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Spinal Cord Disease
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
Progressive bulbar palsy: about 10% of patients present in this manner and most progress to classical MND. Incidence of bulbar-onset disease increases with age (>40% of patients are over the age of 70 years).
Station 2: History Taking
Published in Saira Ghafur, Parminder K Judge, Richard Kitchen, Samuel Blows, Fiona Moss, The MRCP PACES Handbook, 2017
Saira Ghafur, Parminder K Judge, Richard Kitchen, Samuel Blows, Fiona Moss
Progressive bulbar palsy Only lower cranial nerves affected (IX, X, XII).‘Donald Duck’/nasal speech.Weakness of palatal muscles results in swallowing difficulties.
The epidemiology of amyotrophic lateral sclerosis in Moscow (Russia)
Published in Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 2020
Lev Brylev, Anastasia Ataulina, Vera Fominykh, Vadim Parshikov, Anna Vorobyeva, Elena Istomina, Rafiz Shikhirimov, Alexandr Salikov, Mariya Zakharova, Alla Guekht, Ettore Beghi
The incidence of ALS in the Russian Federation (1.25/100,000/year) is lower than in several other world countries (2) but is comparable to Estonia (1.27 [0.92–1.63]) (7), a nearby country with a partly overlapping ethnic structure, as the citizens of slavic origin constitute about 35% of the entire population (8). In that study, multiple sources were investigated and, along with ALS, other motor neuron diseases were included (progressive bulbar palsy and progressive muscular atrophy). Our lower estimates, when compared to most published reports, can be in part the result of poor diagnostic ascertainment, especially in the elderly population. The incidence of ALS in Europe, where several prospective population-based registries have been activated in the last 20 years, was 2.16 per 100,000 person years (95% CI 2.0–2.3), with similar rates across all registries (9).
An epidemiological profile of dysarthria incidence and assistive technology use in the living population of people with MND in Scotland
Published in Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 2020
Elizabeth Elliott, Judith Newton, Phillipa Rewaj, Jenna M. Gregory, Lynda Tomarelli, Shuna Colville, Siddharthan Chandran, Suvankar Pal
Motor neurone disease (MND) collectively describes a group of progressively fatal muscle wasting conditions caused by the selective degeneration of brain and spinal cord motor neurones. Amyotrophic lateral sclerosis (ALS) is the commonest form and the median survival time for people with ALS from diagnosis is less than 2 years (1). Additional subtypes of the disease include; progressive bulbar palsy (PBP) which primarily affects the muscles of speech and swallowing, lower motor neurone predominant progressive muscular atrophy (PMA), and upper motor neurone predominant primary lateral sclerosis (PLS) (2). It is estimated that cognitive impairment affects 50% of pwMND and the criteria for co-morbid dementia in MND-Frontotemporal dementia (MND-FTD) is met in 10–15% of cases (3,4).
Tracheostomy ventilation in motor neurone disease: a snapshot of UK practice
Published in Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 2022
Jonathan Palmer, Ben Messer, Michelle Ramsay
Median survival for patients who were not weaned and continued TV was 24 months (range 1–60) post intubation. For those eight patients extubated and successfully weaned onto NIV, the median survival post decannulation was 37 months (range 7–46). Only one of these patients had a diagnosis of progressive bulbar palsy, the remainder had ALS. For patients who chose elective tracheostomy, an increase in survival of 10 months was demonstrated compared to those receiving emergency TV with known MND (34 vs. 24 months). This was further improved when compared to those who received emergency tracheostomy before a formal diagnosis of MND (34 vs. 22 months).