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Acute amnesia and seizures in a young female
María Eugenia García García1, Sergio Muñiz Castrillo1, Irene Garcia Morales1
1Neurology Department, Hospital Clínico San Carlos, Madrid, Spain.
Abstract:
Limbic encephalitis is a condition characterised by an acute or sub-acute onset of memory disorder, associated with seizures and psychiatric manifestations. Investigations such as brain MRI usually reveal a high intensity signal in the medial temporal lobe and cerebrospinal fluid analysis shows mild pleocytosis and oligoclonal bands. It may occur in association with cancer, infection, or as an isolated clinical condition, often accompanying autoimmune disorders. Immune-mediated limbic encephalitis is now subclassified according to the presence and type of autoantibodies, which has significant consequences regarding the effectiveness of treatment and prognosis. Glutamic acid decarboxylase (GAD) is an enzyme that catalyses glutamic acid into gamma aminobutyric acid. Anti-GAD antibodies are associated with different neurological and non-neurological disorders, but only a few cases of limbic encephalitis associated with anti-GAD antibodies have been reported in the literature, most of them non-paraneoplastic. Here, we report the case of a young female patient with a medical history of psoriasis who developed an acute onset and chronic evolution of anterograde amnesia, associated with drug-resistant epilepsy. Brain MRI showed hyperintensity in the medial temporal lobes and the biochemical studies revealed intrathecal synthesis of anti-GAD antibodies. Screening tests for tumours were negative. Despite antiepileptic drugs, intravenous immunoglobulins and immunosuppressive treatment, the patient did not show clinical improvement and one year later, she continues to present refractory temporal epilepsy and cognitive deficits.
Insights
Limbic encephalitis, an autoimmune disorder, can be linked to anti-glutamic acid decarboxylase (GAD) antibodies. This case highlights a patient with refractory epilepsy and cognitive deficits despite treatment, emphasizing the challenges in managing anti-GAD antibody-associated limbic encephalitis.
Area of Science:
- Neuroimmunology
- Neurology
Background:
- Limbic encephalitis is an inflammatory brain condition often associated with autoimmune disorders.
- Immune-mediated limbic encephalitis is subclassified based on autoantibodies, impacting treatment and prognosis.
- Glutamic acid decarboxylase (GAD) antibodies are linked to various neurological disorders, with rare associations to limbic encephalitis.
Observation:
- A young female patient with psoriasis presented with acute anterograde amnesia and drug-resistant epilepsy.
- Brain MRI revealed medial temporal lobe hyperintensity, and cerebrospinal fluid analysis showed intrathecal synthesis of anti-GAD antibodies.
- Cancer screening tests were negative, suggesting a non-paraneoplastic autoimmune etiology.
Findings:
- The patient exhibited refractory temporal epilepsy and persistent cognitive deficits.
- Standard treatments including antiepileptic drugs, intravenous immunoglobulins, and immunosuppressants were ineffective.
- Intrathecal synthesis of anti-GAD antibodies was confirmed, indicating an autoimmune attack on the central nervous system.
Implications:
- This case underscores the diagnostic challenge and limited treatment efficacy for anti-GAD antibody-associated limbic encephalitis.
- Further research is needed to understand the pathogenesis and develop targeted therapies for this specific autoimmune encephalitis subtype.
- Early identification and novel therapeutic strategies are crucial for improving outcomes in patients with refractory autoimmune limbic encephalitis.
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