运动神经元病课件

1、Clinical features of als and other forms of motor neuron diseaseChen YanClinical features of als and 运动神经元病（motor neuron disease MND）：是一组病因未明的选择性侵犯脊髓前角细胞、脑干后组运动神经元、皮质锥体细胞及锥体束的慢性进行性神经变性疾病。 运动神经元病（motor neuron diseaseSPECTRUM OF MOTOR NEURON DISEASEAmyotrophic lateral sclerosis (ALS）Progressive muscul

2、ar atrophy（PMA）Primary lateral sclerosis（PLS ）Progressive bulbar palsy （ PBP ）Flail arm syndromeFlail leg syndromeALS-plus syndromeSPECTRUM OF MOTOR NEURON DISEAALS is the most common form of motor neuron disease and includes upper and lower motor neuron pathology.PMA is a progressive lower motor ne

3、uron disorder. Some experts believe it represents a form of ALS . ALS is the most common form oFrontotemporal dementia may be associated with ALS in 15 to 50 percent of cases.Flail arm syndrome is characterized by progressive lower motor neuron weakness and wasting that predominantly affects the pro

4、ximal arm.Upper extremity onset is most often heralded by hand weakness but may begin in the shoulder girdle muscles.PMA is a progressive lower motor neuron disorder.Clinical patterns of progressionThese may produce impairment affecting limb(table 1) ,bulbar (table 2) ,axial (table 3) and respirator

5、y (table 4) function.Upper extremity onset is most often heralded by hand weakness but may begin in the shoulder girdle muscles.Flail arm syndromeCognitive dysfunction does not exclude the diagnosis of ALS.Cognitive symptomsProgressive muscular atrophy（PMA）Clinically Probable ALS is defined on clini

6、cal evidence alone by UMN and LMN signs in at least two regions with some UMN signs necessarily rostral to (above) the LMN signs.临床、肌电图或神经病理学检查有下运动神经元损害的证据Cognitive dysfunction does not exclude the diagnosis of ALS.Progressive muscular atrophy（PMA）In patients with unilateral arm (leg) onset the most

7、 common pattern of spread is to the contralateral arm (leg), then to the ipsilateral leg (arm), then to the contralateral remaining leg (arm), and then to the bulbar muscles.Sensory symptomsIn patients with bulbar onset the most common pattern of spread is to one arm and then to the contralateral ar

8、m .PLS is a progressive isolated upper motor neuron disorder . Compared with ALS, it is characterized by slower progression, lack of weight loss, and absence of lower motor neuron findings on examination or electromyography in the first four years after symptom onset .PBP is a progressive upper and

9、lower motor neuron disorder of cranial muscles. This condition may occasionally stay isolated to the bulbar segment, but more commonly, upper and lower motor neuron signs and symptoms spread to involve other segments.Frontotemporal dementia may beFlail arm syndrome is characterized by progressive lo

10、wer motor neuron weakness and wasting that predominantly affects the proximal arm. It usually begins proximally and spreads distally to the point where arm and hand function is severely impaired. It is often asymmetric.Flail leg syndromeis characterized by progressive lower motor neuron weakness and

11、 wasting with onset in the distal leg.ALS-plus syndrome some patients have all of the clinical features of ALS along with features of other disorders such as frontotemporal dementia, autonomic insufficiency, parkinsonism, supranuclear gaze paresis, and/or sensory loss.Flail arm syndrome is characteC

12、LINICAL SYMPTOMS AND SIGNS The loss of motor neurons results in the primary clinical symptoms and signs of ALS. These may produce impairment affecting limb(table 1) ,bulbar (table 2) ,axial (table 3) and respiratory (table 4) function.CLINICAL SYMPTOMS AND SIGNS Amyotrophic lateral sclerosis (ALS）有能

13、解释上和（或）下运动神经元损害的其他疾病的电生理依据Sensory symptomsAmyotrophic lateral sclerosis (ALS）The loss of motor neurons results in the primary clinical symptoms and signs of ALS.Clinically Probable ALS is defined on clinical evidence alone by UMN and LMN signs in at least two regions with some UMN signs necessarily

14、rostral to (above) the LMN signs.Cognitive symptomsFlail arm syndromePrimary lateral sclerosis（PLS ）Asymmetric limb weakness is the most common presentation of ALS (80 percent).Sensory symptomsAmyotrophic lateral sclerosis 运动神经元病课件运动神经元病课件运动神经元病课件Initial presentation and patient complaints Asymmetri

15、c limb weakness is the most common presentation of ALS (80 percent). Upper extremity onset is most often heralded by hand weakness but may begin in the shoulder girdle muscles. Lower extremity onset of ALS most often begins with weakness of foot dorsiflexion (foot drop), while proximal pelvic girdle

16、 onset is less common. Initial presentation and patieUpper motor neuron symptomsLower motor neuron symptomsCognitive symptomsAutonomic symptomsParkinsonism and supranuclear gaze palsySensory symptomsClinical patterns of progressionUpper motor neuron symptomsCognitive impairment, typically related to

17、 frontotemporal executive dysfunction, may precede or follow the onset of upper motor neuron and/or lower motor neuron dysfunction in patients with ALS. Frontotemporal dementia may be associated with ALS in 15 to 50 percent of cases.Cognitive dysfunction does not exclude the diagnosis of ALS. Cognit

18、ive symptomsCognitive impairment, typicallAutonomic symptoms, parkinsonism, supranuclear gaze paresis, and/or sensory loss may occur as part of an ALS-plus syndrome.Autonomic symptoms, parkinsoniIn patients with unilateral arm (leg) onset the most common pattern of spread is to the contralateral arm

19、 (leg), then to the ipsilateral leg (arm), then to the contralateral remaining leg (arm), and then to the bulbar muscles. In patients with bulbar onset the most common pattern of spread is to one arm and then to the contralateral arm .Clinical patterns of progressionZIn patients with unilateral arDi

20、agnosis The diagnosis of ALS is based upon clinical criteria that include the presence of upper motor neuron and lower motor neuron signs, progression of disease, and the absence of an alternative explanation.Diagnosis The diagnosis of ADiagnostic criteria The clinical standard for the diagnosis of

21、ALS is the revised El Escorial World Federation of Neurology crite诊断依据：临床、肌电图或神经病理学检查有下运动神经元损害的证据临床检查有上运动神经元损害的证据症状或体征在一个部位内进行性扩展或扩展到其他部位同时排除以下两点有能解释上和（或）下运动神经元损害的其他疾病的电生理依据有能解释临床体征和电生理特点的其他疾病的神经影像学依据Diagnostic criteria The cliniThe body is divided into four regions: cranial, cervical, thoracic and

22、lumbosacral.Clinically Definite ALS is defined on clinical evidence alone by the presence of UMN, as well as LMN signs, in three regions.Clinically Probable ALS is defined on clinical evidence alone by UMN and LMN signs in at least two regions with some UMN signs necessarily rostral to (above) the L

23、MN signs.Cinically Probable - Laboratory-Supported ALS is defined when clinical signs of UMN and LMN dysfunction are in only one region, or when UMN signs alone are present in one region, and LMN signs defined by EMG criteria are present in at least two limbs, with proper application of neuroimaging and clinical laboratory protocols to exclude other causes.Clinically Possible ALS is defined when clinical signs of