Clinical characteristics and treatment analysis of the facial onset sensory and motor neuronopathy
10.19845/j.cnki.zfysjjbzz.2021.0087
- VernacularTitle:面部起病的感觉运动神经元病临床特点及治疗分析
- Author:
Lifang MA
1
;
Ruofan LIU
1
;
Li ZHOU
1
Author Information
1. Department of Neurology,Dongzhimen Hospital,Beijing University of Chinese Medicine,Beijing 100700,China
- Publication Type:Journal Article
- Keywords:
Facial onset sensory and motor neuronopathy;
Neurodegenerative disease;
Sensory motor neuron disease
- From:
Journal of Apoplexy and Nervous Diseases
2021;38(4):340-344
- CountryChina
- Language:Chinese
-
Abstract:
Objective To retrospectively analyze the clinical characteristics and treatment of patients with facial onset sensory and motor neuronopathy (FOSMN).Methods FOSMN syndrome were searched in PubMed,EMbase,CBM,CNKI,WanFang Data database,from the date of database establishment to 2019,and combined with a case admitted to our hospital.The clinical characteristics and treatment were analyzed from above data.Results A total of 27 studies were included,42 males and 25 females,with an average onset age of (51.0±12.4) years.Clinical features:85.1% had numbness in the face or mouth as the initial symptom,53.7% had facial muscle weakness,56.7% had facial muscle atrophy,50.7% had abnormal corneal reflex,more than 80% had bulbar syndrome,and more than 50% had neck flexion or upper limbs weakness,47.8% had neck or upper limb muscle atrophy,and 43.3% had tongue muscle atrophy.Pathological examination of autopsy or muscle biopsy showed loss of involvement nucleus and spinal cord neurons,as well as muscle neurogenic atrophy.TDP-43-positive inclusions were found by autopsy.Neuroelectrophysiological examination:83.6% had prolonged or disappeared blink reflex,88.1% had acute or chronic progressive denervation,and 55.2% had lower SNAP amplitude in upper limbs.Treatment:Of the,1/30 cases had a clear improvement with IVIg therapy.Some patients continued to progress after IVIG,glucocorticoids,and plasma exchange therapy;16 patients were died with an average duration of 7.7 years.Conclusion FOSMN syndrome begins with abnormal sensory in the trigeminal nerve distribution area.The lesions spread from top to bottom in the brainstem,gradually involving the motor nucleus in bulbar,and spinal cord anterior horn.The abnormal blink reflexes,neurogenic damage,and SNAPs amplitude reduction in neuroelectrophysiological examinations have diagnostic value.There is a high possibility of degenerative diseases and no effective treatment for the disease currently.
- Full text:2024073013254042836Clinical characteristics and treatment analysis of the facial onset sensory and motor neuronopathy.pdf
