The first of the clinical sessions covered a variety of topics related to the clinical care and symptom management of patients with ALS/MND.
This session opened with Professor Orla Hardiman from Ireland speaking about the importance of specialist multidisciplinary clinics in the care of ALS/MND patients. There is evidence that patients who attend such clinics survive longer and have a better quality of life. Identifying the precise mechanisms by which these improvements are achieved are challenging hence the presentation title – Multidisciplinary care in ALS: Measuring the immeasurable? However health care professionals in this setting are able to work interdependently to guide patients through the complex multiple levels of decision making combining experience, judgement, scientific evidence and patient’s preferences and values in order to optimise the timing and effectiveness of interventions. This is not to ignore the importance and value of community based services in the support of patients with MND/ALS and Professor Hardiman talked about an ecosystem – which includes the patient/caregiver, community services and the specialist multidisciplinary clinic. If we can better understand the range and pattern of decision making practices associated with successful outcomes for patients and their carers this will inform the creation of more effective models of care.
The next speaker was Dr Richard Smith from California giving the results of a trial following anecdotal reports which suggested that Nuedexta may improve bulbar symptoms in ALS. Nuedexta containing dextromethorphan and quinidine is a drug that has been licensed and available in the USA since 2010 for the treatment of emotional lability (pseudobulbar affect). It was licensed by the EU in 2013 but is still not available in the UK.
The trial (cross over trial design) involved 60 patients treated for one month with Nuedexta or placebo then switched after a washout period for a second one month course of either drug or placebo as appropriate. They used a self report scale (Center for Neurologic Study-Bulbar Function Scale CNS-BFS) as the primary outcome measure which showed a statistically significant improvement in the mean score for the patient group compared to the placebo group with speech, swallow and saliva responding positively to treatment. Patients without emotional lability benefitted as well as those with emotional lability. Speech rate and swallowing time (secondary outcome measures) also responded to treatment although not statistically significant.
Dr Smith concluded that further work is needed to determine the duration of the treatment effect and whether Nuedexta is potentially disease modifying.
Dr Ed Kasarskis from the University of Kentucky then spoke about a small case series of the use of radiotherapy as a treatment for siallorhea which had not responded to medication. They used electron beam radiotherapy (EBRT) to a single parotid gland in 32 patients and found a reduction in saliva production which lasted over 22 months with minimal side effects. The non irradiated side also improved. The use of anticholinergic medication was able to be reduced or discontinued.
Finally Dr Bjorn Oskarsson from the University of California gave the results of a national study of muscle cramps in ALS. 282 ALS participants took part in an online survey and 92% reported cramps and for 20% this was their first symptom. For 62% of respondents cramps were the only source of pain. 36% took prescribed medication which included baclofen and gabapentin, 17% took over the counter remedies and 47% took no treatment. They did not find any correlation between disease duration or ALSFRS and frequency of cramps. 48% reported that their cramps had increased over time while 29% reported a decrease in frequency. Dr Oskarsson concluded that this study highlighted the clinical importance of cramps in ALS and further research on cramp symptom management is in progress in order to develop better guidelines for care.
Overall it was an interesting session with lots to take back to discuss with the team at King’s. Particularly keen to find out when/whether Nuedexta may become available in the UK. A good start to the rest of the symposium.
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