Rare disease: woman sees reptiles instead of people, literally

A resident of the Netherlands suffered from a rare disease, called prosopometamorphopsia, when the brain distorts the visual perception of other people’s faces. 

For more than a century, scientists have recorded only 80-100 such cases. Meanwhile, the distorted faces, the woman saw, looked like reptiles or dragons. This gave doctors the opportunity to get an idea, of what how fragile and complex the human vision system is.

It is noted, that a 52-year-old resident of the Hague sought psychiatric help after hallucinations began to interfere with her normal daily life. According to her, when she looked at the faces of other people, she saw, that they were gradually changing: the skin became like that of reptiles, the ears stretched, the faces were forward, and the eyes began to glow unnatural color. 

The woman also adds, that even when no one was around, she could see the faces of dragons, looming over her from the walls, sockets and screens. The doctors conducted standard tests, taking blood samples, conducting a neurological examination and making an EEG, which measures the electrical activity of the brain. All the results were normal. 

The MRI results showed lesions near the lenticular nucleus — this is an area of the brain, which is responsible for cognitive functions, memory and attention. The damage was detected in the white matter of the brain — the nerve fibers, that connect different areas of the brain to each other. 

The damage was probably caused by ruptures of small blood vessels, that occurred a long time ago, possibly at birth, as a result of oxygen deprivation. According to doctors, these old injuries disrupted the normal transmission of signals between the ventral occipito-temporal cortex — the area responsible for recognizing objects and faces — and other visual circuits. 

The woman’s brain distorted only human faces and perceived other objects and animals normally. Doctors diagnosed her with prosopometamorphosis, which covers the entire face, not just one side. First, he was prescribed valproic acid, a drug, used for epilepsy, migraines, and bipolar disorder.

This reduced the woman’s visual hallucinations, but caused a loud snoring in her sleep. Because of this, the woman was prescribed the following instead of valproic acid rivastigmine — a drug, commonly used during treatment of Alzheimer’s and Parkinson’s diseases. Thanks to this drug, the visual hallucinations significantly decreased and became controlled, and in 3 years the woman returned to normal life. 

For most people with post-traumatic arthritis, the disorders last only a few days or weeks. In others, they persist for years. Symptoms often resemble psychosis, which can lead to misdiagnosis and inadequate treatment. However prostropometamorphopsia has nothing to do with delusions, as patients know, that their brains distort visual perception. This distinction is very important for doctors, trying to distinguish rare visual processing disorders from mental illnesses, such as schizophrenia.

Scientists still don’t know exactly, what causes the disease post-traumatic osteomyelitis. Some patients have a history of brain damage, strokes, migraines, or epilepsy, while other structural changes are not observed. In the future, the researchers hope to use more advanced neuroimaging and computer modeling techniques to recreate what patients with post-traumatic osteomyelitis see. 

Source: ZME Science