Dr Judith Guedalia

"He Is Blind But He Can See": More Diagnostic Tales from a Neuropsychologist's Journal.

"We'd like you to come up to see a young boy", said Dr. A, the then pediatric chief resident.  "We have a patient here, an 8 year old boy from a small Bedouin village, he is blind but he can see."


That immediately caught my attention.  As a neuropsychologist I am always interested in the brain/behavior relationship.  Here was an obvious example of the doctor reporting his own ‘cognitive dissonance'.  Cognitive dissonance can be defined as a psychological phenomenon which refers to the
discomfort felt at a discrepancy between what you already know or believe, and new information or interpretation and there is a need to accommodate new ideas.  In this situation it occurred when the ‘blind' patient could really ‘see'. 

When I told the floor nurse who I was looking for, she said she had seen something ‘different' about his behavior too.  She told me that as opposed to other children in the unit, he did not show ‘fear' when they came to take blood, or inject him with antibiotic, in general she said, ‘his affect was flat'.

Hum, I said, in my most professional manner, where I could find him.  She told me that he was in the ‘school'.  Every large hospital in Israel also has a ‘school', a one room schoolhouse, under the auspices of the Ministry of Education.  The teachers are in contact with the inpatient's school so that a hospitalized child can keep up with homework and curriculum.  At Shaare Zedek Medical Center (which is a 500 bed medical center), we have both Israeli and Arab teachers who teach the relevant curriculum.

Fuad (not his real name), had been hospitalized for severe diarrhea, shigellosis.  He was being treated; one morning his worried parents told the doctor that he didn't recognize them.    When I saw him, Fuad was being pushed in a wheel chair by his father.  Marchaba (‘Hello' in Arabic); Sho Ismac? (What is your name), I said --I speak a lot of languages poorly!  He looked in my general direction and said his name.   I asked him to imitate some hand signals.  He did so, but at the same time he moved his ‘pinky' and eyebrow.  I did some more, and noticed that these seemingly peripheral movements continued.  As it happened, his back was toward the wall and I was standing facing a mirror which was hanging in the play area of ‘the school'.  As I recorded his ‘odd' eye and pinky movements, I suddenly noticed that he was imitating my eyebrow and small finger moving.  Interesting, I thought, he is attending to extraneous movements in his field of vision. 

I gave him a cup of water to drink.  I had wanted to see if he noticed the cup coming at him and wondered what he would do.  He moved his mouth to the cup I held, and started to lap it up, as a cat might lap up milk in a dish.

Then quite unexpectedly he sneezed; I gave him a tissue; he ate the tissue.

To make a long story short, the intern and I looked at each other disbelievingly.  In graduate school, especially in the field of neurology, the professors always bring up these ‘weird' cases that you think in a million years you'll never see yourself (most of the time that is correct).  With the exception of ‘odd' sexual behavior-which one wouldn't have expected seeing in a 9 year old anyway, little Fuad seemed to demonstrate the criteria of Kluver-Bucy Syndrome.  

The syndrome is named for Heinrich Kluver and Paul Bucy, who in 1939 bilaterally removed the temporal lobes in rhesus monkeys, in an attempt to determine its function. This caused the monkeys to develop psychic blindness (visual agnosia), emotional changes, altered sexual behavior, hypermetamorphosis and oral tendencies.  People with lesions in their temporal lobes show similar behaviors. They may display oral or tactile exploratory behavior (socially inappropriate licking or touching); hypersexuality; bulimia; memory disorders; flattened emotions (placidity); and an inability to recognize objects or to recognize faces (Prosopagnosia or ‘face blindness').  They also show signs of visual agnosia or "psychic blindness," i.e. an inability to visually recognize objects.

Further research in the hospital's medical library turned up a few cases of Kluver-Bucy (by now I was calling it by the ‘familiar' KBS) in humans with ‘trauma' to there temporal lobes.  After a EEG and CT Scan, it seemed that Fuad did indeed have some ‘unusual' activity in the temporal areas of his brain, probably related to seizures he had had as a younger child and Shigellosis which is a bacterial infection affecting the intestinal tract. We hypothesized that the Shigellosis, (causing temporary metabolic deficiencies and high fever) may have created a transient form of bi-lateral temporal dysfunction, which in turn produced the symptoms of Kluver-Bucy Syndrome. His condition improved over a week, which made it unusual for science, as KBS is rarely transient.

I love the dynamism of the field of neuropsychology, it is like solving a mystery.  One never knows for sure what the days' patient list will present.  I guess among the skills the field teaches, is being able to ‘see' and understand ‘clues' when they are presented to you.  We wrote it up and published it in an international neuropsychological journal as an unusual case of transient Kluver-Bucy Syndrome.


Originally published in the Jewish Press on January 25, 2006.

Tags: Brain/Behavior Relationship | Cognitive Dissonance | Face Blindness | Jewish Press | KBS | Kluver-Bucy Syndrome | Prosopagnosia | Psychic Blindness | Shigellosis | Visual Agnosia