Neuromuscular

Patient Population
The Neuromuscular Clinic at Toronto Western Hospital specializes in the tertiary care and research of patients with disorders of the nerve, muscle, and neuromuscular junction. The clinic is a tertiary referral Centre for many neurologists (including other neuromuscular specialists) in Ontario and across Canada. It's the largest program in Canada for management of patients with myasthenia gravis.

Approximately 3,500 patients attend this clinic annually. About 1,600 procedures are performed annually including highly specialized investigations such as single fibre electromyography (EMG) and quantitative EMG. To improve efficiency and service, the EMG laboratory has state-of-the-art electrodiagnostic equipment, with extensive networking and immediate reporting capabilities.

Education
The clinic plays an important role in teaching at the post-graduate, medical school and undergraduate levels. Fellows in neuromuscular disorders, neurology residents, medical residents and summer students all learn about the investigation and care of patients with nerve, muscle and neuromuscular diseases. These trainees participate in important clinical research projects involving neuromuscular patients.

Research
Research activities are focused on new treatments for patients with: diabetic neuropathy, inflammatory demyelinating polyneuropathy (acute and chronic), and myasthenia gravis (MG).

• We have defined the optimal methodology for electrophysiolgocial testing in neuropathy trials. Our studies have helped establish the major role of electrodiagnositic evaluation in the assessment of patients with chronic polyneuropathy.

• We are completing a large-scale screening program in approximately 500 diabetic patients to assess the optimal simple clinical test for the detection of diabetic neuropathy in the general diabetes clinic or physician's office.

• We are actively investigating aspects of autonomic nervous system control and quantitative sensory function in patients with diabetic neuropathy.

• Multiple therapeutic drugs for diabetic neuropathy have been tested including: aldose reductase inhibitors (sorbinil, statil, FK-366, zopolrestat, zenarestat), non-specific metabolic drugs (acetyl-l-carnitine), neurotrophic factors (nerve growth factor) and phospholipase kinase C inhibitor (PKCi). Zopolrestat, zenarestat and PKCi trials are ongoing.

• Studies of painful symptom treatment in patients with diabetic neuropathy have been completed in the clinic and a study on the effectiveness of a new drug, pregabalin, in painful symptoms is underway.

• In patients with MG, we have documented a comparable long-term clinical outcome after transcervical thymectomy as compared to more invasive surgical approaches. Our experience in MG patients with thymoma has been published.

• An active area of clinical investigation is the effectiveness of different immunomodulation therapies in patients with motor neuropathies, inflammatory demyelinating polyneuropathies (acute and chronic). Currently, we are investigating the usefulness of the new immune suppressive agent, neoral in the treatment of patients with autoimmune neuromuscular disorders (MG, polymyositis and chronic inflammatory demyelinating polyneuropathy [CIDP]). Clinical research comparing different immunomodulation therapies (intravenous immunoglobulin and plasma exchange) in patients with CIDP is planned.