Differences and diversity of autoimmune-mediated encephalitis in 77 cases from a single tertiary care center

Abstract Background The incidence of autoimmune encephalitis has risen globally. There are two general categories of disease-associated antibodies that can be tested for: neuronal surface and intracellular. However, testing both groups of autoantibodies are costly. This study aims to identify differences between groups by comparing clinical presentations, radiological findings and CSF profile of patients, and determine if any parameters are indicative of one group of autoantibodies over another. Additionally, we aim to report the local incidence of less common groups of disease-associated antibodies as well. Methods Seventy-seven records of autoimmune encephalitis/encephalomyelitis patients admitted to King Chulalongkorn Memorial Hospital, Bangkok, Thailand, between October 2010 and February 2017 were reviewed. Patients with infections or those with classic central nervous system demyelinating features were excluded. Results Of 77 patients, 40% presented with neuronal surface antibodies and 33% had intracellular antibodies. The most common autoantibody detected in each group was anti-NMDAr antibody (25/31, 81%) and anti-Ri antibody (7/25, 28%) respectively. In the neuronal surface antibody group, behavioral change was the most common complaint (45%), followed by seizures (39%) and abnormal movements (29%). In the latter group, seizure was the most common presenting symptom (32%), followed by motor weakness (20%), behavioural change (16%) and abnormal movements (16%). Patients with neuronal surface antibodies were younger (35 vs 48 years old, p = 0.04) and more likely to present with behavioral change (45% vs 16%, p = 0.02). Mortality rate was higher in the intracellular group (16% vs 3.2%, p = 0.09). No differences were detected in magnetic resonance imaging (MRI) and CSF profile. Conclusions In the early stages of the disease, both groups have comparable clinical outcomes. Although there were significant differences in age and percentage of patients with behavioral change, both groups of autoimmune encephalitis still shared many clinical features and could not be distinguished based on MRI and CSF profiles. Therefore, we recommend that patients with features of autoimmune encephalitis should be screened for both the neuronal surface and intracellular antibodies regardless of clinical presentation.

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Differences and diversity of autoimmune-mediated encephalitis in 77 cases from a single tertiary care center

10.21203/rs.2.1811/v4 ◽

2019 ◽

Author(s):

Abhinbhen Wasontiwong Saraya ◽

Kanthita Worachotsueptrakun ◽

Kritchai Vutipongsatorn ◽

Chanikarn Sonpee ◽

Thiravat Hemachudha

Keyword(s):

Behavioral Change ◽

Care Center ◽

Behavioural Change ◽

Tertiary Care ◽

Autoimmune Encephalitis ◽

Motor Weakness ◽

Abnormal Movements ◽

Clinical Presentations ◽

Magnetic Resonance Imaging Mri ◽

Surface Antibodies

Abstract Background The incidence of autoimmune encephalitis has risen globally. There are two general categories of disease-associated antibodies that can be tested for: neuronal surface and intracellular. However, testing both groups of autoantibodies are costly. This study aims to identify differences between groups by comparing clinical presentations, radiological findings and CSF profile of patients, and determine if any parameters are indicative of one group of autoantibodies over another. Additionally, we aim to report the local incidence of less common groups of disease-associated antibodies as well. Methods 77 records of autoimmune encephalitis/encephalomyelitis patients admitted to King Chulalongkorn Memorial Hospital, Bangkok, Thailand, between October 2010 and February 2017 were reviewed. Patients with infections or those with classic central nervous system demyelinating features were excluded. Results Of 77 patients, 40% presented with neuronal surface antibodies and 33% had intracellular antibodies. The most common autoantibody detected in each group was anti-NMDAr antibody (25/31, 81%) and anti-Ri antibody (7/25, 28%) respectively. In the neuronal surface antibody group, behavioral change was the most common complaint (45%), followed by seizures (39%) and abnormal movements (29%). In the latter group, seizure was the most common presenting symptom (32%), followed by motor weakness (20%), behavioural change (16%) and abnormal movements (16%). Patients with neuronal surface antibodies were younger (35 vs 48 years old, p=0.04) and more likely to present with behavioral change (45% vs 16%, p=0.02). Mortality rate was higher in the intracellular group (16% vs 3.2%, p=0.09). No differences were detected in magnetic resonance imaging (MRI) and CSF profile. Conclusions In the early stages of the disease, both groups have comparable clinical outcomes. Although there were significant differences in age and percentage of patients with behavioral change, both groups of autoimmune encephalitis still shared many clinical features and could not be distinguished based on MRI and CSF profiles. Therefore, we recommend that patients with features of autoimmune encephalitis should be screened for both the neuronal surface and intracellular antibodies regardless of clinical presentation.

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Differences and diversity of autoimmune-mediated encephalitis in 77 cases from a single tertiary care center

10.21203/rs.2.1811/v1 ◽

2019 ◽

Author(s):

Abhinbhen Wasontiwong Saraya ◽

Kanthita Worachotsueptrakun ◽

Kritchai Vutipongsatorn ◽

Chanikarn Sonpee ◽

Thiravat Hemachudha

Keyword(s):

Behavioral Change ◽

Care Center ◽

Tertiary Care ◽

Autoimmune Encephalitis ◽

Numerical Data ◽

Clinical Findings ◽

Exact Test ◽

Abnormal Movements ◽

Surface Antibody ◽

Surface Antibodies

Abstract Background Since the discovery of N-methyl-D-aspartate receptor (NMDAr) antibody in 2007, the incidence of autoantibody-mediated encephalitis has risen globally. Here we analyzed and compared groups of autoantibody-associated encephalitis patients based on clinical findings and laboratory results in order to find differences between two major groups of autoantibody-mediated encephalitis: intracellular and neuronal surface antibodies. Methods 77 records of autoimmune encephalitis/encephalomyelitis patients admitted to King Chulalongkorn Memorial Hospital (KCMH) between October 2010 and February 2017 were reviewed. Patients with infections or those with classic central nervous system demyelinating features were excluded. Categorical data was analyzed using chi-square and Fisher’s exact test. Unpaired, two-tailed t-test was performed to analyze numerical data. Results Of 77 patients, 40% presented with neuronal surface antibodies and 33% had intracellular antibodies. The most common autoantibody detected in each group was anti-NMDA receptor antibody (25/31, 81%) and anti-Ri antibody (7/25, 28%) respectively. In the neuronal surface antibody group, behavioral change was the most common complaint (45%), followed by seizures (39%), abnormal movements (29%) and psychosis/mood disorder (23%). In the latter group, seizure was the most common presenting symptom (32%), followed by motor weakness (20%), behavioral change (16%) and abnormal movements (16%). Patients with neuronal surface antibodies were significantly younger (35 vs 48 years old, p=0.04) and were more likely to present with behavioral change (45% vs 16%, p=0.02). Mortality rate was higher in the intracellular group although this was statistically insignificant (16% vs 3.2%, p=0.09). No significant differences were detected in magnetic resonance imaging and cerebrospinal fluid (CSF) profile. Conclusions The prevalence of neuronal surface antibody group was much higher than the intracellular group. In the earlier stages of the disease, both groups have comparable clinical outcomes. Furthermore, it is difficult to distinguish autoantibody-associated encephalitis patients based on clinical data, neuroimaging and CSF profile. Therefore, we recommend that patients with features of autoimmune encephalitis should be screened for both the neuronal surface and intracellular antibodies regardless of clinical presentation.

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Differences and diversity of autoimmune-mediated encephalitis in 77 cases from a single tertiary care center

10.21203/rs.2.1811/v2 ◽

2019 ◽

Author(s):

Abhinbhen Wasontiwong Saraya ◽

Kanthita Worachotsueptrakun ◽

Kritchai Vutipongsatorn ◽

Chanikarn Sonpee ◽

Thiravat Hemachudha

Keyword(s):

Behavioral Change ◽

Care Center ◽

Tertiary Care ◽

Autoimmune Encephalitis ◽

Numerical Data ◽

Clinical Findings ◽

Exact Test ◽

Abnormal Movements ◽

Surface Antibody ◽

Surface Antibodies

Abstract Background Since the discovery of N-methyl-D-aspartate receptor (NMDAr) antibody in 2007, the incidence of autoantibody-mediated encephalitis has risen globally. Here we analyzed and compared groups of autoantibody-associated encephalitis patients based on clinical findings and laboratory results in order to find differences between two major groups of autoantibody-mediated encephalitis: intracellular and neuronal surface antibodies. Methods 77 records of autoimmune encephalitis/encephalomyelitis patients admitted to King Chulalongkorn Memorial Hospital (KCMH) between October 2010 and February 2017 were reviewed. Patients with infections or those with classic central nervous system demyelinating features were excluded. Categorical data was analyzed using chi-square and Fisher’s exact test. Unpaired, two-tailed t-test was performed to analyze numerical data. Results Of 77 patients, 40% presented with neuronal surface antibodies and 33% had intracellular antibodies. The most common autoantibody detected in each group was anti-NMDA receptor antibody (25/31, 81%) and anti-Ri antibody (7/25, 28%) respectively. In the neuronal surface antibody group, behavioral change was the most common complaint (45%), followed by seizures (39%), abnormal movements (29%) and psychosis/mood disorder (23%). In the latter group, seizure was the most common presenting symptom (32%), followed by motor weakness (20%), behavioral change (16%) and abnormal movements (16%). Patients with neuronal surface antibodies were significantly younger (35 vs 48 years old, p=0.04) and were more likely to present with behavioral change (45% vs 16%, p=0.02). Mortality rate was higher in the intracellular group although this was statistically insignificant (16% vs 3.2%, p=0.09). No significant differences were detected in magnetic resonance imaging and cerebrospinal fluid (CSF) profile. Conclusions The prevalence of neuronal surface antibody group was much higher than the intracellular group. In the earlier stages of the disease, both groups have comparable clinical outcomes. Furthermore, it is difficult to distinguish autoantibody-associated encephalitis patients based on clinical data, neuroimaging and CSF profile. Therefore, we recommend that patients with features of autoimmune encephalitis should be screened for both the neuronal surface and intracellular antibodies regardless of clinical presentation.

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Scrub Typhus with Varied Clinical Presentations

Journal of Pediatric Infectious Diseases ◽

10.1055/s-0037-1605363 ◽

2017 ◽

Vol 13 (04) ◽

pp. 319-322

Author(s):

Susrita Banerjee ◽

Satyabrata Roychowdhuri ◽

Mihir Sarkar

Keyword(s):

Scrub Typhus ◽

Care Center ◽

Tertiary Care ◽

Clinical Manifestations ◽

Case Series ◽

Dengue Shock Syndrome ◽

Tertiary Care Center ◽

Eastern India ◽

Clinical Presentations ◽

Diagnostic Difficulties

AbstractWe present four cases of pediatric scrub typhus from a tertiary care center of eastern India, emphasizing on varied unusual clinical manifestations posing diagnostic difficulties. A low index of suspicion is required to prevent under diagnosis. We report a case series of four patients with scrub typhus having uncommon clinical manifestations (two patients with hemophagocytic lymphohistiocytosis, one patient with myocarditis, and one patient presenting with shock reminiscent of dengue shock syndrome). The response to doxycycline was good, with fever subsiding within 48 to 72 hours of starting the treatment. Three out of four cases completely recovered once appropriate medication was given.

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Cerebrospinal Fluid Pleocytosis in Critical Care Patients With Seizures

Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques ◽

10.1017/cjn.2016.442 ◽

2017 ◽

Vol 44 (4) ◽

pp. 343-349

Author(s):

Carly Scramstad ◽

Alan C. Jackson

Keyword(s):

Cerebrospinal Fluid ◽

Critical Care ◽

Care Center ◽

Tertiary Care ◽

Retrospective Chart Review ◽

Critical Care Units ◽

Generalized Seizures ◽

Csf Pleocytosis ◽

Magnetic Resonance Imaging Mri ◽

Critical Care Patients

AbstractObjectives: To assess the etiology of cerebrospinal fluid (CSF) pleocytosis in critical care patients with seizure(s) or status epilepticus (SE). Many previous studies, some performed decades ago, concluded that CSF pleocytosis may be entirely attributable to seizure activity. Methods: We undertook a retrospective chart review of adult patients with an admitting or acquired diagnosis of seizure(s) or SE in critical care units at the Winnipeg Health Sciences Centre between 2009 and 2012. Patients were identified through a critical care information database at a tertiary care center. We limited our study to patients who had lumbar punctures at our center within 5 days of seizure(s) or SE. Results: Of 426 patients with seizures in critical care units, 51 met the inclusion criteria. Seizure subtypes included focal seizures (5 or 10%), generalized seizures (14 or 27%), and SE (32 or 63%). Twelve (seven with SE) of the 51 (24%) were found to have CSF pleocytosis. A probable etiological cause for the CSF pleocytosis was identified in all 12 cases. Conclusions: We conclude that seizures do not directly induce a CSF pleocytosis. Instead, the CSF pleocytosis more likely reflects the underlying acute or chronic brain process responsible for the seizure(s). This was not readily apparent in early studies without magnetic resonance imaging (MRI) of the brain and currently available laboratory investigations. An etiological cause of CSF pleocytosis must always be sought when patients present with seizures and it should never be assumed that seizures are the cause.

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Rapid Growth of Acoustic Neuromas after Stereotactic Radiotherapy in type 2 Neurofibromatosis

Ear Nose & Throat Journal ◽

10.1177/014556130208101210 ◽

2002 ◽

Vol 81 (12) ◽

pp. 831-833 ◽

Cited By ~ 16

Author(s):

Steven Y. Ho ◽

John F. Kveton

Keyword(s):

Stereotactic Radiotherapy ◽

Rapid Growth ◽

Rare Complication ◽

Care Center ◽

Tertiary Care ◽

Jugular Foramen ◽

Appreciable Change ◽

Acoustic Neuromas ◽

Magnetic Resonance Imaging Mri

We describe a rare complication of stereotactic radiotherapy for large acoustic neuromas in a patient with type 2 neurofibromatosis. We retrospectively reviewed the case of a 14-year-old girl who had been referred to our tertiary care center. Prior to referral, the patient had been evaluated for hoarseness. During the work-up, magnetic resonance imaging (MRI) detected two large bilateral acoustic neuromas and two bilateral jugular foramen tumors. The patient was diagnosed with type 2 neurofibromatosis, and she underwent stereotactic radiotherapy for treatment of the two acoustic neuromas; the jugular foramen tumors were not irradiated. The patient's post-treatment course was complicated by hydrocephalus and symptoms of brainstem compression, which required urgent surgical intervention. Follow-up MRI 7 months following radiotherapy demonstrated a rapid growth of the acoustic neuromas, but no appreciable change in the size of the jugular foramen neuromas. These findings suggest that the radiotherapy might have been the cause of the rapid growth of the acoustic neuromas. To our knowledge, no such report has been published in the literature, and this phenomenon might be unique. Our findings suggest that radiotherapy might not be the optimal first-line treatment for acoustic neuromas in patients with type 2 neurofibromatosis.

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Neuromyelitis optica in Brazil: a study on clinical and prognostic factors

Multiple Sclerosis Journal ◽

10.1177/1352458508101935 ◽

2009 ◽

Vol 15 (5) ◽

pp. 613-619 ◽

Cited By ~ 46

Author(s):

DB Bichuetti ◽

EML Oliveira ◽

NA Souza ◽

RLM Rivero ◽

AA Gabbai

Keyword(s):

Neuromyelitis Optica ◽

Age Of Onset ◽

Care Center ◽

Brain Mri ◽

Tertiary Care ◽

Brain Magnetic Resonance Imaging ◽

Brain Lesions ◽

Relapsing Remitting ◽

First Relapse ◽

Magnetic Resonance Imaging Mri

Objectives To describe the clinical characteristics of patients with relapsing neuromyelitis optica (NMO) from a tertiary care center in Brazil and compare the groups with normal and abnormal brain magnetic resonance imaging (MRI). Methods Retrospective review of 41 patients followed at the Neuroimmunology Clinic of the Federal University of São Paulo, Brazil, from 1994 to 2007. Results All patients had relapsing-remitting optic-spinal disease, long extending spinal cord lesions, and brain MRI not meeting Barkhof criteria for multiple sclerosis (MS), thus fulfilling the 1999 and 2006 Wingerchuck criteria for NMO. Mean follow-up time was 52 months; mean age of onset was 32.6 years. The mean relapse rate (RR) and progression index (PI) were 1.0 and 0.9, respectively. Twenty-four patients had brain lesions not compatible with MS on MRI, and there were no statistical differences on PI and RR between patients who had brain lesions and patients who did not. Incomplete recovery, but not the type of first relapse, correlated with a worse prognosis. Seventeen patients were tested for NMO-IgG (anti-aquaporin-4 antibody) with 41% positivity. Conclusions In this series, we did not find a statistical difference of disease progression between patients with and without brain lesions, suggesting that the presence of brain abnormalities is not a marker of disease severity.

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Anti- NMDAR Autoimmune Encephalitis Presenting as Acute Psychosis: A Case Report

Nepal Medical Journal ◽

10.37080/nmj.87 ◽

2020 ◽

Vol 3 (1) ◽

pp. 59-61

Author(s):

Bibek Rajbhandari ◽

Minani Gurung

Keyword(s):

Behavioral Change ◽

Psychiatric Symptoms ◽

Autoimmune Encephalitis ◽

Autoimmune Disorder ◽

Clinical Findings ◽

Acute Psychosis ◽

Abnormal Movements ◽

Nmdar Encephalitis ◽

Long Time ◽

Autonomic Instability

Anti-NMDA receptor (NMDAR) encephalitis is a recently identified autoimmune disorder with prominent psychiatric symptoms. Patients usually present with acute behavioral change, psychosis, catatonic symptoms, memory deficits, seizures, dyskinesias, and autonomic instability. We present a case of a 13-year old who presented with noticeably chirpiness, excessive talking with unknown people and wandering around the neighborhood without purpose.The main symptoms of the patient and the important clinical findings were irrelevant talking which later developed into slurring of speech, abnormal movements and memory loss.This case is an example of how easily we are misled towards diagnosis based on the present symptoms. The patient suffered the unnecessary stigma of a psychiatric illness, which might stay imprinted on her for a long time. In this report we call for attention to the inclusion of anti-NMDAR encephalitis in the differential diagnosis of acute psychosis. It adds on to show that NMDAR might present in the most unexpected and unpredictable ways, sometimes misleading the patient away from medical help.Prompt diagnosis is critical as early immunotherapy and tumor removal could dramatically affect outcomes.

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Clinical and histopathological study of lepra reactions from a tertiary care center in South India

International Journal of Research in Dermatology ◽

10.18203/issn.2455-4529.intjresdermatol20175375 ◽

2017 ◽

Vol 3 (4) ◽

pp. 512

Author(s):

Remya Prasannan ◽

Mamatha George ◽

Binitha M. P. ◽

Lekha T.

Keyword(s):

Clinical Features ◽

South India ◽

Care Center ◽

Tertiary Care ◽

Descriptive Statistics ◽

Histopathological Study ◽

Neutrophilic Infiltration ◽

Clinical Presentations

Background: Clinical features and histopathology of leprosy and type 1 and type 2 reactions do not always match, though they are generally accepted as important for arriving at a diagnosis. We studied the varied clinical presentations and the correlation with histology in patients presenting with leprosy and type 1 and type 2 reactions.Methods: All patients with clinical features of leprosy and those with features of type 1 and type 2 lepra reactions attending the department of Dermatology from August 2008 to August 2009 were included. Detailed history, clinical examination and skin biopsy findings were noted and correlated using descriptive statistics.Results: Of the 138 patients included in the study, 24 cases of reactions were detected. Eighteen had type 1 (75%) and six had type 2 (25%) reaction. Correlation showed that dermal edema on histology was helpful in diagnosis of lepra reactions while neutrophilic infiltration favoured the diagnosis of type 2 reactions. Conclusions: Histopathological features are helpful in the diagnosis of lepra reactions, however in cases of doubt, the diagnosis should be made on clinical grounds.

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