Abstract: Objective To explore the clinical features and auxiliary examination of neuronal intranuclear inclusions(NIID), in order to raise awareness toward the disease.Methods A total of 8 NIID patients who were admitted to Department of Neurology, the Affiliated Hospital of Xuzhou Medical University from January 2020 to January 2022, andpathologically and genetically diagnosed were enrolled. Their clinical, imaging and electrophysiological data were retrospective analyzed.Results For the patients enrolled, their clinical manifestations included cognitive impairment in six cases, episodic psychiatric symptoms in one case, limb weakness in two cases, sensory disturbance in one case, autonomic dysfunction in six cases, tremor in four cases, and ataxia in four cases. Magnetic resonance imaging(MRI) showed abnormally high signals of diffusion-weighted image(DWI) along the corticomedullary junction in all eight cases, with brain atrophy to various degree in six cases. According to electrophysiological results, there were abnormal nerve conduction in eight cases, including slowing of motor nerve conduction velocity in eight cases, decreases in motional amplitudein one case, slowing of sensory nerve conduction in seven cases, decreases in sensory amplitude in two cases, and abnormal sympathetic skin response in seven cases. Synchronized tremor(4-7 Hz) was detected in four cases.Conclusions The clinical manifestations of NIID are different, where both the central and peripheral nervous systems are involved. Cerebral MIR shows abnormally high signals along the corticomedullary junction. Electrophysiological results presents obvious peripheral myelin damage and autonomic nerve injury, with tremor in some pateints.