Abstract
BackgroundImmune-mediated cerebellar ataxias (IMCAs) are common in paraneoplastic cerebellar degeneration (PCD) but rarely occur in patients with neuronal surface antibodies (NSAbs). Although cerebellar ataxias (CAs) associated with anti-NMDAR and anti-CASPR2 have been reported in a few cases, they have never been studied systematically. This study aimed to analyze the characteristics of anti-NSAbs-associated CAs.MethodsA retrospective investigation was conducted to identify patients using the keywords IMCAs and NSAbs. We collected the clinical data of 14 patients diagnosed with anti-NSAbs-associated CAs.ResultsThe median age was 33 years (16-66), and the male-to-female ratio was 4:3. Nine were positive for NMDAR-Ab, two for LGI1-Ab, two for CASPR2-Ab, and one for AMPA2R-Ab. CAs were initial symptoms in three patients and presented during the first two months of the disease course (10 days on average) among the rest of the patients. After the immunotherapy, two cases were free from symptoms, and eight cases recovered satisfactorily (10/14, 71.4%). Compared with other causes of IMCAs, anti-NSAbs were more frequently associated with additional extra-cerebellar symptoms (85.7%), mostly seizures (78.6%) and mental abnormalities (64.3%). In the CSF analysis, pleocytosis was detected in ten patients (71.4%) and oligoclonal bands (OB) were observed in nine patients (64.3%). Moreover, compared with PCD and anti-GAD65-Ab-associated CAs, anti-NSAbs-associated CAs showed a better response to immunotherapy.ConclusionIMCAs are rare and atypical in autoimmune encephalitis with neuronal surface antibodies. Compared with other forms of IMCAs, more symptoms of encephalopathy, a higher rate of pleocytosis and positive OB in CSF, and positive therapeutic effect were the key features of anti-NSAbs-associated CAs.
Highlights
Over the past few decades, with the discovery of anti-NMDAR encephalitis in 2007 [1], an increasing number of specific neuronal surface antibodies (NSAbs) have been discovered, including LGI1-Ab, CASPR2-Ab, AMPA1/2R-Ab, GABAR-A/ B-Ab, and so on [2–5]
Among 54 patients, patients were diagnosed with paraneoplastic cerebellar degeneration (PCD) (7 with Yo-Ab, 3 with Hu-Ab 2 with Tr-Ab, and 1 with SOX1-Ab), 7 patients with anti-GAD65-Ab-associated CA, 6 with autoimmune disease-associated CAs (4 with Hashimoto’s Encephalopathy and 2 with Systemic Lupus Erythematosus), with unknown etiology and the remaining 14 patients were positive for NSAbs, Abbreviations: NSAbs, Neuronal surface antibodies; PCD, Paraneoplastic cerebellar degeneration; onconeural antibodies (ONAs), Onconeural antibodies; magnetic resonance imaging (MRI), Magnetic resonance imaging; modified Rankin score (mRS), Modified Rankin score; WBC, White blood cell counts; oligoclonal bands (OB), Oligoclonal bands; IMCAs, Immune-mediated cerebellar ataxias; gluten ataxia (GA), Gluten ataxia; opsoclonus myoclonus syndrome (OMS), Opsoclonus myoclonus syndrome
185 patients were positive with one antibody (108 with NMDAR-Ab, 52 with LGI1-Ab, 12 with CASPR2-Ab, 4 with AMPA2R-Ab, and 9 with GABAR-B-Ab), and 6 patients were positive with two types of antibodies (4 with NMDAR-Ab and CASPR2-Ab, and 2 with CASPR2-Ab and LGI1-Ab) (Table 3)
Summary
Over the past few decades, with the discovery of anti-NMDAR encephalitis in 2007 [1], an increasing number of specific neuronal surface antibodies (NSAbs) have been discovered, including LGI1-Ab, CASPR2-Ab, AMPA1/2R-Ab, GABAR-A/ B-Ab, and so on [2–5]. Immune-mediated cerebellar ataxias (IMCAs) are one of the most common symptoms of paraneoplastic cerebellar degeneration (PCD) [9]. Cerebellar ataxia has been reported in a few patients with anti-NMDAR encephalitis [10, 11]. CAs associated with anti-NMDAR and anti-CASPR2 have been studied in a few cases, they have not been studied systematically. The present study focused on the clinical characteristics of patients with anti-NSAbs-associated CAs. Immune-mediated cerebellar ataxias (IMCAs) are common in paraneoplastic cerebellar degeneration (PCD) but rarely occur in patients with neuronal surface antibodies (NSAbs). Cerebellar ataxias (CAs) associated with antiNMDAR and anti-CASPR2 have been reported in a few cases, they have never been studied systematically. This study aimed to analyze the characteristics of anti-NSAbsassociated CAs
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