Hebatallah R Rashed, Nathan P Staff, Margherita Milone, Michelle L Mauermann, Sarah Berini, William P Cheshire, Elizabeth A Coon, Robert D Fealey, Eric Sorenson, Jeremy Cutsforth-Gregory, Eduardo E Benarroch, Paola Sandroni, Phillip A Low, Wolfgang Singer, Kamal Shouman
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引用次数: 0
Abstract
Purpose: Prior studies reported evidence of autonomic involvement in motor neuron disease and suggested more severe dysfunction in upper motor neuron predominant syndromes. Hence, we sought to characterize autonomic impairment in primary lateral sclerosis.
Methods: Neurological evaluations, thermoregulatory sweat tests, and autonomic reflex screens were analyzed retrospectively in 34 primary lateral sclerosis patients (28 definite and 6 probable). Patients with other potential causes of autonomic failure and patients with autonomic testing results compromised by artifact were excluded.
Results: A total of 17 patients reported autonomic symptoms. Orthostatic lightheadedness was most frequent (8 patients), followed by bladder (7), bowel (5), and erectile dysfunction (3). The autonomic reflex screens of 33 patients were reviewed; 20 patients had abnormal studies. The thermoregulatory sweat tests of 19 patients were reviewed; 11 patients had abnormal studies. Composite Autonomic Severity Score was calculated for 33 patients and found abnormal in 20/33 patients (60.6%): 15/20 patients (75%) had mild impairment, and 5/20 patients (25%) had moderate impairment. The frequencies of testing abnormalities were: sudomotor 18/20 (90%), cardiovagal 9/20 (45%), and adrenergic 6/20 (30%). Sweat loss pattern analysis showed global, regional, and mixed patterns to be more common than length-dependent and distal patterns.
Conclusion: We found evidence of frequent autonomic dysfunction in primary lateral sclerosis, which is generally of modest severity akin to prior reports for amyotrophic lateral sclerosis, but more commonly in a pattern consistent with preganglionic/ganglionic localization. This suggests that primary lateral sclerosis, as with amyotrophic lateral sclerosis, is a multisystem disease that affects the autonomic nervous system.
期刊介绍：
Clinical Autonomic Research aims to draw together and disseminate research work from various disciplines and specialties dealing with clinical problems resulting from autonomic dysfunction. Areas to be covered include: cardiovascular system, neurology, diabetes, endocrinology, urology, pain disorders, ophthalmology, gastroenterology, toxicology and clinical pharmacology, skin infectious diseases, renal disease.
This journal is an essential source of new information for everyone working in areas involving the autonomic nervous system. A major feature of Clinical Autonomic Research is its speed of publication coupled with the highest refereeing standards.
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