Cardiac sympathetic function in the patients with amyotrophic lateral sclerosis: analysis using cardiac [123I] MIBG scintigraphy
- 312 Downloads
Amyotrophic lateral sclerosis (ALS), which is the most serious form of degenerative motor neuron disease in adults, is characterized by upper and lower motor neuron degeneration, skeletal muscle atrophy, paralysis, and death. Some patients with respiratory-dependent ALS die of sudden cardiac arrest or anoxic encephalopathy following circulatory collapse, which may be associated with sympathetic hyperactivity. Cardiac [123I] MIBG scintigraphy is a diagnostic method of cardiac sympathetic function. However, few reports have addressed cardiac sympathetic function in ALS patients using this technique. We investigated cardiac sympathetic function in 63 ALS patients and 10 healthy volunteers using cardiac [123I] metaiodobenzylguanidine (MIBG) scintigraphy [heart/mediastinum ratio (H/M ratio) in the early phase and washout ratio (WR)] at the time of diagnosis. The WR of cardiac [123I] MIBG scintigraphy, which indicates cardiac sympathetic activity, was significantly increased in ALS patients compared with controls. ALS patients with an increased WR exhibited a significantly higher progression rate compared with those with normal WR. Moreover, the survival of ALS patients with increased WR was significantly decreased compared with those with normal WR. These results suggested that some patients with ALS have sympathetic hyperactivity at the time of diagnosis. ALS patients may suffer from chronic cardiac sympathetic hyperactivity, which is associated with sudden cardiac death and stress induced cardiomyopathy. Increased WR in cardiac [123I] MIBG scintigraphy may be a predictive factor in ALS patients.
KeywordsAmyotrophic lateral sclerosis Cardiac sympathetic function Cardiac sympathetic hyperactivity [123I]-Metaiodobenzylguanidine (MIBG) scintigraphy
Conflicts of interest
The authors state that they have no conflicts of interest.
- 4.Hayashi H (1994) Long-term in-hospital ventilatory care for patients with ALS. In: Mitsumoto H, Norris FH (eds) Amyotrophic lateral sclerosis: a comprehensive guide to management. Demos publications, New York, pp 127–138Google Scholar
- 13.Brooks BR (1994) El Escorial World Federation of Neurology criteria for the diagnosis of amyotrophic lateral sclerosis. Subcommittee on motor neuron diseases/amyotrophic lateral sclerosis of the world federation of neurology research group on neuromuscular diseases and the El Escorial “clinical limits of amyotrophic lateral sclerosis” workshop contributors. J Neurol Sci 124(Suppl):96–107PubMedCrossRefGoogle Scholar
- 18.Litchy WJ, Low PA, Daube JR, Windebank AJ (1987) Autonomic abnormalities in amyotrophic lateral sclerosis. Neurology 37(Suppl I):162Google Scholar
- 21.Tamura N, Shimazu K, Yamamoto T, Watanabe S, Onoda A, Itokawa K, Hamaguchi K (1991) A supplementary study of sympathetic nervous hypertension in motor neuron disease. Auton Nerv Syst 28:357–363 (in Japanese, abstract in English)Google Scholar
- 23.Low PA, McLeod JG (1993) The autonomic neuropathies. In: Low PA (ed) Clinical autonomic disorders: evaluation and management. Little Brown, Boston, pp 395–421Google Scholar
- 32.Morimoto S, Terada K, Keira N, Satoda M, Inoue K, Tatsukawa H, Katoh S, Ida K, Sugihara H, Takeda K, Nakagawa M (1996) Investigation of the relationship between regression of hypertensive cardiac hypertrophy and improvement of cardiac sympathetic nervous dysfunction using iodine-123 metaiodobenzylguanidine myocardial imaging. Eur J Nucl Med 23:756–761PubMedCrossRefGoogle Scholar
- 46.Tanaka Y, Yoshikura N, Harada N, Yamada M, Koumura A, Sakurai T, Hayashi Y, Kimura A, Hozumi I, Inuzuka T (2012) Late-onset patients with sporadic amyotrophic lateral sclerosis in Japan have a higher progression rate of ALSFRS-R at the time of diagnosis. Intern Med 51:579–584PubMedCrossRefGoogle Scholar