CLINICAL MANIFESTATIONS OF ANTI-NMDA RECEPTOR ENCEPHALITIS IN CHILDREN

The aim of the study was to determine the clinical features of anti-NMDAR encephalitis in children. Materials and methods of research: 11 patients were selected from the group of children with autoimmune encephalitis, who met the diagnostic criteria for reliable anti-NMDAR encephalitis. A retro-prospective analysis of clinical symptoms, laboratory, neurophysiological and neuroimaging data, treatment, duration of primary hospitalization and long-term results of treatment was carried out, neuropsychological testing of patients was performed in the follow-up. Results: the age of the patients was 8.5±4.4 years, the gender composition of boys/girls was 1/10 (9.1%/90.9%). The average follow-up period was 17±12 months. In 73% of cases, there was an acute onset of the disease without a prodromal phase and with rapidly growing signs of neurological dysfunction: behavior change (11/100%), epileptic seizures (11/100%), speech impairment (10/90.9%), movement disorders (10/90.9%), disturbed sleep/wakefulness rhythm (9/81.8%), hallucinations (5/45.5%), autonomic disorders (6/54.5%), sensory disturbance (1/9,1%). In 82% of cases, therapy was required in the intensive care unit. When analyzing EEG monitoring in children, the extreme delta brush pattern was revealed in only one patient. Neuroimaging revealed no specific changes in the substance of the brain. The paraneoplastic nature of the disease could not be established in any case. 4 patients (36.36%) had relapses of the disease after 1.5–27 months. from the onset of the disease. All children showed a favorable outcome without a gross neurological deficit, but complaints of increased fatigue, headaches, poor memory, a decrease in vocabulary, and impulsive behavior persisted.

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Autoimmune encephalitis in a South Asian population

BMC Neurology ◽

10.1186/s12883-021-02232-6 ◽

2021 ◽

Vol 21 (1) ◽

Author(s):

Nilanka Wickramasinghe ◽

Dhanushka Dasanayake ◽

Neelika Malavige ◽

Rajiva de Silva ◽

Thashi Chang

Keyword(s):

Sri Lanka ◽

Psychiatric Symptoms ◽

South Asians ◽

Laboratory Data ◽

Clinical Manifestations ◽

Autoimmune Encephalitis ◽

Asian Population ◽

South Asian Population ◽

Clinical Presentations

Abstract Background Autoimmune encephalitis (AE) is now considered a main, potentially curable cause of encephalitis, but remains conspicuously underreported from South Asia. We studied the clinical characteristics in relation to their antibody status and outcomes of patients presenting with AE in Sri Lanka. Methods Patients admitting to government hospitals who were clinically suspected of AE by an on-site neurologist were prospectively recruited over a period of 12 months. Sera and cerebrospinal fluid were tested for NMDAR, AMPAR1, AMPAR2, LGI1, CASPR2, GABARB1/B2 antibodies (Ab) using commercial cell-based assays. Demographic, clinical and laboratory data were compiled into an investigator-administered proforma. Patients were reviewed at 1 year follow up either in person or via telephone. Results One-hundred and forty-two patients from 21 of 25 districts in Sri Lanka (median age = 20.5 years; range 1–86 years; females = 61.3%) were recruited. Of them, 65 (45.8%; median age = 19 years; range 1–86 years; females = 64.6%) fulfilled diagnostic criteria for probable NMDAR-antibody encephalitis (NMDARE) and 6 (4.2%; median age = 44 years; range 28–71 years; females = 83.3%) limbic encephalitis (LE). Abnormal behaviour (95.3%), seizures (81.5%) and movement disorders (69.2%) were the most frequent clinical manifestations of probable NMDARE. NMDAR-antibodies were detectable in 29 (44.6%) and not detectable in 36 in CSF of probable-NMDARE patients. Abnormal EEG was more frequent (p = 0.003) while a worse outcome (OR = 2.78; 95% CI = 0.88–9.09) and deaths (OR = 2.38; 95% CI = 0.67–8.33) were more likely in antibody-negative than antibody-positive probable-NMDARE. Most patients with LE had amnesia (50%) and/or confusion (100%) with agitation (83.3%) and seizures (100%) but none had detectable antibodies to any of the antigens tested. Conclusions NMDARE is the commonest type of AE among South Asians as is the case worldwide. Clinical presentations of NMDARAb-positive and NMDARAb-negative AE patients do not significantly differ but EEG may be a useful marker of an autoimmune basis for psychiatric symptoms.

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The diffuse involvement of anti-N-methyl-D-aspartate receptor encephalitis in brain: a case report

BMC Neurology ◽

10.1186/s12883-019-1456-6 ◽

2019 ◽

Vol 19 (1) ◽

Cited By ~ 1

Author(s):

Yun Jiang ◽

Jianpeng Ma ◽

Tao Gong ◽

Hongjun Hao ◽

Haibo Chen

Keyword(s):

Basal Ganglia ◽

Brain Mri ◽

Clinical Manifestations ◽

Autoimmune Encephalitis ◽

Ovarian Teratoma ◽

Mass Effects ◽

Glucose Levels ◽

Aspartate Receptor ◽

Nmdar Encephalitis ◽

Case Presentations

Abstract Background Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a severe and most common autoimmune encephalitis in patients under 40 years old. Anti-NMDAR encephalitis has various clinical and neuroimaging findings. Here we report a special case of an anti-NMDAR encephalitis who had diffuse lesions in bilateral hemispheres with mild mass effects in left basal ganglia area. Case presentations A 28-year-old female anti-NMDAR encephalitis patient mainly presented with headache and fever. Brain magnetic resonance image (MRI) showed slightly contrasted diffuse lesions, involving the left temporal and frontal lobes, left basal ganglia area and splenium of corpus callosum, as well as the right frontal lobe, with mild edema surrounded in the left basal ganglia area. Cerebrospinal fluid (CSF) revealed a moderate pleocytosis with normal protein and glucose levels. Anti-NMDAR antibodies were identified in CSF. Transvaginal ovarian ultrasound did not reveal an ovarian teratoma. The patient was treated with immunoglobulin and steroid, and had a good recovery. Conclusions Anti-NMDAR encephalitis has no special clinical manifestations and brain MRI is highly variable, which could be unremarkable or abnormal involving white and grey matters. The extensive lesions in frontal and temporal lobes, and basal ganglia area, with mild mass effects, have not been described previously. Recognition of various changes in brain MRI will enable the early detection of anti-NMDAR antibody and then effective treatments.

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Percutaneous Closure of PFO in Patients with Reduced Oxygen Saturation at Rest and during Exercise: Short- and Long-Term Results

Journal of Interventional Cardiology ◽

10.1155/2020/9813038 ◽

2020 ◽

Vol 2020 ◽

pp. 1-8

Author(s):

Céline De Cuyper ◽

Tristan Pauwels ◽

Eric Derom ◽

Michel De Pauw ◽

Daniël De Wolf ◽

...

Keyword(s):

Oxygen Saturation ◽

Clinical Symptoms ◽

Systemic Circulation ◽

Percutaneous Closure ◽

Long Term Results ◽

Sf 36 ◽

Short And Long Term

Background. A patent foramen ovale (PFO) is a rare cause of hypoxemia and clinical symptoms of dyspnea. Due to a right-to-left shunt, desaturated blood enters the systemic circulation in a subset of patients resulting in dyspnea and a subsequent reduction in quality of life (QoL). Percutaneous closure of PFO is the treatment of choice. Objectives. This retrospective multicentre study evaluates short- and long-term results of percutaneous closure of PFO in patients with dyspnea and/or reduced oxygen saturation. Methods. Patients with respiratory symptoms were selected from databases containing all patients percutaneously closed between January 2000 and September 2018. Improvement in dyspnea, oxygenation, and QoL was investigated using pre- and postprocedural lung function parameters and two postprocedural questionnaires (SF-36 and PFSDQ-M). Results. The average follow-up period was 36 [12–43] months, ranging from 0 months to 14 years. Percutaneous closure was successful in 15 of the 16 patients. All patients reported subjective improvement in dyspnea immediately after device deployment, consistent with their improvement in oxygen saturation (from 90 ± 6% to 94 [92–97%] on room air and in upright position) (p<0.05). Both questionnaires also indicated an improvement of dyspnea and QoL after closure. The two early and two late deaths were unrelated to the procedure. Conclusion. PFO-related dyspnea and/or hypoxemia can be treated successfully with a percutaneous intervention with long-lasting benefits on oxygen saturation, dyspnea, and QoL.

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The diffuse involvement of Anti-N-methyl-D-aspartate receptor encephalitis in brain: a case report

10.21203/rs.2.1815/v1 ◽

2019 ◽

Author(s):

Yun Jiang ◽

Jianpeng Ma ◽

Tao Gong ◽

Hongjun Hao ◽

Haibo Chen

Keyword(s):

Basal Ganglia ◽

Brain Mri ◽

Clinical Manifestations ◽

Autoimmune Encephalitis ◽

Ovarian Teratoma ◽

Mass Effects ◽

Glucose Levels ◽

Aspartate Receptor ◽

Nmdar Encephalitis ◽

Case Presentations

Abstract Background Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a severe and most common autoimmune encephalitis in patients under 40 years old. The anti-NMDAR encephalitis has various clinical and neuroimaging findings. Here we report a special anti-NMDAR encephalitis patient who had diffuse lesions in bilateral hemispheres with mild mass effects in left basal ganglia area. Case presentations A 28-year-old female anti-NMDAR encephalitis patient mainly presented with headache and fever. Brain magnetic resonance image (MRI) showed slightly contrasted diffuse lesions, involving the left temporal and frontal lobes, left basal ganglia area and splenium of corpus callosum, and the right frontal lobe, with mild edema surrounded in the left basal ganglia area. Cerebrospinal fluid (CSF) revealed a moderate pleocytosis with normal protein and glucose levels. Anti-NMDAR antibodies were identified in CSF. Transvaginal ovarian ultrasound did not reveal an ovarian teratoma. The patient was treated with immunoglobulin and steroid, and had a good recovery. Conclusions The anti-NMDAR encephalitis has no special clinical manifestations and brain MRI is highly variable, which could be unremarkable or abnormal involving white and grey matters. The extensive lesions in frontal and temporal lobes, and basal ganglia area, with mild mass effects, have not been described previously. The recognition of various changes in brain MRI will enable the early detection of anti-NMDAR antibody and then effective treatments.

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Autoimmune encephalitis associated with antibodies against the metabotropic glutamate receptor type 1: case report and review of the literature

Therapeutic Advances in Neurological Disorders ◽

10.1177/1756286419847418 ◽

2019 ◽

Vol 12 ◽

pp. 175628641984741 ◽

Cited By ~ 4

Author(s):

Monika Christ ◽

Torsten Müller ◽

Corinna Bien ◽

Thomas Hagen ◽

Markus Naumann ◽

...

Keyword(s):

Cerebellar Ataxia ◽

Glutamate Receptor ◽

Metabotropic Glutamate Receptor ◽

Clinical Symptoms ◽

Clinical Course ◽

Autoimmune Encephalitis ◽

Receptor Type ◽

Metabotropic Glutamate

Autoimmune encephalitis associated with antibodies against the metabotropic glutamate receptor type 1 is a rare autoimmune disease with only 18 cases being described in the literature so far. Most patients present with subacute cerebellar ataxia. In more than one third of cases a paraneoplastic aetiology has been suspected. Here we report a case of a 45-year-old man without known malignancy, who presented with progressive dysarthria and subsequently developed subacute cerebellar ataxia. Immunotherapy with glucocorticoids, i.v. immunoglobulins and rituximab improved clinical symptoms and resulted in a stable disease course up to the present. The article describes the clinical course of the patient with a follow-up-period of approximately 24 months and reviews the cases reported in the literature so far.

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Current Progress on Assessing the Prognosis for Anti-N-Methyl-D-Aspartate Receptor (NMDAR) Encephalitis

BioMed Research International ◽

10.1155/2020/7506590 ◽

2020 ◽

Vol 2020 ◽

pp. 1-8

Author(s):

Hao Wang ◽

Zheng Xiao

Keyword(s):

Positive Impact ◽

Treatment Options ◽

Clinical Manifestations ◽

Autoimmune Encephalitis ◽

Antibody Testing ◽

Aspartate Receptor ◽

Nmdar Encephalitis ◽

Behavioral Abnormalities ◽

Positron Emission ◽

The Impact

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is the most common antineuronal antibody encephalitis in autoimmune encephalitis found at present. It has complex clinical manifestations such as psychiatric and behavioral abnormalities, seizures, movement disorders, consciousness, and autonomic dysfunction. The relationship between those manifestations and prognosis is unclear. Electroencephalography (EEG) is gradually becoming useful in the evaluation of the prognosis of anti-NMDAR encephalitis patients, while imaging and antibody testing have a limited prognostic value. Starting the patients on adequate treatments (such as immunotherapy) in a timely fashion has a positive impact on their prognosis. Nevertheless, research on the prognosis of anti-NMDAR encephalitis remains scarce. Here, we review the current advances of prognosis-related research from the clinical manifestations of the disease and auxiliary examinations such as EEG, magnetic resonance imaging (MRI), 18F fluorodeoxyglucose positron emission tomography (FDG-PET), and antibody measurement. In addition, we also discuss the impact of different treatment options on prognosis. In-depth research on the prognosis of patients with anti-NMDAR encephalitis will contribute to a better understanding of this disease, leading to better treatments options and, ultimately, a better prognosis.

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Anti-N-methyl-D-Aspartate Receptor Encephalitis Mimicking Sporadic Creutzfeldt–Jakob Disease

Frontiers in Neurology ◽

10.3389/fneur.2020.593680 ◽

2020 ◽

Vol 11 ◽

Author(s):

Jiao Liu ◽

Liyan Chen ◽

Jing Yang ◽

Lan Wang ◽

Huifang Shang ◽

...

Keyword(s):

Psychiatric Symptoms ◽

Clinical Manifestations ◽

Progressive Dementia ◽

Memory Problems ◽

Aspartate Receptor ◽

Nmdar Encephalitis ◽

Jakob Disease ◽

Laboratory Investigations ◽

Positive Results

Objectives: Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis and sporadic Creutzfeldt–Jakob disease (sCJD) share similar clinical features. Here, we present two unusual cases of anti-NMDAR encephalitis who were misdiagnosed as sCJD at first.Methods: We described two patients' clinical manifestations, as well as the string of symptomatological evolution, treatments, and follow-up results.Results: Our patients presented with rapidly progressive dementia, memory problems, psychiatric symptoms, and movement disorders, and we considered all these symptoms as a presenting feature of sCJD at first, but the cerebrospinal fluid examination showed positive results for both the 14-3-3 protein and antibodies against NMDAR. Immunomodulatory treatment led to a resolution of these deficits, and both of them remained in remission after treatment.Conclusion: Anti-NMDAR encephalitis can present with rapidly progressive cognitive decline, and sometimes laboratory investigations can be misleading. The examination for the presence of NMDAR antibodies is necessary, even with the presence of 14-3-3 protein. Early immunomodulatory therapy should be considered, especially for patients with high titers of NMDAR antibodies.

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Anti-NMDAR Encephalitis Following Herpes Simplex Virus Encephalitis: 2 Cases From Portugal

The Neurohospitalist ◽

10.1177/1941874419870987 ◽

2019 ◽

Vol 10 (2) ◽

pp. 133-138

Author(s):

Ana Brás ◽

Ana André ◽

Laura Sá ◽

João Carvalho ◽

Anabela Matos ◽

...

Keyword(s):

Herpes Simplex Virus ◽

Herpes Simplex ◽

Specific Treatment ◽

Autoimmune Encephalitis ◽

Herpes Simplex Virus Encephalitis ◽

Virus Encephalitis ◽

Nmdar Encephalitis ◽

Initial Improvement ◽

Simplex Virus

Herpes simplex virus encephalitis (HSVE) usually presents as a monophasic disease. Symptomatic HSVE relapsing with seizures, encephalopathy, or involuntary movements associated with anti- N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis have been recently reported. We report 2 cases of adult post-HSVE anti-NMDAR encephalitis from Portugal. Two female patients aged 50 years and 30 years were diagnosed with herpes simplex virus type 2 and type 1 encephalitis, respectively. After the initial improvement with specific treatment and despite virologic negativization, both patients suffered clinical, electroencephalographic, and imaging deterioration. The autoimmune encephalitis hypothesis was confirmed with the demonstration of anti-NMDAR antibodies in both cerebrospinal fluid and serum. Both responded to human immunoglobulin and methylprednisolone, with progressive gain of autonomy along the follow-up period. Thymectomy for thymic hyperplasia diagnosed during follow-up was performed in 1 patient. Although being rare, post-HSVE anti-NMDAR encephalitis should be considered in all cases of symptomatic recrudescence after HSVE, since adequate immune-modulating treatment improves the outcome. The role of thyme hyperplasia in autoimmune encephalitis pathogenesis needs better understanding.

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Spinal Capillary Hemangioma: Report of Five Surgical Cases and Literature Review

Translational Neuroscience and Clinics ◽

10.18679/cn11-6030_r.2017.029 ◽

2017 ◽

Vol 3 (4) ◽

pp. 196-203

Author(s):

Liang Zhang ◽

Zhifeng Zhang ◽

Jifeng Shang ◽

Wenqing Jia ◽

Jun Yang ◽

...

Keyword(s):

Management Strategy ◽

Clinical Symptoms ◽

Clinical Manifestations ◽

Capillary Hemangioma ◽

Neurological Deficits ◽

En Bloc ◽

Limb Weakness ◽

Intradural Extramedullary ◽

Early Surgical Intervention

Objective Capillary hemangioma is a benign vascular malformation that is usually encountered in soft-tissue. Rarely, it may occur in the neuraxis, and spinal capillary hemangioma (SCH) is a rare variant of it. Existing literature on SCH is limited because of its rarity. As a result, epidemiological and clinical characteristics as well as management strategy for SCH are still lacking. Here, we present a report on five patients with pathologically proven SCH, treated in Beijing Tiantan Hospital between 2013 and 2015. Methods Patients' age, gender, clinical manifestations, radiological features, operative methods, and surgical outcomes were retrospectively reviewed, and an updated review of the literature was also provided. Results Four patients were men and one was a woman, with a median age at presentation of 43 years (range: 15–66 years). Two lesions were intramedullary, two epidural, and one intradural extramedullary. The thoracic segment was most commonly affected (n = 3, 60%), followed by the cervical (n = 1, 20%) and lumbar (n = 1, 20%) segments. Common symptoms, in descending order, were numbness and paresthesia, limb weakness, and pain. The surgical procedure was successfully performed with total resection of the tumor achieved in 4 patients and subtotal in 1 patient. During an average follow-up period of 32 months (range: 27–43 months), recovery of the clinical symptoms was observed in all five patients. Conclusions SCH is a rare benign vascular disease, for which surgical resection of the lesion, by the en bloc method, is recommended. Clinically, it usually manifests with progressive myelopathy, but early surgical intervention usually produces good results and may prevent permanent neurological deficits.

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Left renal vein stenting: indications, disputable points, immediate and long-term results

Andrology and Genital Surgery ◽

10.17650/1726-9784-2021-22-1-28-37 ◽

2021 ◽

Vol 22 (1) ◽

pp. 28-37

Author(s):

A. Е. Vasiliev ◽

О. В. Zhukov ◽

V. Е. Sinitsyn ◽

A. N. Sulima

Keyword(s):

Renal Vein ◽

Left Renal Vein ◽

Clinical Manifestations ◽

Long Term Results ◽

Technical Performance ◽

Significant Compression

The article presents the results of stenting of the left renal vein in 35 patients with proven hemodynamically significant compression of the vein in “aorto-mesenteric tweezers”. Clinical manifestations of lesions of the left renal vein are very diverse, and pathogenetic mechanisms remain not fully understood. Therefore, the determination of indications for stenting and perfect technical performance are predictors of success in the near and long-term periods of postoperative follow-up. Our experience in stenting the left renal vein demonstrates the possibility of achieving intraoperative technical success in 100 % of cases, as well as a significant improvement in the quality of life in most patients after endovascular intervention, due to a significant reduction in the clinical manifestations of the pathological phenomenon.

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