What Is CADASIL, The Disease Featured In The Fall Of The House Of Usher? Our Doctor Explains
Contains mild spoilers for "The Fall of the House of Usher"
Movies, books, and TV series have a way of leaving pieces of them with us. This is exactly what happened when Netflix premiered Mike Flanagan's miniseries, "The Fall of the House of Usher" on October 12. The gothic horror drama is based largely on Edgar Allan Poe's 1839 short story of the same name and pulls heavily from other works by this famed writer of everything mystery and macabre. It's a meeting of two minds unlike any other.
Starring Bruce Greenwood as Roderick Usher, the tormented CEO of a corrupt pharmaceutical company called Fortunato responsible for killing millions of people, Carla Gugino as Verna, the supernatural shape-shifting being who also portrays another one of Poe's characters — the black raven from "The Raven," and Madeline Usher, sister of Roderick and COO of Fortunato Pharmaceuticals, "The Fall of the House of Usher" is disturbing to say the least. It's a clever play between what's real and what's not, and it is in this realm that viewers are left with another piece that's hard to put out of their heads — the reference to a disease called CADASIL. Roderick Usher, who's lost all six of his children to gruesome deaths within a week, says he has it.
New York City neuropsychologist and director of Comprehend the Mind, Dr. Sanam Hafeez, told Health Digest exclusively that CADASIL stands for Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy. "[It] is a rare genetic condition affecting the brain's blood vessels," she explained. In the series, we're led to believe that Usher hallucinates because of CADASIL. Does this happen with the disease?
CADASIL results in a narrowing of the brain's blood vessels
Viewers of "The Fall of the House of Usher" follow the narrator, a former friend-turned-rival of Roderick Usher, who's been invited to hear the latter's confession after the tragic and disturbing deaths of his children. This takes place in the long-abandoned house Usher and his sister grew up in, which he seems afraid of, and is portrayed as eery, cheerless, and depressing. On top of such a setting, we're also led to believe that Usher hallucinates and sees things like a frightening figure in a costume as well as the mutilated remains of his children because of his disease – CADASIL.
"Think of your brain as a network of tiny blood vessels that supply it with blood and oxygen. In CADASIL, there is a problem with these blood vessels. They become narrower and less efficient over time," explained Dr. Hafeez. Are hallucinations a part of the symptoms of this disease as portrayed in the Netflix show? Not according to the doctor. "Hallucinations are generally not considered a common symptom of CADASIL." (Perhaps, we're better off thinking Roderick Usher was plagued by guilt instead and this was the reason for his hallucinations).
She notes that the most typical symptoms of CADASIL include migraine headaches as well as strokes or Transient Ischemic Attacks (TIAs) which can manifest in sudden weakness, numbness, or trouble with speaking. Other symptoms can include cognitive impairment like thinking, memory, and reasoning problems that progress over time. Afflicted people can also experience mood and psychiatric changes such as depression, anxiety, personality changes, mobility as well as balance-related issues like problems with walking, coordination, and balance, and vision and hearing impairments, added the doctor.
How rare is CADASIL?
In "The Fall of the House of Usher," it's not only Roderick Usher who's plagued by the disease. So was his mother, Eliza. Just how rare is this genetic condition?
"CADASIL is estimated to affect a relatively small portion of the population, with the exact prevalence varying by region," explained Dr. Hafeez. "In some regions, the prevalence may be as low as 1 in 25,000 people, while in others, it could be slightly higher." The doctor added, however, that these numbers are just rough estimates. "The rarity of CADASIL means that it might not be well-known or frequently encountered in healthcare settings." No wonder most of us weren't aware of the disease before "The Fall of the House of Usher."
It's also important to note that CADASIL doesn't affect or progress in everyone the same way, much like how Alzheimer's disease progresses. "Some people may experience a milder course with slower progression, while others may have more severe and rapidly progressing symptoms," shared the neuropsychologist. There is also no cure for this genetic condition, but those who have it can manage symptoms with treatments that include medications and lifestyle changes, according to the doctor. "[These] may help control issues like headaches, blood pressure, and mood changes," she added. Managing the disease also involves stroke prevention and management, dealing with the cognitive problems that affect daily life, and managing the physical symptoms that affect mobility and balance.