A RARE CASE OF CAUSALGIC DYSTONIADiagnosis + Cure
Dystonia is a medical condition that affects more than 300,000 adults and children across the United States and is somewhat of a medical mystery. Dystonia consists of abnormal involuntary muscle activity caused by abnormal firing of nerves. It can affect writers (referred to as ‘writer’s cramp’ ), musicians, athletes, and others who have a genetic predisposition and are engaged in concentrated repetitive activities.
Dr. Marko Bodor, Director of Research for Napa Medical Research Foundation, diagnosed an unusual case of dystonia and then in conjunction with Dr. Tom Sampson of San Francisco, developed a series of operations that helped restore the patient to normal function.
A healthy, very active 65-year-old woman developed pain in her groin after a 6-mile hike among the redwoods of Big Basin State Park. Over a period of several weeks, she developed a worsening contracture of her hip, such that her thigh was flexed up against her chest and could not be straightened. Suffering from significant pain and discomfort and unable to walk, sit, or function with any degree of normalcy, the mysterious dystonia had turned an avid hiker into an invalid. She sought the help of multiple neurologists and orthopedic surgeons including at Stanford and UCSF, most of whom had never seen anything like it before.
Her UCSF neurologists referred her to Dr. Bodor for assessment and consideration for an ultrasound-guided injection of botulinum toxin into her hip flexor muscles to block neural impulses resulting in the muscle contracture. This is normally a routine procedure for neurologists specializing in movement disorders, but given the technical difficulty of her thigh being flexed up against her chest, she was referred to Dr. Bodor.
Dr. Bodor determined that she had two problems – one, an injury at the hip joint and two, a dystonia. This combination was most consistent with a diagnosis of ‘causalgic dystonia’, which had only been described once before. Dr. Bodor concluded that the only way to resolve her problem would be surgically. He referred her to surgeons specializing in children with cerebral palsy, who tend to develop similar contractures, but they felt her case was too difficult and risky. Finally she saw Dr. Sampson in San Francisco, one of the pioneers of arthroscopic hip surgery, who agreed with Dr. Bodor’s assessment. In a long and technically complicated operation he was able to reduce her contracture from 135-degrees to 45-degrees. However, according to Dr. Bodor, the dystonia would come back unless its trigger, pain in the hip, was resolved. Since she had developed arthritis which had finally reached a tipping point, the only way to resolve her pain would be with a hip replacement. Considering the technical difficulties related to her hip flexion contracture, she was referred to Dr. Matta in Los Angeles for the hip replacement, which she underwent.
Finally, she was pain-free, but stuck with a 45-degree flexion contracture, requiring her to stand on the tip of her toes of the affected leg and not being able to walk normally. She saw Dr. Bodor again for the next step.
Through a series of operations using high-frequency ultrasound, Dr. Bodor was able to determine the precise location of additional tight bands and scar tissue that were limiting her hip. Using a 3 mm meniscotome (a small scalpel on the end of thin 20 cm rod), Dr. Bodor was able to navigate using ultrasound, avoid the lateral femoral cutaneous nerve, cut the tight bands and reduce her hip contracture to 0 degrees. The technique used in this case has not been published yet for any tendon contractures in live humans, let alone for causalgic dystonia.
At this time, the patient has regained a normal gait pattern and is able to conduct her daily living activities. Thanks to the support of the Napa Medical Research Foundation and the assistance of Yvette Uribe BS, Dr. Bodor will be able to publish a case report on causalgic dystonia and how its contracture was effectively treated with one hospital-based arthroscopic operation and a series of office-based ultrasound-guided operations.
Neurologists, neurosurgeons, neuroscience and rehabilitation specialists around the world are working collaboratively to better understand the physiological processes that trigger abnormal neuromuscular activity in dystonia with the goal of discovering a way to prevent and cure them. The experience and technique developed here in Napa adds to that knowledge and provides an effective treatment for the consequences of one type of dystonia among the many that affect patients around the world.
Reduce Pain. Increase Mobility + Function. Improve Quality of Life.
All the research conducted through the Napa Medical Research Foundation, a 501(c)(3) nonprofit organization, is fully funded through generous donations received from individuals and family foundations.