What is Opsoclonus-Myoclonus Syndrome? 

Opsoclonus is an unusual disorder of eye movement in which both eyes dart involuntarily (dancing eyes).  Myoclonus simply means brief muscle jerks and ataxia indicating in-coordination.

Symptoms of Myoclonus occur most when movement is attempted, worsens with agitation or stimulation, but may be present at rest.   The child may appear tremulous or have gross jerking.  Face, eyelids, limbs, fingers, head and trunk are involved.  During the peak of the illness, sitting or stand is difficult or impossible.  Patients also have trouble speaking, eating, or sleeping, and exhibit drooling, rage attacks, head tilts, or other abnormalities.  Children appear to be nervous, irritable or lethargic.

The brain is an innocent bystander caught in the “cross-fire” between the body’s immune system and the tumor, which is trying to destroy it.  Brain regions like the cerebellum, brainstem, and limbic system appear to bear the brunt of the onslaught.

*Neuroblastoma is the deadliest form of pediatric cancer with a 55% survival rate.  Only 2-3% of children with Neuroblastoma will develop Opsoclonus Myoclonus Syndrome.  Survival from Neuroblastoma  is much better in children with OMS than those without.

N-Myc Amplified Localized Stage 1 Neuroblastoma Tumors  are extremely rare.  Only 87 tumors have been reported worldwide in a ten year study.  This particular tumor is not typically found in OMS patients.  Joey’s age of onset, his unusual symptoms, and type of tumor makes Joey’s disease most rare.