Chiari 1; Philosophy of Treatment; Jorge Lazareff, MD

SUMMARY: 10 years ago I (Jim Ausman-JIA) was asked to visit Dr. Tom Milhorat in New York to review his work on Chiari abnormalities. I had known Tom from the NIH where he was an excellent scientist. He devoted his career to the study of CSF and Chiari Disorders. He and his interdisciplinary team of Neurologists and Neuroscientists studied the Chiari deformities in a large series of patients. He and his team took detailed histories of these patients, measured the dimensions of the posterior fossa in detail, categorized other anomalies they saw; recorded the psychological state of the patients, documented those with tethered cord and more. At the time no such exhaustive study of this disease had been made. He concluded that the Chiari disorders was spectrum of disorders, likely genetically based, with differing penetrance of clinical signs and symptoms. Included in this spectrum of disorders was craniosynostosis, Chiari 1,2 ,3, tethered cord, osteogenesis imperfecta, Cranial facial disorders, neurological signs of nystagmus, and cranial nerve disease, psychological disorders indicating brain neural development disorders, and syringomyelia . He was thoroughly criticized for his radical views of this disease spectrum and surgical treatments such as cutting the filum by the pediatric neurosurgical community. He was another pioneer whose ideas were ruthlessly rejected. Some, related to jealousy.

Dr. Jorge Lazareff in this challenging, thought provoking, analysis of reviews how the treatments for Chiari 1 developed over time based on clinical observations mostly centered in increased CSF pressure. Yet in his insightful analysis, not all of the symptoms and signs found with Chiari 1 disorders can be explained by increased intracranial pressure. Surgery is an answer in many cases but is seriously a deficient analysis of the cause of this disease. The disease has a much wider spectrum of presentations now most likely related to a genetic disorder (s) of the CNS, still not well recognized. Dr Milorhat was likely correct in his detailed studies. Dr. Lazareff describes how 'we come to believe what we believe is true' by being rewarded with some surgical successes for a disease that is not totally surgical, but has another cause. His analysis is humbling and shows how, in treating patients, we are trying to do the best we can without a full understanding of the disease and can make some serious mistakes. Like the. treatment of Epilepsy in the past for a disease which was not understood and believed related to Gods and Demons, feared and treated with isolation of the patients. How can one recognize these mistakes? See what you decide after seeing this outstanding challenging debate on the fundamentals of Medicine. What do we really know about the diseases we treat and how we are treating them? What about the treatment of back pain? One of the best "Lecture-Discussions" on SNI Digital®. Essential viewing for all physicians and students at all levels. 60 minutes (JIA) From the Subsaharan African Grand Rounds meeting sponsored by SNI Digital and the Subsaharan Neurosurgeons.