Severe dysautonomia as a main feature of anti-GAD encephalitis: Report of a paediatric case and literature review. - Archive ouverte HAL Accéder directement au contenu
Article Dans Une Revue European Journal of Paediatric Neurology Année : 2018

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

Résumé

Introduction Anti-glutamic acid decarboxylase (anti-GAD65) antibodies are a rare cause of autoimmune encephalitis. This entity is mainly recognized in adults and very few cases were reported in children. We report on a paediatric case of anti-GAD encephalitis with severe presentation and uncontrollable dysautonomia. CASE STUDY A 9-year-old girl was referred to our department for refractory seizures and behavioral disturbances. Brain magnetic resonance imaging (MRI) was normal. Repeat screening for antineuronal antibodies showed negative results for anti-NMDA receptor antibodies but positive results for anti-GAD65 with a low positivity of anti-Ma2 antibodies. Although a transient improvement was noticed after immunomodulatory treatment, the patient developed severe intractable autonomic imbalance including dysrythmia, alternating bradycardia/tachycardia, hypotension/hypertension, hypothermia/hyperthermia and hyperhidrosis. She deceased six months after onset. CONCLUSION Our report intends to raise awareness of autoimmune encephalitis with anti-GAD65 antibodies which may involve extralimbic brain regions and manifest with fatal dysautonomia. We highlight the need for prompt diagnosis and aggressive management for this underdiagnosed entity in children. Case study A 9-year-old girl was referred to our department for refractory seizures and behavioral disturbances. Brain magnetic resonance imaging (MRI) was normal. Repeat screening for antineuronal antibodies showed negative results for anti-NMDA receptor antibodies but positive results for anti-GAD65 with a low positivity of anti-Ma2 antibodies. Although a transient improvement was noticed after immunomodulatory treatment, the patient developed severe intractable autonomic imbalance including dysrythmia, alternating bradycardia/tachycardia, hypotension/hypertension, hypothermia/hyperthermia and hyperhidrosis. She deceased six months after onset. Conclusion Our report intends to raise awareness of autoimmune encephalitis with anti-GAD65 antibodies which may involve extralimbic brain regions and manifest with fatal dysautonomia. We highlight the need for prompt diagnosis and aggressive management for this underdiagnosed entity in children.
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Dates et versions

hal-01876256 , version 1 (18-09-2018)

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Citer

Nedia Ben Achour, Thouraya Ben Younes, Ibtihel Rebai, Melika Ben Ahmed, Ichraf Kraoua, et al.. Severe dysautonomia as a main feature of anti-GAD encephalitis: Report of a paediatric case and literature review.. European Journal of Paediatric Neurology, 2018, 22 (3), pp.548--551. ⟨10.1016/j.ejpn.2018.01.004⟩. ⟨hal-01876256⟩

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