Clinical and Radiologic Findings of Acute Necrotizing Encephalopathy in Young Adults.
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ABSTRACT:
SUBMITTER: Vanjare HA
PROVIDER: S-EPMC7963253 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description:ObjectiveTo identify and define clinical associations and radiologic findings of posterior reversible encephalopathy syndrome (PRES).Patients and methodsPatients prospectively diagnosed as having PRES from October 1, 2005, through April 30, 2009, were pooled with retrospectively identified patients admitted from August 1, 1999, through September 30, 2005. We performed a detailed review of clinical information, including demographics, presenting symptoms, medical history, and risk factors. All patients underwent computed tomography of the brain or magnetic resonance imaging. Findings on magnetic resonance imaging were analyzed independently by 2 neuroradiologists.ResultsWe identified 120 cases of PRES in 113 patients (mean age, 48 years). Mean peak systolic blood pressure was 199 mm Hg (minimum-maximum, 160-268 mm Hg), and mean peak diastolic blood pressure was 109 mm Hg (minimum-maximum, 60-144 mm Hg). Etiologies of PRES included hypertension (n=69 [61%]), cytotoxic medications (n=21 [19%]), sepsis (n=8 [7%]), preeclampsia or eclampsia (n=7 [6%]), and multiple organ dysfunction (n=1 [1%]). Autoimmune disease was present in 51 patients (45%). Clinical presentations included seizures (n=84 [74%]), encephalopathy (n=32 [28%]), headache (n=29 [26%]), and visual disturbances (n=23 [20%]). In the 115 cases (109 patients) for which magnetic resonance imaging findings were available, the parieto-occipital regions were the most commonly involved (n=108 [94%]), followed by the frontal lobe (n=88 [77%]), temporal lobe (n=74 [64%]), and cerebellum (n=61 [53%]). Cerebellar involvement was significantly more frequent in patients with a history of autoimmunity (P=.008), and patients with sepsis were more likely to have cortical involvement (P<.001).ConclusionA substantial proportion of patients with PRES have underlying autoimmune conditions that may support endothelial dysfunction as a pathophysiologic mechanism. On brain imaging, the location and severity of vasogenic edema were mostly similar for the different clinical subgroups.
| S-EPMC2861971 | biostudies-literature
Project description:Genetic (also known as familial) acute necrotizing encephalopathy (ANE1) is a rare disease presenting with encephalopathy often following preceding viral febrile illness in patients with a genetic predisposition resulting from a missense mutation in the gene encoding RAN Binding Protein 2 (RANBP2). The acute episode is characterized by deterioration in consciousness, often with focal neurologic deficits and seizures. Additionally, symmetric multifocal brain lesions are seen in the bilateral thalami as well as other characteristic regions, involving both gray and white matter. Prognosis is variable, with a high mortality rate and most surviving patients having persistent neurologic deficits. Early treatment with high dose steroids is associated with a more favorable outcome, however the diagnosis is often overlooked resulting in delayed treatment. The RANBP2 mutation associated with ANE1 causes an incompletely penetrant predisposition to encephalopathy in the setting of febrile illness through a mechanism that remains elusive. There are several non-mutually exclusive hypotheses suggesting possible etiologies for this phenotype based on the many functions of RANBP2 within the cell. These include dysfunctions in nucleocytoplasmic trafficking and intracellular metabolic regulation, as well as cytokine storm, and abnormal distribution of mitochondria. This narrative review explores these key concepts of the RANBP2 mutation and its clinical and therapeutic implications in pediatric populations.
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Project description:IntroductionAn increasing number of published reports on SARS-CoV-2 neurological manifestations have revealed a wide spectrum of symptoms, diagnostic features, and outcomes. We report a fatal case of a COVID-19-associated acute necrotizing encephalopathy (ANE).Case reportWe report a 70-year-old man brought to the hospital after a generalized tonic-clonic seizure. He was confused and disoriented. Nasopharyngeal swab testing for SARS-CoV-2 was positive. A head computed tomography (CT) scan and cerebrospinal fluid (CSF) analysis showed no signs of acute pathology. After recurrent seizures, he was sedated and intubated. Throughout the days that followed he remained in a therapeutic coma. After discontinuation of sedatives, he remained unconscious. A repeated head CT scan showed signs of pontine edema, and brain magnetic resonance imaging (MRI) revealed inhomogeneous hyperintensities with microhemorrhages and small autonecrotic cavities in both thalami, brain stem, and cerebellar peduncles. With a high suspicion of a COVID-19-associated ANE, the patient was started on high-dose glucocorticoids; however, he died the next day. The CSF tested negative for SARS-CoV-2.DiscussionA variety of COVID-19 neurological manifestations have been reported to date, including various forms of encephalitis and encephalopathy. In our patient, encephalopathy with seizures was the presenting symptom of SARS-CoV-2 infection. The radiological findings on days 8 and 9 were consistent with an ANE. The precise pathogenesis of ANE remains unclear; however, an immune-mediated mechanism is suspected. Early diagnostics with prompt administration of immunomodulators may be lifesaving. Suspicion of a COVID-19-related encephalopathy/encephalitis should be raised in all patients with altered mental status, seizures, and/or coma.
| S-EPMC8653160 | biostudies-literature
Project description:For many encephalitis cases, the cause remains unidentified. After 2 children (from the same family) received a diagnosis of acute necrotizing encephalopathy at Centre Hospitalier Universitaire (Tours, France), we attempted to identify the etiologic agent. Because clinical samples from the 2 patients were negative for all pathogens tested, urine and throat swab specimens were added to epithelial cells, and virus isolates detected were characterized by molecular analysis and electron microscopy. We identified a novel reovirus strain (serotype 2), MRV2Tou05, which seems to be closely related to porcine and human strains. A specific antibody response directed against this new reovirus strain was observed in convalescent-phase serum specimens from the patients, whereas no response was observed in 38 serum specimens from 38 healthy adults. This novel reovirus is a new etiologic agent of encephalitis.
| S-EPMC3381585 | biostudies-literature
Project description:Infection-induced acute encephalopathy 3 (IIAE3) is an autosomal dominant disease resulting from a pathogenic variant in the RANBP2 gene. IIAE3 results in the susceptibility to the recurrence of acute necrotizing encephalopathy (ANE1) which presents as bilateral symmetric thalamic, midbrain and/or hindbrain lesions that typically develops within 1-4 days post-acute viral infection, commonly occurring before age 6.1-6 These case reports highlight a retrospective analysis of clinical data and radiographic studies on 2 ANE1 cases from our institution. The novel p.Leu450Phe variant of the RANBP2 gene was analyzed using in silico algorithms (PolyPhen-2, SIFT, Mutationtaster) which suggests the p.Leu450Phe variant is probably deleterious.7 An expansion of documented ANE1 case presentations and clinically significant RANBP2 gene mutations has the potential to improve long term outcomes if more informed therapeutic decision making can be achieved.
| S-EPMC8326991 | biostudies-literature
Project description:Here, we report a case of COVID-19-related acute necrotizing encephalopathy where SARS-CoV-2 RNA was found in CSF 19 days after symptom onset after testing negative twice. Although monocytes and protein levels in CSF were only marginally increased, and our patient never experienced a hyperinflammatory state, her neurologic function deteriorated into coma. MRI of the brain showed pathologic signal symmetrically in central thalami, subinsular regions, medial temporal lobes, and brain stem. Extremely high concentrations of the neuronal injury markers neurofilament light and tau, as well as an astrocytic activation marker, glial fibrillary acidic protein, were measured in CSF. Neuronal rescue proteins and other pathways were elevated in the in-depth proteomics analysis. The patient received IV immunoglobulins and plasma exchange. Her neurologic status improved, and she was extubated 4 weeks after symptom onset. This case report highlights the neurotropism of SARS-CoV-2 in selected patients and emphasizes the importance of repeated lumbar punctures and CSF analyses in patients with suspected COVID-19 and neurologic symptoms.
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Project description:Neurological complications are frequent non-respiratory complications associated with coronavirus disease 2019 (COVID-19), and acute encephalopathy (AE) has been reported to occur in 2.2% of patients. Among many phenotypes of AEs, acute necrotizing encephalopathy (ANE) is associated with multiple organ failure (MOF), leading to severe neurological morbidity and mortality. A previously healthy seven-year-old girl presented with a one-day history of fever followed by 12 hours of vomiting and altered consciousness. On arrival, the patient was in shock. Blood tests revealed severe acute liver failure and kidney injury, accompanied by coagulopathy. The serum interleukin-6 levels were also elevated. PCR testing for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) was positive. A head CT scan showed heterogeneous low-density areas in the bilateral thalamus, without brainstem involvement. She was diagnosed as ANE complicated with MOF (ANE severity score = 6). Intravenous methylprednisolone and therapeutic plasma exchange (TPE) were initiated with neurocritical care. After the introduction of TPE, hemodynamics improved rapidly, followed by gradual improvement in neurological manifestations. Upon follow-up after two months, no neurological or systemic sequelae were noted. Although further studies are needed, our case suggests that early immunomodulatory therapy and TPE may have contributed to the improvement in ANE and MOF associated with COVID-19.
| S-EPMC10838156 | biostudies-literature
Project description:We report acute encephalopathy associated with influenza A infection in 3 adults. We detected high cerebrospinal fluid (CSF) and plasma concentrations of CXCL8/IL-8 and CCL2/MCP-1 (CSF/plasma ratios > or =3), and interleukin-6, CXCL10/IP-10, but no evidence of viral neuroinvasion. Patients recovered without sequelae. Hyperactivated cytokine response may play a role in pathogenesis.
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Project description:Clinical features of human metapneumovirus (HMPV) infection have not been well documented for adults. We investigated clinical and radiologic features of HMPV infection in 849 adults in a tertiary hospital in South Korea. We classified patients into groups on the basis of underlying diseases: immunocompetent patients, solid tumor patients, solid organ transplantation recipients, hematopoietic stem cell transplant recipients, hematologic malignancy patients, and patients receiving long-term steroid treatment. Of 849 HMPV-infected patients, 756 had community-acquired infections, 579 had pneumonia, and 203 had infections with other pathogens. Mortality rates were highest in hematopoietic stem cell transplantation recipients (22% at 30 days). Older age, current smoking, and underlying disease were associated with HMPV pneumonia. Body mass index and an immunocompromised state were associated with 30-day mortality rates in HMPV-infected patients. Bronchial wall thickening, ground-glass opacity, and ill-defined centrilobular nodules were common computed tomography findings for HMPV pneumonia. Macronodules and consolidation were observed in <50% of patients.
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Project description:Background and purposeIn acute hepatic encephalopathy, MR imaging abnormalities have been described in the PVWM, thalami, and corticospinal tracts. We sought to determine characteristic regions of involvement on FLAIR and DWI, to evaluate their reversibility, and to correlate MR imaging extent with clinical severity.Materials and methodsTwenty patients who presented clinically with acute hepatic encephalopathy and MR imaging <21 days after symptom onset were reviewed retrospectively. Two neuroradiologists recorded involved regions on FLAIR and DWI in each, measured ADC values in affected regions and NAWM, and scored the MR imaging severity/extent. The initial severity (West Haven grade), follow-up clinical severity (degree of improvement), and maximal PAL within ±8 days of MR imaging were recorded and correlated with the MR imaging severity.ResultsOn FLAIR and DWI respectively, there were abnormalities in the thalami (85%, 70%), PLIC (75%, 80%), PVWM (80%, 85%), and DBS (70%, 35%) and diffuse cortical involvement (30%, 25%). There were relatively strong significant (P < .005) correlations of FLAIR (r = 0.680, P = .001) and DWI severity (r = 0.690, P = .001) with PAL, and of PAL with the clinical outcome (r = 0.691, P = .001). Both FLAIR (r = 0.592, P = .006) and DWI (r = 0.487, P = .029) severity correlated moderately with the clinical outcome but were not significant at the P < .005 level after Bonferroni correction.ConclusionsPatients with acute hepatic encephalopathy may exhibit characteristic regions of involvement on FLAIR with DWI findings that can be reversible. The MR imaging extent on FLAIR and DWI strongly correlates with the maximal PAL, and PAL correlates well with the clinical outcome. Diffuse cortical involvement has a higher potential for neurologic sequelae but can be reversible.
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