Streaming through a case of SREAT

Abstract

Background Hashimoto’s encephalopathy, also known as steroid responsive encephalopathy associated with autoimmune thyroiditis (SREAT) is an autoimmune neuroendocrine disorder marked by impaired brain function. It is a diagnosis of exclusion with variable nature of presentation and no gold standard investigation of choice. Case presentation Here, we report a case of SREAT in a 26-year-old female who presented to our Emergency Department with altered sensorium and generalised tonic clonic seizures. After thorough clinical examination and initial resuscitation, a provisional diagnosis of neuroglycopenic injury or possible encephalitis was made. Broad-spectrum antibiotics were initiated. Routine investigations and cerebrospinal fluid (CSF) study were inconclusive except for neutrophilic leucocytosis. Magnetic resonance imaging (MRI) depicted hyper-intense signal changes around bilateral hippocampus and thalamus. Serum anti-thyroid peroxidase (anti-TPO) was strongly positive while other serum and CSF autoantibodies were within normal limits. A diagnosis of SREAT was made and she responded brilliantly to systemic corticosteroids. Incidentally, anti-SSA (anti-Ro) and anti-SSB (anti-La) were positive and a possible association between Sjogren’s syndrome and SREAT was insinuated. Conclusion There is a long list of differentials for SREAT and a proper diagnostic criteria must be followed to reach at a conclusion. It can be easily missed and remain underreported due to its overlapping nature and ambiguous presentation. Hence, clinicians must have high index of suspicion for the disease and optimal therapy should be initiated early to improve the long term mortality.

Cerebellar Ataxia in the Setting of Hashimoto’s Thyroiditis: A Case Report Update and Review

“…It should be suspected in any case of autoimmune thyroiditis with obvious neuro/psychiatric symptoms, regardless of the thyroid function status, since it is oftentimes normal at presentation [4,6]. Antithyroid antibodies are a hallmark of this disease; nonetheless, they are non-specific since they can be present in up to 13% of healthy individuals, 27% in healthy white women in their 60s, and patients with other autoimmune encephalitis [2,8,9,11]. Anti-TPO levels do not correlate with the severity of the disease, but some authors have considered them to be a good marker for treatment response [2,8].…”

“…Antithyroid antibodies are a hallmark of this disease; nonetheless, they are non-specific since they can be present in up to 13% of healthy individuals, 27% in healthy white women in their 60s, and patients with other autoimmune encephalitis [2,8,9,11]. Anti-TPO levels do not correlate with the severity of the disease, but some authors have considered them to be a good marker for treatment response [2,8]. Antibodies against a-enolase (amino NH2/terminal domain) have been identified in up to 68% of cases with HE; nonetheless, these have also been found in patients with other autoimmune disorders and in a few healthy people [6,8].…”

“…At this time, HE continues to be a diagnosis of exclusion as no specific biomarkers for the disease have been identified [6,7]. The initial treatment is corticosteroids, but in corticosteroid-resistant HE, or when there are relapses after treatment, other pharmacological approaches should be considered [6,8]. In resistant cases, combinations of corticosteroids with azathioprine, methotrexate, rituximab, or cyclophosphamide have been recommended [1,8,9].…”

“…The initial treatment is corticosteroids, but in corticosteroid-resistant HE, or when there are relapses after treatment, other pharmacological approaches should be considered [6,8]. In resistant cases, combinations of corticosteroids with azathioprine, methotrexate, rituximab, or cyclophosphamide have been recommended [1,8,9]. Plasmapheresis and intravenous immunoglobulin (IVIG) have also proved to be effective in selected patients [1,8].…”

“…In resistant cases, combinations of corticosteroids with azathioprine, methotrexate, rituximab, or cyclophosphamide have been recommended [1,8,9]. Plasmapheresis and intravenous immunoglobulin (IVIG) have also proved to be effective in selected patients [1,8].…”