de Morsier’s Syndrome

Image2The video of Lou, which follows, evokes the mellifluous voice and musical ability which sometimes accompanies the congenital disorder, de Morsier’s Syndrome. Also known as ONH (Optic Neural Hypoplasia) or SOD (septo-optic dysplasia) this condition has become increasingly more common and is now a major cause of congenital blindness. Limited intellectual ability, obsessive-compulsive and autistic traits and seizures are frequently seen. The pituitary gland may not form properly and as a consequence sufferers may require replacement of thyroid and other hormones. When vision is quite limited, nystagmus, a jerky repetitive movement the eyes may be noted, though this is not unique to de Morsier’s.

We don’t know for sure what causes de Morsier’s Syndrome (which was named for the Swiss physician who discovered the condition). Definitive tests for the disease do not exist, though I am told by a radiologist acquaintance that an MRI may reveal that a part of the brain called the septum pellucidum is under-developed.

The number and degree of all of these symptoms and findings can vary a great deal from one individual to another. Parents describe sufferers as requiring very rigid routines and often they are very sensitive to environmental stimuli. For example a patient of mine who suffers from de Morsier’s used to become very agitated by the sound of running water.

Speech may be repetitive and limited, and echolalia, the tendency to repeat phrases back, is quite common. Sufferers may manifest a lilting vocal tone and musical talent out of keeping with their other abilities.

This ability should be encouraged in order to maximize the potential of the child and allow him or her to interact in positive and meaningful manner…and this is exactly what Lou’s family does.

Enjoy the song!

Lou je m’appelle Lou

Lou’s Foundation

 

Photo Credit

Photo Of Lou is a screen capture from the video

 

 

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