KLHL11 antibody-associated autoimmune encephalomyelitis in a middle-aged female patient: a case report and literature review
KLHL11 antibody-associated autoimmune encephalomyelitis in a middle-aged female patient: a case report and literature review
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DOI：
10.1080/00207454.2023.2269306
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发表时间：
2023
期刊：
影响因子：
--
通讯作者：
Haining Li
中科院分区：
文献类型：
--
作者：
Wanqiu Yang;Juan Yang;Junmei Zhang;Na Wei;Tingting Xuan;Zhenhai Wang;Haining Li
Kelch-like protein 11antibody is a recently identified biomarker for paraneoplastic neurological syndromes associated with germ-cell tumors that was first described as an onconeural antibody causing autoimmune encephalitis associated with seminoma in 2019. Ataxia is the most prevalent presenting symptom, with other neurological symptoms including vertigo, double vision, hearing loss, tinnitus, and dysarthria. MRI scans reveal that the lesions are mostly located in the cerebellum and brainstem, particularly in the pontine region, and may also exhibit cerebellar atrophy. In this report, we present the case of a middle-aged female patient who presented with vertigo, cognitive decline, ataxia, and limb weakness. A cell-based assay (CBA) showed positive IgG Kelch-like protein 11 in both her serum and CSF, as well as positive oligoclonal bands in her CSF. She was diagnosed with KLHL11 antibody-associated autoimmune encephalomyelitis and received high-dose intravenous methylprednisolone pulse therapy. Clinical outcomes suggest that patients with KLHL11-Abs mostly have poor prognoses, excepting our case. We propose that early and appropriate treatments are critical for timely diagnosis and rapid improvement.
