Details

Autor(en) / Beteiligte

• Karvouniari, P.
• Hartinger, S.
• Geis, C.
• Witte, O.W.

Titel

P 107 Antibody-negative autoimmune encephalitis?

Ist Teil von

• Clinical neurophysiology, 2017-10, Vol.128 (10), p.e382-e383

Ort / Verlag

Elsevier B.V

Erscheinungsjahr

2017

Quelle

Alma/SFX Local Collection

Beschreibungen/Notizen

• We report about a 66-year-old woman which has been hospitalized with suspected antibody-negative autoimmune encephalitis. The patient experienced relapsing episodes with disorientation, amnestic aphasia, short-term memory dysfunction, hallucinations, apraxia, ataxia, seizures and severe delirium during the years 2012–2013. In medical history the patient has had a breast cancer right sided (first diagnosis 2010) with breast-conserving therapy, radiation and hormone therapy. MRI and cerebrospinal fluid (including antibodies for autoimmunencephalitis) did not show relevant abnormalities. The symptoms completely remitted after cortison therapy. After several episodes, azathioprin therapy was started resulting in clinical stability without relapses. In July 2016 azathioprin was discontinued. In October 2016 the patient developed similar symptoms and hospitalized. As in previous brain scans, MRI was normal without signs of encephalitis. Electroencephalogram showed a severe slowing most pronounced in frontal regions without epileptiform patterns. In cerebrospinal fluid cell count was normal, total protein was elevated (∼1300mg/l). There was no evidence of bacterial or viral infection. F-18-FDG-PET showed a reduced tracer uptake frontal, parietal and temporal on both sides. A whole body PET-CT, skeletal scintigraphy and mammography did not reveal any malignant tumors. During the following days the patient experienced a rapid worsening in orientation, developed anarthria and was unable to eat independently. Routine blood investigations were without any abnormalities. Autoantibodies to surface antigens and paraneoplastic antibodies as well as vasculitis associated parameters were negative. However, we found highly elevated antibodies against thyreoperoxidase (2726U/ml, normal<60). We started steroid pulse therapy and the patient improved rapidly. She regained orientation and improved in ability to communicate. She could move and eat independently. Complete remission was achieved within one week. The patient described complete amnesia regarding this episode. We restarted immunosuppressive therapy with azathioprin. At discharge the patient is able to carry out usual activities. In conclusion we diagnosed a SREAT (steroid responsive encephalopathy in association with autoimmunthyreoditis) with relapsing episodes of amnestic aphasia, disorientation, memory-dysfunctions, delirium, apraxia, and ataxia with highly elevated TPO-antibodies and complete remission after cortison therapy. MRI findings: Unspecific T2/FLAIR Hyperintensities bifrontal. Electroencephalogram: severe slowing most pronounced in frontal regions without epileptiform patterns.