Dados do Trabalho

Título

Case report of Autoimmune Anti-NMDA Encephalitis in adolescents in the state of Rio Grande do Sul

RESUMO

Case presentation:
Patient GCS, female, 18 years old, in July 2020 started to present behavioral changes, hypersexualization, depression, anxiety, crying crises, emotional lability, refractory to psychiatric treatment. In April 2021, the exacerbation of the symptoms evolved, followed by language alteration, agitation, aggressiveness, visual hallucinations, and seizures, evolving to status epilepticus. On further investigation she presented small area of hypersignal in T2W and FLAIR in bilateral mesial temporal region topography, no pathological enhancement on skull MRI and pleocytosis with 69 cells, exclusive intrathecal oligoclonal bands and presence of anti-NMD-receptor antibodies in cerebrospinal fluid. She was initially approached with acyclovir, pulse therapy in another service, with no response. We followed treatment with immunoglobulin, again without response, with ongoing epilepsy and orofacial dyskinesias. Therapy with Rituximab was subsequently followed, resulting in severe pharmacoderma in the condition of Toxic Epidermal Necrolysis (TEN), combined to secondary complications such as multidrug-resistant infections, that greatly increased morbidity. At the first opportunity of clinical stabilization, cyclophosphamide therapy was proceeded, obtaining good response in neuropsychiatric manifestations. The patient maintained the rehabilitation with a slow and gradual recovery, and today she has returned to her family, walks without help, and eats orally.
Discussion and Comments:
In this case, we discuss the importance of autoimmune encephalitis among the differential diagnoses of neuropsychiatric conditions, its diagnosis delay, mainly due to the onset of psychiatric manifestations and consequently delay in treatment. We also present the reality of a committed neurology service in the northern interior of the state of Rio Grande do Sul. The N-methyl-D-aspartate (NMDA) anti-receptor encephalitis first described in 2007 is the most common, and children and young adults should always be considered. The research of these receptors in the cerebrospinal fluid confirms the diagnosis, since brain images may be normal. The therapeutic approach consisting of immunosuppression should be initiated as early as possible to improve the outcome. The possibility of adverse reactions to potent and consolidated medications should always be taken into consideration.

Palavras Chave

Behavioral changes, seizures, anti-NMD-receptor antibodies, autoimmune, encephalitis.

Área

Neuroimunologia