Dados do Trabalho

Título

LIMBIC ENCEPHALITIS ASSOCIATED WITH ANTI-GAD65: BENEFITS OF EARLY IMMUNOTHERAPY IN CONTROLLING EPILEPTIC SEIZURES.

Apresentação do caso único

A previously healthy 7-year-old girl was referred to our service with an acute history of behavioral changes
and focal motor epileptic seizures, preceded by epigastric aura and followed by generalization. On
examination, there was confusion and horizontal nystagmus with lateral gaze, without motor deficits or
movement disorders. Subjected to brain tomography and cerebrospinal fluid puncture and no changes
were identified. He developed recurrent epileptic seizures, requiring three anti-seizure drugs in high doses
to control. Brain MRI showed an increase in bilateral hippocampal signal more evident on the right on T2/
FLAIR and an electroencephalogram showed bursts of slow waves projected in the right anterior temporal
region. In view of the findings, methylprednisolone 1g was prescribed for 5 days followed by human
immunoglobulin 2g/kg for 3 days and a panel for autoimmune encephalitis was performed, and anti-GAD65
was subsequently identified in high titers in the CSF and serum. He presented clinical improvement and
was discharged for outpatient follow-up in conjunction with endocrinology due to the discovery of diabetes
mellitus. At the moment, she is under seizure control while on monotherapy with oxcarbazepine and
awaiting release of rituximab, in addition to neuropsychological evaluation due to amnestic cognitive
impairment.

Discussão

Glutamic acid decarboxylase (GAD) antibody-associated limbic encephalitis generally results from anti-
GAD65-mediated autoimmunity. It determines temporal lobe epilepsy, affective disorders and cognitive
deficit with marked memory impairment. It frequently coexists with systemic autoimmune diseases and is
generally not paraneoplastic in nature. Brain MRI may show bilateral mesial temporal hypersignal with
progression to hippocampal sclerosis, cerebrospinal fluid is normal in most cases and the
electroencephalogram shows temporal epileptiform activity, while the anti-GAD65 dosage is positive.
Immune therapy should be early and preferably in the immunological activation phase, when there is still no
permanent brain damage, aiming to alleviate cognitive sequelae and progression to difficult-to-control
epilepsy.

Comentários finais

The case report, showing seizure control with monotherapy, reinforces the importance of early diagnosis, as
immunotherapy in the early stages has the potential to prevent the development of permanent damage to
brain tissue, avoiding the development of difficult-to-control epilepsy.

Referências

Muñoz-Lopetegi A, de Bruijn MAAM, Boukhrissi S, et al. Neurologic syndromes related to anti-GAD65: clinical and serologic response to treatment. Neurol Neuroimmunol Neuroinflamm. 2020;7(3):e696. doi.org/10.1212/NXI.0000000000000696
Daif A et al. Antiglutamic acid decarboxylase 65 (GAD65) antibody-associated epilepsy. Epilepsy & Behavior 80 (2018) 331–336. doi.org/10.1016/j.yebeh.2018.01.021
Li X et al. Immune-mediated epilepsy with GAD65 antibodies. Journal of Neuroimmunology 341 (2020) 577189. doi.org/10.1016/j.jneuroim.2020.577189

Palavras Chave

Anti-GAD65; Limbic encephalitis; Immune-mediated epilepsy