The clinical and electrophysiological characteristics of 8 cases of sensory neuronopathies
10.3760/cma.j.cn113694-20250123-00053
- VernacularTitle:感觉神经元病8例临床及电生理特征
- Author:
Ruizhi ZHENG
1
;
Li TIAN
1
;
Zhecheng ZHANG
1
;
Lei CUI
1
;
Lei WANG
1
;
Yanping REN
1
;
Xiaohui SUN
1
;
Ju ZHU
1
Author Information
1. 天津市第三中心医院神经内科 天津市重症疾病体外生命支持重点实验室 天津市人工细胞工程技术研究中心 天津市肝胆疾病研究所，天津 300170
- Publication Type:Journal Article
- Keywords:
Ataxia;
Electrophysiology;
Paraneoplastic syndromes, nervous system;
Sensory neuronopathies
- From:
Chinese Journal of Neurology
2025;58(4):380-386
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To analyze the clinical and electrophysiological characteristics of patients with sensory neuronopathies (SNN), and to evaluate the significance of electrophysiological markers in the diagnosis and assessment of disease progression.Methods:A retrospective analysis was performed to evaluate the clinical manifestations, electrophysiological characteristics, and spinal cord magnetic resonance imaging (MRI) features of 8 cases diagnosed with SNN at the Third Central Hospital of Tianjin between 2015 and 2023. The neurophysiological examination mainly included limb nerve conduction study (NCS), same core needle electrode electromyography, somatosensory evoked potential (SEP), skin sympathetic reflex (SSR), and contact heat evoked potential (CHEP).Results:Among the 8 cases with SNN, 7 cases exhibited asymmetrical onset and a non-length-dependent pattern. All the 8 cases presented with severe deep sensory ataxia, accompanied by superficial sensory abnormalities and tendon areflexia. Paraneoplastic SNN were the most prevalent etiological subtype (4 cases), all of whom presented peripheral neuropathy as the initial symptom. Among these 4 cases, malignancies were identified in 3 cases and 3 cases presented with anti-Hu antibodies. Among the remaining 4 patients, 2 cases were autoimmune and the other 2 cases were idiopathic. NCS results of the 8 cases revealed decrease or absence of sensory nerve action potential (SNAP) amplitudes, with normal sensory conduction velocities. Six cases showed abnormal SEP, including 2 cases of central damage and 4 cases of peripheral damage, 5 cases had abnormal SSR, and 2 cases exhibited abnormal CHEP. Motor nerve conduction studies were normal in all 8 cases. Six patients underwent spinal MRI, and 4 exhibited abnormal signals in dorsal columns.Conclusions:The hallmark clinical manifestation of SNN is sensory ataxia, characterized by substantial impairment of superficial sensation, typically manifesting in a non-length-dependent distribution. Beyond the widespread and significant reduction in SNAP amplitudes, SNN may also exhibit additional electrophysiological impairments, such as those observed in SEP, SSR and CHEP.SEP combined with spinal cord MRI can improve the detection rate for damages in the central sensory conduction pathway.
