Anti-Ma2-encephalitis in a 2 year-old child: A newly diagnosed case and literature review

Mrabet, S.; Achour, N. Ben; Kraoua, Ichraf; Benrhouma, Hanène; Klaa, Hédia; Rouissi, Aida; Ahmed, Mélika Ben; Turki, Ilhem

doi:10.1016/j.ejpn.2015.06.005

Cited by 19 publications

(11 citation statements)

References 25 publications

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“…[ 2 ] According to a recent review and analysis, only 68 cases of anti-Ma2-associated neurological syndromes have been reported to date. [ 6 ] Most cases occurred in adult men with testicular germ cell tumor or non-small-cell lung cancer, whereas some were associated with tumors such as lymphoma, prostatic adenocarcinoma, gastric adenocarcinoma, and recurrent cervical cancer. [ 7 , 8 ] Ma2 is an intracellular onconeural protein; anti-Ma2 antibodies recognize 2 antigens—PNMA1 and PNMA2 [ 9 ] —which are mainly expressed in neurons in the brain, spinal cord, dorsal root ganglia, and tumors.…”

Section: Discussionmentioning

confidence: 99%

Anti-Ma2–associated limbic encephalitis with coexisting chronic inflammatory demyelinating polyneuropathy in a patient with non-Hodgkin lymphoma

Ju¹,

Wang³

et al. 2017

Medicine

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Rationale:We report the rare case of a 74-year-old man with anti-Ma2–associated paraneoplastic neurologic syndrome (PNS), and review and analyze the clinical manifestations, diagnosis, and treatment of the disease.Patient concerns:The patient presented with a 5-month history of muscle weakness, progressive body aches, and weakness and numbness in both lower extremities. Before his hospitalization, he had experienced cognitive function decline; ptosis, inward gaze, and vertical gaze palsy in the right eye; and occasional visual hallucinations. Brain and spinal cord magnetic resonance imaging (MRI) yielded normal results. Anti-Ma2 antibodies were detected in both serum and cerebrospinal fluid. A 4-hour electroencephalogram showed irregular sharp slow waves and δ waves in the temporal region. Electromyography showed peripheral nerve demyelination. Positron-emission tomography/computed tomography (PET-CT) examination revealed hypermetabolism in the lymph nodes of the whole body. Biopsy of the lymph nodes showed non-Hodgkin lymphoma.Diagnosis:A clinical diagnosis of lymphoma and PNS was made.Interventions:The patient was treated with intravenous dexamethasone (15 mg/day) for 3 days.Lessons:We have presented a rare case of a PNS involving both the central and peripheral nervous systems. The clinical features of this case indicated anti-Ma2–associated encephalitis and chronic inflammatory demyelinating polyneuropathy. PET-CT played a critical role in enabling early diagnosis and prompt treatment in this case.

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Section: Discussionmentioning

confidence: 99%

Anti-Ma2–associated limbic encephalitis with coexisting chronic inflammatory demyelinating polyneuropathy in a patient with non-Hodgkin lymphoma

Ju¹,

Wang³

et al. 2017

Medicine

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“…Pediatric cases presented with subacute onset of focal seizures, behavioral changes, speech disturbance, and dystonia. [ 16 17 ] MRI abnormalities are frequent in the limbic system, diencephalon, and brainstem. Inflammatory changes were typically present in the CSF.…”

Section: P Ediatric a Utoimmune mentioning

confidence: 99%

Pediatric autoimmune encephalitis

et al. 2017

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Autoimmune (antibody mediated) encephalitis (AE) is emerging as a more common cause of pediatric encephalopathy than previously thought. The autoimmune process may be triggered by an infection, vaccine, or occult neoplasm. In the latter case, onconeural autoantibodies are directed against intracellular neuronal antigens, but a recent heterogeneous group of encephalitic syndromes has been found not to have underlying tumor but is associated with autoantibodies to the neuronal surface or synaptic antigens. Neuropsychiatric symptoms are very common in autoimmune encephalopathy; as a result, affected children may be initially present to psychiatrists. Neurological features are movement disorders, seizures, altered conscious level, and cognitive regression. Hypoventilation and autonomic features may be an aspect. Inflammatory findings in the cerebrospinal fluid may be present but are relatively nonspecific. Magnetic resonance imaging (MRI) may also demonstrate abnormalities that provide clues for diagnosis, particularly on fluid-attenuated inversion recovery or T2-weighted images. AE is well responsive to immune therapy, with prompt diagnosis and treatment strongly beneficial. Patients with paraneoplastic encephalitis are more refractory to treatment compared to those in whom no malignancy is identified. Herein, the authors present an update of literature data on the clinical presentation, laboratory and imaging findings, therapy, and outcomes for the most common autoimmune encephalitides.

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“…In children, oncologic association is more often the exception than the rule. 48,[69][70][71] As opposed to encephalitides associated with neuronal surface antigens exhibiting an antibody-/complementmediated immune response, encephalitis associated with intracellular antigens (namely anti-Hu and anti-Ma) appears to be associated with T-cellemediated neuronal cytotoxicity. 72 Anti-Hu encephalitis typically demonstrates a poor response to immunotherapy.…”

Section: Anti-hu Encephalitismentioning

confidence: 99%

“…73 Only a few cases of anti-Ma2 encephalitis have been reported in children. 48,71 Reported pediatric cases presented with subacute onset of focal seizures, behavioral changes, speech disturbance, and dystonia. Imaging findings are relatively nonspecific and varied.…”

Section: Anti-ma2 Encephalitismentioning

confidence: 99%

“…Clinical outcome is typically poor, with medically refractory seizures. 48,71 Anti-GADeassociated encephalitis Glutamic acid decarboxylase (GAD) is an intracellular enzyme responsible for the synthesis of the inhibitory neurotransmitter GABA. It is selectively expressed in GABAergic neurons and in pancreatic b-cells and is considered a major autoantigen in type 1 diabetes mellitus.…”

Section: Anti-ma2 Encephalitismentioning

confidence: 99%

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