Israeli Woman Shares Family Struggle With Rare, Fatal Brain Disease Creutzfeldt-Jakob

Shanel Biton, a 20-year-old from Ramat Gan, recounts the devastating impact of Creutzfeldt-Jakob Disease (CJD) on her family after her mother, Yarit Biton, was diagnosed about a year ago. Initially mistaken for a stroke due to symptoms like stammering and unusual behavior, Yarit’s condition rapidly deteriorated, leading to her current hospice care in Jerusalem. Shanel emphasizes the emotional toll of watching her mother lose her personality and speech, urging others to cherish their loved ones while they can.

CJD, also known as "mad cow disease" in its bovine form, is a rare, incurable neurological disorder caused by prion proteins that progressively destroy brain tissue. Professor Eyal Leshem from Sheba Medical Center explains that most cases arise spontaneously, typically in people aged 50 to 70, with about 10 to 20 new cases annually in Israel. Unlike the UK outbreak linked to contaminated beef in the 1980s and 1990s, no Israeli cases have been traced to infected meat. Around 10% of cases are hereditary, with some genetic mutations more common among Libyan Jews.

The disease leads to severe cognitive decline, memory loss, movement disorders, and eventually death within months. Diagnosis relies on clinical symptoms and specialized laboratory and imaging tests. Dr. Nurit Omer of Ichilov Medical Center highlights that their neurology department serves as Israel’s national center for diagnosing and treating CJD, offering advanced molecular diagnostics and clinical trials. She notes that the term "mad cow disease" is misleading for human CJD, which is not contagious through daily contact.

Shanel’s story, shared widely on social media, has raised awareness about this rare disease. She encourages families facing similar challenges to remember their loved ones as they were and to value the time they have together. The Biton family also copes with the severe health decline of Shanel’s younger brother, adding to their hardships. Medical experts continue to seek effective treatments, with experimental therapies underway at Ichilov since 2023.