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Medicine (Baltimore). 2019 May;98(20):e15706. doi: 10.1097/MD.0000000000015706.

Autoimmune encephalitis with anti-IgLON5 and anti-GABAB-receptor antibodies: A case report.

Abstract

RATIONALE:

Anti-IgLON5 disease is a complex neurological illness which is characterized by progressive sleep and movement disorders and defined by specific autoantibodies to IgLON5. We here describe the first case of a patient with coexisting anti-IgLON5 as well as anti-γ-aminobutyric acid B (GABAB)-receptor antibodies and predominant clinical features of anti-IgLON5 disease.

PATIENT CONCERNS:

The patient initially presented with subacute symptoms of severe sleep disorder, gait stability, dysarthria, cognitive impairment, depressive episode and hallucinations.

DIAGNOSES:

The patient was diagnosed with autoimmune encephalitis, based on clinical features and positive anti-IgLON5 antibodies in serum as well as in cerebrospinal fluid and anti-GABAB-receptor antibodies in serum only.

INTERVENTIONS:

Initially, the patient was treated with high dosages of methylprednisolone and subsequently with plasmapheresis. Due to the lack of clinical improvement immunosuppressive treatment with intravenous cyclophosphamide was initiated.

OUTCOMES:

Following the first year of cyclophosphamide treatment, neurological examination revealed an improvement in gait instability, visual and acoustic hallucinations and sleep disorder.

LESSONS:

The case report demonstrates that anti-IgLON5 and anti-GABAB-receptor antibodies can coexist in the same patient whereas clinical leading symptoms are determined by those antibodies that were tested positive in cerebrospinal fluid.

PMID:
31096519
PMCID:
PMC6531245
DOI:
10.1097/MD.0000000000015706
[Indexed for MEDLINE]
Free PMC Article

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