Journal article
Demographics and clinical characteristics of primary lateral sclerosis: case series and a review of literature
Neurodegenerative disease management, v 8(1), pp 17-23
01 Feb 2018
PMID: 29316850
Featured in Collection : UN Sustainable Development Goals @ Drexel
Abstract
Aim: Primary lateral sclerosis (PLS) is a form of motor neuron disease involving only upper motor neurons. In some patients presenting as PLS, the disease progresses to involve lower motor neurons and thereby converting to amyotrophic lateral sclerosis (ALS). However, pure forms of PLS do exist. Our aim was to study epidemiological and clinical characteristics of pure PLS patients treated at our neuromuscular clinic. Methods: We retrospectively reviewed 15 patients from July 2011 to October 2014 with PLS treated at the neuromuscular disorder clinic at our hospital. Data collection included patient demographics, age and site of onset, duration of symptoms and duration of follow-up. We also studied clinical features such as bulbar involvement; pseudobulbar affect; depression; spasms/pain; bladder involvement; diagnostic work up, in other words, MRI; brain/electromyography findings; clinical course, namely years to wheelchair; and need for gastrostomy tube requirement baclofen pump placement. We also tried to find a correlation between PLS and environmental factors such as urban/suburban/rural living, consumption of well water, socioeconomic status/occupation and history of trauma. Results: Male-to-female ratio was 1: 2, mean age at onset of symptoms was 58.6 years, with the oldest patient being an 84-year-old female at the time of onset of symptoms. Mean duration of follow-up was 51 months. Mean duration of symptoms was 77.4 months. About eight (53%) patients presented with bulbar symptoms in the form of spastic speech and dysphagia, pseudobulbar affect, developed depression and had bladder involvement. Seven (47%) patients presented with symmetric spasticity in the extremities. A third of the patients required baclofen for spasticity and a third required gastrostomy tube placement for dysphagia. None of them had abnormal neuroimaging or electrodiagnostic testing. Only one patient had history of trauma. About half of the patients were from lower socioeconomic status as well as middle class. One of the patients had consumed well water during younger years and three (20%) patients lived in the rural area. Conclusion: Though on review of literature there is no clear consensus about the existence of PLS as a distinct disease entity, we believe that there are rare cases of motor neuron disease with progressive upper motor neuron symptoms that throughout their course never convert to ALS. Our series highlights the demographic and clinical features of these patients and underscores the longer survival of these patients when compared with ALS.
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Details
- Title
- Demographics and clinical characteristics of primary lateral sclerosis: case series and a review of literature
- Creators
- Ramnath Santosh Ramanathan - Neurology, IncSandeep Rana - Allegheny Health Network
- Publication Details
- Neurodegenerative disease management, v 8(1), pp 17-23
- Publisher
- Future Medicine Ltd
- Number of pages
- 7
- Resource Type
- Journal article
- Language
- English
- Academic Unit
- Neurology
- Web of Science ID
- WOS:000427354600005
- Scopus ID
- 2-s2.0-85058732542
- Other Identifier
- 991021958689004721
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- Collaboration types
- Domestic collaboration
- Web of Science research areas
- Clinical Neurology