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When Alzheimer's was unknown

September 18, 2006|ELENA CONIS

In the fall of 1901, a confused, forgetful, disoriented and paranoid 51-year-old woman named Auguste D. was admitted to Germany's Frankfurt Hospital. She was the first documented case of a degenerative disorder that afflicts about 4.5 million people in the U.S. today. And, in the fullness of time, her striking condition would bring lasting recognition to a German doctor, Alois Alzheimer.




Working at Frankfurt's mental asylum in the 1800s, the young Alzheimer became intrigued by physical abnormalities in the brains of the mentally ill. Using the latest techniques to study brain tissue, he and a colleague wrote a six-volume tome on the brain's normal and diseased states.

Alzheimer described the brain changes associated with epilepsy and Huntington's chorea, but it was August D.'s unique condition that would earn him a place in medical history. Alzheimer examined Auguste D. in 1901, documenting her hallucinations, "strong feelings of jealousy toward her husband" and "rapidly increasing memory impairments."

"She was disoriented carrying objects to and fro in her flat and sometimes hid them," he wrote. "Sometimes she felt that someone wanted to kill her and would scream loudly." Auguste D.'s symptoms worsened gradually until her death five years later of an infection of the blood.

When he heard of her death, Alzheimer, who had by then moved on to the psychiatric clinic at Munich University, requested her records -- and her brain.

Examining her brain tissue, Alzheimer noticed hardened blood vessels and clusters of cells and proteins, known as plaques, previously seen only in the brains of much older victims of senility.

He also saw, for the first time, abnormal tangles of nerve fibers.

This combination of plaques and tangles, which Alzheimer described at a meeting of German psychiatrists in 1906, ultimately led to the definition of a new disease. Over the next few years, Alzheimer and physicians in Europe and the U.S. shared stories of similar cases: relatively young patients with symptoms of severe senility that worsened with age, and -- visible at an autopsy after death -- tangles of neurons and plaques in the brain.

Alzheimer's name wasn't attached to the condition until 1910 -- and even then, the naming of the disease caught him by surprise. It was his former teacher and colleague, renowned psychiatrist Emil Kraepelin, who coined the term "Alzheimer's disease" in his 1910 Textbook of Psychiatry.

Because Auguste D. and other early cases were relatively young, Alzheimer's was originally defined as a form of dementia that set in before old age, sometimes as early as age 40.

That notion held for decades. But then, studies in the 1960s and '70s showed that Alzheimer's tangles and plaques were common in many much-older patients with senile dementia.

The mystery of Alzheimer's continues to unfold slowly. Researchers now know that it very rarely affects young people, instead largely striking only those over 60. Four genes have been linked to the disease, but scientists still don't know what causes it, and there is no cure.

Auguste D. was undoubtedly not the first to suffer Alzheimer's disease, she was merely the first to be so diagnosed.

Alois Alzheimer died in his 50s of an infection of the heart. He probably had little idea what a household name "Alzheimer" would eventually become.

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