Journal of Neuroimmunology 287 (2015) 9397

Contents lists available at ScienceDirect

Journal of Neuroimmunology
journal homepage: www.elsevier.com/locate/jneuroim

The spectrum of autoimmune encephalopathies,,

Divyanshu Dubey a,b,, Anshudha Sawhney b, Benjamin Greenberg b, Andrea Lowden b, Worthy Warnack b,
Pravin Khemani b, Olaf Stuve b,c,d, Steven Vernino b
a

Parkland Memorial Hospital, 5201 Harry Hines Blvd, Dallas, TX, USA
Department of Neurology & Neurotherapeutics, University of Texas Southwestern Medical Center, 5323 Harry Hines Blvd, Dallas, TX, USA
Neurology Section, VA North Texas Health Care System, Medical Service, Dallas, TX, USA
d
Department of Neurology, Klinikum rechts der Isar, Technische Universitt Mnchen, Germany
b
c

a r t i c l e

i n f o

Article history:
Received 25 January 2015
Received in revised form 4 August 2015
Accepted 19 August 2015
Available online xxxx
Keywords:
Encephalitis
Seizure
Limbic encephalitis
Autoimmune
Paraneoplastic

a b s t r a c t
Despite being a potentially reversible neurological condition, no clear guidelines for diagnosis or management of
autoimmune encephalitis exist. In this study we analyzed clinical presentation, laboratory and imaging characteristics, and outcome of autoimmune encephalitis from three teaching hospitals. Non-paraneoplastic autoimmune encephalitis associated with antibodies against membrane antigens was the most common syndrome,
especially in the pediatric population. Clinical outcome was better for patients with shorter latency from symptom onset to diagnosis and initiation of immunomodulation. Patients with underlying malignancy were less likely to respond well to immunomodulatory therapy. The clinical spectrum of autoimmune encephalitis is fairly
broad, but prompt recognition and treatment often leads to excellent outcome.
2015 Elsevier B.V. All rights reserved.

1. Introduction
There is growing interest in the etiopathogenesis and management
of autoimmune encephalitis. Identication of specic antibodies and
their availability for commercial testing have assisted in diagnosis
(Ramanathan et al., 2014). In the California encephalitis project
(between September 2007 and February 2011) 32 cases of anti-NMDA
receptor antibody mediated encephalitis were identied among patients 30 years (Gable et al., 2012). This number was even higher
than the combined frequency of Herpes Simplex virus, Varicella Zoster
virus and West Nile virus encephalitis. Clinically these disorders are

Funding: none.
Conict of interest: none.
Disclosures: Dr. Benjamin Greenberg has received consulting fees from EMD Serono,
Novartis and Medimmune. Dr. Olaf Stuve serves on the editorial boards of JAMA
Neurology, Multiple Sclerosis Journal, and Therapeutic Advances in Neurological
Disorders. Dr. Stuve has served on data monitoring committees for Pzer and SanoAventis without monetary compensation. Dr. Stuve has served on an advisory board for
Sano Genzyme and Genentech, and he has advised Huron Life Sciences and Navigant
Consulting. Dr. Stuve counselled Novartis in front of a Scientic Advisory Group at the
European Medicines Agency (EMA). Dr. Stuve currently receives grant support from
Teva Pharmaceuticals and Opexa Therapeutics. Dr. Stuve received travel support from
Pzer. Dr. Stuve is funded by a Merit grant from the US Department of Veterans Affairs.
Dr. Steven Vernino receives personal compensation as a scientic consultant for Athena diagnostics and on speaker bureau for Lundbeck. The other authors report no conicts of interest in this work.
Corresponding author at: Department of Neurology & Neurotherapeutics, University
of Texas Southwestern Medical Center, 5323 Harry Hines Blvd, Dallas, TX, USA.

http://dx.doi.org/10.1016/j.jneuroim.2015.08.014
0165-5728/ 2015 Elsevier B.V. All rights reserved.

challenging to diagnose due to myriad neurological manifestations

(Graus and Saiz, 2008; Graus et al., 2008; Gultekin et al., 2000). Diverse
clinical presentations include seizure, memory loss, behavioral changes,
altered sensorium, and dyskinesias (Dubey et al., 2014; Davis and
Dalmau, 2013; Sharma et al., 2012; Vernino et al., 2007). MRI changes
are likewise diverse with a predilection for mesial temporal lobe involvement. Cerebrospinal uid (CSF) inammatory ndings, presence
of specic antibodies in CSF or serum and response to immunomodulatory therapy can help identify these cases and distinguish autoimmune
encephalitis from other causes such as viral infections (Gultekin et al.,
2000; Sharma et al., 2012; Vernino et al., 2007). Several autoantibodies
have been associated with autoimmune encephalitis. Some of these
antibodies are directed against intracellular neuronal antigens such as
Hu, Ma-2, and CRMP-5 (Davis and Dalmau, 2013; Sharma et al., 2012;
Vernino et al., 2007; Hughes et al., 2010). Others are directed against
neuronal cell membrane antigens including voltage gated potassium
channel complex (VGKCc), NMDA receptors, GABAB receptor, voltagegated calcium channels (VGCC) and others (Davis and Dalmau, 2013;
Vernino et al., 2007; Lancaster and Dalmau, 2012). Prior studies have
shown that the neuronal nuclear and cytoplasmic antibodies have
stronger association with paraneoplastic syndromes, whereas antibodies against cell membrane antigens have a weaker association with cancer (Graus et al., 2008; Gultekin et al., 2000; Davis and Dalmau, 2013;
Lancaster and Dalmau, 2012). Dalmau et al. have reported that the response to immunomodulatory therapy varies based on the type of antibodies which may reect different forms of central nervous system
inammation (Dalmau and Bataller, 2007).

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D. Dubey et al. / Journal of Neuroimmunology 287 (2015) 9397

The purpose of this study was to analyze the clinical presentation, diagnostic features and management of autoimmune encephalitis in three
academic teaching hospital settings (a public county hospital, a university hospital and a pediatric tertiary care hospital). Our study also aimed
to assess the effect of early diagnosis and initiation of immunomodulatory treatment on clinical outcome.
2. Methods
We conducted a retrospective chart review at three afliated teaching hospitals [Parkland Memorial Hospital (PMH), UT Southwestern
University Hospital (UTSW), and Children's Health (CH)]. Clinical data
extending from January 2008 to December 2013 was collected. UT
Southwestern Medical Center institutional review board approved the
study on behalf of all sites. An initial case ascertainment was performed
based on diagnostic code of encephalitis (ICD-9 codes 323.0, 323.9). All
cases with encephalitis diagnosis during a hospital encounter were further screened based on the pre-specied inclusion and exclusion
criteria listed in Table 1.
Thyroid antibodies were not included as part of the serological inclusion criteria due to lack of specicity of this antibody for autoimmune
encephalopathy (Olmez et al., 2013). However, cases which met inclusion criteria but did not have a specic antibody were further divided
based on the presence or absence of high levels of thyroid peroxidase
(TPO) antibodies (N 100 IU/ml). Testing for subtypes of VGKCc antibodies (such as LGI1) was not available during the time period of this study.
Clinical improvement was dened based on the patients' main
symptoms; more than 50% reduction of seizure frequency, complete
or near complete resolution of memory impairment (MOCA or MMSE
score N25), complete or near complete resolution of behavioral changes,
or complete or near complete resolution of involuntary movements.
Data was collected using the electronic medical record system at the
three institutions. Clinical data was analyzed using SPSS 21 software.
Categorical variables were analyzed using Chi Square. Continuous variables were examined for normative or non-normative distribution. Normative data and non-normative data were analyzed using independent
t-test and MannWhitney U test respectively, p-value of b0.05 was considered statistically signicant.
Table 1
Depicting pre-specied inclusion and exclusion criteria.
Inclusion criteria

Exclusion criteria

1. Patients with rapidly progressive

mental status changes over 16 weeks
or new onset seizure activity
Plus at least two of the following
criteria:
1. CSF ndings consistent with
inammation (elevated CSF protein
N50 mg/dl and/or lymphocytic
pleocytosis)
2. Brain MRI showing signal changes
consistent with limbic encephalitis
(mesial temporal FLAIR signal
changes)
3. Autoimmune/paraneoplastic
antibodies in serum or CSF which
have been associated with
autoimmune encephalitis in previous
studies [any neuronal
nuclear/cytoplasmic antibody such as
anti-Hu or anti-CRMP-5, or any
neuronal membrane antibody
including anti-VGKCc, anti-NMDA-R,
anti-GABAB receptor, or anti-glutamic
acid decarboxylase-65 (GAD-65)
antibody (titer 0.5 nmol/L)]
4. New onset seizure or mental status
changes responding to
immunomodulatory therapies.

1. Evidence of CNS infection based on

acute serology or PCR analysis from CSF
2. Presence of metabolic or sytemic
abnormalities which would explain the
symptoms (such as severe renal or
hepatic failure or malignant
hypertension)

3. Results
During the 6 year study period, a total of 224 potential cases were
identied based on ICD-9 coding. Following screening of these cases
based on inclusion and exclusion criteria, 68 patients (11.33 patients
per year) were selected. Four patients were excluded due to limited
availability of medical records, and 64 cases of autoimmune encephalitis
were included in the nal analysis.
Of the 64 cases, 36 (56.3%) were males, and the mean age was
40.41 years (range: 2 to 86 years). The three study hospitals, PMH,
UTSW and CH contributed 27, 25 and 12 patients respectively. 34 of
these patients were included in a previous study on autoimmune epilepsy (Dubey et al., 2015).
Sixteen (25.0%) patients had antibodies against NMDA-receptor,
nine (14.1%) against voltage gated potassium channel complex antigens
(VGKCc), six (9.4%) against Glutamic Acid Decarboxylase-65 (GAD-65),
three (4.7%) against GABAB receptor and one (1.6%) had anti-Hu antibody. Nine patients (14.1%) had thyroperoxidase (TPO) as the only serological marker. Half of the anti-NMDA receptor antibody cases were
from CH. Among the remaining pediatric cases, 1 had antibody against
GAD-65 and in 3 patients no encephalitis-related antibody detected.
No encephalitis-related antibody was indentied in 20 (31.3%) patients,
but these patients' met the other inclusion criteria and infectious etiologies were ruled out. Underlying malignancy was detected only in 12
(18.8%) patients. Among the 12 patients with malignancy; 2 had breast
cancer, 2 ovarian teratomas, 1 neuro-endocrine prostate cancer, 1 adenocarcinoma of the lung, 1 small cell lung cancer, 1 pancreatic cancer,
1 testicular cancer, 1 papillary thyroid cancer, 1 thymoma and 1
lymphoma.
The clinical presentation correlated with the type of encephalitisrelated antibody (Table 2, Fig. 1). The majority of patients with antiNMDA-R antibody were less than 40 years of age (87.5%) whereas
those with anti-VGKCc antibody were clustered between 4060 years
(77.8%). 37.5% of patients with NMDA-R antibody and 22.2% patients
with VGKCc antibody had prodromal symptoms. Behavioral changes
were the most common initial symptoms among NMDA-R antibody
(43.7%) and VGKCc antibody (44.4%) groups, while 33.3% of patients
with GAD-65 antibody developed involuntary movements or ataxia as
their rst clinical symptom. 68.7% of the patients with anti-NMDA-R antibody had change in their level of consciousness, but impaired mental
status was only seen in one of the six anti-GAD antibody positive patients. The majority of patients in the anti-GAD antibody group developed movement disorder (66.7%) and speech changes (50%). 62.5% of
patients with anti-NMDA-R antibodies were also found to have involuntary movements. Autonomic instability (presenting as labile
blood pressure, labile heart rate, persistent tachycardia, postural hypotension) seemed to be more prevalent among the anti-NMDA-R antibody (56.25%) group. Only 33.3% of anti-VGKCc antibody, 33.3% of
anti-GAD antibody and 11% anti-TPO antibody patients had autonomic
dysfunction.
Seizures were prevalent in patients with anti-VGKCc antibody
(88.9%), anti-NMDA-R antibody (75%), anti-GAD antibody (66.7%) and
anti-TPO antibody (55.6%) groups. 70% of the patients with no specic
antibodies had seizures during the course of their illness. 44 patients included in the study had seizures attributed to autoimmune encephalitis.
Among these, 36 patients had temporal lobe onset (31 unilateral temporal and 5 bilateral temporal) and 8 had extra-temporal onset or multiple ictal foci.
Among the 64 patients studied, 60 had lumbar puncture for CSF
analysis. Median time from symptom onset to CSF collection was
22 days (1 to 308 days). In 36.7% patients, the CSF was unremarkable
with normal protein and cell count. Median cell count was 2 cells/l
(range 0 to 137 cells/l) and median CSF protein was 42.4 mg/dl
(range 0 to 434 mg/dl). All cases were evaluated with MRI of the
brain. Median duration of symptom onset to MRI was 18 days (1 to
309 days). 40.6% patients had no MRI abnormalities suggestive of

D. Dubey et al. / Journal of Neuroimmunology 287 (2015) 9397

95

Table 2
Demographic and clinical characteristics of autoimmune encephalitis patients.
Type of
antibodies

Number

Age range (years)

Gender

First symptom

MRI T2/FLAIR
abnormality (%)

Malignancy

Clinical
improvement

NMDA

16

62.5% (10)

77.8

33.3

4 (breast Ca, lymphoma,

thymoma, PTCA)
0

44.4% (4)

GAD

Behavioral changes (43.7%),

seizure (31.2%)
Behavioral changes (33%),
seizure (22.2%)
Memory loss (40%)

2 (ovarian teratoma)

TPO

Behavioral changes (33%)

66.7

55.6% (5)

GABAB

Seizures (66%)

66.7

1 (SCC lung)

66.7% (2)

Hu

M8
F8
M7
F2
M4
F2
M3
F6
M1
F2
M0
F1
M 13
F7

43.75

VGKCc

15.5
(244)
55
(4586)
39.5
(1572)
47
(2574)
53
(3469)
66

No Antibody

20

44
(566)

Memory loss (100%)

Seizure (41%)

0
70

0
5 (breast Ca, prostate Ca, pancreatic Ca,
ADCA lung, testicular Ca)

50% (3)

0
65% (13)

VGKCc voltage gated potassium channel complex antibody; NMDA N-methyl-D-aspartate receptor antibody; GAD glutamic acid decarboxylase receptor antibody; GABAB -aminobutyric
acid B receptor antibody; M Male; F Female; Ca Cancer; PTCA Papillary thyroid cancer; SCC small cell cancer; ADCA adenocarcinoma; NL Normal.

encephalitis. Unilateral temporal lobe involvement, bilateral temporal

lobe involvement and extra-temporal involvement were seen in 18
(28.1%), 8 (12.5%) and 12 (18.8%) patients respectively. Repeat MRI to
assess for interval change in imaging was performed in 46 patients. In
the majority of patients, there was no interval change. Only 4 patients'
showed complete resolution and 5 had partial resolution of lesions.
Immunomodulatory therapy was initiated in 59 (92.2%) patients
(Fig. 2). Median time from symptom onset to immunomodulation therapy was 29 days (6 to 613 days). Intravenous corticosteroid was used in
82.8% (53) of the patients, typically as 1000 mg methylprednisolone

intravenously daily for 5 days. Plasmapheresis was used in 60.9% (39)

patients, usually given as 5 exchanges on alternate days during inpatient admission. Intravenous immunoglobulin, rituximab, mycophenolate, cyclophosphamide were used in 18 (28.1%), 13 (20.3%), 10
(15.6%) and 8 (12.5%) patients respectively. 12 patients were found to
have an underlying tumor, only 5 of them underwent tumor resection.
Three patients underwent neo-adjuvant chemotherapy (chemotherapy
plus radiation therapy), two patients received only chemotherapy, and
for two patients no chart documentation of cancer treatment was
found.

Fig. 1. (A) Initial symptoms and signs of autoimmune encephalitis. Among the entire cohort, the most common initial symptoms were behavioral changes (38%) and seizure (28%).
(B) Symptoms present at any time in the disease course differed somewhat between sub-groups. Abnormal movements were most likely to occur in patients with anti-NMDA antibody
(dyskinesias) or anti-GAD-65 antibody (ataxia). Short-term memory impairment and seizures were nearly always present in patients with VGKCc antibody. Among patients with anti-TPO
antibody and those without an encephalitogenic antibody, behavioral changes were the most common clinical feature.

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D. Dubey et al. / Journal of Neuroimmunology 287 (2015) 9397

Clinical improvement was evident in more than half (57.8%) of the

patients included in the study at the time of rst outpatient follow-up
visit. Mean time from inpatient discharge to initial clinic follow-up
was 52 days (12 to 117 days), and this interval was not different between patients that improved and those that did not. Time from symptom onset to diagnosis among patients who showed neurological
improvement (Table 3) compared to those with no clinical improvement was signicantly shorter. Earlier initiation of immunomodulation
was also associated with clinical improvement (Table 3). Even after adjustment for baseline characteristics (age, gender, race, type of antibody), time from symptom onset to diagnosis (OR 0.98, CI 0.970.99,
p b 0.05) and time from symptom onset to immunomodulatory treatment (OR 0.99, CI 0.980.99, p b 0.05) continued to be signicantly
lower in group showing clinic improvement.
Among patients with seizures, 26 out of 44 had at least 50% reduction in seizure frequency at the time of follow-up. With respect to
other symptoms, 50.0%, 64.8% and 59.1% patients showed considerable
improvement in memory, personality/behavior and movement disorders respectively. There was no signicant difference in clinical outcome
in patients who received high dose corticosteroids compared to those
receiving plasmapheresis as initial immunomodulatory therapy. Patients with underlying malignancy were less likely to improve with
treatment (p b 0.05), while patients who gave a history of preceding
u-like prodromal symptoms were signicantly more likely to have
good clinical outcome (p b 0.005).
Comparing cases from the adult hospitals (UTSW and PMH) and pediatric hospital (CH), the time of symptom onset to diagnosis (33 days
versus 18 days, p b 0.05) and symptom onset to immunodulatory
therapy initiation (32 days versus 19 days, p b 0.05) were signicantly
lower at CH. There was no statistically signicant difference in good
neurological outcome based on location (PMH & UTSW: 55.7%, CH:
66.7%, p = 0.491).
4. Discussion
Autoimmune encephalitis is not rare and may present in patients
of all ages. Consistent with other reports, we found that nonparaneoplastic cases associated with antibodies against cell surface antigens are more common than classic paraneoplastic limbic encephalitis. Early diagnosis and initiation of immunomodulatory therapy is
associated with signicantly better clinical outcome in patients with autoimmune encephalitis, and patients with paraneoplastic encephalitis
were more refractory to treatment compared to those in whom no malignancy was identied.
Our study adds to existing data and understanding of clinical presentation of autoimmune encephalitis. Behavioral changes and seizures
were found to be the most common initial presenting symptoms
(Fig. 1A). These were also the most prevalent symptoms along with
memory loss and altered sensorium experienced by patients throughout the disease course. Recognition of these clinical symptoms and
signs as features of autoimmune encephalitis should lead to earlier diagnosis. Variations in clinical presentation were noted to be associated
with specic antibody types (Lancaster and Dalmau, 2012; Dalmau,
Table 3
Clinical improvement was signicantly associated with earlier diagnosis and initiation of
treatment; median values of the variables (range) and p values for the association with
good clinical outcome.
Clinical improvement
Yes
Duration of symptom onset to diagnosis
(days)
Duration of symptom onset to
immunomodulation (days)
Duration of symptom onset to clinic
follow up (days)

No

2009). The presence of involuntary movement and ataxic speech with

normal mental status suggests anti-GAD antibody encephalitis whereas
young patients (b40 years) presenting with seizures, altered sensorium,
involuntary movements, and autonomic instability would point towards NMDA-R encephalitis.
Patients included in our study came from three institutions; a county/community hospital, a university hospital/tertiary care center and a
tertiary care pediatric hospital. Eight of the 12 pediatric autoimmune
encephalitis cases that we identied were associated with antiNMDA-R antibodies, highlighting the importance of this type of autoimmune encephalopathy in children. Interestingly, in comparison to adult
patients, diagnosis and initiation of immunomodulatory therapy was
sooner at CH, although difference in clinical outcome did not reach statistical signicance. Earlier initiation of immunomodulation of pediatric
NMDA encephalitis patients compared to adults was also reported by
Titulaer et al. (21 days versus 28 days), without any signicant difference in clinical outcome (Titulaer et al., 2013). This observation suggests
that suspicion of NMDA-R encephalitis is higher for children presenting
with sub-acute encephalopathy, perhaps because there are fewer comorbidities and fewer alternative explanations for rapidly progressive
behavioral changes in children.
With 64 patients, our study is one of the larger series of autoimmune
encephalitis published to date. We not only included NMDA encephalitis cases but also those with other antibody subgroups and cases of
suspected autoimmune encephalitis with no detectable
encephalopathy-related antibody. Thus, the relative prevalence of different autoimmune encephalitis syndromes cases can be assessed.
Most of the cases in our series had neuronal cell membrane associated
antibodies, whereas many of the previously published case series included mostly patients with classic paraneoplastic antibodies
(Gultekin et al., 2000; Lawn et al., 2003). This reects the growing recognition of non-paraneoplastic autoimmune encephalitidies and the
higher association of these disorders with antibodies against cell membrane antigens (Ramanathan et al., 2014). The prevalence of seronegative cases and of those with non-specic antibody markers such as
anti-TPO antibody remains unclear. These continue to represent an important but challenging clinical group. It is also important to recognize
that treatment-responsive autoimmune encephalitis can be associated
with normal CSF and brain imaging.
As a retrospective study with specic inclusion criteria, the number
of autoimmune encephalitis cases is likely underestimated. Limited
numbers and the diversity of clinical syndromes make the assessments
of association or correlation difcult. Since many of the patients were
not evaluated with PET imaging, small underlying tumors could have
been missed. Also since long term follow up data was not a part the
study, underlying malignancy which was too small to be detected at
the time of initial screening could have been missed.
This study highlights certain important clinical aspects of autoimmune encephalitis. Early identication and prompt initiation of immunomodulatory agents appears to be benecial for these patients. In
future, prospective studies evaluating the impact of type or duration
of initial and maintenance immunomodulatory therapy on clinical outcome and disease relapse are required. To facilitate early diagnosis, clinicians need to maintain a high level of suspicion when evaluating
patients with new onset seizures or encephalopathy since autoimmune
encephalopathies likely remain under-diagnosed.
Supplementary data to this article can be found online at http://dx.
doi.org/10.1016/j.jneuroim.2015.08.014.

p
value

16 (4209)

54 (7513)

0.001

19 (7221)

52.5 (9513)

0.001

59 (38290) 93.5 (37553) 0.001

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