Characteristics of pain syndrome in motor neuron disease

V.V. Kostenko


Background. Motor neuron disease, or amyotrophic lateral sclerosis (ALS), is primarily characterized by progressive motor deficit and various non-motor symptoms. One of such symptoms is pain syndrome, which continues to be poorly understood and often not detected by clinicians. The purpose of our study was to determine the frequency of pain syndrome in the early stages of motor neuron disease and the anatomical localization of pain. Materials and methods. We examined 49 ALS patients aged 27 to 79 years (on average 56.7 years). The ALS diagnosis was based on the revised El Escorial criteria. The visual analog scale was used to determine the intensity of pain, and the ALS functional rating scale (ALSFRS-R) — to monitor disease progression. Results. In this study, 69 % of patients had experienced pain. The presence of pain reported by ALS patients was not sex and age dependent. The most frequent descriptors of pain syndrome reported by the patients were nagging, pulling, throbbing, and burning sensations. The disease progresses statistically slowly in patients without pain (40.4 ± 4.4 points) compared to those with pain sensations (36.5 ± 4.9 points, p = 0.01) according to ALSFRS-R. We found that pain was mainly localized in the upper extremities (38 %), lower extremities (27 %) and lower back (15 %). The presence of pain syndrome depended significantly on the onset of the disease. The risk of pain in patients with the onset in the neck was 1.5 times higher. Patients with bulbar onset rarely complained about pain (4.0 ± 5.5 %). Conclusions. Clinicians should detect pain syndrome in the early stages of motor neuron disease. Since the presence of pain in ALS patients correlates with a more rapid disease progression, therapeutic measures should be adjusted to the presence of such symptom. Pain heterogeneity requires additional study and appropriate planning of rehabilitation.


motor neuron disease; amyotrophic lateral sclerosis; pain syndrome


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