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On Friday 23 October, patients, carers, researchers and healthcare professionals gathered together at the Oxford Spires Four Pillars Hotel for the first ever UK day dedicated to primary lateral sclerosis.

Patients carers and researchers discuss a rare form of motor neuron disease

What is PLS?

Primary lateral sclerosis (PLS) is a very rare form of motor neurone disease (MND) that progresses slowly. Typically, it affects people over 50, but it can affect younger people too. PLS affects the nerve cells (upper motor neurons) that send messages from the brain down to the spinal cord to control other cells (lower motor neurons) that are directly responsible for making muscles work. The consequence of this damage is that muscles develop stiffness and loss of function. Although the limbs are affected in almost all cases, symptoms in the muscles used for speech and swallowing are also common.

It is not known what causes PLS, and research is ongoing. Researchers believe that a combination of environmental and genetic factors play a part, although it is not thought to be an inherited condition.

The day's programme

About 100 people attended the event, which was organised by the Oxford MND Centre and supported by the MND Association. It included expert talks from researchers at the University of Oxford and the University of Sheffield, as well as two question and answer sessions. For many people, one of the main benefits of the day was to meet others who had been diagnosed with the same condition. In particular, there is a Facebook group for PLS patients and their carers, and many of them were meeting in person for the first time.

Martin Turner and Kevin Talbot from the Nuffield Department of Clinical Neurosciences (NDCN) at the University of Oxford talked about the history of the condition and its clinical features, discussing its relationship to more common types of motor neuron disease. Olaf Ansorge, also based in NDCN, gave an overview of the histopathology of PLS, and Christopher McDermott from Sheffield explained what other diseases can teach us about the condition.

Ricarda Menke (NDCN & Oxford Centre for Functional Magnetic Resonance of the Brain) and Malcom Proudfoot (NDCN & Oxford Centre for Human Brain Activity) presented two key techniques for furthering research into PLS: magnetic resonance imaging and magnetoencephalography. A key feature of all these presentations was the honesty with which the researchers presented their limited understanding of this rare disease, along with their determination and optimism regarding future discoveries.

Dr Mary-Kay Floeter from the National Institutes of Health in Washington, USA, gave a much-anticipated talk in the afternoon on her unique work to understand the natural history of the condition. This was followed by a presentation from Oxford MND Centre staff Kevin Talbot and Rachel Marsden on symptom management. The day concluded with a session where patients’ questions on how to manage symptoms were addressed by the panel.

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