Diagnosis dilemma: In rare circumstances, women considered people as dragons

Patient: A 52-year-old woman from The Hague, Netherlands

Symptoms: The woman visited an outpatient psychiatry clinic and told the doctor that she saw people’s faces transform into a dragon’s face. She also saw a dragon-like face appear before nature, even when there were no people. These hallucinations got in the way and influenced her ability to interact with people.

The patient reported that the initially normal human face “could turn black, long, pointy ears and protruding nose showed huge eyes of dark yellow, green, blue or red.” She added that she had experienced these hallucinations since she was a child.

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What happened next: Clinicians performed blood tests and neurological tests using a fierce brain technique called EEG. The results were all normal. However, MRI of the patient’s brain showed several lesions near the lentisphere nucleus. Damage to this part of the brain is associated with cognitive impairment, particularly attention and memory problems associated with schizophrenia.

These lesions that appear in the brain’s insulation wiring, known as the white matter, may have been caused by rupture of small blood vessels in the brain. However, the damages were not recent, according to the report.

Although EEG did not reveal any abnormalities, doctors suspected that the patient’s visual hallucinations were caused by atypical electrical activity in parts of the brain that process the colour and face, particularly the ventral occipital cortex. This area on the back of the brain is involved in regulating object recognition. The lesions revealed by MRI may have caused this electrical activity, and doctors theorized, and may have been present since birth, possibly caused by temporary oxygen detachment before and after birth.

Diagnosis: The doctor determined that the woman had a form of propometamoamopsis (PMO). This unusual condition affects how a person perceives a human face, making facial features appear dramatically distorted. The functionality may appear to have been enlarged or reduced. They may hang down, stretch sideways, or excavate their posture.

In people with Hemi-prosopometamorphophopsia, or Hemi-PMO, only one side of the observed face is affected. In a full-face propometamoamorphopia, or full-face PMO, distortions distort the entire face that a person is looking at. This condition is associated with changes in brain structure and disorders that affect brain function, such as epilepsy, migraine, and stroke.

Treatment: Doctors prescribed daily patients with valproic acid, an anticonvulsant that prevents seizures and relieves the migraines and symptoms of bipolar disorder. This treatment controlled visual hallucinations in women.

However, she then began to experience hallucinations of pounding sounds while she was sleeping. The doctor switched her to a daily regimen of rivastigmine, a drug commonly used to treat dementia symptoms caused by Alzheimer’s and Parkinson’s disease. This medication reduced the frequency of these auditory hallucinations and reduced visual symptoms to manageable levels. Three years after this treatment, women reported that their work situation had stabilized and social relationships had improved.

According to a 2021 review, this case is unique: Prosopometamorphophopsia is extremely rare, with only 81 accounts of the medical literature over the past 100 years.

For most people experiencing this condition, the effects of distortion on human faces last for days or weeks. The PMO-triggered distortion is known to make people’s faces look “devils”, but in the case of women it is unclear exactly why it caused her very specific perception of the reptile dragon.