Recurrent panic attack and bilateral hippocampus lesions as main manifestation in an autoimmune encephalitis associated with primary biliary cirrhosis
Autoimmune encephalitis (AE) is a type of brain inflammation where the immune system mistakenly attacks healthy neural cells, leading to symptoms like memory loss, seizures, or emotional changes. While AE often co-occurs with other autoimmune conditions—such as lupus or myasthenia gravis—its connection to primary biliary cirrhosis (PBC), a chronic liver disease that damages bile ducts, is rarely documented.
Researchers from the Department of Neurology at Nanfang Hospital (Southern Medical University, Guangzhou) and Bao’an District People’s Hospital (Shenzhen) recently described a unique case of a young woman with both AE and PBC in the Chinese Medical Journal, shedding light on how these two conditions can present together.
The Case: A 27-Year-Old Woman with Panic Attacks and Memory Loss
In February 2019, a 27-year-old woman sought care at Nanfang Hospital after a month of worsening short-term memory and frequent, sudden panic attacks. Her symptoms included transient waves of intense fear, paired with nausea, dizziness, chest tightness, and trembling limbs. Local doctors had diagnosed her with moderate depression and significant anxiety using the Hamilton Depression (HAMD) and Anxiety (HAMA) Scales, but standard anti-anxiety medications—paroxetine (20 mg daily), estazolam (0.5 mg three times daily), and olanzapine (2.5 mg daily)—failed to improve her condition.
Her medical history revealed a prior diagnosis of autoimmune hepatopathy during pregnancy in May 2018, treated with ursodeoxycholic acid (250 mg daily for one month). She delivered a healthy baby via natural labor in September 2018, with no family history of autoimmune or neurological conditions. A neurological exam found only impaired short-term memory; other cognitive functions were normal. The patient provided written consent for her clinical information and images to be published, with efforts to protect her identity.
Diagnostic Tests: Liver Enzymes, Antibodies, and Brain Imaging
Blood tests showed elevated liver enzymes: alanine aminotransferase (ALT, 75 U/L, normal 7–10 U/L), aspartate aminotransferase (AST, 54 U/L, normal 13–35 U/L), alkaline phosphatase (ALP, 295 U/L, normal 35–100 U/L), and gamma glutamyltransferase (GGT, 677 U/L, normal 7–45 U/L). These results, combined with a strongly positive anti-mitochondrial antibody M2 (AMAM2)—a hallmark biomarker for PBC—and a positive anti-nuclear antibody (ANA), confirmed a PBC diagnosis. Other autoimmune markers, including anti-neutrophil cytoplasmic antibodies (ANCA), were normal. Metabolic tests (amino acid and acyl carnitine spectra in blood and urine) and cerebrospinal fluid (CSF) analysis (for infection or known AE-related antibodies) were unremarkable.
Electroencephalograms (EEGs)—both short-term (three times) and long-term—showed no epileptic activity. However, a brain MRI revealed symmetric swelling and high-intensity signals in the bilateral hippocampus (the brain region critical for memory formation) on T2-weighted and FLAIR imaging—classic findings for AE.
Treatment and Recovery
The team first managed her PBC with ursodeoxycholic acid (500 mg twice daily), which improved her liver function. But her panic attacks and memory loss persisted until they treated her for AE: five days of intravenous immunoglobulin (IVIG, 0.4 g/kg daily) followed by oral methylprednisolone (44 mg daily, tapered by 8 mg every two weeks).
By her 3-month follow-up, her panic attacks and memory loss had fully resolved. She declined repeat testing for AMAM2 or a follow-up MRI for personal reasons.
What This Case Teaches Us
This case is notable because the woman had no obvious liver symptoms—her primary issues were neurological. While AMAM2 is well-established as a PBC marker, its role in AE remains unclear. Previous studies have linked AMAM2 to liver, muscle, or heart damage, but its connection to brain inflammation is largely unstudied. The team couldn’t test AMAM2 in her CSF due to laboratory limitations, so more research is needed to determine if this antibody directly affects the central nervous system.
Other researchers have reported similar cases: a 2006 study described a PBC patient with limbic encephalitis (a type of AE affecting the hippocampus) where immunosuppression improved symptoms and imaging. While there’s no definitive proof of a “PBC/AE overlap syndrome,” the fact that both conditions are autoimmune suggests a possible shared mechanism.
Conclusion
This case highlights a rare presentation of AE associated with PBC: recurrent panic attacks, short-term memory loss, and bilateral hippocampus lesions, alongside a positive AMAM2 antibody. It raises important questions: Could AMAM2 be a new AE-related antibody? Is there a true overlap between PBC and AE? Or are other, untested factors at play?
While more research is needed to answer these questions, the case underscores the importance of considering autoimmune overlaps when patients present with unexpected neurological symptoms—especially if they have a history of autoimmune liver disease.
This study was supported by a grant from the President Fund of Nanfang Hospital (No. 2016B017). The authors thank the Laboratory Department and Neurology Laboratory of Nanfang Hospital for their support.
This case was originally published in the Chinese Medical Journal in 2020 by Shu-Min Chen, Yong-Fang Zhang, Guang-Hui Liu, Ling Li, Hai-Jun Zhang, and Liang Zhou. The full study is available at doi.org/10.1097/CM9.0000000000000611
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