Thyrotoxic autoimmune encephalopathy in a female patient: only partial response to typical immunosuppressant treatment and remission after thyroidectomy.
Hashimoto's encephalopathy (HE) is a rare immune-mediated encephalopathy developing in patients with high serum concentrations of anti-thyroid antibodies usually in an euthyroid or hypothyroid state. We report a 31-year-old female patient with thyrotoxic HE whose daughter has been followed up with the same diagnosis. Suboptimal response was observed with intravenous methylprednisolone (IVMP), intravenous immunoglobulin (IVIG) and plasmapheresis. Reduction of the anti-thyroid auto-antibody concentrations marked the patient's improvement in each episode. She relapsed under oral immunosuppressive therapy. After removing the thyroid tissue, full recovery has been achieved for the last 18 months. These data may contribute to clarification of the pathogenetic role of anti-thyroid antibodies in HE. Thyroidectomy can be considered as one of the treatment options especially in thyrotoxic HE patients with uncontrolled relapses. Our patient is the first reported HE case with a family history. Genetic background can underlie the etiopathogenesis of HE as is the case in other autoimmune disorders.
Yuceyar
Nur
N
Ege University Medical School of Hospital, Department of Neurology, Bornova, Izmir, Turkey. nuryuceyar@yahoo.com
Karadeniz
Muammer
M
Erdogan
Mehmet
M
Copur
Asli
A
Akgun
Aysegul
A
Kumral
Emre
E
Ozgen
Gokhan
G
D000328:Adult; D001323:Autoantibodies / Q000097:blood; D001324:Autoantigens / Q000276:immunology; D001927:Brain Diseases / Q000175:diagnosis / Q000235:genetics / Q000276:immunology / Q000628:therapy*; D003937:Diagnosis, Differential; D005260:Female; D050031:Hashimoto Disease / Q000175:diagnosis / Q000235:genetics / Q000276:immunology / Q000628:therapy*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007262:Infusions, Intravenous; D007453:Iodide Peroxidase / Q000276:immunology; D033862:Iron-Binding Proteins / Q000276:immunology; D008775:Methylprednisolone / Q000627:therapeutic use*; D012008:Recurrence; D013954:Thyroglobulin / Q000276:immunology; D013965:Thyroidectomy; D013971:Thyrotoxicosis / Q000175:diagnosis / Q000235:genetics / Q000276:immunology / Q000628:therapy*
D001323:Autoantibodies; D001324:Autoantigens; D007166:Immunosuppressive Agents; D033862:Iron-Binding Proteins; D013954:Thyroglobulin; C056658:TPO protein, human; D007453:Iodide Peroxidase; D008775:Methylprednisolone