Limbic Encephalitis Associated With GAD65 Antibodies: Brief Review of the Relevant literature

Gagnon, Marc-Aurèle; Savard, Martin

doi:10.1017/cjn.2016.13

Cited by 44 publications

(42 citation statements)

References 53 publications

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“…For two of our patients, epilepsy diagnosis was not obvious and long-term video-EEG was essential to correlate this exclusive subjective semiology to recurrent temporal lobe status epilepticus. In the literature, the description of seizures is limited, but intense subjective impairment (such as dysgueusia and dysosmia) has previously been reported in a review (Gagnon and Savard, 2016). The two first patients presented with LE semiology with temporo-mesial seizures, whereas the third patient clearly presented with lateral temporal and frontal seizures arguing for non-LE.…”

Section: Discussionmentioning

confidence: 99%

GAD65‐Ab encephalitis and subtle focal status epilepticus

Goudot

Frismand

Hopes

et al. 2019

Epileptic Disorders

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Aims . To delineate common epilepsy features associated with the presence of glutamic acid decarboxylase autoantibodies (GAD65‐Ab). Methods . Three consecutive cases of GAD65‐Ab encephalitis patients, followed in our neurological department, were investigated with regards to clinical semiology and EEG. Results . These patients presented new‐onset subtle ictal clinical features. Patients 1 and 2 described prolonged and transitory feelings of “ déjà vu ‐ déjà vécu ” and a “ dreamy state ”. Patient 3 was admitted for subsequent transient aphasia events followed by paroxysmal behavioural disturbances. Epileptic origin of the symptoms was confirmed using either a standard EEG (observation of temporal status epilepticus in one case) or a prolonged EEG (focal epileptiform activity during an asymptomatic period for two patients). All patients suffered from clinical focal status epilepticus. Patients 1 and 2 presented with temporo‐mesial seizures in agreement with the definition for limbic encephalitis, whereas Patient 3 presented with neocortical (lateral temporal and frontal lobe) seizures arguing for a non‐limbic encephalitis. A high level of GAD65‐Ab was found in cerebral spinal fluid, confirming a diagnosis of epilepsy associated with GAD65‐Ab encephalitis. Conclusion . Encephalitis seems to be a frequent neurological syndrome associated with GAD65‐Ab disorders. Epilepsy may be more frequent and severe than currently suggested, as ictal semiology may be subtle for these outpatients in whom standard EEG is commonly falsely reassuring. Subtle focal status epilepticus is a particular semiology of the GAD65‐Ab encephalitis spectrum.

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Section: Discussionmentioning

confidence: 99%

GAD65‐Ab encephalitis and subtle focal status epilepticus

Goudot

Frismand

Hopes

et al. 2019

Epileptic Disorders

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“…Among 58 literature patients [60], 45/58 (78%) patient MRIs were abnormal with specific involvement of the temporal lobes in 34 (59%), and multifocal abnormalities in 9 (16%); and 7 patient MRIs were normal.…”

Section: Laboratory Investigationsmentioning

confidence: 99%

“…Gangnon & Savard [60] reviewed the clinical experience of 58 cases of GAD65-antibody LE beginning with the first reported case [61] inclusively through 2016,in 7 observational studies, 3 case series, and 21 published case reports [31,49,. They provide a useful summary of the literature of anti-GAD65 associated LE.…”

Section: Clinical Aspectsmentioning

confidence: 99%

Autoimmune Encephalitides

Younger¹

2017

NNOAJ

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Autoimmune encephalitis is a severe inflammatory disorder of the brain with diverse causes and a complex differential diagnosis. A recent advance in the past decade has led to the identification of new syndromes and biological markers that have transformed the approach to diagnosis and management of autoimmune encephalitis. Limbic encephalitis, the commonest form of autoimmune encephalitis, combines common presentations of cognitive, psychiatric, and epileptic disorders and has until recently been considered paraneoplastic or postinfectious in origin. The autoimmune encephalitides are clinically and histopathologically associated with serum and intrathecal antibodies to intracellular and surface neuronal antigens, and constituents of the limbic system neuropil. This has led to a reconsideration of a number of neuropsychiatric and neurocognitive disorders as having shared mechanisms of origin. This chapter reviews their historical background, clinical presentation, laboratory evaluation, histopathology, diagnosis and management.

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“…Clinical Aspects: Gangnon and Savard [60] reviewed the clinical experience of 58 cases of GAD65-antibody LE beginning with the first reported case [61] inclusively through 2016, in 7 observational studies, 3 case series, and 21 published case reports [31] [49] [62]- [88]. They provide a useful summary of the literature of anti-GAD65 associated LE.…”

Section: Anti-gad65 Encephalitismentioning

confidence: 99%

“…In the series of 58 literature cases summarized by Gangnon and Savard [60] follow-up was available in 53 cases. Full recovery was noted in 4 (8%) cases, 3 of whom received corticosteroids alone, with intravenous (IV) immunoglobulins, or in combination with PE; a fourth case received no immunosuppressant therapy and recovered.…”

Section: Anti-gad65 Encephalitismentioning

confidence: 99%

Autoimmune Encephalitides

Younger¹

2017

WJNS

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Autoimmune encephalitis is a potentially severe disorder of the brain of diverse causes with a complex differential diagnosis. Recent advances in the past decade have led to the elucidation of new syndromes and biological markers transforming the approach to diagnosis and management of autoimmune encephalitis. Limbic encephalitis, the commonest form of autoimmune encephalitis, combines common presentations of cognitive, psychiatric, and epileptic disorders and has until recently been considered paraneoplastic or postinfectious in origin. The autoimmune encephalitides are clinically and histopathologically associated with serum and intrathecal antibodies to intracellular and surface neuronal antigens, and constituents of the limbic system neuropil. This has led to a reconsideration of a number of neuropsychiatric and neurocognitive disorders as having shared mechanisms of origin. This chapter reviews their historical background, clinical presentation, laboratory evaluation, histopathology, diagnosis and management. KeywordsAutoimmune, Encephalitis, Hashimoto, Encephalopathy Historical PerspectiveCorsellis and colleagues [1] coined the term limbic encephalitis (LE) in 1968, noting a relation to bronchial cancer in three patients in the sixth to eighth decade of life, and showing close clinicopathologic similarity to cases described by Brierley and colleagues [2] six years earlier. All three cases had subacute temporal lobe seizures, neuropsychiatric, and memory disturbances for two years before death. Postmortem examination showed inflammatory lesions in limbic grey matter sections of the brain, especially in medial temporal lobe structures of the uncus and amygdala nuclei, and hippocampal, cingulum and dentate gyri. Case 2 had an undifferentiated non-metastatic lung carcinoma removed six months after onset of neurological symptoms, while two others had a clinically unsus- The formulation of the relation of GAD antibodies to SPS has been especially instructive in understanding how far the science of autoimmune neurologic disorder has advanced. In 1988, Solimena and colleagues [28] investigated the existence of non-paraneoplastic CNS autoimmunity in a patient with SPS, epilepsy and type-1 diabetes (T1D), and increased titers of oligoclonal CSF IgG. Both the serum and CSF produced identical intense staining of all gray-matter regions. GAD65 was thence an important autoantigen in T1D, being highly expressed in the cytoplasm of pancreatic β cells. GAD-derived peptides were presentable by main histocompatibility complex (MHC) class I molecules and recognized by CD8+ cells on the surface of β cells [29]. Activation of CD8+ GAD-specific T-cells further differentiated into memory cells suggesting a pathogenic role in T1D [30]. However, only patients with very high titers of GAD were associated with LE [31], and they typically presented with recent-onset temporal lobe epilepsy (TLE) and intrathecal secretion, defining a form of non-paraneoplastic LE. Other patients within the SPS spectrum harbored antibodies agains...

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Limbic Encephalitis Associated With GAD65 Antibodies: Brief Review of the Relevant literature

Cited by 44 publications

References 53 publications

GAD65‐Ab encephalitis and subtle focal status epilepticus

GAD65‐Ab encephalitis and subtle focal status epilepticus

Autoimmune Encephalitides

Autoimmune Encephalitides

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