- Email: [email protected]
23.1 Neuropsychiatric Presentations of Autoimmune Encephalopathies: A Clinical Case Series
CLINICAL PERSPECTIVES 23.1 — 23.4
23.1 NEUROPSYCHIATRIC PRESENTATIONS OF AUTOIMMUNE ENCEPHALOPATHIES: A CLINICAL CASE SERIES Lisa B. Namerow, MD, Institute of Living at Hartford Hospital, [email protected] Objectives: This presentation will provide an introduction to the literature summarizing the current complexities in detecting patients with autoimmune encephalopathies (AEs) and will review three cases, which presented with neuropsychiatric findings that led the clinician to consider the diagnosis of AE. In addition, these case presentations will highlight the potential obstacles in making the diagnosis and in collaborating with other medical disciplines to provide a consistent approach to both treatment and recovery. Methods: Three clinical case presentations of patients with AE will be presented, highlighting the acute pattern of the onset of symptoms, the initial “negative” medical workup, the treatments used, the multidisciplinary nature of the treatment team, and the complexity of the clinical course. The first patient presented with acute confusion, disorientation, irritability, and mild paranoia in the setting of mycoplasma disease; another patient developed marked hypervigilance, agitation, and confusion initially thought to be secondary to Hashimoto’s disease; and the last patient developed acute onset Capgras-type delusions with associated impairments in cognition. In all three cases, the patients had normal findings on neurological exam, CT, MRI, EEG, and initial laboratory testing, leading to difficulties in developing a consensus in both diagnosis and appropriate treatment. Results: Participants will gain a more complete understanding of the clinical presentation, course of recovery, and the use of pharmacologic, immunologic, and nonpharmacologic treatments in order to promote a resolution of symptoms. Conclusions: Enhancing the ability of the child and adolescent psychiatrist to recognize the patterns of the neuropsychiatric aspects of the clinical presentation of AEs has become increasingly essential in helping with the detection and treatment of these conditions, given that the identification of the autoantibodies, in the setting of initial negative medical or neurologic focal findings, can take time. The current evidence suggests that delays in the detection and treatment of these syndromes may contribute to a worse prognosis. Therefore, the child and adolescent psychiatrist is well positioned, especially those who work in medical settings, to help to promote and enhance improved outcomes for these patients.
PSY, NI, CON http://dx.doi.org/10.1016/j.jaac.2017.07.132
23.2 UTILITY OF THE MONTREAL COGNITIVE ASSESSMENT AND NEUROPSYCHOLOGICAL SCREENING TOOLS TO IDENTIFY AND MONITOR AUTOIMMUNE ENCEPHALOPATHY Kevin P. Young, PhD, Institute of Living at Hartford Hospital, [email protected] Objectives: Psychiatric manifestations of autoimmune encephalopathy (AE) can confound and delay diagnosis, and treatment delays can have deleterious effects on prognosis. Standard laboratories aimed at identifying the cause of AE can take weeks to process, and tests that can rapidly distinguish psychiatric from medical patients are needed to begin the differential process. As many individuals with AE will present with cognitive symptoms, it is critical to develop concrete methods for identification and ongoing monitoring. Methods: The Montreal Cognitive Assessment (MoCA) was originally designed as a brief screen for mild cognitive impairment in adults but has demonstrated use as a general cognitive screener across numerous populations; mocatest.org currently lists almost 500 citations. It is translated into 62 languages and has multiple forms, increasing utility. We will show how to use the MoCA to guide probabilistic thinking about etiology of psychiatric symptoms associated with AE and subsequent monitoring of course. More advanced neuropsychological testing techniques will also be presented for cases wherein a more comprehensive approach is indicated.
S34
www.jaacap.org
Results: Our sample group of 168 patients with mental illness aged 12–17 years (44% female) from diverse backgrounds (61% Caucasian) produced a mean MoCA performance of 24.54 (SD ¼ 3.67), which was statistically no different from our sample group of 238 patients aged 18–25 years [mean ¼ 24.55 (SD ¼ 4.08), P > 0.1]. Neither mental illness diagnosis nor demographic variables influenced scores from patients aged 12–25 years, and results were consistent with large, population-based adult norms. Given these MoCA norms within a mental illness population, the use of the MoCA for identification, monitoring, and/or triggering consultations within the management of AE will be considered. Conclusions: The MoCA appears to be a useful and an easily available tool in identifying possible cases of AE and in monitoring treatment efficacy. More detailed but still brief, bedside evaluation can be used to empirically demonstrate degree of change in cognition and confirm MoCA findings. Use of these tools by appropriately trained individuals can expedite treatment, although traditional work-up is still pending, and provide the basis for monitoring treatment efficacy.
DIAG, NEPSYC, PSY http://dx.doi.org/10.1016/j.jaac.2017.07.133
23.3 HOW TO DEFINE AND EVALUATE AUTOIMMUNE ENCEPHALOPATHY: A NEUROLOGIST’S VIEW William Gallentine, DO, Duke University, william.gallentine@ duke.edu Objectives: Autoimmune encephalopathy (AE) has become a more widely recognized cause of abrupt onset neuropsychiatric syndrome. It affects all age groups, and presenting symptoms often vary with age. Several observational studies describing clinical symptoms and diagnostic criteria have been published. At the conclusion of this talk, the learners will be able to 1) recognize clinical signs that should prompt an evaluation for AE; 2) perform an appropriate diagnostic evaluation to assess for AE; and 3) understand newly proposed diagnostic criteria. Methods: Clinical symptoms commonly seen in AE, as described in the literature, as well as our clinical experience at the Duke Children’s Autoimmune Brain Disease clinic, will be discussed. Diagnostic approaches and diagnostic criteria, as defined by experts in the field, will be reviewed. Results: Clinical symptoms that are abrupt in onset and cross multiple domains (cognition, behavioral, seizures, movement disorders, dysautonomia, and sleep disruption) without other clear etiology should raise concern for a possible AE and warrant further evaluation. Psychiatric symptoms can be profound and are often the presenting symptoms in adolescents and adults. Current diagnostic criteria are quite helpful in providing a framework for which this diagnosis may be approached but may have some limitations as they apply to children. Conclusions: AE is an important cause of abrupt onset neuropsychiatric symptoms that requires recognition and appropriate diagnostic evaluation.
DIAG, NI, PSY http://dx.doi.org/10.1016/j.jaac.2017.07.134
23.4 TREATMENT CONSIDERATIONS IN AUTOIMMUNE ENCEPHALOPATHY Heather A. Van Mater, MD, MSc, Duke University, heather. [email protected] Objectives: This presentation will focus on the treatment of autoimmune encephalopathies (AEs), including immunomodulatory treatments and supportive therapies. AE is a broad category of autoimmune brain disease, representing numerous diagnoses, with a range of symptoms and severity. As a result, there are different treatment recommendations based on the clinical features and specific diagnosis. It is important for all providers to recognize the heterogeneity in treatment responses across the spectrum of AE and across immunomodulatory therapies. Understanding the expected pace of improvement is essential when determining treatment effectiveness and assessing which supportive therapies may be necessary over the course of the disease process. Methods: This presentation will include information on current clinical guidelines surrounding the treatment of children and adolescents with AEs.
J OURNAL
OF THE
AMERICAN A CADEMY OF CHILD & ADOLESCENT P SYCHIATRY VOLUME 56 NUMBER 10S OCTOBER 2017
23.1 NEUROPSYCHIATRIC PRESENTATIONS OF AUTOIMMUNE ENCEPHALOPATHIES: A CLINICAL CASE SERIES Lisa B. Namerow, MD, Institute of Living at Hartford Hospital, [email protected] Objectives: This presentation will provide an introduction to the literature summarizing the current complexities in detecting patients with autoimmune encephalopathies (AEs) and will review three cases, which presented with neuropsychiatric findings that led the clinician to consider the diagnosis of AE. In addition, these case presentations will highlight the potential obstacles in making the diagnosis and in collaborating with other medical disciplines to provide a consistent approach to both treatment and recovery. Methods: Three clinical case presentations of patients with AE will be presented, highlighting the acute pattern of the onset of symptoms, the initial “negative” medical workup, the treatments used, the multidisciplinary nature of the treatment team, and the complexity of the clinical course. The first patient presented with acute confusion, disorientation, irritability, and mild paranoia in the setting of mycoplasma disease; another patient developed marked hypervigilance, agitation, and confusion initially thought to be secondary to Hashimoto’s disease; and the last patient developed acute onset Capgras-type delusions with associated impairments in cognition. In all three cases, the patients had normal findings on neurological exam, CT, MRI, EEG, and initial laboratory testing, leading to difficulties in developing a consensus in both diagnosis and appropriate treatment. Results: Participants will gain a more complete understanding of the clinical presentation, course of recovery, and the use of pharmacologic, immunologic, and nonpharmacologic treatments in order to promote a resolution of symptoms. Conclusions: Enhancing the ability of the child and adolescent psychiatrist to recognize the patterns of the neuropsychiatric aspects of the clinical presentation of AEs has become increasingly essential in helping with the detection and treatment of these conditions, given that the identification of the autoantibodies, in the setting of initial negative medical or neurologic focal findings, can take time. The current evidence suggests that delays in the detection and treatment of these syndromes may contribute to a worse prognosis. Therefore, the child and adolescent psychiatrist is well positioned, especially those who work in medical settings, to help to promote and enhance improved outcomes for these patients.
PSY, NI, CON http://dx.doi.org/10.1016/j.jaac.2017.07.132
23.2 UTILITY OF THE MONTREAL COGNITIVE ASSESSMENT AND NEUROPSYCHOLOGICAL SCREENING TOOLS TO IDENTIFY AND MONITOR AUTOIMMUNE ENCEPHALOPATHY Kevin P. Young, PhD, Institute of Living at Hartford Hospital, [email protected] Objectives: Psychiatric manifestations of autoimmune encephalopathy (AE) can confound and delay diagnosis, and treatment delays can have deleterious effects on prognosis. Standard laboratories aimed at identifying the cause of AE can take weeks to process, and tests that can rapidly distinguish psychiatric from medical patients are needed to begin the differential process. As many individuals with AE will present with cognitive symptoms, it is critical to develop concrete methods for identification and ongoing monitoring. Methods: The Montreal Cognitive Assessment (MoCA) was originally designed as a brief screen for mild cognitive impairment in adults but has demonstrated use as a general cognitive screener across numerous populations; mocatest.org currently lists almost 500 citations. It is translated into 62 languages and has multiple forms, increasing utility. We will show how to use the MoCA to guide probabilistic thinking about etiology of psychiatric symptoms associated with AE and subsequent monitoring of course. More advanced neuropsychological testing techniques will also be presented for cases wherein a more comprehensive approach is indicated.
S34
www.jaacap.org
Results: Our sample group of 168 patients with mental illness aged 12–17 years (44% female) from diverse backgrounds (61% Caucasian) produced a mean MoCA performance of 24.54 (SD ¼ 3.67), which was statistically no different from our sample group of 238 patients aged 18–25 years [mean ¼ 24.55 (SD ¼ 4.08), P > 0.1]. Neither mental illness diagnosis nor demographic variables influenced scores from patients aged 12–25 years, and results were consistent with large, population-based adult norms. Given these MoCA norms within a mental illness population, the use of the MoCA for identification, monitoring, and/or triggering consultations within the management of AE will be considered. Conclusions: The MoCA appears to be a useful and an easily available tool in identifying possible cases of AE and in monitoring treatment efficacy. More detailed but still brief, bedside evaluation can be used to empirically demonstrate degree of change in cognition and confirm MoCA findings. Use of these tools by appropriately trained individuals can expedite treatment, although traditional work-up is still pending, and provide the basis for monitoring treatment efficacy.
DIAG, NEPSYC, PSY http://dx.doi.org/10.1016/j.jaac.2017.07.133
23.3 HOW TO DEFINE AND EVALUATE AUTOIMMUNE ENCEPHALOPATHY: A NEUROLOGIST’S VIEW William Gallentine, DO, Duke University, william.gallentine@ duke.edu Objectives: Autoimmune encephalopathy (AE) has become a more widely recognized cause of abrupt onset neuropsychiatric syndrome. It affects all age groups, and presenting symptoms often vary with age. Several observational studies describing clinical symptoms and diagnostic criteria have been published. At the conclusion of this talk, the learners will be able to 1) recognize clinical signs that should prompt an evaluation for AE; 2) perform an appropriate diagnostic evaluation to assess for AE; and 3) understand newly proposed diagnostic criteria. Methods: Clinical symptoms commonly seen in AE, as described in the literature, as well as our clinical experience at the Duke Children’s Autoimmune Brain Disease clinic, will be discussed. Diagnostic approaches and diagnostic criteria, as defined by experts in the field, will be reviewed. Results: Clinical symptoms that are abrupt in onset and cross multiple domains (cognition, behavioral, seizures, movement disorders, dysautonomia, and sleep disruption) without other clear etiology should raise concern for a possible AE and warrant further evaluation. Psychiatric symptoms can be profound and are often the presenting symptoms in adolescents and adults. Current diagnostic criteria are quite helpful in providing a framework for which this diagnosis may be approached but may have some limitations as they apply to children. Conclusions: AE is an important cause of abrupt onset neuropsychiatric symptoms that requires recognition and appropriate diagnostic evaluation.
DIAG, NI, PSY http://dx.doi.org/10.1016/j.jaac.2017.07.134
23.4 TREATMENT CONSIDERATIONS IN AUTOIMMUNE ENCEPHALOPATHY Heather A. Van Mater, MD, MSc, Duke University, heather. [email protected] Objectives: This presentation will focus on the treatment of autoimmune encephalopathies (AEs), including immunomodulatory treatments and supportive therapies. AE is a broad category of autoimmune brain disease, representing numerous diagnoses, with a range of symptoms and severity. As a result, there are different treatment recommendations based on the clinical features and specific diagnosis. It is important for all providers to recognize the heterogeneity in treatment responses across the spectrum of AE and across immunomodulatory therapies. Understanding the expected pace of improvement is essential when determining treatment effectiveness and assessing which supportive therapies may be necessary over the course of the disease process. Methods: This presentation will include information on current clinical guidelines surrounding the treatment of children and adolescents with AEs.
J OURNAL
OF THE
AMERICAN A CADEMY OF CHILD & ADOLESCENT P SYCHIATRY VOLUME 56 NUMBER 10S OCTOBER 2017