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Table 2 Descriptions of dramatic behavioral manifestations associated with c9FTD/ALS

From: Cognitive and behavioral features of c9FTD/ALS

•      The patient believed that others were spying on her and required all blinds to be closed in the home and doors to be locked.
•      The patient believed that pieces of plastic were emanating from his head, leading him to pick repetitively at his scalp to remove the 'plastic bits' embedded in his skin.
•      The patient believed that someone was about to harm her and carried a knife and pistol for self-defense.
•      The patient believed he had a weakness of the gluteal muscles and that he therefore had to keep his finger in his anus to prevent incontinence.
•      The patient believed that he was under surveillance.
•      The patient believed that he was infested by mites, which crawled under his skin and into his extremities. He reported that the mites congregated in his earlobe and that he could reduce their number by pinching his earlobe at regular 10-minute intervals.
•      The patient believed that his son was trying to kill him, so the patient barricaded himself in his home.
•      The patient believed he was being contacted by letter or phone by dead friends and hatched plans to meet them.
•      The patient believed that characters on the television screen were communicating with her.
•      The patient believed that someone's wife was trying to harm him and threatened to shoot her.
•      The patient believed that people around him and on the television screen were talking about him and calling him names.
•      The patient had visions of the devil.
•      The patient heard the voice of God.
•      The patient perceived that men, including a man dressed in a gorilla outfit, were hiding in her garden and saw disembodied faces, which she believed to be spirits. As a result, the police were contacted.
•      The patient sat in the yard, held rifles in both arms, and 'shot anything that moved' (illustrative case).
•      The patient spoke in a high-pitched and child-like voice and behaved as if she was a child.
•      The patient underwent a dramatic change in religious beliefs and arranged ritualistic and candle-lit meetings with spirits.
•      The patient felt the need to carry a handgun in her purse. When her husband hid the gun, she proceeded to purchase another (and was able to do so without raising the suspicion of the gunshop owner).
•      The patient threw lit fireworks though his neighbor's letterbox.
•      The patient complained of excessive heat. He threw open doors and windows, refused to allow any heating in the home, and dressed in summer attire in mid-winter. He took to pouring cold water over tepid food to 'cool it down'.
•      The patient combed his hair repetitively and vigorously, leading to bleeding of the scalp. The patient wore multiple watches on his arm and donned clothes on the wrong part of his body (for example, trousers on his head and underpants on his arms). He used objects inappropriately (for example, a spoon to clean his teeth and a toilet brush to brush his hair).
•      The patient constantly wiped surfaces and washed pots and carried with him a cloth to wipe his shoes before getting into his car and plastic bags to wipe his hands.
•      The patient washed his hands, filed his nails, and combed his hair repetitively and drank excessive quantities of water as a remedy for her symptoms, leading to hyponatremia.
•      The patient cleaned the house obsessively and followed his dog around, cleaning the surfaces that it had walked on.
  1. Examples were compiled from the clinical series of the authors [21] and that of Snowden and colleagues [28]. c9FTD/ALS, frontotemporal dementia or amyotrophic lateral sclerosis (or both) linked to chromosome 9.