Prosopometamorphopsia and facial hallucinations

In July, 2011, a 52-year-old woman presented to our psychiatric outpatient clinic in The Hague with a life-long history of seeing people’s faces change into dragon-like faces and hallucinating similar faces many times a day. She could perceive and recognise actual faces, but after several minutes they turned black, grew long, pointy ears and a protruding snout, and displayed a reptiloid skin and huge eyes in bright yellow, green, blue, or red. She saw similar dragon-like faces drifting towards her many times a day from the walls, electrical sockets, or the computer screen, in both the presence and absence of face-like patterns, and at night she saw many dragon-like faces in the dark. During her childhood the faces had not bothered her, but in early adolescence they became more prominent and she realised that this was not how other people saw each other’s faces. She felt isolated, became depressed, and abused alcohol for many years, but managed to graduate from secondary school, get married, have a daughter, and become a school administrator. Her diffi culty sustaining stable perception of other people’s faces led to communication problems and recurrent confl icts forcing her to change jobs often, but she sought professional help only after further deterioration of her symptoms. A local psychiatrist prescribed citalopram and quetiapine with no improvement. In desperation she searched the internet for experts in her condition and emailed Prof Oliver Sacks, who referred her to our team in The Netherlands. Her medical history consisted of birth with a caul, recurrent non-migrainous headaches, urinary tract infections, sensed presence, passage hallucinations (seeing movement from the corner of the eye), and occasional zoopsia (seeing large ants crawling over her hands). Apart from the hallucinations, prosopo metamorphopsias, and a mildly depressed mood, psychiatric assessment showed no other abnormalities. Our patient had full insight into the hallucinatory nature of her perceptions, and explained them in terms of a “brain disorder”, having previously interpreted them in metaphysical terms as a consequence of being born with a caul. Neurological examination, blood tests, and electroencephalogram (EEG) were normal, and MRI brain showed only a few white-matter abnormalities near the lentiform nucleus and in the semioval centre (fi gure). Face recognition is a complicated process with huge ramifi cations for social functioning. It is mainly associated with the fusiform face area and the adjoining occipital face area, within the context of the visual network as a whole. Considering the complexity of the process involved it is remarkable that prosopometamorphopsia has been reported only rarely. The condition, fi rst described in 1947 by Bodamer, is usually transient, and is attributed to structural brain changes or functional disorders such as epilepsy, migraine, or eye disease. Despite the negative EEG fi ndings we attributed our patient’s visual events to aberrant electrophysiological activity in the adjacent regions of the brain that are specialised for face and colour in the ventral occipito-temporal cortex. Another possibility we considered was peduncular hallucinosis (appendix). We provided psychoeducation, stopped all her previous medication, and started 300 mg of valproic acid daily. She had symptom-free days for the fi rst time in her life but developed an auditory sleep start (loud bangs heard several hours after falling asleep) so we changed valproic acid to 3 mg of rivastigmine daily, which reduced the frequency of auditory symptoms and kept visual symptoms suffi ciently under control for her to function normally. She has remained in the same job for the past 3 years and her interaction with colleagues is greatly improved.