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 × 10-8, legs p < 2.86 × 10-15; LMN phenotype: arms p = 6.74 × 10-9, legs p = 6.26 × 10-6; UMN phenotype: legs p = 4.07 × 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.
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 × 10-8, legs p < 2.86 × 10-15; LMN phenotype: arms p = 6.74 × 10-9, legs p = 6.26 × 10-6; UMN phenotype: legs p = 4.07 × 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 ALSpatients. 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.
Entities:
Keywords:
Motor neuron disease; amyotrophic lateral sclerosis; lower motor neuron; symptom development; upper motor neuron
Authors: Kate L Osman; Sabrina Kohlberg; Alexis Mok; Ryan Brooks; Lori A Lind; Katelyn McCormack; Andries Ferreira; Matan Kadosh; Mary K Fagan; Elizabeth Bearce; Nicole L Nichols; Joan R Coates; Teresa E Lever Journal: Dysphagia Date: 2019-07-12 Impact factor: 3.438
Authors: Sudarshan Ragunathan; Laura C Bell; Natenael Semmineh; Ashley M Stokes; Jeremy M Shefner; Robert Bowser; Shafeeq Ladha; C Chad Quarles Journal: Tomography Date: 2021-05-05