Motor Neuron Diseases
Conditions
Interventions
non-motor neuron diseases or healthy group:No
motor neuron diseases group:none
Sponsors
Department of Neurology of Sun Yat-sen Memorial Hospital
Eligibility
Sex/Gender
All
Age
18 Years to 80 Years
Inclusion criteria
Inclusion criteria: Experimental Group: Those whose families and patients have informed consent and can perfect relevant examinations; Clinically clearly diagnosed patients with motor neuron disease: occult onset after middle age, chronic progressive exacerbation, clinical manifestations are mainly different combinations of myasthenia, amyotrophy, bulbar paralysis and pyramidal sign caused by upper and lower motor neuron damage. There was no sensory disturbance, electromyography showed neurogenic damage, cerebrospinal fluid was normal, and imaging was not abnormal. Including the following types: amyotrophic lateral sclerosis (ALS), progressive bulbar palsy (PBP), progressive spinal muscular atrophy (PMA), primary lateral sclerosis (PLS), etc. The inclusion of ALS: 1. Necessary conditions: (1) Evidence of lower motor neuron disease shown by clinical, electrophysiological or neuropathological examination. (2) Evidence of upper motor neuron disease shown by clinical examination. (3) The medical history or examination confirms that the above symptoms or signs are progressing in one part or from one part to other parts. 2. At the same time, the following 2 points should be excluded: (1) Electrophysiological or pathological examination indicates that the patient may have other diseases causing upper and lower motor neuron diseases. (2) Neuroimaging suggests that patients may have other diseases that lead to the above clinical symptoms or electrophysiological changes. 3. The definite diagnosis of ALS by further grading diagnosis according to clinical manifestations: there are signs of upper and lower motor neuron disease in at least 3 parts. The inclusion of PBP conforms to the diagnostic criteria of motor neuron disease and is manifested by unilateral or bilateral medullary disorders, dysarthria and other medullary disorders. Clinical or electrophysiological indications indicate that there is no evidence of spinal cord motor neuron injury: limb weakness, muscle atrophy, tendon reflex weakening, muscle tremor or pyramidal symptoms, muscle spasm, tendon hyperreflexia and motor disorders. The incorporation of PMA is in line with the diagnostic criteria for motor neuron disease. Clinical or electrophysiological findings suggest evidence of motor neuron paralysis (myasthenia gravis, amyotrophy, and fascicular tremor) under 1-4 parts (medulla oblongata, neck, chest, lumbosacral), without upper motor neuron paralysis, including pseudobulbar paralysis such as crying and laughing, pharyngeal hyperreflexia, sensory disturbance, and elimination of similar diseases such as poliomyelitis, diabetic amyotrophy, hereditary SMA family history, etc. PLS inclusion: According to the diagnosis of motor neuron disease, the onset age is over 40 years old, and only upper motor neuron symptoms exist. EMG of 3 limbs, medullary muscle and paraspinal muscle are normal. Control Group: Those whose families and patients have informed consent and can perfect relevant examinations, clinically clearly diagnosed patients with other diseases or healthy volunteers.
Exclusion criteria
Exclusion criteria: Combined with other cerebrovascular diseases such as acute stroke, brain trauma, epilepsy, poisoning, hereditary degeneration, demyelinating disease, metabolic diseases, hydrocephalus and other non-vascular leukoencephalopathy patients, etc.; Inflammatory diseases of the peripheral system and other central nervous systems, etc.; Have serious underlying diseases; Poor general condition.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| HOCl; | — |
Countries
China
Contacts
Public ContactYing Peng
Department of Neurology, Sun Yat-sen Memorial Hospital, Sun Yat-sen University
Outcome results
None listed