Recurrent post-partum rhombencephalitis associated with anti-centromere antibody: a case report

Abstract

Background Rhombencephalitis (RE) is a serious condition of the brain with multiple etiologies. We report a unique case of recurrent, postpartum RE that is associated with positive anti-centromere antibody (ACA). A discussion of the case, current literature on autoimmune RE and related autoantibodies are reviewed. Case presentation A healthy 33-year-old Caucasian patient (gravida 2, para 2) had two episodes of progressive focal neurological deficits during postpartum periods. Signs and symptoms included right-sided dysmetria, adiadochokinesia, weakness, ataxia, and photophobia. MRI revealed rhombencephalitis involving the mesencephalon of the brainstem. Extensive and comprehensive investigations using blood and cerebrospinal fluid (CSF) were consistently positive only for ACA. The first episode was successfully treated with empiric antimicrobial agents and steroid. Given the negative infectious work up with the prior episode and the nearly identical clinical presentations, the second episode was treated with corticosteroid only. This led to complete resolution of her symptoms and reversal of the brain magnetic resonance imaging (MRI) lesions. Conclusion To the author's knowledge, this is the first report of a primary autoimmune RE during postpartum period that is associated with ACA. Immunologic causes should be considered early with any encephalitis. Given the risk of recurrence, relapse, and neurologic deterioration, regular monitoring is recommended, especially for female patients of child-bearing age. Consistent with the current literature on autoimmune RE, steroid seems to be an effective treatment for ACA-associated RE.

Suspected pregnancy‐associated meningoencephalitis of unknown origin in a dog

“…that concluded similar total drug exposure with this dosing pattern as compared to the previously used dosage (50 mg/m 2 SC every 12 hours) for 2 days 30 . In humans, methylprednisolone is used as the first‐line therapy 14,24 . In veterinary medicine, the use of IgG has been reported in treatment of other immune‐mediated disorders such as immune‐mediated haemolytic anaemia or immune‐mediated thrombocytopenia 31 ; however, they are not often employed in the treatment of MUO.…”

“…This autoimmune encephalitis has been reported in several patients during pregnancy or in the postpartum period, suggesting that pregnancy could affect self‐antigen tolerance and trigger anti‐NMDAR encephalitis 12,14,15 . Autoimmune rhombencephalitis has also been linked with pregnancy 24 . The underlying mechanism is not clear, but autoantibodies (anti‐centromere antibodies) with unknown effects on the neurons have been detected and seem to have an important role 24 .…”

“…12,14,15 Autoimmune rhombencephalitis has also been linked with pregnancy. 24 The underlying mechanism is not clear, but autoantibodies (anti-centromere antibodies) with unknown effects on the neurons have been detected and seem to have an important role. 24 The identification of immunoglobulin G (IgG) antibodies against neural antigens in patients with autoimmune encephalitis has been developed in recent years.…”

“…24 The underlying mechanism is not clear, but autoantibodies (anti-centromere antibodies) with unknown effects on the neurons have been detected and seem to have an important role. 24 The identification of immunoglobulin G (IgG) antibodies against neural antigens in patients with autoimmune encephalitis has been developed in recent years. 23,25 Since commercial antibody testing has become available, the prevalence of these diseases has increased.…”

“…30 In humans, methylprednisolone is used as the first-line therapy. 14,24 In veterinary medicine, the use of IgG has been reported in treatment of other immune-mediated disorders such as immune-mediated haemolytic anaemia or immune-mediated thrombocytopenia 31 ; however, they are not often employed in the treatment of MUO. Intravenous immunoglobulin (IV-Ig) treatment and plasmapheresis therapies have been reported in cases of severe encephalitis in human medicine, hereby guiding the decision in this case to pursue IV-Ig treatment when the patient deteriorated despite aggressive immunosuppressive treatment.…”