VGKC-complex/LGI1-antibody encephalitis: Clinical manifestations and response to immunotherapy

Shin, Yong-Won; Shin, Jung-Won; Moon, Jangsup; Lim, Jong-Deock; Byun, Jung-Ick; Kim, Tae‐Joon; Lee, Keon‐Joo; Kim, Young-Su; Park, Kyung‐Il; Jung, Keun‐Hwa; Lee, Sang Kun; Chu, Kon

doi:10.1016/j.jneuroim.2013.10.005

Cited by 205 publications

(259 citation statements)

References 25 publications

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“…We did not find extratemporal lesions, i.e. basal ganglia hyperintensities in our patients including FBDS cases in contrast to earlier studies [9,10,14,30,31]. Nevertheless, we found that higher volume of the putamen was associated with better cognition measured by ACE test at 23.4±7.6 months indicating the affection of the basal ganglia also in our studies, and that basal ganglia atrophy may affect functional outcomes in patients with LGI1 encephalitis.…”

Section: Discussioncontrasting

confidence: 99%

“…The putamen is actively involved in a variety of cognitive functions such as episodic memory, cognitive control and category learning [38], and its inter-connection with frontal areas and hippocampus play an important role in cognition [39,40]. Hyperintensities previously published in VGKC and LGI1 encephalitis/FBDS pointed to the affection of these structures, and a very recent study reported correlation between longer duration of FBDS and pallidum volume [9,14,[28][29][30]. Although we found decreased hippocampal volumes compared to matched controls, hippocampal volumetry did not correlate with ACE and mRS outcomes.…”

Section: Accepted Manuscript 12mentioning

confidence: 99%

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Global brain atrophy and metabolic dysfunction in LGI1 encephalitis: A prospective multimodal MRI study

Szőts

Blaabjerg

Orsi

et al. 2017

Journal of the Neurological Sciences

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Background: Chronic cognitive deficits are frequent in leucin-rich glioma-inactivated 1 protein (LGI1) encephalitis. We examined structural and metabolic brain abnormalities following LGI1 encephalitis and correlated findings with acute and follow-up clinical outcomes. Methods:Nine patients underwent prospective multimodal 3 Tesla MRI 33.1±18 months after disease onset, including automated volumetry, diffusion tensor imaging (DTI) and magnetic resonance spectroscopy (MRS). Data were compared to 9 age-and sex-matched healthy controls. Conclusions:Poor clinical outcome following LGI1 encephalitis is associated with global brain atrophy and disintegration of white matter tracts. The pathological changes affect not only temporomesial structures but also frontal lobes and the cerebellum.

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Section: Discussioncontrasting

confidence: 99%

Section: Accepted Manuscript 12mentioning

confidence: 99%

Global brain atrophy and metabolic dysfunction in LGI1 encephalitis: A prospective multimodal MRI study

Szőts

Blaabjerg

Orsi

et al. 2017

Journal of the Neurological Sciences

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“…neuropathological examination reveals chronically active inflammatory processes in medial temporal lobe and/or the presence of a tumour diagnosed within 5 years of the occurrence of neurological symptoms, and/or the presence of autoantibodies, and/or the signs of inflammation in medial temporal lobe in MRI, which cannot be explained otherwise) [9][10][11]/ Co najmniej jeden objaw z badań morfologicznych (w badaniu neuropatologicznym stwierdzenie przewlekłego zapalenia przyśrodkowej części płata skroniowego i/lub obecność guza zdiagnozowanego w ciągu 5 lat od wystąpienia objawów neurologicznych, i/lub obecność autoprzeciwciał, i/lub w badaniu MRI cechy zapalenia w przyśrodkowej części płata skroniowego, których nie można wyjaśnić inaczej) [9][10][11] the potassium channel (alpha subunit) are rarely detected. Most frequently detected antibodies are those directed against the LGI1 protein, which plays an important role in synaptic transmission as it binds to preand post-synaptic protein complexes [24][25][26][27][28][29], against the CASPR2-associated protein contaktin-2. Limbic encephalitis may have some characteristic medical signs depending on the type of antibodies present.…”

Section: Dunclassified

“…In the T1 sequence, the temporal-limbic area may be hypointense and atrophic, and rarely enhances after contrast injection [16]. In PLE variants, extratemporal lobe epilepsy was also reported [26]. If MR imaging is normal, fluorodeoxyglucose-positron emission tomography (FDG-PET) should show increased signal in medial temporal lobe in acute inflammatory phase [35,36].…”

Section: Dmentioning

confidence: 99%

Non-paraneoplastic variant of limbic encephalitis – case report

Głuszak¹,

Pazderska-Głuszak²,

Gut-Lepiech³

et al. 2017

ppn

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Purpose: Limbic encephalitis (LE) is a rare but necessary condition to consider when experiencing cognitive disorders, seizures, and psychiatric disorders. Case description: This article presents a case of a 28-year-old patient with uncontrolled disease history, with systemic symptoms of viral infection and prolonged convulsive seizure status. Based on clinical symptoms, magnetic resonance imaging of the brain and cerebrospinal fluid test results revealed LE. After treatment and rehabilitation, the patient returned to work. Comment: Limbic encephalitis was first described in 1960. In terms of aetiology, we divide LE into non-infectious (a para nitrogen syndrome primarily associated with small cell lung cancer) and infectious cases (caused by herpes simplex virus). Other cases of autoimmune diseases have also been reported -non-paraneoplastic limbic encephalitis (NPLE), in which the presence of antibodies against the antigen of the neuronal membrane is observed. Non-paraneoplastic LE variants are examples of potentially reversible LE variants. Key words: limbic encephalitis, paraneoplastic syndrome, onconeuronal antibodies. StreszczenieCel: Limbiczne zapalenie mózgu (LE) jest chorobą rzadką, którą należy jednak uwzględnić w przypadku wystąpienia u pacjenta zaburzeń poznawczych, napadów padaczkowych i zaburzeń psychicznych. Opis przypadku: W pracy przedstawiono przypadek 28-letniej pacjentki z nieobciążonym wywiadem chorobowym, u której wystąpiły objawy ogólnoustrojowe infekcji wirusowej oraz przedłużający się drgawkowy stan padaczkowy. Na podstawie objawów klinicznych, obrazu rezonansu magnetycznego mózgu i wyników badań płynu mózgowo-rdzeniowego rozpoznano LE. Po leczeniu i rehabilitacji pacjentka wróciła do poprzednio wykonywanej pracy. Komentarz: Limbiczne zapalenie mózgu zostało opisane po raz pierwszy w 1960 r. Pod względem etiologicznym wyróżniamy przypadki nieinfekcyjne LE (o charakterze zespołu paranowotworowego związanego głównie z rakiem drobnokomórkowym płuc) oraz przypadki infekcyjne LE (spowodowane przez wirusa opryszczki pospolitej). Opisano również przypadki związane z chorobami

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“…However, some findings indicate that combined and earlier (within the first 2 months after initial manifestation) treatment is associated with a better prognosis [49,63]. A retrospective study (n = 14) found that combination therapy of IVIG plus steroids was superior to steroid monotherapy [64]. In an open prospective study, all patients (n = 9) improved when treated with the combination of plasma exchange, IVIG and steroid pulse therapy followed by oral steroid treatment [65].…”

mentioning

confidence: 99%

Management of Immune-Mediated Paraneoplastic Neurological Disorders

Ayzenberg

Gold

Kleiter

2017

Neurology International Open

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AbsTR Ac TParaneoplastic neurological disorders are rare and clinically heterogeneous diseases. They can affect both the central and peripheral nervous system as well as the neuromuscular junction and muscle. Neurological deficits develop in 2/3 of cases prior to cancer diagnosis. The diagnostic approach includes screening for antineuronal antibodies and a search for the underlying tumor. A prompt tumor therapy in combination with immunotherapy is the cornerstone in the management of these diseases. Due to lack of clinical trials, treatment recommendations are based on case series and expert opinions. Highdose corticosteroids, intravenous immunoglobulins and apheresis therapies are often used in the acute stage of the disease. These therapies should be started as early as possible, e. g., during tumor screening, in order to prevent irreversible damage. Long-term treatment is mostly immunosuppressive and depends on the specific paraneoplastic syndrome. Outcomes vary depending upon the prognosis of the underlying cancer and the nature of the antineuronal antibodies. Disorders with antibodies directed against antigens on the neuronal cell surface are highly sensitive to B cell-directed therapies and mostly associated with a favorable outcome. A thorough review of published data on actual treatment recommendation is provided along with discussion of currently not validated, but potentially effective new therapies. E264Ayzenberg I et al. Management of Immune-mediated Paraneoplastic … Neurology International Open 2017; 1: E264-E274 course, treatment response and prognosis of PNS [2,3]. Depending on tumor association, obligate and facultative paraneoplastic Abs are differentiated (Tab. 1).Clinically, the following PNS exist [3,4]: A. In the central nervous system: ▪ Encephalomyelitis (incl. brainstem encephalitis) ▪ Limbic encephalitis ▪ Subacute cerebellar degeneration ▪ Opsoclonus-myoclonus syndrome ▪ Optic neuritis/neuromyelitis optica ▪ Myelopathy/myelitis ▪ Stiff person syndrome ▶Table 1 Paraneoplastic antibodies: associated neurological syndromes and tumors. Target antigen clinical presentation# Association with tumorObligate paraneoplastic Abs (directed against intracellular antigens; tumor association > 95 %) In this review we discuss the diagnosis and management of PNS. With regard to facultative paraneoplastic syndromes which are more frequently of autoimmune origin, such as neuromyelitis optica or myasthenia gravis, the reader should refer to pertinent reviews [5,6]. Diagnostic and Pathogenetic Significance of Antibodies to Intracellular and Surface AntigensAntibodies (Abs) to intracellular antigens or so-called "classical" Abs are almost always tumor-associated (exception: GAD-Abs). They can be associated with various tumors and a variety of neurological conditions [7]. However, a direct pathogenetic significance of these Abs has not yet been demonstrated. Abs to intracellular antigens serve as useful diagnostic markers in the targeted tumor search in PNS. Here, cell-mediated immune responses play a major p...

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VGKC-complex/LGI1-antibody encephalitis: Clinical manifestations and response to immunotherapy

Cited by 205 publications

References 25 publications

Global brain atrophy and metabolic dysfunction in LGI1 encephalitis: A prospective multimodal MRI study

Global brain atrophy and metabolic dysfunction in LGI1 encephalitis: A prospective multimodal MRI study

Non-paraneoplastic variant of limbic encephalitis – case report

Management of Immune-Mediated Paraneoplastic Neurological Disorders

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