Leukoaraiosis is a chronic recurrent inflammatory disease of unknown etiology, with clinical manifestations of recurrent oral aphthous ulcers, uveitis, genital ulcers and skin damage. Neuroleukodystrophy is the neurological manifestation of the disease, and 10-25% of leukodystrophies are combined with central nervous system damage. There are no characteristic laboratory manifestations of the disease, and a proportion of patients have increased blood sedimentation and C-reactive protein in the acute phase. Neuroleukopenia relies mainly on clinical diagnosis and is often misdiagnosed, often as multiple sclerosis, etc. A typical case: Wang xx, male, 34 years old. 2 years ago, the patient developed choking cough with no obvious cause, and did not pay attention to it at that time. 1 year ago, the choking cough and slurred speech worsened, and the patient was consulted at the local hospital, and a cranial MRI was performed, suggesting “abnormal signals in the brainstem and bilateral cerebral hemispheres”, which was diagnosed as “cerebral infarction and brain atrophy”. Ten months ago, he developed numbness and weakness in the left limbs; seven months ago, he developed weakness in the right limbs, slurred speech and unsteadiness in walking, which was diagnosed as “multiple sclerosis” and treated with a-interferon. One month ago, he suddenly became unstable in walking and the strength of the left limb was increased compared with before. The patient had a history of recurrent oral ulcers and vulvar genital ulcers, and a positive pinprick test. The blood, urine and stool routine, biochemical and rheumatic immunological indicators were normal, and the blood sedimentation and C-reactive protein were normal. Lumbar puncture cerebrospinal fluid routine and biochemistry did not show abnormalities. Oligoclonal zone band (-), blood and cerebrospinal fluid water channel protein-4 antibodies were negative. MRI suggested multiple abnormal intracranial signals, including brainstem, basal ganglia area, and thalamus, and circumferential enhancement was seen in the thalamic lesions. The patient was diagnosed with “neuroleukopenia” based on the characteristic clinical manifestations and a series of examination results. After regular treatment, the patient’s symptoms are now stable and there are no obvious episodes of oral ulcers or vulvar genital ulcers.