, intramyelinic oedema rather than a typical intracellular cytotoxic oedema, which is not reversible.

Reversible splenial lesion syndrome (RESLES) is a clinico-radiological entity that defines a reversible lesion in the splenium of the corpus callosum (SCC) on magnetic resonance imaging (MRI). The clinical and radiological characteristics of RESLES are poorly defined and most RESLES literature is in the form of case reports. We reviewed the clinical and radiological data from 11 RESLES patients in order to more clearly describe the characteristics of this disorder in adults.Patients included in this study were diagnosed with RESLES from May 2012 to March 2018. We collected clinical, imaging, and laboratory data of 11 adult patients from Neurology Department of the Affliated Yantai Yuhuangding Hospital of Qingdao University. After analyzing various clinico-radiological features and laboratory parameters, including serum sodium, pathogen testing, cerebrospinal fluid (CSF) studies, electroencephalography (EEG), and MRI findings, we made a diagnosis of RESLES based on the criteria proposed previously by Garcia-Monco et al.Of the 11 patients, 7 (63.63%) were male and 4 (36.36%) were female, ranging in age from 24 to 62 years with an average age of 31.48 ± 11.47 years. Seven cases occurred in the months of winter and spring (December–March). The primary clinical symptoms were headache, seizure, disturbance of consciousness, mental abnormality, and dizziness. All 11 patients had lesions in the SCC and all the lesions disappeared or significantly improved on follow-up imaging that was done within a month of symptom resolution.We found 5 (45.45%) patients had a CSF opening pressure >180 mmH2O, in addition to elevated protein and(or) leukocytes levels in 3 (27.27%) patients. The serum sodium concentration in 6 (54.55%) patients was low (<137 mmol/L) and EEG showed nonspecific slowing in waves 4 (36.36%) patients.When we encounter clinical manifestations such as headache accompanied with mental symptoms, disturbance of consciousness or epilepsy, and brain MRI finds lesions of the corpus callosum, we should consider whether it is RESLES. In order to find out the possible cause of the disease, we should carefully inquire about the history of the disease, complete etiology examination, and CSF tests. Of course, it is one of the necessary conditions for the diagnosis that the lesions in the corpus callosum are obviously relieved or disappeared.