Apheresis in Autoimmune Encephalitis and Autoimmune Dementia

Rößling, Rosa; Prüß, Harald

doi:10.3390/jcm9092683

Cited by 14 publications

(14 citation statements)

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“… 15 , 16 , 17 The binding of the antibody may result in dysfunction of the receptor, internalization, antibody mediated cytotoxicity, or activation of complement. The frequency of cancer is less (~ 50%) and these disorders tend to respond well to immunotherapy, particularly antibody depleting therapy [e.g., plasma exchange (PLEX), 18 rituximab]. 19 …”

Section: Pathophysiologymentioning

confidence: 99%

Autoimmune encephalopathies presenting as dementia of subacute onset and rapid progression

Banks

Sechi

Flanagan

2021

Ther Adv Neurol Disord

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The terms autoimmune dementia and autoimmune encephalopathy may be used interchangeably; autoimmune dementia is used here to emphasize its consideration in young-onset dementia, dementia with a subacute onset, and rapidly progressive dementia. Given their potential for reversibility, it is important to distinguish the rare autoimmune dementias from the much more common neurodegenerative dementias. The presence of certain clinical features [e.g. facio-brachial dystonic seizures that accompany anti-leucine-rich-glioma-inactivated-1 (LGI1) encephalitis that can mimic myoclonus] can be a major clue to the diagnosis. When possible, objective assessment of cognition with bedside testing or neuropsychological testing is useful to determine the degree of abnormality and serve as a baseline from which immunotherapy response can be judged. Magnetic resonance imaging (MRI) head and cerebrospinal fluid (CSF) analysis are useful to assess for inflammation that can support an autoimmune etiology. Assessing for neural autoantibody diagnostic biomarkers in serum and CSF in those with suggestive features can help confirm the diagnosis and guide cancer search in paraneoplastic autoimmune dementia. However, broad screening for neural antibodies in elderly patients with an insidious dementia is not recommended. Moreover, there are pitfalls to antibody testing that should be recognized and the high frequency of some antibodies in the general population limit their diagnostic utility [e.g., anti-thyroid peroxidase (TPO) antibodies]. Once the diagnosis is confirmed, both acute and maintenance immunotherapy can be utilized and treatment choice varies depending on the accompanying neural antibody present and the presence or absence of cancer. The target of the neural antibody biomarker may help predict treatment response and prognosis, with antibodies to cell-surface or synaptic antigens more responsive to immunotherapy and yielding a better overall prognosis than those with antibodies to intracellular targets. Neurologists should be aware that autoimmune dementias and encephalopathies are increasingly recognized in novel settings, including post herpes virus encephalitis and following immune-checkpoint inhibitor use.

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Section: Pathophysiologymentioning

confidence: 99%

Autoimmune encephalopathies presenting as dementia of subacute onset and rapid progression

Banks

Sechi

Flanagan

2021

Ther Adv Neurol Disord

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“…Plasmapheresis was more effective for encephalitis caused by cell-surface antigens (approximately 85%) than by intracellular or synaptic antigens (66.7%) [ 149 , 150 , 151 ]. Response rate was higher with early initiation of therapy [ 152 ].…”

Section: Treatmentmentioning

confidence: 99%

Neuropsychiatric Disorders Due to Limbic Encephalitis: Immunologic Aspect

Kao

Lin²,

Weng

et al. 2020

IJMS

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Limbic encephalitis (LE) is a rare cause of encephalitis presenting as an acute and subacute onset of neuropsychiatric manifestations, particularly with memory deficits and confusion as core features, along with seizure occurrence, movement disorders, or autonomic dysfunctions. LE is caused by neuronal antibodies targeting the cellular surface, synaptic, and intracellular antigens, which alter the synaptic transmission, especially in the limbic area. Immunologic mechanisms involve antibodies, complements, or T-cell-mediated immune responses in different degree according to different autoantibodies. Sensitive cerebrospinal fluid markers of LE are unavailable, and radiographic findings may not reveal a typical mesiotemporal involvement at neurologic presentations; therefore, a high clinical index of suspicions is pivotal, and a neuronal antibody testing is necessary to make early diagnosis. Some patients have concomitant tumors, causing paraneoplastic LE; therefore, tumor survey and treatment are required in addition to immunotherapy. In this study, a review on the molecular and immunologic aspects of LE was conducted to gain awareness of its peculiarity, which we found quite different from our knowledge on traditional psychiatric illness.

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“…This mechanism was recently discussed, and CSF biomarkers of neurodegeneration have proven to correlate inversely with clinical improvement [ 26 , 27 ]. A potential association between autoantibodies and cognitive decline in autoimmune dementia has been thoroughly investigated, and currently recruiting clinical trials investigating the impact of immunotherapy in suspected Alzheimer’s disease associated with novel autoantibodies have been reviewed elsewhere [ 28 , 29 ]. Autoantibodies exhibit variable associations with paraneoplastic syndromes sometimes resembling neurocognitive syndromes such as Alzheimer’s disease, which we had to reconsider as the second patient had a positive tumor history.…”

Section: Discussionmentioning

confidence: 99%

KCNA2 Autoimmunity in Progressive Cognitive Impairment: Case Series and Literature Review

et al. 2021

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Autoimmune dementia is a novel and expanding field which subsumes neuropsychiatric disorders with predominant cognitive impairments due to an underlying autoimmune etiology. Progressive dementias with atypical clinical presentation should trigger a thorough diagnostic approach including testing for neural surface and intracellular antibodies to avoid a delay in accurate diagnosis and initiating appropriate therapy. Here, we present two emerging cases of progressive dementia with co-existing serum autoantibodies against the KCNA2 (potassium voltage-gated channel subfamily A member 2) subunit. We found various cognitive deficits with dominant impairments in the memory domain, particularly in delayed recall. One patient presented a subacute onset of then-persisting cognitive deficits, while the other patient’s cognitive impairments progressed more chronically and fluctuated. Cognitive impairments coincided with additional neuropsychiatric symptoms. Both had a potential paraneoplastic background according to their medical history and diagnostic results. We discuss the potential role of KCNA2 autoantibodies in these patients and in general by reviewing the literature. The pathogenetic role of KCNA2 antibodies in cognitive impairment is not well delineated; clinical presentations are heterogeneous, and thus a causal link between antibodies remains questionable. Current evidence indicates an intracellular rather than extracellular epitope. We strongly suggest additional prospective studies to explore KCNA2 antibodies in specifically-defined cohorts of cognitively impaired patients via a systematic assessment of clinical, neuropsychological, neuroimaging, as well as laboratory and CSF (cerebrospinal fluid) parameters, and antibody studies to (1) determine the epitope’s location (intracellular vs. extracellular), (2) the mode of action, and (3) seek co-existing, novel pathogenetic autoantibodies in sera and CSF.

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Apheresis in Autoimmune Encephalitis and Autoimmune Dementia

Cited by 14 publications

References 50 publications

Autoimmune encephalopathies presenting as dementia of subacute onset and rapid progression

Autoimmune encephalopathies presenting as dementia of subacute onset and rapid progression

Neuropsychiatric Disorders Due to Limbic Encephalitis: Immunologic Aspect

KCNA2 Autoimmunity in Progressive Cognitive Impairment: Case Series and Literature Review

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