Admission diagnoses of patients later diagnosed with autoimmune encephalitis

Baumgartner, Annette; Rauer, Sebastian; Hottenrott, Tilman; Leypoldt, Frank; Ufer, Friederike; Hegen, Harald; Prüß, Harald; Lewerenz, Jan; Deisenhammer, Florian; Stich, Oliver

doi:10.1007/s00415-018-9105-3

Cited by 43 publications

(49 citation statements)

References 20 publications

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“…The first dog had a history and multifocal neurological signs typical of encephalitis as well as both multifocal MRI changes and CSF pleocytosis. The second dog had severe cerebellar signs, consistent with the atypical presentation reported in people more recently, as well as having CSF pleocytosis. In both dogs, infectious causes were excluded and their response to immunosuppression supports an autoimmune etiology although a causal relationship with the anti‐NMDAR antibodies cannot be established from these observations.…”

Section: Discussionsupporting

confidence: 81%

Presence of cerebrospinal fluid antibodies associated with autoimmune encephalitis of humans in dogs with neurologic disease

Stafford

Kortum

Castel

et al. 2019

Veterinary Internal Medicne

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Background Presumed autoimmune diseases affecting the central nervous system (CNS) of dogs are common. In people, antibodies against neuronal cell surface antigens that are associated with a wide variety of neurological syndromes have been identified. The presence of cerebrospinal fluid (CSF) autoantibodies that target neuronal cell surface proteins has not been reported in dogs with neurologic disorders. Objectives Autoantibodies to neuronal cell surface antigens can be found in the CSF of dogs with inflammatory CNS disease. Our aim was to determine whether 6 neuronal cell surface autoantibodies were present in the CSF of dogs diagnosed with inflammatory and noninflammatory CNS disease. Animals Client‐owned dogs with CNS disease and complete diagnostic evaluation including magnetic resonance imaging and CSF analysis were included. One healthy dog was included as a negative control. Methods Cerebrospinal fluid was tested for 6 antigenic targets with a commercially available indirect immunofluorescence assay test kit. Results There were 32 dogs with neurological disease, 19 diagnosed with inflammatory disease (encephalitis and meningitis), 10 with noninflammatory disease (neoplasia, intervertebral disk disease, degenerative myelopathy, and epilepsy), 2 with no diagnosis, and 1 with neoplasia and meningoencephalitis. Anti‐N‐methyl‐d‐aspartate receptor 1 (NMDAR1) antibodies were detected in 3 dogs (3/32; 9.38%). All 3 dogs responded to treatment of meningoencephalomyelitis of unknown etiology (MUE). Conclusions and Clinical Importance Further evaluation of the prevalence and clinical relevance of CSF and serum antibodies to neuronal cell surface antigens is warranted. Defining antigenic targets associated with encephalitis in dogs might allow diagnostic categorization of MUE antemortem.

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Section: Discussionsupporting

confidence: 81%

Presence of cerebrospinal fluid antibodies associated with autoimmune encephalitis of humans in dogs with neurologic disease

Stafford

Kortum

Castel

et al. 2019

Veterinary Internal Medicne

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“…The most discussed antibody in psychotic disorders at the moment is the N-methyl-D-aspartate receptor (NMDA-R) antibody. It has been reported that as many as 74% of patients suffering from NMDA-R encephalitis experience psychotic symptoms (53, 54), and a recent smaller study found that 13% were initially admitted to the hospital with a psychiatric diagnosis (55). Multiple studies have investigated the frequency of NMDA-R antibodies in schizophrenia, but so far most have only had access to serum not CSF, most have had no healthy control group, and results have varied markedly (56).…”

Section: Epidemiological Associationsmentioning

confidence: 99%

Autoimmune Diseases and Psychotic Disorders

2019

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The notion of immunological pathways playing a role in the etiology of a subset of psychotic disorders has received increased interest in the last decades. One of the findings that has spiked interest herein, is an apparent link between autoimmune diseases and psychotic disorders. This is supported by genetic findings associating immune-related genetic markers with schizophrenia and clinical studies finding increased levels of inflammatory markers in patients with psychosis. Several large-scale epidemiologic studies have found positive associations between autoimmune diseases and psychosis. Particularly, autoimmune diseases as multiple sclerosis and lupus are known to have higher frequencies of neuropsychiatric symptoms, including psychosis, compared to healthy controls. Cross sectional studies have found higher prevalence of psychiatric diagnoses among those with autoimmune diseases, and longitudinal studies have shown bidirectional associations between several autoimmune diseases and increased risks associated with schizophrenia. Moreover, a family history of autoimmune diseases has been shown to be associated with an increased risk of psychotic disorders and vice versa. In this review we will summarize the epidemiologic evidence on associations between autoimmune diseases and psychosis. Possible mechanisms accountable for the association will be discussed, amongst others the probable role of shared genetic risk factors, the impact of infections on both autoimmunity and the development of psychotic disorders, and the potential role of the microbiome. We discuss the findings on and influence of autoantibodies and dysregulation of T- and B-cells in both disease categories, and why further research hereon is needed. In addition to the potential importance of autoimmunity in etiological mechanisms of psychotic disorders, the association also brings important attention to somatic comorbidity in patients with psychotic disorders.

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“…A retrospective study examining admission diagnoses of 50 patients with autoimmune encephalitides such as ALE found that two-thirds were initially thought to have conditions other than encephalitis, including primary psychiatric disorders, idiopathic epilepsy, cerebral ischemia or neurodegeneration. 15 Even among the one-third of patients in whom encephalitis was considered, an infectious rather than autoimmune cause was more commonly assumed. 15 The potential symptom overlap between these 2 disease processes was highlighted in a prospective study of 203 patients with encephalitis, which found that many traditionally infectious symptoms such as fever did not readily distinguish between an infectious and immune-mediated cause.…”

Section: Why Is Prompt Correct Diagnosis Essential?mentioning

confidence: 99%

“…15 Even among the one-third of patients in whom encephalitis was considered, an infectious rather than autoimmune cause was more commonly assumed. 15 The potential symptom overlap between these 2 disease processes was highlighted in a prospective study of 203 patients with encephalitis, which found that many traditionally infectious symptoms such as fever did not readily distinguish between an infectious and immune-mediated cause. 16 Identification of ALE is important because it facilitates prompt use of immunotherapy which, in observational studies, has been associated with reduced seizure frequency, recovery of cognition and likely even improved survival.…”

Section: Why Is Prompt Correct Diagnosis Essential?mentioning

confidence: 99%