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Queen Square Centre for Neuromuscular Diseases

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About Neuromuscular Diseases

The severity of neuromuscular diseases can vary from mild weakness or numbness that has little impact on day to day life to life threatening weakness affecting breathing muscles. Neuromuscular diseases affect approximately 150,000 people in the UK and around 14,000,000 people globally.

Who do we see?

We see people with all types of neuromuscular conditions in our clinics: from Motor Neuron Disease that affects the motor nerve control centre located right at the top of a motor nerve near the spinal cord, to distal myopathies that affect the muscles furthest from the trunk.

We see patients with both genetic and acquired diseases, e.g. Guillan Barre Syndrome, an autoimmune condition that affects nerves and may be triggered by a preceding infection, and Charcot Marie Tooth Disease, one of the most common inherited nerve diseases.

Patients of all ages from birth to the elderly can be affected by neuromuscular diseases. Paediatric clinics are held at Great Ormond Street Hospital, adult clinics are held at the National Hospital for Neurology and Neurosurgery and paediatric transition clinics may be held in both locations.

Our research

Our clinical services are complemented by a very active and successful neuromuscular research centre.

Our research focuses on translating scientific discoveries into new ways to diagnose and treat patients with neuromuscular diseases. This means that patients benefit from research advances that we translate into improvements in patient care.

What happens when this stops working?

Neuromuscular diseases are genetic or acquired conditions that can affect the somatic motor neuron, sensory neuron or muscle (see diagram). The symptoms of a condition depend on where the problem is.

ganglion


For example a disease affecting the somatic motor neuron may cause weakness and wasting of the muscle (see picture). Whilst diseases affecting the sensory neuron may cause patients to inadvertently harm themselves as they are not aware of something being too hot or too cold or to have difficulty coordinating their movements as they are not getting proper feedback on where their body part is in space.

HandsWasted legs

A functioning neuromuscular system is critical to interact safely and effectively with the environment. For movement to occur your brain emits an electrical signal. This signal travels down your spinal cord and makes contact with somatic motor neurons or nerves that leave the front (ventral) part of the spinal cord and communicate with the muscles you need to contract or relax (see diagram).

Meanwhile, information about what your body is feeling e.g. if your hand or foot is in the right place or if something is too hot or too cold, is encoded in electrical signals that travel through a sensory neuron (see diagram), into the back (dorsal) part of the spinal cord and on to the brain. Your brain decodes these signals, integrates them with other signals for example visual, and then acts on the information. This allows you to carry out accurate voluntary movement e.g. step over an obstacle or wave to someone you have seen, do up a button and pick up a pen and write.

However, the neuromuscular pathway also enables reflex movements that help protect your body from damage. This is because sensory neurons also connect indirectly with a motor neuron via an interneuron in the spinal cord (see diagram). This pathway bypasses the brain and the rest of the spinal cord and enables very fast movements to occur without conscious thought e.g. withdrawing your hand from a hot object, or adjusting your leg position to prevent falling if your foot hits an unexpected obstacle.