Severe dysautonomia as a main feature of anti-GAD encephalitis: Report of a paediatric case and literature review

Achour, N. Ben; Younes, Thouraya Ben; Rebai, Ibtihel; Ahmed, Mélika Ben; Kraoua, Ichraf; Youssef–Turki, Ilhem Ben

doi:10.1016/j.ejpn.2018.01.004

Cited by 35 publications

(23 citation statements)

References 10 publications

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“…Preceding these features, cardiac block and syncope could have been secondary to dysautonomia as described. Severe dysautonomia related to anti-GAD-related limbic encephalitis was also reported to be the cause of death in a 9year-old child that developed after immunotherapy [8]. However, our patient had the pacemaker at the start of his illness which could have been associated with a positive outcome.…”

Section: Discussionmentioning

confidence: 72%

Anti-GAD-associated limbic encephalitis: an unusual clinical manifestation from northwest of Ireland

Mansoor

Murphy

2020

Egypt J Neurol Psychiatry Neurosurg

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Section: Discussionmentioning

confidence: 72%

Anti-GAD-associated limbic encephalitis: an unusual clinical manifestation from northwest of Ireland

Mansoor

Murphy

2020

Egypt J Neurol Psychiatry Neurosurg

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“…First, we excluded studies for which it was difficult to assess the efficacy of methylprednisolone, immunoglobulin or a combination of methylprednisolone and immunoglobulin because patients received immunosuppressive therapy in addition to IVMP or IVIG meanwhile, such as plasma exchange [14][15][16], immunoadsorption [17], azathioprine [18], rituximab [19], mycophenolate mofetil [20] or others [21]. Second, we excluded studies for which we could not obtain the patients' response to IVMP or IVIG or their combination because of the ambiguous descriptions or the inaccessible follow-up information in the original literature [22][23][24][25][26][27]. Third, we excluded studies for which the different clinical manifestations of patients responded inconsistently to treatment; for example, in patient D in the literature [28], seizure frequency did not respond to IVMP, while CA improved.…”

Section: Methodsmentioning

confidence: 99%

Intravenous methylprednisolone or immunoglobulin for anti-glutamic acid decarboxylase 65 antibody autoimmune encephalitis: which is better?

Li¹,

Zhang²,

Wang³

et al. 2020

BMC Neurosci

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Background: Patients positive for anti-glutamic acid decarboxylase 65 (GAD65) antibodies have attracted increasing attention. Their clinical manifestations are highly heterogeneous and can be comorbid with tumors. Currently, there is no consensus on the therapeutic regimen for anti-GAD65-associated neurological diseases due to the clinical complexity, rarity and sporadic distribution. We reported six anti-GAD65 autoimmune encephalitis (AE) patients who received intravenous methylprednisolone (IVMP) or immunoglobulin (IVIG) or both. Then, we evaluated the therapeutic effect of both by summarizing results in previous anti-GAD65 AE patients from 70 published references. Results: Our six patients all achieved clinical improvements in the short term. Unfortunately, there was no significant difference between IVMP and IVIG in terms of therapeutic response according to the previous references, and the effectiveness of IVMP and IVIG was 45.56% and 36.71%, respectively. We further divided the patients into different subgroups according to their prominent clinical manifestations. The response rates of IVMP and IVIG were 42.65% and 32.69%, respectively, in epilepsy patients; 60.00% and 77.78%, respectively, in patients with stiff-person syndrome; and 28.57% and 55.56%, respectively, in cerebellar ataxia patients. Among 29 anti-GAD65 AE patients with tumors, the response rates of IVMP and IVIG were 29.41% and 42.11%, respectively. There was no significant difference in effectiveness between the two regimens among the different subgroups. Conclusion: Except for stiff-person syndrome, we found that this kind of AE generally has a poor response to IVMP or IVIG. Larger prospective studies enrolling large numbers of patients are required to identify the optimal therapeutic strategy in the future.

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“…Antibodies to GABA B1 receptor are found in the serum and/or CSF in most patients. 31 Increased signals are seen in the majority on MRI T2-FLAIR sequences in the medial temporal lobes and the hippocampi. CSF analysis reveals lymphocytic pleocytosis in most patients and some with elevated intrathecal oligoclonal bands.…”

Section: Gamma-amino Butyric Acid Subunit-b Receptor Encephalitismentioning

confidence: 99%

“…28 Pediatric patients who present acutely often have nonspecific prodromal symptoms with fever and vomiting, followed by autonomic instability (changes in heart rate and blood pressure) in addition to seizure activity. 29,31 Individuals with this form of encephalitis have a poor response to AEDs, and those with status epilepticus are often medically refractory. There is moderate response to IVIg, corticosteroids, and plasma exchange.…”

Section: Glutamic Acid Decarboxylase Antibodies-associated Encephalitismentioning

confidence: 99%

Antibody-Associated Autoimmune Encephalitis and Epilepsies in Children

Govil

Williams

2018

J Pediatr Epilepsy

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Autoimmune causes of epilepsy have become an increasingly recognized etiology, especially in treatment refractory cases. Over the past two decades, there has been an explosion of new biomarkers that have led to the identification of new syndromes. As the underlying pathophysiology is immune driven, their management and treatment are directed at the modulation of this abnormal immune response against self-antigens. These antibody-specific syndromes show variation between adult and pediatric populations. We focus this review article on those identified autoimmune encephalopathies and epilepsies and clinical differences between them as well as age-related variations that have been reported.

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Severe dysautonomia as a main feature of anti-GAD encephalitis: Report of a paediatric case and literature review

Cited by 35 publications

References 10 publications

Anti-GAD-associated limbic encephalitis: an unusual clinical manifestation from northwest of Ireland

Anti-GAD-associated limbic encephalitis: an unusual clinical manifestation from northwest of Ireland

Intravenous methylprednisolone or immunoglobulin for anti-glutamic acid decarboxylase 65 antibody autoimmune encephalitis: which is better?

Antibody-Associated Autoimmune Encephalitis and Epilepsies in Children

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