REVIEW

CURRENT
OPINION Autoimmune encephalitis as differential diagnosis
of infectious encephalitis
Thaı´s Armangue a, Frank Leypoldt b, and Josep Dalmau a,b,c

Purpose of review
This review describes the main types of autoimmune encephalitis with special emphasis on those associated
with antibodies against neuronal cell surface or synaptic proteins, and the differential diagnosis with
infectious encephalitis.
Recent findings
There is a continuous expansion of the number of cell surface or synaptic proteins that are targets of
autoimmunity. The most recently identified include the metabotropic glutamate receptor 5 (mGluR5),
dipeptidyl-peptidase-like protein-6 (DPPX), and g-aminobutyric acid-A receptor (GABAAR). In these and
previously known types of autoimmune encephalitis [N-methyl-D-aspartate receptor (NMDAR), a-amino-3-
hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR), g-aminobutyric acid-B receptor (GABABR),
leucine-rich glioma inactivated protein 1 (LGI1), contactin-associated protein-like 2 (CASPR2)], the
prodromal symptoms or types of presentations often suggest a viral encephalitis. We review here clues
that help in the differential diagnosis with infectious encephalitis. Moreover, recent investigations indicate
that viral encephalitis (e.g., herpes simplex) can trigger synaptic autoimmunity. In all these disorders,
immunotherapy is usually effective.
Summary
Autoimmune encephalitis comprises an expanding group of potentially treatable disorders that should be
included in the differential diagnosis of any type of encephalitis.
Video Abstract
http://links.lww.com/CONR/A25
Keywords
autoimmune encephalitis, herpes simplex encephalitis, immunotherapy, neuronal surface antibodies,
viral encephalitis

INTRODUCTION encephalitis surpassed that of any individual

Encephalitis is a significant cause of morbidity and viral etiology in young individuals [7]. Moreover,
mortality worldwide. In order to find the etiology of recent studies have shown that some forms of auto-
the disorder patients frequently undergo extensive immune encephalitis can be triggered by herpes
& &

testing, but despite this the cause remains unknown simplex encephalitis (HSE) [11 ,12 ]. This review
in about 60% of the cases [1–3]. The discovery that focuses on the diagnosis and treatment of auto-
several forms of encephalitis result from antibodies immune encephalitis, mainly those associated with
against neuronal cell surface or synaptic proteins
&
and that they are potentially treatable [4 ] has led
a
to a paradigm shift in the diagnostic approach of August Pi i Sunyer Biomedical Research Institute (IDIBAPS), Service of
&& && Neurology, Hospital Clinic, University of Barcelona, bCatalan Institution
encephalitis [5–8,9 ,10 ]. A recent multicenter
for Research and Advanced Studies (ICREA), Barcelona, Spain and
population-based prospective study found that in c
Department of Neurology, University of Pennsylvania, Philadelphia, USA
42 of 203 patients (21%), the etiology was immune Correspondence to Josep Dalmau, MD, PhD, Centre de Recerca Bio-
mediated and 38% of them occurred with neuronal mèdica, CELLEX, Facultat de Medicina, Universitat de Barcelona,
antibodies [6]. Another study by the California C/Casanova 143, Recepció CELLEX, 08036 Barcelona, Spain.
Encephalitis Project, a center focused on the epi- Tel: +34 932 271 738; e-mail: Jdalmau@clinic.ub.es
demiology of encephalitis, found that the frequency Curr Opin Neurol 2014, 27:361–368
of anti-N-methyl-D-aspartate receptor (NMDAR) DOI:10.1097/WCO.0000000000000087

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 Inflammatory diseases and infection

encephalitis, particularly in patients with limbic

KEY POINTS encephalitis. Most patients with autoimmune
 A rapidly expanding subset of autoimmune encephalitis or paraneoplastic limbic encephalitis have uni- or
occurs in association with antibodies to neuronal cell bilateral increased T2/fluid-attenuated inversion
surface or synaptic proteins. recovery (FLAIR) signal in the medial temporal
lobes without contrast enhancement or abnormal
 Symptoms of autoimmune encephalitis are diverse and
diffusion-weighted images; an exception is the
include psychiatric manifestations (psychosis, catatonia,
abnormal behavior), seizures, abnormal movements, paraneoplastic encephalitis with antibodies against
decrease of level of consciousness, or autonomic the intracellular protein Ma2, in which MRI often
dysfunction. shows contrast enhancement [15]. The syndromes
with classical findings of limbic encephalitis
 Detection of antibodies to cell surface or synaptic
include those associated with antibodies against
proteins often associates with response to
immunotherapy. the a-amino-3-hydroxy-5-methyl-4-isoxazolepropio-
nic acid receptor (AMPAR), g-aminobutyric acid-B
 Autoimmune encephalitis can mimic infectious receptor (GABABR), leucine-rich glioma inactivated
encephalitis. Comprehensive testing for autoantibodies protein 1 (LGI1), and less frequently the metabo-
should include cerebrospinal fluid and serum.
tropic glutamate receptor 5 (mGluR5) [16–19]. In
patients with anti-NMDAR encephalitis, the brain
MRI is normal in approximately 60% of the patients
antibodies to cell surface or synaptic proteins and shows nonspecific findings in the rest including
(Table 1), with emphasis on the differential diag- cortical–subcortical FLAIR changes in brain or
nosis with infectious etiologies. posterior fossa, transient meningeal enhancement,
&
or areas of demyelination [20 ]. The brain MRI in
COMPARISON BETWEEN AUTOIMMUNE other autoimmune encephalitis types, such as those
AND INFECTIOUS ENCEPHALITIS associated with antibodies against contactin-associ-
Autoimmune encephalitis occurs more frequently ated protein-like 2 (CASPR2) or dipeptidyl-pepti-
in immunocompetent than immunocompromised dase-like protein-6 (DPPX) is frequently abnormal
patients (22 versus 3%) [6]. Most patients with anti- but rarely suggestive of focal limbic encephalitis
body-associated encephalitis and HSE have seizures &
[21,22 ]. Patients with high titer serum and CSF
[6]. In contrast, patients with encephalitis associated GABAAR antibodies may develop extensive cortical
with varicella zoster virus (VZV) or Mycobacterium and subcortical T2/FLAIR changes during the course
tuberculosis infrequently develop seizures [6]. Psy- &
of the disease [23 ].
chosis, language dysfunction, autonomic instability, Only a few infectious encephalitis types associ-
and abnormal movements are a hallmark of anti- ate with MRI findings similar to those occurring
NMDAR encephalitis [5,7,13]. Most patients with in autoimmune limbic encephalitis; they include
infectious encephalitis have fever, but approxi- posttransplant acute limbic encephalitis related
mately 50% of cases with autoimmune encephalitis to human herpes virus 6 (HHV6), exceptional cases
present or develop fever during the course of the of neurosyphilis, and HSE. Of note, HSE typically
disease [6,7]. Prodromal symptoms such as headache shows asymmetric medial temporal lobe necrosis
or flu-like symptoms occur frequently in auto- along with involvement of cingulate and insular
immune encephalitis and may lead to the suspicion regions. Some patients, usually children, may
of an infectious etiology [5]. Skin lesions can assist in develop more extensive MRI abnormalities in fron-
the recognition of VZV; however, central nervous tal, occipital, or parietal lobes [24]. The polymerase
system (CNS) VZV reactivation may occur in the chain reaction (PCR) for herpes simplex virus (HSV)
absence of rash [14]. can be false-negative during the first 48 h of HSE
Most autoimmune encephalitis cases are [24].
associated with cerebrospinal fluid (CSF) lympho-
cytic pleocytosis that is usually milder than that
found in viral etiologies [5,7]. Patients with viral AUTOIMMUNE ENCEPHALITIS WITH
and autoimmune encephalitis have normal glucose ANTIBODIES AGAINST INTRACELLULAR
levels and normal or mildly increased protein ANTIGENS
concentration [5,7], whereas patients with bacterial Most of the antibodies to intracellular proteins
infections such as Mycobacterium tuberculosis have considered here are paraneoplastic and, therefore,
a decrease of CSF glucose concentration [6]. they occur in middle-aged or elderly patients
Magnetic resonance imaging (MRI) of the who sometimes have a previous history of cancer.
brain can be useful in the differential diagnosis of They include antibodies to Hu, Ma2, Ri, CRMP5, and

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 Autoimmune encephalitis and infectious encephalitis Armangue et al.

Table 1. Clinical features of encephalitis associated with well characterized antibodies to intracellular and neuronal cell
surface antigens
Age, sex, presence of tumor, response
Antigen Neurological symptoms to immunotherapy

Intracellular antigens
Hu (ANNA I) Encephalomyelitis, PCD, brainstem encephalitis, Mostly adults, 96–98% associated with cancer. Mostly
focal cortical encephalitis, limbic encephalitis SCLC (Hu, CV2, amphiphysin, Ri), thymoma (CRMP5),
CRMP5 Encephalomyelitis, chorea, PCD, limbic breast (amphiphysin, Ri, Yo), ovary (Yo, Ri), testes
encephalitis (Ma2)
Amphiphysin Stiff-person syndrome, myelopathy and Limited response to immunotherapy, and treatment of
myoclonus, encephalomyelitis the tumor
Ri (ANNA 2) Brainstem encephalitis, opsoclonus myoclonus
Ma2 Diencephalitic, limbic encephalitis, brainstem
encephalitis
GAD65 Ataxia, stiff-person syndrome, epilepsy Adults, <10% tumors, limited response to immunotherapy
Neuronal surface antigens
NMDAR receptor Psychiatric symptoms, language dysfunction, Children (40%) and young adults (median 19 years),
(NR1 subunit) abnormal movements, seizures, decreased 80% women
level of consciousness, autonomic instability Presence of a tumor varies with age, sex, and race
(9–55%), mostly ovarian teratomas, 80% good
recovery with immunotherapy
GABAAR High titers in serum and CSF: refractory seizures, Limited experience, 39% in children; no clear cancer
or status epilepticus. association (some patients may have thymoma).
Low titers in serum: more broad spectrum of Severe disorder (two of six patients with high titers
symptoms including seizures, stiff-person died, but the other four had substantial response to
syndrome, opsoclonus myoclonus syndrome immunotherapy
GABABR (B1 subunit) Classic limbic encephalitis. Early and prominent Adults (median 62 years, 50% women) 60% small-cell
seizures (GABABR), isolated psychiatric lung cancer
symptoms (AMPAR), hyponatremia and brief Good response to immunotherapy
tonic-myoclonic seizures (LGI1)
AMPAR (Glu R1/2 Adults (median 80 years, 90% women) 70% tumors
subunit) (lung, breast, thymus)
Good response to immunotherapy
LGI1 Adults (median 60 years, 65% men) <10% tumors
(thymoma)
CASPR2 Morvan’s syndrome, encephalitis, peripheral Adults (median 60 years, male predominance)
nerve hyperexcitability Limited experience, 30% thymoma
DPPX Diffuse encephalitis, prodromal severe diarrhea. Adults
Psychiatric symptoms, tremor, myoclonus, ataxia, Limited experience, no association with cancer,
nystagmus, hyperekplexia response to immunotherapy

AMPAR, a-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor; CASPR2, contactin-associated protein-like 2; CSF, cerebrospinal fluid; DPPX, dipeptidyl-
peptidase-like protein-6; GABABorAR, g-aminobutyric acid-B or A receptor; GAD65, 65 kDa glutamic acid decarboxylase; LGI1, leucine-rich glioma inactivated
protein 1; NMDAR, N-methyl-D-aspartate receptor; PCD, paraneoplastic cerebellar degeneration; SCLC, small-cell lung carcinoma.

amphyphisin [25]. In approximately 70% of the encephalitis, 16% underwent duodenal biopsy for
cases, the development of neurological symptoms suspected Whipple’s disease before the final diag-
precedes the cancer diagnosis [25,26]. Patients with nosis was made [28].
any of these antibodies can develop limbic ence- A subset of patients with limbic or nonfocal
phalitis, usually in the context of encephalomyeli- encephalitis with or without seizures has antibodies
tis. Some patients with Hu antibodies develop focal against GAD65 [29]. These antibodies rarely associ-
cortical encephalitis and epilepsia partialis continua ate with cancer, and also occur in patients with
suggesting a focal infectious process [27]. Patients cerebellar degeneration, stiff-person syndrome,
with Ma2 antibodies may develop prominent brain- and nonneurological disorders such as type I
stem dysfunction with abnormal gaze and facial diabetes mellitus, vitiligo, or pernicious anemia.
movements, which frequently suggest Whipple’s Moreover, GAD65 antibodies may associate with
disease. In a series of 38 patients with anti-Ma2 encephalitis related to other more relevant

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 Inflammatory diseases and infection

antibodies, such as AMPAR, GABABR, or GABAAR cases (overall less than 5% of the cases) positive
&
[18,23 ]. All patients with encephalitis or seizures serologies for Mycoplasma pneumoniae, HHV6, or
with GAD65 antibodies should be assessed for enterovirus have been described; the significance
the co-occurrence of other antibodies against cell of these findings is currently unclear [30,32]. Detec-
surface proteins. tion of HHV6 or 7 in the CSF by PCR may represent
detection of a latent rather than an active viral
infection [36]. A link between HSE and anti-NMDAR
AUTOIMMUNE ENCEPHALITIS WITH encephalitis (and other types of synaptic autoim-
ANTIBODIES TO CELL SURFACE OR munity) was recently identified (discussed below).
SYNAPTIC PROTEINS The antibodies of patients with anti-NMDAR
Several forms of autoimmune encephalitis with encephalitis cause a specific internalization of these
antibodies to cell surface or synaptic proteins receptors, and alter the NMDAR synaptic currents
should be considered in the differential diagnosis [30]. A similar antibody mediated internalization
of infectious encephalitis, including anti-NMDAR of receptors was observed after infusing patients’
encephalitis, encephalitis with predominant limbic antibodies into the hippocampus of rats. Autopsies
involvement, and other autoimmune encephalitis, of patients with these antibodies show a decrease
each of them discussed below. of NMDAR in areas of deposits of antibodies
along with absence of cytotoxic T-cell infiltrates
or deposits of complement [37].
Anti-N-methyl-D-aspartate receptor
encephalitis
This disorder predominates in young women and Encephalitis with predominant limbic
children although it can affect males and people involvement
of all ages (the youngest and the oldest patient The term limbic encephalitis refers to an inflamma-
described were 2 months and 85 years old) tory process of the limbic system including the
& &
[12 ,20 ]. The presence of a tumor (mostly an medial temporal lobes, amygdala, and cingulate
ovarian teratoma) is age dependent, and rarely gyri, resulting in severe memory deficits, behavioral
encountered in patients younger than 12 years changes, psychiatric symptoms, and temporal lobe
&
[20 ]. The antibodies target the GluN1 subunit of seizures [38]. The most frequent cell surface target
the NMDAR receptor [30]. The neuropsychiatric antigen of limbic encephalitis is LGI1. The median
symptoms are often preceded by prodromal head- age of patients with these antibodies is 60 years, and
ache, fever, or other features that may suggest the neurological symptoms are often accompanied
an infection. In teenagers and young women, the by hyponatremia [17,39]. Patients rarely have an
onset is characterized by prominent psychiatric underlying tumor, and if so, it is usually a thymoma.
manifestations (delusional thoughts, bizarre beha- Some patients develop myoclonic-like movements,
vior, psychosis, catatonia), followed by a decrease of also described as faciobrachial dystonic seizures,
consciousness, seizures, orofacial or limb dyskine- but with electroencephalographic features of tonic
sias, and autonomic instability [30]. In children seizures [40,41]. These seizures can precede or occur
and adult male patients, the first symptom can be simultaneously with symptoms of limbic dys-
&
seizures or movement disorders [31,32,33 ,34]. function and may lead to an early recognition of
The differential diagnosis often includes a primary the disorder. Approximately 70% of the patients
psychiatric disorder, drug abuse, neuroleptic malig- with LGI1 antibodies improve with immunotherapy
nant syndrome, or infectious encephalitis [5]. In although residual memory deficits are frequent
some instances, the diagnosis of rabies has been (unpublished observation). There is evidence that
considered because of the presence of extreme agita- LGI1 antibodies may disrupt the normal interaction
tion, prominent sialorrhea, and abnormal move- of LGI1 with the synaptic proteins ADAM22 and
ments [5]. In contrast to anti-NMDAR encephalitis ADAM23, resulting in a decrease of postsynaptic
&&
in which the brain MRI is frequently normal [30], AMPAR [42 ].
the MRI of patients with rabies often shows sym- Other cell surface antigens related to limbic ence-
metric involvement of the gray matter of the dorsal phalitis include AMPARs and GABABRs [16,18]. More
brainstem, thalamus, basal ganglia, or central region than half of the patients with these antibodies have
of the spinal cord [35]. cancer; the type of tumor varies with the antibodies
Because of the frequent presence of prodromal [small-cell lung carcinoma (SCLC) predominantly
symptoms (hyperthermia, headache, and other), with GABABR and breast cancer and thymomas with
most patients with anti-NMDAR encephalitis are AMPAR]. Patients with SCLC may have other anti-
investigated for an infectious etiology. In a few bodies suggesting the presence of this tumor, such as

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 Autoimmune encephalitis and infectious encephalitis Armangue et al.

SOX1 or N-type voltage-gated calcium channel cause a specific decrease of these receptors at syn-
&
(VGCC). Patients’ antibodies against AMPAR cause apses [23 ].
internalization of receptors and decrease of AMPAR- Several studies have indicated the presence of
mediated currents, strongly suggesting a pathogenic antibodies to dopamine receptor 2 (DR2) in some
role of these antibodies [16]. patients with basal ganglia encephalitis or Sydenham
&
chorea [47 ,48]. At this time, the frequency and
pathogenic significance of these antibodies are
Other autoimmune encephalitis unclear.
A subset of patients with autoimmune encephalitis
&
harbor antibodies to DPPX [22 ], a critical regulatory
subunit of the Kv4.2 potassium channel. These HERPES SIMPLEX ENCEPHALITIS
patients develop agitation, confusion, psychiatric TRIGGERS SYNAPTIC AUTOIMMUNITY
symptoms, seizures, tremor, myoclonus, and less There is recent evidence that HSE triggers synaptic
& & &
frequently hyperekplexia [22 ,43]. Characteristi- autoimmunity [11 ,12 ]. This finding likely explains
cally, most of these patients have diarrhea or other cases with prolonged or atypical neurological
gastrointestinal symptoms leading to profound symptoms after successful control of the viral infec-
weight loss. The etiology of these gastrointestinal tion, or patients who develop a syndrome described
symptoms is unclear, but may be related to the as relapsing post-HSE or choreoathetosis post-HSE
& &
expression of DPPX in the myenteric plexus [22 ]. [12 ,32,49–51]. These disorders are important to
This clinical presentation often leads to extensive recognize because the outcome without immuno-
gastrointestinal studies for a malignancy or infec- therapy is usually poor [52]. In contrast, aggressive
tious etiology, which in all cases has been negative. immunotherapy appears to be beneficial, sometimes
A form of nonfocal encephalitis (although often with substantial recoveries [32,49,53]. Choreoathe-
referred to as limbic encephalitis) associates with tosis post-HSE usually develops a few weeks after
Hodgkin’s lymphoma, and is known as Ophelia patients have recovered from HSE [52,54]; the
syndrome [44]. These patients usually have anti- main differences between true viral relapses and
bodies to mGluR5 [19]. Identification of this autoimmune encephalitis post-HSE are shown in
disorder is important because it is highly responsive Table 2. The clinical features of autoimmune
to treatment of the tumor and immunotherapy encephalitis post-HSE are similar to those of anti-
[19,45]. Autoantibodies to mGluR5 can also occur NMDAR encephalitis, although some patients
in patients with autoimmune encephalitis without develop fragments of this syndrome. A recent study
Hodgkin’s lymphoma. showed that the novel synthesis of NMDAR anti-
&
CASPR2 is the target antigen of antibodies bodies occurred after the viral encephalitis [12 ].
of some patients with Morvan’s syndrome, ence- Some patients may develop antibodies to DR2 [51]
phalitis (sometimes focal limbic encephalitis), or a and other yet unknown cell surface neuronal
&
subset of cases with neuromyotonia. Autoantibodies proteins [12 ].
against CASPR2 and those directed against LGI1
were previously reported as voltage-gated potassium
channels (VGKCs) antibodies. About 30% of patients DIAGNOSIS AND TREATMENT OF
with CASPR2 antibodies have an underlying thy- ENCEPHALITIS WITH ANTIBODIES TO
moma [21,39,46]. CELL SURFACE ANTIGENS
The most recently identified autoimmune ence- Current experience suggests that any rapidly progress-
phalitis occurs with antibodies against the GABAARs ive encephalopathy of unclear etiology, particularly
&
[23 ]. High titers of these antibodies in serum and if accompanied by lymphocytic CSF pleocytosis
CSF usually result in refractory seizures and status (although routine CSF studies can be normal), and
epilepticus, along with extensive MRI cortical/ multifocal symptoms with or without MRI changes,
subcortical FLAIR changes. Approximately, 40% of should raise concern for an immune-mediated
the patients are children. Low titers of serum anti- process. FLAIR/T2 MRI abnormalities (without sub-
bodies associate with encephalitis and seizures, but stantial enhancement) involving medial temporal
also with opsoclonus and stiff-person syndrome lobes occur frequently in patients with typical limbic
(with or without GAD65 antibodies). Patients encephalitis, and should increase the suspicion of
with GABAAR antibodies are often misdiagnosed an immune-mediated process, keeping in mind that
as having anti-GAD65-associated encephalitis the MRI findings could be the result of seizures or a
or Hashimoto’s encephalitis due to the frequent viral infection.
co-occurrence of GAD65 or thyroid peroxidase Antibody testing cannot replace the clinical
(TPO) antibodies. Patient’s GABAAR antibodies evaluation. Determination of antibodies should be

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 Inflammatory diseases and infection

Table 2. Relapsing symptoms post-herpes simplex encephalitis

Nonviral-related post-HSE or
Viral-related post-HSE encephalitis ‘choreathetosis post-HSE’

Time HSE to relapse Variable 4–6 weeks (also described as early as

7 days after onset of HSE)
Neurological symptoms Focal neurological signs, seizures, behavioral In children frequent abnormal movements
abnormalities, low frequency of abnormal (choreoathetosis, ballism), adults and
movements adolescents (abnormal behavior)
HSV PCR in CSF Positive Negative
New necrotic lesions Yes No
on MRI
Response to antiviral Yes No
therapy
Etiology Infectious Suspected autoimmune. A substantial number
of patients have NMDAR antibodies.
Some patients may have antibodies to DR2
and against unknown cell surface antigens

CSF, cerebrospinal fluid; DR2, dopamine receptor 2; HSE, herpes virus encephalitis; HSV, herpes simplex virus; MRI, magnetic resonance imaging; NMDAR,
N-methyl-D-aspartate receptor; PCR, polymerase chain reaction. Adapted from [55].

considered a supportive test to confirm the etiology Mycoplasma pneumoniae) can be positive and the
of a disorder clinically suspected to be immune patient still have an immune-mediated process.
mediated. In our experience, the association of If studies for an infectious or autoimmune
some syndromes with one or a restricted number etiology are negative, but there is concern for
of antibodies is so high that in many patients an underlying autoimmune process, one should
the type of syndrome directs the antibody testing. consider examining the CSF and serum in a research
This high syndrome antibody specificity is obtained laboratory. The rate of novel autoantibodies des-
when comprehensive testing for one or a specific cribed (approximately 1–2 per year) and the fact
subset of antibodies is applied, including immuno- that for many of them the initial assessment of
histochemistry with brain tissue and cell-based patient’s CSF was critical emphasize the importance
assays with patient’s serum and CSF. If studies are of banking or keeping aliquots of CSF.
less comprehensive (e.g., serum only with cell- The extent of tumor search depends on the type
based assays only), the specificity decreases and of antibody, age, and sex of the patient (as discussed
the number of false-positive or false-negative cases above) [26]. Immunotherapy (steroids, intravenous
&
increases [56 ]. The importance of a comprehen- immunoglobulin, or plasma exchange) can be
sive evaluation including CSF and serum was effective, but patients often require more aggressive
&
recently demonstrated in a study on anti-NMDAR therapies (rituximab, cyclophosphamide) [20 ].
&
encephalitis [56 ]. Although the follow-up of CSF and serum antibody
Comprehensive testing also identifies cases that titers may assist in some assessments (e.g., relapses
might erroneously be considered variants of a syn- or effects of treatment), clinical decisions about
drome or ‘widening’ of the spectrum of symptoms, changing or discontinuing treatments should rely
whereas in fact represent the overlap of two different more on clinical assessment (e.g., antibody titers
syndromes with independent immune responses may remain detectable after neurological recovery)
&
[e.g., myelitis or optic neuritis with aquaporin 4 [56 ].
or myelin oligodendrocyte glycoprotein (MOG) in
patients with anti-NMDAR encephalitis] [57]. There-
fore, it is important to store aliquots of CSF when CONCLUSION
spinal taps for viral studies are done. Depending on Autoimmune encephalitis comprises an expanding
the syndrome and degree of clinical suspicion, the group of potentially treatable disorders that should
study for autoantibodies can be initiated at the same be in the differential diagnosis of any type of ence-
time that viral studies are conducted or wait until phalitis. They can resemble infectious encephalitis,
PCR or serological studies for the most common and sometimes are triggered by infectious disorders
viruses are completed. However, it is important (e.g., HSE). Aggressive immunotherapy is often
to keep in mind that some tests (e.g. for HSV or effective.

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 Autoimmune encephalitis and infectious encephalitis Armangue et al.

13. Thomas L, Mailles A, Desestret V, et al. Autoimmune N-methyl-D-aspartate

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