[Hashimoto's encephalopathy associated with scleromyxedema: coincidental or the overlapping of two syndromes with a possible autoimmune origin?].
BACKGROUND
The encephalopathies that may accompany Hashimoto's thyroiditis and scleromyxedema, both of which are diseases that probably have an autoimmune origin, are clinically similar. The presence of both Hashimoto's thyroiditis and scleromyxedema in a patient with encephalopathy led us to compare the clinical symptoms and the different possible mechanisms accounting for the pathology.


METHODS
We describe the case of a 54-year-old male who was admitted to hospital because of several occurrences of transient neurological focus that finally developed into a clinical picture of aphasia, acalculia and right homonymous hemianopsia. The patient had previously been diagnosed as suffering from scleromyxedema, which responded poorly to treatment, as well as IgG kappa paraproteinemia and, six months before admission to hospital, Hashimoto's thyroiditis. No abnormalities were found in the complementary tests, except for slightly high protein levels in the cerebrospinal fluid, a diffuse slowing of brain waves in the electroencephalogram and alterations due to thyroiditis and paraproteinemia. Neurological symptoms improved after a few days of corticoid therapy.


CONCLUSIONS
The presentation of encephalopathy as a stroke and its excellent response to treatment with corticoids pointed to a case of Hashimoto's encephalopathy, although we cannot completely rule out the possibility of it being a dermato-neuro syndrome, which is associated to scleromyxedema. In this paper we review the different pathological mechanisms proposed for the two clinical entities, as well as the clinical features that are similar in both syndromes and which could be an indication of some common mechanism shared by them.
Caicoya
A G
AG
Servicio de Neurología, Hospital Clínico San Carlos, Madrid, Spain. caicoya@pluri.ucm.es
Barco-Nebreda
L
L
González-Gutiérrez
J L
JL
Egido-Herrero
J A
JA
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D002545:Brain Ischemia / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology* / Q000503:physiopathology; D003240:Connective Tissue Diseases / Q000150:complications* / Q000276:immunology; D006801:Humans; D008297:Male; D008875:Middle Aged; D009422:Nervous System Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology / Q000503:physiopathology; D012871:Skin Diseases / Q000150:complications* / Q000276:immunology; D013967:Thyroiditis, Autoimmune / Q000150:complications* / Q000276:immunology; D016896:Treatment Outcome
D000305:Adrenal Cortex Hormones