How do you know you have ALS?

The average age of ALS was 56 years in sporadic patients and 46 years in patients with positive family history. The average course of disease was 3 ~ 5 years, but the course of disease was also different in different subtypes. Generally speaking, patients under 55 years of age have a longer survival period. In addition, the course of disease in familial ALS patients was different from that in sporadic patients, and was associated with specific gene mutations.

However, no matter what type of ALS patients, they eventually died of respiratory failure. The main manifestations of ALS were myasthenia muscle atrophy muscle bundle tremor and muscle tension hyperreflexia and pathological sign. General no sensory abnormalities and urination disorders. Muscle weakness, muscle atrophy, muscle bundle tremor were involved in the lower motor nervous system, muscle tension increased, tendon reflex hyperactivity, pathological sign positive were the main manifestation of upper motor nervous system involvement. For the purpose of diagnosis, the skeletal muscles of the whole body are usually divided into four segments according to their location: the ball, the neck, the thoracic and the lumbosacral. The evidence of the damage of the upper and lower motor neurons in the above four parts is sought in turn. Cognitive impairment is a common feature of ALS. Frontotemporal dementia (FTD) is a common disease in patients with ALS. According to statistics, about 5% ALS patients with the diagnostic criteria of FTD, while 30% of ALS patients do not meet the diagnostic criteria of FTD, but they also show signs of executive dysfunction. For the patients with advanced cortical dysfunction such as cognition or behavior, but who do not meet the diagnostic criteria of FTD, if the main manifestation is behavioral change, it is called “ALS accompanied with behavioral disorder”, if the main manifestation is cognitive dysfunction. The clinical manifestations of patients with ALS with cognitive impairment include: attentional impairment, executive dysfunction, decreased planning and problem-solving ability, fluency or non-fluency aphasia, personality change, irritability. Advanced cortical dysfunction, such as mental retardation, but memory is usually not involved or mild. At present, there is no reliable screening test for ALS cognitive impairment. Verbal fluency is a sensitive indicator, but also screening frontal lobe executive function and so on.

 

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