Since we announced Bruce’s diagnosis of aphasia in spring 2022, Bruce’s condition has progressed and we now have a more specific diagnosis: frontotemporal dementia (known as FTD). Unfortunately, challenges with communication are just one symptom of the disease
Friday, February 17, 2023 at 4:20 AM GMT+8·2 min rea... Friday, February 17, 2023 at 4:20 AM GMT+8·2 min readNEW YORK (AP) — Nearly a year after Bruce Willis' family announced that he would step away from acting after being diagnosed with aphasia, his family says his “condition has progressed.”病程進展
There are two main variants of FTD: primary progressive aphasia, which hampers a patient’s ability to communicate, and behavioral variant frontotemporal dementia, which manifests as personality and behavioral changes. “It hits the parts of the brain that make us the most human,” said Dr.Bruce Miller, a professor of neurology at the UniversityofCalifornia
On average, it takes patients more than three years to get an accurate diagnosis, Ms. Dickinson said.What are the first signs of frontotemporal dementia?People with primary progressive aphasia may struggle to speak in full sentences or have difficulty comprehending conversations. They may have a hard time writing or reading
What are the treatment options for FTD? Generally, patients with FTD will live for around five to seven years after they first display obvious symptoms, Dr. Grant said. There is no cure — “but there’s a difference between a disease not having a cure and saying, ‘There’s nothing we can do,’”
Patients often benefit from steady, predictable routines, Ms. Dickinson said. And many patients, as well as family members, find comfort in support groups, she added.
Doctors also work to ensure that families are looking out for themselves. “The family often suffers as much as the patient,” Dr. Miller said.
There are at least 15 gene mutations associated with FTD, and each may be a “different type of FTD,” said Paul Schulz, professor of neurology and neurodegenerative disorders at the McGovern Medical School of UTHealth Houston. “That means that each could potentially have different causes.”
Abnormal proteins such as tau and TDP-43 accumulating in the brain is the most likely cause of FTD, the experts said. In the long term, these misfolded disease proteins cause neurotoxicity and, ultimately, cell death.
A patient with frontal lobe-focused abnormality would show behavioral issues of impulsivity and disinhibition. That’s called behavioral variant activity, which is the more common subvariant of FTD.
“For example, a polite person may become rude and a kind person may become self-centered,”
“The agrammatic type tends to be due to the tau problem in the brain, which affects the frontal lobe, mostly. Whereas the semantic type tends to be due to the TDP-43 type, which tends to affect the temporal lobe of the brain.”Some others can develop abnormal protein in parts of the brain associated with motor functioning & eye movement, the experts said.
＊＊＊Lin said. These cholinesterase inhibitors, known as Donepezil or Rivastigmine, might be used in the mild stages of Alzheimer’s and FTD. The second treatment is memantine, an NMDA receptor antagonist. “And typically, we would add that in the moderate to severe stage,” he said.＊＊＊
＊＊“In people with Alzheimer’s, by adding this medication, the cognitive decline rate could be potentially slower,” Lin said. “However, in FTD patients, there’s no such convincing data. But that’s just what we have.”＊＊
＊＊manage symptoms of the disease. Patients may need help managing issues such as anxiety, depression, aggressiveness, agitation and obsessional behavior. The SSRI (selective serotonin reuptake inhibitors) medications in the Prozac family of drugs have been shown to help with some of the problems in behavioral variant frontotemporal dementia, Budson said.＊＊
Friday, February 17, 2023 at 4:20 AM GMT+8·2 min readNEW YORK (AP) — Nearly a year after Bruce Willis' family announced that he would step away from acting after being diagnosed with aphasia, his family says his “condition has progressed.”病程進展
Generally, patients with FTD will live for around five to seven years after they first display obvious symptoms, Dr. Grant said. There is no cure — “but there’s a difference between a disease not having a cure and saying, ‘There’s nothing we can do,’”
Doctors also work to ensure that families are looking out for themselves. “The family often suffers as much as the patient,” Dr. Miller said.
“For example, a polite person may become rude and a kind person may become self-centered,”
常以記性不好表現，其他類型失智症都難以在早期察覺，例如血管失智症，或較少見的額顳葉型失智症。這些失智症除了目前無法用健
保藥物減緩其退化之外，臨床表現通常不以記性變差為主，例如血管型
早期可能就有明顯妄想症幻覺、人物辨認錯誤等症狀。額顳葉型
失智症可以在早期即出現性格改變激躁對於環境刺激無反應社交退縮，
或其他因語言問題導致功能退化。這些症狀（尤其是精神行為症狀，通常是
令家屬最難照護與共同生活。另外，精神行為症狀治療用藥通常會造成頭暈、
走路不穩、跌倒、白天精神嗜睡，甚至加速認知退化的副作用，在非必需使用
下通常不建議使用，特別是失智症的族群多為老年人。最後，雖然阿茲海默失
智症用藥（愛憶欣、憶必佳）已經證實可以「減緩」認知的減退，但**