Clinical manifestations, diagnostic criteria and therapy of Hashimoto's encephalopathy: report of two cases.
Hashimoto's encephalopathy (HE) is a rare, still not well understood, autoimmune disease with neurological and psychiatric manifestations. and elevated titers of antithyroid antibodies in serum and cerebrospinal fluid (CSF) as a hallmark of the disease. Patients are mostly women. Current diagnostic criteria include corticosteroide responsiveness, but it is the case in only 50% of patients with HE. In steroid non-responders other immunomodulatory therapies or plasmapheresis could be applied. Disease course can be acute, subacute, chronic or relapsing-remitting. Two distinct forms emerged from the reported cases: a vasculitic type characterized by multiple relapsing-remitting stroke-like episodes and mild cognitive impairment and a diffuse progressive type characterized by dementia and psychiatric symptoms. Both forms may be accompanied by depressed level of consciousness (stupor or coma), tremor, seizures, or myoclonus. We present two patients with two distinct forms of HE who had different clinical manifestations and response to therapy.
Mijajlovic
Milija
M
Institute of Neurology, Clinical Center of Serbia, Dr Subotica 6, 11000 Belgrade, Serbia. mmijajlovic@mail.neurologija.bg.ac.yu
Mirkovic
Mihailo
M
Dackovic
Jelena
J
Zidverc-Trajkovic
Jasna
J
Sternic
Nadezda
N
D000368:Aged; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001379:Azathioprine / Q000627:therapeutic use; D003937:Diagnosis, Differential; D004569:Electroencephalography; D004660:Encephalitis / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology; D005260:Female; D050031:Hashimoto Disease / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D006801:Humans; D007166:Immunosuppressive Agents; D008279:Magnetic Resonance Imaging; D008569:Memory Disorders / Q000209:etiology; D008775:Methylprednisolone / Q000627:therapeutic use; D008875:Middle Aged; D018908:Muscle Weakness / Q000209:etiology; D007703:Peptidyl-Dipeptidase A / Q000097:blood / Q000134:cerebrospinal fluid; D020521:Stroke / Q000175:diagnosis; D013960:Thyroid Function Tests
D000893:Anti-Inflammatory Agents; D007166:Immunosuppressive Agents; D007703:Peptidyl-Dipeptidase A; D001379:Azathioprine; D008775:Methylprednisolone