• Unfavorable prognosis | |
• Survival time after onset 3–4 years | |
• Progressive loss of voluntary muscle functions | |
• In the course of the disease, most patients suffer from | |
➢ dyspnoe requiring ventilatory treatment | |
➢ dysphagia requiring enteral nutrition | |
• Severe cognitive and behavioral impairment manifested as frontotemporal dementia (FTD) among 3–5% of patients |