Dr Judith Guedalia

Finding A Name For A Situation
Yossi (not his real name), aged 15, underwent three ophthalmologic surgeries within one month (in another hospital in the country). The first two were for cataracts in both eyes, and the third was for a detached retina. He had general anesthetic and was in appropriate medical/psychiatric/psychological condition after the first two surgeries. Following the third surgery, Yossi had many deficits in cognitive and motor behavior (he was unable to walk or speak and showed poor/or no memory of family members).
Medical tests, neuro-physiological examinations including MRI (Magnetic Resonance Imaging) CT (Computerized Axial Tomography) and metabolic evaluations, found no cause for this extreme behavior change.
Conversion disorder (hysterical disorder) was suspected, and he was placed on observation as an outpatient in a pediatric psychiatric hospital and received anti-psychotic medication that did not seem to relieve his condition. He was referred for neuropsychological assessment at the unit that I head at Shaare Zedek Medical Center.
He arrived with his mom and grandmother. I was impressed at how he seemed so placid, yet did attempt to complete the assessment.
His test scores and behavior showed significant cognitive dysfunction (functioning four or five standard deviations below his age norm). His mother said he was completely normal until his last eye surgery. He had studied in regular, i.e. not a special education, classes from kindergarten through current, his eighth-grade year. I perused his report cards and could not find any specific learning problems.
I observed language inconsistencies (during neuropsychological assessment) of articulation, phonology and syntax that presented (to me) a foreign accent.
I asked if he spoke another language or if he heard one while growing up, and the answer was no.
I am a member of a number of International ListServs (mailing list programs for communicating with other people who have subscribed to the same list; in my case, professionals in the field of adult and pediatric neuropsychology). As the brain is the final "midbar" - desert - in medical knowledge, there are always interesting syndromes, on the ListServ, we consult with each other about them. I recalled that a few years ago I had read a thread (a set of posts on a newsgroup, composed of an initial post about a topic and all responses to it) about Foreign Accent Syndrome.
"We neuropsychologists get excited over weird things," I said to myself. Could this be an example of this rare foreign accent syndrome?
As no one else noticed this accent, how would I be able to prove that he had this disorder? I woke up in the middle of the night and sat up and said, "He's 15 years old; I bet he has a Bar Mitzvah video."
The next morning, I called his mom, and indeed there was one and she would drop it off at my office by noon. I located a VCR in the hospital and watched and listened to Yossi at his Bar Mitzvah Celebration. No accent!
As I researched the disorder more fully, I found articles on the rare cases of FAS (foreign accent syndrome) in professional literature, which pointed to disorders of: "left parietal hemorrhagic stroke," "posterior lateral aspect of the left pre-central gyrus," "left basal ganglia infarct."
I called the neurologist who had seen him and gave him my theory. To say the least, he was not taken by my off-the-wall theory, as he had not noticed an accent when he examined Yossi.
When I found the following article: "Foreign Accent Syndrome Following a Catastrophic Second Injury: MRI correlates, linguistic and voice pattern analysis" by: Carbary TJ, Patterson JP, Snyder PJ; PMID 10857668 which stated:
"We suggest that FAS has a primary subcortical involvement. We also show that this case is accompanied by a deficit linguistic but not affective, prosodic expression. We agree that the foreign quality of the FAS speech is a perceptual impression of the listener and not inherent in the patient's vocalization.
I referred Yossi to a speech and language specialist who also thought there was some sort of accent, though she couldn't put her finger on it. Another listener heard an accent of some kind.
My next step was convincing the psychiatrist and his staff that the problem may be neurological and not psychiatric.

I had no hard data. Neither the CT Scan nor other tests described any specific findings.

I asked the neurologist to call a meeting and went on line and downloaded a format for a legal brief. I had no data but I thought I might be able to make a case.
Using that model, I presented my case to the various medical specialists. My goal was to have Yossi tested by an advanced neuro-radiological test, a PET Scan. Positron emission tomography (PET) is a nuclear medicine, medical imaging technique, which produces a three-dimensional image or map of functional processes in the body. Because of the need to inject radioactive material into patients - and in this case, a child - and it is not advisable to subject any one participant to too many scans, strict clearance is required. The test is also quite expensive, and the insurance company would have to give their approval as well.
To make this long story shorter, there was a left hemispheric parietal finding, though a non-conclusive one. Unfortunately, neither providing the syndrome with a name, nor identifying the locale have improved Yossi's outcome, and at this point in time, his cognitive abilities have not improved. This remains the single saddest finding.
Originally published in the Jewish Press on August 2,2006.

Tags: Cognitive Dysfunction | FAS | Foreign Accent Syndrome | Jewish Press | Language Inconsistencies