Objective: To describe clinical and paraclinical spectrum of Steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT) in a Tunisian cohort.

Background: SREAT is a heterogenous group of disorders commonly featured with encephalopathy and positive antithyroid antibodies as well as movement disorders. However, their clinical spectrum remains controversial.

Method: A descriptive retrospective study was conducted in the neurology department of National Institute Mongi Ben Hamida of Neurology of Tunis. Patients diagnosed with cognitive impairment and positive for anti-thyroid peroxidase (anti-TPO) and/or anti-thyroglobulin antibodies (anti-Tg) in serum were included.

Demographic, clinical and paraclinical data were assessed. Evolution after corticosteroid treatment was noted.

Results: Eight female patients were included. Age at onset range was [38-66] years old. No history of hypothyroidism was noted in three patients. Inaugural symptoms were hallucinations (n=2), rapidly progressive dementia (n=3), focal epilepsy (n=2), cerebellar ataxia (n=3), tremor (n=4), parkinsonism (n=4) and mild cognitive impairment with frontal neuropsychological profile (n=3). EEG was normal in two patients, showed background slowing (n=1) and temporal sharp waves (n=4). Brain MRI was normal in all patients. The range of serum anti-TPO and anti-Tg antibodies level were 158 to 610 IU/ml and 1000 to 1100 IU/ml respectively. These antibodies in cerebrospinal fluid (CSF) were negative in 2 tested patients. All patients received a 5-day pulse of intravenous Methylprednisolone followed by oral steroid therapy with significant clinical improvement.

Conclusion: Our study showed the diversity of clinical spectrum of SREAT. The atypical clinical and radiological presentation in some patients highlights the difficulty of diagnosing this disease. Steroid responsiveness and positivity of serum anti-TPO are the only constant features in our patients. Anti-TPO antibodies were negative in CSF. The relevance of anti-thyroid antibodies testing in CSF remains controversial. Some patients did not meet the proposed criteria by Graus et al 2016. The steroid responsiveness remains crucial. Parkinsonism was found in a majority of patients despite being reported only once in literature. This incites towards boardening SREAT clinical spectrum.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/broadening-the-spectrum-of-movement-disorders-in-steroid-responsive-encephalopathy-associated-with-autoimmune-thyroiditis/