Hashimoto encephalopathy in the 21st century.

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.

• Authors: Simone Mattozzi, Lidia Sabater, Domingo Escudero, Helena Ariño, Thais Armangue, Mateus Simabukuro, Takahiro Iizuka, Makoto Hara, Albert Saiz, Stefano Sotgiu, Josep Dalmau, Francesc Graus
• Journal: Neurology (Neurology) Vol. 94 Issue 2 Pg. e217-e224 (01 14 2020) ISSN: 1526-632X [Electronic] United States
• PMID: 31882532 (Publication Type: Journal Article)
• Copyright: © 2019 American Academy of Neurology.

Chemical References

• Steroids

Topics

• Adolescent
• Adult
• Aged
• Child
• Encephalitis (complications, drug therapy, physiopathology)
• Female
• Hashimoto Disease (complications, drug therapy, physiopathology)
• Humans
• Male
• Middle Aged
• Steroids (therapeutic use)
• Young Adult