Age of onset
ALS can also be classified based on the age of onset. While the peak age of onset is 58 to 63 for sporadic ALS and 47 to 52 for familial ALS, about 10% of all cases of ALS begin before age 45 ("young-onset" ALS), and about 1% of all cases begin before age 25 (juvenile ALS). People who develop young-onset ALS are more likely to be male, less likely to have bulbar onset of symptoms, and more likely to have a slower progression of disease. Juvenile ALS is more likely to be familial than adult-onset ALS; genes known to be associated with juvenile ALS include ALS2, SETX, SPG11, FUS, and SIGMAR1. Although most people with juvenile ALS live longer than those with adult-onset ALS, some of them have specific mutations in FUS and SOD1 that are associated with a poor prognosis. Late onset (after age 65) is associated with a more rapid functional decline and shorter survival. Signs and symptoms
The disorder causes muscle weakness, atrophy, and muscle spasms throughout the body due to the degeneration of the upper motor and lower motor neurons. Individuals affected by the disorder may ultimately lose the ability to initiate and control all voluntary movement, although bladder and bowel function and the extraocular muscles (the muscles responsible for eye movement) are usually spared until the final stages of the disease.Cognitive or behavioral dysfunction is present in 30–50% of individuals with ALS.
Pyromania is an impulse control disorder in which individuals repeatedly fail to resist impulses to deliberately start fires, to relieve some tension or for instant gratification. The term pyromania comes from the Greek word πῦρ (pyr, fire). Pyromania is distinct from arson, the deliberate setting of fires for personal, monetary or political gain. Pyromaniacs start fires to release anxiety and tension, or for arousal. Other impulse disorders include kleptomania and intermittent explosive disorder. There are specific symptoms that separate pyromaniacs from those who start fires for criminal purposes or due to emotional motivations not specifically related to fire. Someone with this disorder deliberately and purposely sets fires on more than one occasion, and before the act of lighting the fire the person usually experiences tension and an emotional buildup. When around fires, a person with pyromania gains intense interest or fascination and may also experience pleasure, gratification or relief. Another long term contributor often linked with pyromania is the buildup of stress. When studying the lifestyle of someone with pyromania, a buildup of stress and emotion is often evident and this is seen in teens attitudes towards friends and family. At times it is difficult to distinguish the difference between pyromania and experimentation in childhood because both involve pleasure from the fire. Classification
ICD
The World Health Organizations International Classification of Diseases (11th Revision) ICD-11, regarded as the global standard, was released in June 2018 and came into full effect from January 2022.