Simone Mattozzi1, Lidia Sabater1, Domingo Escudero1, Helena Ariño1, Thais Armangue1, Mateus Simabukuro1, Takahiro Iizuka1, Makoto Hara1, Albert Saiz1, Stefano Sotgiu1, Josep Dalmau1, Francesc Graus2. 1. From the Neuroimmunology Program (S.M., L.S., H.A., T.A., A.S., J.D., F.G.), Institut d'Investigació Biomèdica August Pi i Sunyer, Barcelona, Spain; Department of Medical, Surgical and Experimental Medicine (S.M., S.S.), University of Sassari, Italy; Service of Neurology (D.E., A.S.), Hospital Clinic, and Pediatric Neuroimmunology Unit (T.A.), Neurology Department, Sant Joan de Déu Children's Hospital, University of Barcelona, Spain; Neurology Division (M.S.), Hospital das Clínicas, São Paulo University, Brazil; Department of Neurology (T.I.), Kitasato University School of Medicine, Sagamihara; Division of Neurology (M.H.), Department of Medicine, Nihon University School of Medicine, Tokyo, Japan; Department of Neurology (J.D.), University of Pennsylvania, Philadelphia; and Institució Catalana de Recerca i Estudis Avançats (J.D.), Barcelona, Spain. 2. From the Neuroimmunology Program (S.M., L.S., H.A., T.A., A.S., J.D., F.G.), Institut d'Investigació Biomèdica August Pi i Sunyer, Barcelona, Spain; Department of Medical, Surgical and Experimental Medicine (S.M., S.S.), University of Sassari, Italy; Service of Neurology (D.E., A.S.), Hospital Clinic, and Pediatric Neuroimmunology Unit (T.A.), Neurology Department, Sant Joan de Déu Children's Hospital, University of Barcelona, Spain; Neurology Division (M.S.), Hospital das Clínicas, São Paulo University, Brazil; Department of Neurology (T.I.), Kitasato University School of Medicine, Sagamihara; Division of Neurology (M.H.), Department of Medicine, Nihon University School of Medicine, Tokyo, Japan; Department of Neurology (J.D.), University of Pennsylvania, Philadelphia; and Institució Catalana de Recerca i Estudis Avançats (J.D.), Barcelona, Spain. francesc.graus@idibaps.org.
Abstract
OBJECTIVE: To report the presenting syndromes and to determine whether pretreatment criteria of Hashimoto encephalopathy (HE) predict response to steroids. METHODS: We assessed symptoms and steroid responsiveness in 24 patients with pretreatment criteria of HE, including (1) subacute onset of cognitive impairment, psychiatric symptoms, or seizures; (2) euthyroid status or mild hypothyroidism; (3) serum thyroid peroxidase antibodies (TPOAb) >200 IU/mL; (4) absent neuronal antibodies in serum/CSF; and (5) no other etiologies. Additional studies included determination of TPOAb (>200 IU/mL) in 74 patients with criteria of possible autoimmune encephalitis (AE) without neuronal antibodies and 205 patients with different neuroimmunologic diseases, psychosis, or new-onset refractory status epilepticus (NORSE). Serum antibodies to the amino (ΝΗ2)-terminal of α-enolase (NH2-α-enolaseAb) were examined in the indicated 24 patients and 13 controls. RESULTS: The 24 patients (14 women) with suspected HE had a median age of 48 years (range 8-79 years). Four syndromes were identified: psychiatric (7, 29%), encephalopathy (7, 29%), NORSE-like (6, 25%), and limbic encephalitis (4, 17%). Only 6 of 19 (31.6%) patients completely responded to steroids. The frequency of TPOAb in the 74 patients with possible AE (6 of 74, 8.1%) was similar to that of the 205 controls (17 of 205, 8.2%; p = 0.84). NH2-α-enolaseAb were identified in 1 of 24 suspected HE cases and 1 of 13 controls. CONCLUSION: Current pretreatment criteria of HE do not predict steroid responsiveness. The detection of TPOAb across all control groups reveals their poor disease-specificity. NH2-α-enolaseAb did not help in the diagnosis of HE. These findings imply a redefinition of HE that requires a systematic exclusion of antibody-mediated encephalitis.
OBJECTIVE: To report the presenting syndromes and to determine whether pretreatment criteria of Hashimoto encephalopathy (HE) predict response to steroids. METHODS: We assessed symptoms and steroid responsiveness in 24 patients with pretreatment criteria of HE, including (1) subacute onset of cognitive impairment, psychiatric symptoms, or seizures; (2) euthyroid status or mild hypothyroidism; (3) serum thyroid peroxidase antibodies (TPOAb) >200 IU/mL; (4) absent neuronal antibodies in serum/CSF; and (5) no other etiologies. Additional studies included determination of TPOAb (>200 IU/mL) in 74 patients with criteria of possible autoimmune encephalitis (AE) without neuronal antibodies and 205 patients with different neuroimmunologic diseases, psychosis, or new-onset refractory status epilepticus (NORSE). Serum antibodies to the amino (ΝΗ2)-terminal of α-enolase (NH2-α-enolaseAb) were examined in the indicated 24 patients and 13 controls. RESULTS: The 24 patients (14 women) with suspected HE had a median age of 48 years (range 8-79 years). Four syndromes were identified: psychiatric (7, 29%), encephalopathy (7, 29%), NORSE-like (6, 25%), and limbic encephalitis (4, 17%). Only 6 of 19 (31.6%) patients completely responded to steroids. The frequency of TPOAb in the 74 patients with possible AE (6 of 74, 8.1%) was similar to that of the 205 controls (17 of 205, 8.2%; p = 0.84). NH2-α-enolaseAb were identified in 1 of 24 suspected HE cases and 1 of 13 controls. CONCLUSION: Current pretreatment criteria of HE do not predict steroid responsiveness. The detection of TPOAb across all control groups reveals their poor disease-specificity. NH2-α-enolaseAb did not help in the diagnosis of HE. These findings imply a redefinition of HE that requires a systematic exclusion of antibody-mediated encephalitis.
Authors: Dominique Endres; Frank Leypoldt; Karl Bechter; Alkomiet Hasan; Johann Steiner; Katharina Domschke; Klaus-Peter Wandinger; Peter Falkai; Volker Arolt; Oliver Stich; Sebastian Rauer; Harald Prüss; Ludger Tebartz van Elst Journal: Eur Arch Psychiatry Clin Neurosci Date: 2020-03-12 Impact factor: 5.270