Journal
AMYOTROPHIC LATERAL SCLEROSIS AND FRONTOTEMPORAL DEGENERATION
Volume 19, Issue 1-2, Pages 21-28Publisher
TAYLOR & FRANCIS LTD
DOI: 10.1080/21678421.2017.1386688
Keywords
Motor neuron disease; amyotrophic lateral sclerosis; symptom development; upper motor neuron; lower motor neuron
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Funding
- ALS Foundation Netherlands
- Prinses Beatrix Spierfonds
- European Community's Health Seventh Framework Programme [259867]
- SOPHIA - EU Joint Programme - Neurodegenerative Disease Research, JPND
- Biogen Idec
- Netherlands Organization for Health Research and Development (Veni scheme)
- MQ
- Netherlands Organization for Health Research and Development (Vici scheme)
- Baxalta
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Objective: To investigate whether symptom development in motor neuron disease (MND) is a random or organized process. Methods: Six hundred patients with amyotrophic lateral sclerosis (ALS), upper motor neuron (UMN) or lower motor neuron (LMN) phenotypes were invited for a questionnaire concerning symptom development. A binomial test was used to examine distribution of symptoms from site of onset. Development of symptoms over time was evaluated by Kaplan-Meier analysis. Results: There were 470 respondents (ALS = 254; LMN = 100; UMN = 116). Subsequent symptoms were more often in the contralateral limb following unilateral limb onset (ALS: arms p = 1.05 x 10(-8), legs p < 2.86 x 10(-15); LMN phenotype: arms p = 6.74 x 10(-9), legs p = 6.26 x 10(-6); UMN phenotype: legs p = 4.07 x 10(-14)). In patients with limb onset, symptoms occurred significantly faster in the contralateral limb, followed by the other limbs and lastly by the bulbar region. Patterns of non-contiguous symptom development were also reported: leg symptoms followed bulbar onset in 30%, and bulbar symptoms followed leg onset in 11% of ALS patients. Conclusions: Preferred spread of symptoms from one limb to the contralateral limb, and to adjacent sites appears to be a characteristic of MND phenotypes, suggesting that symptom spread is organized, possibly involving axonal connectivity. Non-contiguous symptom development, however, is not uncommon, and may involve other factors.
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