Sometimes good things really do come to those who wait. That could be the message of the year for people with neuromuscular disorders — terrible, uncommon diseases that steal patients’ muscle strength and often their lives. This is the year that the Ice Bucket Challenge became an Internet sensation, bringing an unprecedented flood of exposure and research funds to the “orphan” disease ALS, one of the most sinister neuromuscular disorders. And this is the week that the University of Cincinnati Neuroscience Institute’s Neuromuscular Program became a little more muscular. It joins nine other neuroscience specialties as an official Center of Excellence.

This is welcome news for patients and families affected by the spectrum of adult neuromuscular disorders, which include:

• Acquired neuropathies (diabetes related to toxins and chemotherapy, etc.)
• ALS (amyotrophic lateral sclerosis or Lou Gehrig’s disease)
• Cervical and lumbar radiculopathies
• Charcot Marie Tooth disease
• Guillain-Barre
• Inflammatory myopathies (dermatomyositis, polymyositis, inclusion body myositis)
• Metabolic myopathies (mitochondrial disorders, myophosphorylase deficiency)
• Muscular dystrophy
• Myasthenia gravis and neuromuscular junction disorders
• Spinal muscular atrophy

“Becoming a center of excellence gives assurance to our patients that we provide cutting-edge neuromuscular care,” says John Quinlan, MD, Medical Director of the Neuromuscular Center. “It will help in the long term to raise awareness for these poorly understood diseases and to help us develop future benchmarks in three core areas: research, treatment and education.”

Center status also brings new visibility within the region, says Hani Kushlaf, MD, right, Director of the Neuromuscular Medicine Fellowship Program. “We are known for our expertise in delivering tertiary and quaternary neuromuscular care. Our elevated status will help us going forward to achieve additional milestones.”

The Center’s clinical team is led by five board-certified neuromuscular specialists. Patients with the most complicated neuromuscular disorders are seen in two MDA Clinics (one at the UC Medical Center and the other at University Pointe in West Chester, Ohio).  Robert Neel, MD, heads up the recently established UC Health ALS Clinic at the Daniel Drake Center for Post-Acute Care.

“Our team provides a full range of neurophysiological testing for neuromuscular diseases — from the most common carpal tunnel syndrome to the rarest botulism poisoning,” Dr. Quinlan says. “We offer neuromuscular junction testing, and Dr. Kushlaf performs single-fiber EMG for diagnosis of the most difficult neuromuscular junction diseases.

In the educational arena, the neuromuscular team has a long track record of excellence. Dr. Quinlan is a legendary professor at the UC College of Medicine, and his collection of silver apples awarded by his students is sparkling proof. Dr. Neel directs an annual ALS workshop and, looking forward, a dedicated neuromuscular fellowship training program will begin in July 2015 under the direction of Dr. Kushlaf.

Perhaps of greatest importance to desperate patients who often find that little can be done to stop or delay muscle wasting or deterioration, the Center is now primed to play a larger role in laboratory and clinical research. Earlier this year, Laura Sams, MD, began a treatment trial of rituximab for the treatment of myasthenia gravis. A collaboration is under way with Cincinnati Children’s Hospital Medical Center in an effort to correct the primary molecular defect in myotonic dystrophy type I, and Dr. Kushlaf is pursuing additional basic science studies of myasthenia gravis.

As the Neuromuscular Center gains momentum, it also stands to benefit from added community engagement. The neuromuscular team has played a leadership role in the annual MDA Muscle Walk and the Walk to Defeat ALS, and Dr. Neel was not to be left out of the Ice Bucket Challenge. He did his own challenge at the College of Medicine and then got drenched a second time when helping to pour ice on UCMC CEO Lee Ann Liska and his boss, Brett Kissela, MD, the Albert Barnes Voorheis Chair of the Department of Neurology and Rehabilitation Medicine.

– Cindy Starr