Limbic Encephalitis Research Articles

A systematic review of paraneoplastic syndromes associated with thymoma: Treatment modalities, recurrence, and outcomes in resected cases

ObjectiveParaneoplastic syndromes associated with thymomas remain incompletely understood. The objective was to examine the association between surgically resected thymomas and paraneoplastic syndromes over the past half century. MethodsA primary PubMed/MEDLINE search was used to identify published articles describing paraneoplastic syndromes associated with thymomas from 1960 to 2019. A secondary search identified additional articles referenced in the articles found in the primary search. Kaplan–Meier and log-rank test were used for time-to-event data analyses. ResultsFrom 407 articles describing 507 patients, 123 different paraneoplastic syndromes were associated with thymoma. The 5 most common paraneoplastic syndromes were myasthenia gravis, pure red cell aplasia, lichen planus, Good syndrome, and limbic encephalitis. Complete or partial resolution of paraneoplastic syndrome symptoms after surgery was noted in 76% of patients, of whom 21% had a relapse or new paraneoplastic syndrome onset after surgery. The most common adjunctive therapy associated with resolution of paraneoplastic syndrome was corticosteroids (30%). For all patients after surgery, thymoma recurrence was observed in 17% of cases, whereas recurrence of paraneoplastic syndrome was observed in 34% of cases, and both were observed in approximately 11% of cases. The 5- and 10-year overall survivals were 78% and 66%, respectively. Improved overall survival was associated with patients who had total resolution from paraneoplastic syndrome. ConclusionsA comprehensive assessment of publications over the past half century suggests that a multimodal treatment approach that includes surgical resection of thymomas is able to achieve paraneoplastic syndrome resolution in a majority of patients. Onset of new paraneoplastic syndromes after surgery is associated with the recurrence of the first paraneoplastic syndrome, and resolution of paraneoplastic syndrome is associated with improved overall survival.

Continuous EEG Findings in Autoimmune Encephalitis.

Autoimmune encephalitis (AE) is a cause of new-onset seizures, including new-onset refractory status epilepticus, yet there have been few studies assessing the EEG signature of AE. Multicenter retrospective review of patients diagnosed with AE who underwent continuous EEG monitoring. We identified 64 patients (male, 39%; white, 49%; median age, 44 years); of whom, 43 (67%) were antibody-proven AE patients. Of the patients with confirmed antibody AE, the following were identified: N-methyl-D-aspartate receptor (n = 17, 27%), voltage-gated potassium channel (n = 16, 25%), glutamic acid decarboxylase (n = 6, 9%), and other (n = 4, 6%). The remaining patients were classified as probable antibody-negative AE (n = 11, 17%), definite limbic encephalitis (antibody-negative) (n = 2, 3%), and Hashimoto encephalopathy (n = 8, 13%). Fifty-three percent exhibited electrographic seizures. New-onset refractory status epilepticus was identified in 19% of patients. Sixty-three percent had periodic or rhythmic patterns; of which, 38% had plus modifiers. Generalized rhythmic delta activity was identified in 33% of patients. Generalized rhythmic delta activity and generalized rhythmic delta activity plus fast activity were more common in anti-N-methyl-D-aspartate AE (P = 0.0001 and 0.0003, respectively). No other periodic or rhythmic patterns exhibited AE subtype association. Forty-two percent had good outcome on discharge. Periodic or rhythmic patterns, seizures, and new-onset refractory status epilepticus conferred an increased risk of poor outcome (OR, 6.4; P = 0.0012; OR, 3; P = 0.0372; OR, 12.3; P = 0.02, respectively). Our study confirms a signature EEG pattern in anti-N-methyl-D-aspartate AE, termed extreme delta brush, identified as generalized rhythmic delta activity plus fast activity in our study. We found no other pattern association with other AE subtypes. We also found a high incidence of seizures among patients with AE. Finally, periodic or rhythmic patterns, seizures, and new-onset refractory status epilepticus conferred an increased risk of poor outcome regardless of AE subtype.

Paraneoplastic Limbic Encephalitis Mimicking Acute Herpetic Encephalitis

Paraneoplastic limbic encephalitis (PLE) is a rare disorder that typically follows a chronic or subacute course of personality changes, memory loss, seizures, and hallucinations. Early diagnosis is difficult, and characteristic symptoms can be mimicked by a variety of conditions. We present a case of PLE, initially presenting as acute herpetic encephalitis.

A paraneoplastic limbic encephalitis from an anorectal small cell neuroendocrine carcinoma: a case report

BackgroundParaneoplastic limbic encephalitis (PLE) is a rare autoimmune neurological syndrome observed in cancer patients. PLE is difficult to diagnose and presents a variable response to treatment, depending on the characteristics of the tumor and neuronal autoantibodies.Case presentationA 64-year-old, Caucasian, non-smoker man presented with a rapidly developing cognitive impairment, personality change, spatial disorientation, and short-term memory loss associated with anorexia and cervical and inguinal lymph nodes. The 18F-FDG PET scan documented intensely hypermetabolic lymph nodes, which histologically corresponded to a metastasis from a small cell neuroendocrine carcinoma. The brain MRI revealed a high T2-weighted FLAIR signal of the hippocamps, consisted with a PLE. The presence of anti-neuronal Hu antibodies confirmed the diagnosis. The patient underwent plasmapheresis, associated to a systemic chemotherapy resulting in a partial and temporary improvement of the neurological symptoms. Four cycles of intravenous immunoglobulins were also necessary. After six cures of chemotherapy, the lymph node metastases regressed. However, a new anorectal lesion was detected and was histologically confirmed as a primary small cell neuroendocrine carcinoma, which was treated with concomitant chemoradiotherapy. At the end of this treatment, the patient showed a rapid tumor progression leading to his death.ConclusionsThis case highlights the rare entity, PLE, which is difficult to diagnose and manage. In addition, this is the first published case of PLE associated with an anorectal small cell neuroendocrine carcinoma, which appeared after completion of systemic chemotherapy.

Volumetry of Mesiotemporal Structures Reflects Serostatus in Patients with Limbic Encephalitis.

Limbic encephalitis is an autoimmune disease. A variety of autoantibodies have been associated with different subtypes of limbic encephalitis, whereas its MR imaging signature is uniformly characterized by mesiotemporal abnormalities across subtypes. Here, we hypothesized that patients with limbic encephalitis would show subtype-specific mesiotemporal structural correlates, which could be classified by supervised machine learning on an individual level. T1WI MPRAGE scans from 46 patients with antibodies against glutamic acid decarboxylase and 34 patients with antibodies against the voltage-gated potassium channel complex (including 10 patients with leucine-rich glioma-inactivated 1 autoantibodies) and 48 healthy controls were retrospectively ascertained. Parcellation of the amygdala, hippocampus, and hippocampal subfields was performed using FreeSurfer. Volumes were extracted and compared between groups using unpaired, 2-tailed t tests. The volumes of hippocampal subfields were analyzed using a multivariate linear model and a binary decision tree classifier. Temporomesial volume alterations were most pronounced in an early stage and in the affected hemispheric side of patients. Statistical analysis revealed antibody-specific hippocampal fingerprints with a higher volume of CA1 in patients with glutamic acid decarboxylase-associated limbic encephalitis (P = .02), compared with controls, whereas CA1 did not differ from that in controls in patients with voltage-gated potassium channel complex autoantibodies. The classifier could successfully distinguish between patients with autoantibodies against leucine-rich glioma-inactivated 1 and glutamic acid decarboxylase with a specificity of 87% and a sensitivity of 80%. Our results suggest stage-, side- and antibody-specific structural correlates of limbic encephalitis; thus, they create a perspective toward an MR imaging-based diagnosis.

Pretransplant Risk Factors for Calcineurin Inhibitor-Induced Encephalopathy and Limbic Encephalitis Following Allogeneic Hematopoietic Cell Transplantation

Pretransplant Risk Factors for Calcineurin Inhibitor-Induced Encephalopathy and Limbic Encephalitis Following Allogeneic Hematopoietic Cell Transplantation

NCMP-13. BRAIN MRI FINDINGS IN PATIENTS TREATED WITH CHIMERIC ANTIGEN RECEPTOR T-CELLS (CAR-T) DURING SEVERE NEUROTOXICITY

Abstract INTRODUCTION Severe neurotoxicity occurs in ~30% of Diffuse Large B Cell Lymphoma (DLBCL) patients treated with CAR-T cell therapy. Brain MRI is used to assess and manage patients during neurotoxicity. Brain MRI can help understand which brain locations are affected during neurotoxicity. However, in majority of patients brain MRI is normal. It is unclear if the MRI findings are time dependent. Here we review our institutional experience with MRI findings. METHOD We reviewed MRI brain of 38 patients who received CAR-T at Moffitt Cancer Center from October, 2017 to March, 2019. RESULTS We identified 8 patients (21%) who had abnormal MRI findings. Five (63%) of these patients showed increased T2 FLAIR changes in bilateral mesial temporal lobes consistent with limbic encephalitis. One patient showed restricted diffusion and abnormal T2 FLAIR and T2 weighted images within the splenium of the corpus callosum plus increased T2 FLAIR changes in bilateral mesial temporal lobe, which resolved with follow up MRI in a month. One patient showed increased FLAIR signal intensity in left lateral cerebellum and cerebellar vermis. Two patients showed multiple new tiny areas of acute ischemic changes post CAR-T. One patient had several small and subtle areas of increased FLAIR signal intensity in bilateral frontal and parietal cortex on day 5 post CAR-T and multiple tiny areas of acute ischemia on day 10 post CAR-T. The median time to performing brain MRI was 8 days (range: 5–14). CONCLUSIONS MRI can show T2 FLAIR changes consistent with limbic encephalitis mostly in temporal lobes but also in frontal, parietal lobes, and cerebellum. The MRI changes can happen anytime from day 5 to day 14 post CAR-T infusion. We need larger, controlled studies to have better understanding of MRI findings and whether they are time dependent in relation to when CAR-T was given.

Limbic encephalitis associated with tuberculous mediastinal lymphadenitis

IntroductionLimbic encephalitis represents an autoimmune disorder that is commonly associated with malignancies. It is also seen in association with infectious or systemic autoimmune diseases. The literature reports two case reports of limbic encephalitis associated with tuberculosis. Case ReportWe report the case of a 42 year-old male referred to our clinic for a non-resolving pneumonia. He was found to have a limbic encephalitis associated with mediastinal tuberculous lymphadenitis. The diagnosis was made on a needle aspirate from a mediastinal lymph node obtained through endobronchial ultrasound. A paradoxical radiological progression was noted during therapy. He was successfully treated by anti-tuberculous drugs with clinical and radiological improvement. ConclusionLimbic encephalitis is associated with tuberculosis and should be included as part of the central nervous system involvement with tuberculosis. Endobronchial ultrasound has been shown to be useful in the diagnosis of mediastinal tuberculous lymphadenitis.

Human herpesvirus-6B infection in pediatric allogenic hematopoietic stem cell transplant patients: Risk factors and encephalitis.

Human herpesvirus-6B (HHV-6B) infection after allogenic hematopoietic stem cell transplantation (allo-HSCT) is known to be associated with post-transplant limbic encephalitis in adults. Meanwhile, the association between HHV-6B infection and central nervous system complications remains unclear in pediatric allo-HSCT patients. In this study, HHV-6B infection was monitored for more than 50days after HSCT using virus isolation and real-time PCR. Clinical information such as patient background and encephalitis status was collected retrospectively from medical records. Risk factors for HHV-6B infection were determined by the Cox proportional hazards model, and the clinical features of HHV-6B encephalitis in pediatric allo-HSCT patients were elucidated. Human herpesvirus-6B infection was observed in 74 (33.8%) of 219 patients at 3-47days (median 18, interquartile range 13-20). Risk factors identified in multivariable analysis were hematological malignancy (hazards ratio [HR], 5.0; 95% confidence interval [CI], 2.3/12.5; P<.0001), solid tumor (HR, 4.8; CI, 1.5/16.3; P=.0104), unrelated donor (HR, 2.1; CI, 1.0/4.6; P=.0378), and sex-mismatched donor (HR 1.8; CI, 1.1/3.0; P=.0257). HHV-6B encephalitis occurred in only one of the 219 patients (0.46%); this patient demonstrated the typical clinical course of posterior reversible encephalopathy syndrome. Hematological malignancy, solid tumor, unrelated donor, and sex-mismatched donor were significant risk factors for HHV-6B infection after pediatric allo-HSCT. In pediatric allo-HSCT patients, the incidence of HHV-6B encephalitis was low and the clinical features differed from those in adult patients.

Dopaminergic Hyperactivity in Neurological Patients with Delirium

Delirium has important etiological, prognostic, and therapeutic implications. The study of neurochemical markers in this condition is relevant to the understanding of its pathophysiology. The assessment of the dopamine system is particularly relevant, as dopamine antagonists are the most used drugs in delirium. To analyze neurotransmission markers in patients with delirium, focusing in the dopamine metabolite, homovanillic acid. A case-control study was performed at the National Institute of Neurology and Neurosurgery, Mexico, including hospitalized patients in which lumbar puncture was obtained for diagnostic purposes. Cases were selected if they fulfilled DSM-5 criteria for delirium. Age-paired controls were patients in which delirium was ruled out, selected at the same clinical scenario, during the same period. Neurological and systemic diagnoses were registered. Delirium was assessed using the DRS-98-R instrument. The dopamine metabolite, homovanillic acid (HVA), was measured by means of high-performance liquid chromatography. Other neurotransmission markers were also measured (5-hydroxyindoleacetic acid, glutamate, aspartate, GABA, glycine, arginine, citrulline, nitrites, and nitrates). A logistic regression model was used to determine pathogenic factors associated with the presence of delirium. 68 neurological patients with delirium and 68 patients without delirium were included. Higher homovanillic acid levels in cerebrospinal fluid were significantly associated with delirium. This result was significant after a subanalysis in patients without exposure to antipsychotics. Male gender and autoimmune limbic encephalitis were also associated with the presence of delirium. In hospitalized neurological patients, dopaminergic hyperactivity and autoimmune limbic encephalitis are pathogenic factors associated with the presence of delirium.

Nursing care in anti-N-methyl-D-aspartate receptor encephalitis: A case series.

Anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis is a paraneoplastic limbic encephalitis, recently identified.To summarize our experience in the nursing care of patients with anti-NMDAR encephalitis managed with surgery and pharmacotherapy.This study included 45 patients treated between July 2015 and November 2016. Laparoscopic oophorocystectomy was performed in 11 female patients with teratomas. Eleven patients required tracheal intubation or tracheotomy and ventilation.The patients were hospitalized for an average of 25.2 days. The mental and neurological symptoms were significantly relieved 23.6 ± 4.8 days after surgery or immunotherapy. Near-normal function was restored in 11 patients, while 34 patients had varying degrees of dysfunction at discharge. After follow-up of 1 to 18 months, 24 patients were found to have permanent impairments.Appropriate symptomatic nursing care is required to ensure the safety of patients with anti-NMDAR encephalitis.

Autoantibodies to synapsin I sequestrate synapsin I and alter synaptic function

Synapsin I is a phosphoprotein that coats the cytoplasmic side of synaptic vesicles and regulates their trafficking within nerve terminals. Autoantibodies against Syn I have been described in sera and cerebrospinal fluids of patients with numerous neurological diseases, including limbic encephalitis and clinically isolated syndrome; however, the effects and fate of autoantibodies in neurons are still unexplored. We found that in vitro exposure of primary hippocampal neurons to patient’s autoantibodies to SynI decreased the density of excitatory and inhibitory synapses and impaired both glutamatergic and GABAergic synaptic transmission. These effects were reproduced with a purified SynI antibody and completely absent in SynI knockout neurons. Autoantibodies to SynI are internalized by FcγII/III-mediated endocytosis, interact with endogenous SynI, and promote its sequestration and intracellular aggregation. Neurons exposed to human autoantibodies to SynI display a reduced density of SVs, mimicking the SynI loss-of-function phenotype. Our data indicate that autoantibodies to intracellular antigens such as SynI can reach and inactivate their targets and suggest that an antibody-mediated synaptic dysfunction may contribute to the evolution and progression of autoimmune-mediated neurological diseases positive for SynI autoantibodies.

Unique combination of anti‐paraneoplastic antigen Ma2 antibody‐positive Ophelia syndrome and opsoclonus‐myoclonus syndrome in an adult Hodgkin’s lymphoma patient

AbstractBackgroundParaneoplastic neurological syndromes are a rare presentation of malignant lymphomas at diagnosis or relapse. The most frequent form is the cerebellar degeneration associated with Hodgkin’s lymphoma (HL), but several other syndromes, such as limbic encephalitis, sensorimotor neuropathies and dermatomyositis, have also been described in association with both HL and non‐Hodgkin’s lymphomas.Case presentationHere, we describe a unique case of a 64‐year‐old man with HL presenting at diagnosis with two distinct well‐defined paraneoplastic neurological syndromes – autoimmune limbic encephalitis with memory loss and opsoclonus‐myoclonus syndrome. Intriguingly, the patient tested positive for anti‐paraneoplastic antigen Ma2/Ta antibody, which is almost exclusively detected in solid cancers, such as testicular germ cell tumors and small cell lung cancer. The patient had early‐stage HL, and showed rapid improvement of neurological symptoms after initiation of corticosteroid treatment and specific lymphoma therapy with the adriamycin, bleomycin, vinblastine and dacarbazine regimen.ConclusionsThis case supports the general view of the complex immune system deregulation in HL, which can trigger various autoimmune phenomena, even in early‐stage disease.

Multiple co-existing antibodies in autoimmune encephalitis: A case and review of the literature

A 66-year-old man with a history of chronic tobacco use presented with two months of progressive memory deficits, ataxia, diplopia, and opsoclonus. His brain magnetic resonance imaging (MRI) showed limbic and brainstem encephalitis, and antibody testing was positive for anti-Ma1/Ma2, anti-N-methyl-d-aspartate receptor (anti-NMDA-R) and anti-glutamic acid decarboxylase 65 (anti-GAD65) antibodies. His encephalitis improved with intravenous steroids, plasmapheresis, and rituximab initiation. His PET/CT was suspicious for lung malignancy, but a progressive deterioration of his respiratory status prevented full investigation. Multiple auto-antibodies may be produced in response to a malignancy and overlapping of clinical presentations may occur with multiple auto-antibodies.

Prognostic significance of subsequent extra-temporal involvement in cryptogenic new onset refractory status epilepticus (NORSE) initially diagnosed with limbic encephalitis

IntroductionNew-onset refractory status epilepticus (NORSE) is defined as refractory SE in patients without active epilepsy or relevant neurological disorder with no clear active causes. Diverse types and etiologies of NORSE are reported in various groups. Limbic encephalitis (LE) is reported as one of etiologies of NORSE. In this study, we investigated whether there were any intersections between NORSE and limbic encephalitis, as well as the presence of prognostic factors in intersection patients. MethodsWe retrospectively analyzed patients who met both the definition of NORSE and diagnostic criteria of LE at the initial presentation from our database. Clinical characteristics and blood test, cerebrospinal fluid, electroencephalography, and magnetic resonance imaging results were reviewed. Prognosis was recorded as ICU admission stay, total length of hospitalization, and modified Rankin Scale at discharge. In particular, we determined which factors were associated with patients’ prognosis. ResultsThirteen patients were selected. Nine of the 13 patients had myalgia and 8 patients had fever in the prodromal period. Twelve of the 13 patients had acute memory impairment or confusion before SE development. In addition, 46.2% of the patients showed leukopenia or thrombocytopenia. Median body temperature at hospital arrival was 37.6 °C. Nine patients showed generalized tonic-clonic SE. All patients were treated with immunotherapy and 11 of the 13 patients achieved burst suppression through induced coma therapy. Ten patients showed lesion extension on follow-up imaging. The most common extension site was the claustrum. Patients with more lesion extension showed poorer outcomes than those without lesion extension. ConclusionSubsequent extratemporal lesion extension was closely associated with poor prognosis in NORSE-LE patients. This study explores a new subtype of NORSE and suggests a possible common underlying pathomechanism between NORSE and LE.

Therapeutic plasma exchange in neurological disorders: Experience from a tertiary neuroscience centre

Therapeutic plasma exchange (TPE) involves the extracorporeal separation of plasma from the cellular components of blood with replacement fluid, such as human albumin or fresh frozen plasma. A number of studies across the world revealed that more than one third of TPE procedures were performed for neurological disorders.Myasthenia gravis (MG), Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyneuropathy (CIDP) were the most frequently cited indications for TPE, followed by multiple sclerosis (MS). However, treatments of these conditions have evolved over the years and it is likely that this has impacted on clinical practice. Here we present our experience of using TPE to treat neurological disorders.We reviewed the medical records of all 63 patients who received 349 procedures over 70 therapeutic cycles between 2012 and 2015 in a tertiary neurology centre.In total only 2 patients with GBS and MG were treated with TPE. The commonest indication was voltage gated potassium channel (VGKC) complex antibody associated disorders followed by CIDP and MS. There were 11 patients with limbic encephalitis. Nine of them had antibodies against VGKC complex and two had N-methyl-D-aspartate (NMDA) receptor antibodies. Sixty four percent of patients with limbic encephalitis and overall 78% of patients responded to TPE. The complication rate associated with this procedure was 8.6 per 100 therapeutic cycle. There was no treatment related mortality.We observed a change in indications of TPE compared to historical studies. It was less frequently used to treated GBS and MG. It was found to be safe and effective.

Guilty by Association: KCTD16 and GABABR Antibodies in Paraneoplastic Limbic Encephalitis.

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Auto immune limbic encephalitis; clinical profile and treatment response – a single center experience

Auto immune limbic encephalitis are usually antibody mediated and may be a paraneoplastic manifestation. We report a case series of thirteen patients were admitted with features suggestive of auto-immune encephalitis from January 2014 and March 2019. Age range was 18–37, with male: female ratio of 1:4.5. All of the patients had prodromal respiratory catarrh a week or two preceding onset of neurological symptoms. Psychosis was a prominent initial symptom in all patients. Extrapyramidal symptoms were seen in six. Seizures occurred in eleven patients, three of whom were refractory. Three had associated autonomic instability. Antibody testing was positive for VGKC antibodies in five, anti NMDA antibodies in six and two patients were seronegative for any antibodies. MRI was normal in anti NMDA encephalitis. It showed mesial temporal swelling/hyper intensities in patients with anti VGKC encephalitis, two of whom developed mesial temporal sclerosis at one year follow up. Antibody titers did not correlate with clinical manifestations. Paraneoplastic work up was positive for ovarian teratoma in one patient with anti NMDA encephalitis who had refractory encephalopathy despite tumor removal and aggressive immunotherapy. Most patients had near complete recovery with treatment (methyl prednisolone, immunoglobulins and/or PLEX). Most patients with anti VGKC encephalitis required long term anti epileptic medications. Prominent psychosis, dysautonomia and extrapyramidal symptoms after a prodrome of respiratory catarrh warrant early consideration of autoimmune encephalitis since early treatment initiation determines the outcome. Paraneoplastic etiology portends a poor prognosis.

Limbic encephalitis as the presenting feature of sjögren syndrome

Autoimmune encephalitis associated with serum and intrathecal antibodies against intracellular and surface neuronal antigens of the limbic system neuropiles, and several neuropsychiatric and neurocognitive disorders have shared mechanisms of origin. Sicca syndrome is present in only 50% of patients with neurologic involvement. However, limbic encephalitis (LE) has been recognized on occasion as a feature of Sjogren’s syndrome (SS). A 40-years-old male patient presented with sudden development of headache, episodic memory failure, focal cognitive epileptic and generalized seizures, fever, complex visual hallucinations, fluctuations in awareness. Neurological examination showed drowsiness, as well as high freceuncy generalized tremor at rest. MRI showed an area of increased signal on fluid-attenuated inversion recovery (FLAIR) sequence bilaterally in the temporal lobe. CSF analysis showed a high protein level; viral screening and bacterial cultures, paraneoplastic antibody panel, anti NMDA were negative, EEG showed bilateral temporal focal slowing, PET scan was negative for malignancy, Anti Ro / SSA and anti La / SSB antibodies were elevated, Schirmer test was positive for reduced tear production and minor salivary gland biopsy demonstrated focal lymphocytic sialadenitis. Treatment with azathioprine and prednisone was started as well as rituximab every 6 months with marked improvement shown by lack of seizure recurrence; However, episodic memory failure remained. Recognizing the association between MRI temporal lobe lesions and LE should prompt further evaluation for paraneoplastic and autoantibody-related entities, including SS. This is the first case where the association of neuropsychiatric symptoms is described between LE and SS.

Imaging of other encephalitis and of noncompressive acute transverse myelopathies

Acute encephalitis syndrome (AES) is characterised with acute onset of fever and altered sensorium. Flavivirus infections (West Nile, Dengue, Zika and Japanese encephalitis) are common causes of AES. Subacute sclerosing panencephalitis can present acutely as AES. Bacteria, fungi and parasites are other causes of encephalitis. Autoimmune encephalitis describes a group of brain disorders associated with antineuronal autoantibodies Neuroimaging in various encephalitis is often distinctive. Herpes simplex virus type 1, for example, preferentially affects the mesial temporal lobes. In flavivirus brain infections deep grey matter and periventricular white matter involvement are common. In autoimmune limbic encephalitis, there is preferential involvement of the amygdala and hippocampus. In bacterial, fungal and parasitic disorders, single or multiple enhancing lesions of brain are demonstrated. The term “transverse myelopathies” defines a group of disorders that involves few segments of spinal cord. It clinically manifests with motor, sensory, and bladder involvement. Acute transverse myelopathies, most frequently, are autoimmune in nature. Next, common causes are demyelinating like multiple sclerosis or neuromyelitis optica (NMO) spectrum disorders. A variety of infections, like virus, can directly affect the spinal cord. Acute flaccid myelitis is a polio-like illness. MRI demonstrates a longitudinal lesion in the spinal grey matter. In endemic regions, Mycobacterium tuberculosis is a common cause of transverse myelitis. In transverse myelitis, T2-weighted spinal cord images typically demonstrates hyperintense signals. Pattern of imaging abnormalities differs in different diseases, in multiple sclerosis <2 segments of spinal cord are typically partially involved. In other transverse myelopathies like NMO >3 segments are involved.