Hashimoto's encephalopathy presenting with isolated cerebellar ataxia in 13 children / 中华儿科杂志

Objective: To analyze the clinical characteristics, treatment and prognosis of Hashimoto's encephalopathy presenting with isolated cerebellar ataxia in children. Methods: A retrospective analysis was performed on the clinical features, laboratory tests, neuroelectrophysiological examination, imaging, treatment and outcomes of 13 patients with Hashimoto's encephalopathy presenting with isolated cerebellar ataxia, who were admitted to the Department of Pediatric Neurology of Guangzhou Women and Children's Medical Center from January 2016 to May 2021. Results: Among the 13 cases, 6 were males and 7 were females. The onset age was 2.6 (2.0,3.3) years, 9 children had precursor infection or vaccination before the first course of disease. All the 13 children had gait abnormalities or unsteady sitting, 10 had intentional tremor, 6 had dysarthria, 3 had body tremor, 2 had nystagmus, 3 had fatigue, 3 had hypotonia, 2 had vomiting and 1 had irritability. Thyroglobulin antibody (TgAb) was 500.0 (298.9,587.2) kU/L and thyroid peroxidase antibody (TPOAb) was 621.9 (449.6,869.4) kU/L in 13 cases. Autoantibodies were positive in 9 cases, and cerebrospinal fluid leukocytosis was seen in 4 cases. Regarding electroencephalography result, 4 cases had background slowing and 1 case had occasional sharp waves. Among the 3 patients who had relapses, 1 had cerebellar atrophy shown on cranial magnetic resonance imaging (MRI) during the recurrence. All the patients received intravenous immunoglobulin (IVIG) and intensive methylprednisolone therapy during the first onset, followed by the disappearance of the symptoms, 1 patient had repeated episodes which was decreased after immunosuppressive treatment with Rituximab.Followed up for 25.0 (22.5,33.3) months after the last episode, 12 achieved complete remission and 1 had a wide base gait. Conclusions: Trunk ataxia is the common symptom of Hashimoto's encephalopathy presenting with isolated cerebellar ataxia in children.Children with cerebellar ataxia should be tested for TgAb and TPOAb to detect Hashimoto's encephalopathy, avoiding missed diagnosis and treatment delays; IVIG and intensive steroid therapy is effective, and immunosuppressive therapy for patients with multiple relapses could reduce the recurrence.

Relationship between Cervical Curvature and Spinal Drift Distance after Laminectomy with Screw Fixation and Its Effect on Clinical Outcome / 中国康复理论与实践

Objective:To observe the relationship between cervical curvature and spinal drift distance after laminectomy with lateral mass screw fixation, and its effect on clinical outcome. Methods:From January, 2017 to October, 2018, a total of 117 patients with cervical spondylotic myelopathy (CSM) underwent laminectomy with lateral mass screw fixation, and 90 of them completed the follow-up. According to the patients' cervical curvature (CC), they were divided into three groups: those CC between 0° to 5° were in Group A (n = 28), 5° to 16.5° in Group B (n = 36) and CC > 16.5° in Group C (n = 26). The spinal drift distance, nerve recovery, axial symptoms and C5 palsy were recorded and analyzed. Results:There were significant differences in CC and spinal drift distance (F > 152.119,P < 0.001), and no significant difference was found in laminectomy width and incidence of C5 palsy (P > 0.05) among three groups. The Japanese Orthopaedic Association (JOA) score significantly increased in all the groups post operation and at the last follow-up (t > 8.869,P < 0.001), and no significant difference was found among there groups at the same time (P > 0.05), as well as the incidence of C5 palsy (F = 0.472,P = 0.625). There was significant difference in the score of Visual Analogue Scale of axial symptoms among three groups (F > 34.800,P < 0.001), which was lower in groups B and C than in group A (t > 5.845,P< 0.001), and no significant differene was found between group B and group C. Conclusion:On the basis of the same laminectomy width, the greater the CC was, the more favorable the spinal drift went backwards. The loss of CC was related to the occurrence of axial symptoms, but was not correlated with the neurological recovery and C5 palsy.