| Scheda |
Age
of onset
|
Generally
after 50, with male to female ratio 1.3:1 |
| Type
of lesion |
Degeneration
of motor neurones localized into the spinal cord and brainstem,
responsible for the trophysm/function of skeletal muscles and
of the cortico-spinal tract, connecting the motor cortex to the
motor neurones. |
Prognosis
|
Progressive
and fatal in about three years from the first symptom. |
| Clinical
picture |
Symptomatic
weakness and atrophy in the hands and/or feet and/or bulbar muscles
with difficulties in swallowing, speech, and respiration. |
| Deterioration |
ALS
differs from other neurodegenerative diseases for the rare cognitive
impairment. |
| Onset |
Unrecognized
in the hand, foot, brainstem muscles with weakness, atrophy,
and/or difficulties in swallowing, speech, and respiration. |
Progression
and therapy
|
Therapy
is ineffective: riluzole is the first specif drug approved with
minimal efficacy. Palliative treatment is recommended. |
