Dados do Trabalho

Título

Steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT): a severe case in a young woman.

RESUMO

Case Presentation: A 39-years-old woman, previously healthy, presented in the Emergency department with tonic-clonic seizures, followed by refractory status epilepticus requiring induction of barbituric coma to achieve seizures control using standard antiseizure drugs in high doses. Initial blood test and serologies were normal. Thyroid function tests showed TSH: 7,98, free T4: 0,81, Anti-thyroglobulin: 37,4 and Anti-thyroid-peroxidase: 97,6. Cerebrospinal fluid cell count, protein and glucose were normal. CSF VDRL and Criptococcal antigen were negative. Cultures for bacteria, fungi and BAAR were negative. Brain magnetic resonance imaging revealed in FLAIR/T2 sequences increased signal in hippocampus bilaterally, extending to hypothalamus, with swealling features, exhibiting cortical diffusion restriction. The patient received human immunoglobulin, remaining with consciousness impairment, orofacial and lower limbs mioclonus at the end of immunotherapy. Infusion therapy with methylprednisolone were indicated. After pulse therapy, the patient showed improvement of consciousness level and the mioclonus ceased. However, within ten days, the seizures recurred, associated with drowsiness. Prednisone 40mg daily associated with antiseizures drugs gradually improved the clinical picture. She was discharged within six days, lucid, with mild-cognitive impairment and normal thyroid function tests. She is currently in physical rehabilitation, with regular follow ups. Discution: Previouslly named Hashimoto's encephalopathy, Steroid Responsive Encefalopathy associated with autoimune thyreoiditis is a rare disease, associated with antithyroid antibodies. Women tend to be more affected than men and the mean age of onset is 51 years. It may comprise neuropsychiatric signs, such as progressive cognitive impairment, disturbance of consciousness, seizures, psychosis, behavioral changes, hallucinations, myoclonus, involuntary movements, language barrier and ataxia. This wide range of clinical findings leads to a underrecognition of the syndrome. The pathophysiology, as well as the relationship between Hashimoto thyroiditis remains unclear. SREAT has a pronounced response to corticotherapy, the first-line treatment. Final Considerations: The relevance of this case report lies on the fact that SREAT is a rare and severe, but treatable syndrome. In order to avoid and ease complications that could jeopardize the patient's recovery, the early diagnosis and treatment are decisive.

Palavras Chave

SREAT, Steroid Responsive Encefalopathy associated with autoimune thyreoiditis, Hashimoto's encephalopathy, status epilepticus, myoclonus,

Área

Neuroimunologia