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Imaging of the autonomic nervous system: focus on cardiac sympathetic innervation.

Symptoms or signs of abnormal autonomic nervous system function occur commonly in several neurological disorders. Clinical evaluations have depended on physiological, pharmacological, and neurochemical approaches. Recently, imaging of sympathetic noradrenergic innervation has been introduced and applied especially in the heart. Most studies have used the radiolabeled sympathomimetic amine, (123)I-metaiodobenzylguanidine. Decreased uptake or increased "washout" of (123)I-metaiodobenzylguanidine-derived radioactivity is associated with worse prognosis or more severe disease in hypertension, congestive heart failure, arrhythmias, and diabetes mellitus. This pattern may reflect a high rate of postganglionic sympathetic nerve traffic to the heart. Many recent studies have agreed on the remarkable finding that all patients with Parkinson's disease and orthostatic hypotension have a loss of cardiac sympathetic innervation, whereas all patients with multiple system atrophy, often difficult to distinguish clinically from Parkinson's disease, have intact cardiac sympathetic innervation. Because Parkinson's disease entails a postganglionic sympathetic noradrenergic lesion, the disease appears to be not only a movement disorder, with dopamine loss in the nigrostriatal system of the brain, but also a dysautonomia, with noradrenaline loss in the sympathetic nervous system of the heart. As new ligands are developed, one may predict further discoveries of involvement of components of the autonomic nervous system in neurological diseases.

Adrenergic Fibers↗

Autonomic nervous system evaluation of patients with vasomotor rhinitis.

OBJECTIVE: To demonstrate the utility of quantitative neurological laboratory testing of autonomic nervous system dysfunction and to apply this methodology to further study the relation of chronic vasomotor (nonallergic) rhinitis to the autonomic nervous system. METHODS: It has been suspected that vasomotor rhinitis is due either to a hyperactive parasympathetic nervous system or an imbalance between it and the sympathetic nervous system. The exact relation has not been determined. Recently neurological laboratories have been developed in which a battery of tests can be performed to determine reactivity of the autonomic nervous system. RESULTS: Autonomic nervous system testing was performed on 19 patients with symptoms fulfilling the diagnostic criteria for vasomotor rhinitis and the results were compared with 75 sex- and age-matched control subjects. Patients with vasomotor rhinitis had significant abnormalities of their sudomotor, cardiovagal, and adrenergic subscores. Their composite autonomic scale score was significantly impaired at 2.43, as compared with 0.11 for controls (P < .005). CONCLUSION: Autonomic nervous system dysfunction is significant in patients with vasomotor rhinitis. Possible factors that trigger this dysfunction including nasal trauma and extraesophageal manifestations of gastroesophageal reflux are discussed.

Adult↗

A case of familial atrioventricular heart block affected by alteration of the autonomic nervous system.

Familial atrioventricular heart block affected by the autonomic nervous system has rarely been documented. We describe a 35-year-old man who had first-degree atrioventricular heart block with a PR interval of 0.46 s. He had a family history of 2 members with complete heart block and 1 with documented atrial standstill. The man's P-R interval was shortened by exercise and phenylephrine administration. In the electrophysiolgic study, a "split His" with an H-H' of 220 ms was recognized. Such a case of familial atrioventricular heart block with abnormal His-Purkinje conduction affected by the autonomic nervous system is very rare and worthy of description.

Adult↗

Autonomic nervous system and epilepsy.

Seizures frequently manifest autonomic dysfunction clinically, and seizure discharges commonly spread into and involve autonomic pathways. These associations are direct and simple in some instances, and the result of multiple indirect and complex relationships in others. Effects of epileptic discharge on the autonomic nervous system are mediated through the cortical, limbic, and hypothalamic systems. Some significant consequences of altered autonomic function include convulsive apnea, abnormal sexual function, and potentially fatal effects on the cardiovascular system.

Adult↗

Peripheral and autonomic nervous system involvement in chronic GM2-gangliosidosis.

GM2-gangliosidosis (McKusick 268800 and 272800) is a rare hereditary, progressive disorder of ganglioside metabolism caused by deficiency of lysosomal beta-hexosaminidase (EC 3.2.1.52) activity. It is characterized by severe central nervous system involvement. Involvement of the peripheral and autonomic nervous system has been suspected but rarely documented in published case reports in the chronic form of the disease. Four patients, aged 24-29 years, with chronic GM2-gangliosidosis were examined prospectively for evidence of peripheral and autonomic nervous system dysfunction. All had nerve conduction studies, sympathetic skin responses and cardiac monitoring during the head tilt-table test. Three patients had objective evidence of autonomic dysfunction with abnormal sympathetic nervous skin responses and axonal sensorimotor polyneuropathy. None of the patients had evidence of significant cardiovascular autonomic dysfunction on the head tilt-table test. The peripheral and autonomic nervous system may be involved in patients with chronic GM2-gangliosidosis. In some cases, this may be clinically significant. On the other hand, cardiovascular autonomic instability is apparently not a significant problem in young adult patients with the disease.

Adult↗

Disorders of the autonomic nervous system: Part 1. Pathophysiology and clinical features.

Autonomic dysfunction may result from diseases that affect primarily either the central nervous system or the peripheral autonomic nervous system. The most common pathogenesis of disturbed autonomic function in central nervous system diseases is degeneration of the intermediolateral cell columns (progressive autonomic failure) or disease or damage to descending pathways that synapse on the intermediolateral column cells (spinal cord lesions, cerebrovascular disease, brainstem tumors, multiple sclerosis). The peripheral autonomic nervous system may be damaged in isolation in the acute and subacute autonomic neuropathies or in association with a generalized peripheral neuropathy. The peripheral neuropathies most likely to cause severe autonomic disturbance are those in which small myelinated and unmyelinated fibers are damaged in the baroreflex afferents, the vagal efferents to the heart, and the sympathetic efferent pathways to the mesenteric vascular bed. Acute demyelination of the sympathetic and parasympathetic nerves in the Guillain-Barré syndrome may also cause acute autonomic dysfunction. Although autonomic disturbances may occur in other types of peripheral neuropathy, they are rarely clinically important.

Autonomic Nervous System↗

Autonomic nervous system disorders in stroke.

Disturbances of the autonomic nervous system are common in patients with various cerebrovascular diseases. They are attributed to damage of the central autonomic network, particularly in the frontoparietal cortical areas and in the brain stem, or to a disruption of the autonomic pathways descending from the hypothalamus via the mesencephalon, pons, and medulla to the spinal cord. The most common clinical problems include abnormalities in heart rate and blood pressure regulation, reflecting cardiovascular autonomic dysfunction, and asymmetric sweating with cold hemiplegic limbs, reflecting changes in the sudomotor and vasomotor regulatory systems. Bladder and bowel dysfunction and impotence are also frequent complaints after stroke, but the present knowledge concerning their prevalence and clinical significance is still limited. Cardiovascular autonomic dysfunction, which is mainly related to increased sympathetic activity, is most evident in the acute phase of stroke, whereas other autonomic disorders, such as abnormal sweating, are long-standing or even irreversible. In addition to the well-established sympathetic hyperfunction, abnormalities of the parasympathetic nervous system may also contribute to the autonomic imbalance after stroke. Reliable recognition of autonomic dysfunction using quantitative analysis methods is important, because these disturbances are not only subjectively disabling and uncomfortable, but they may also be prognostically unfavorable. Moreover, quantitative measurements also form the ground for successive treatment of various stroke-related autonomic disorders.

Autonomic Nervous System Diseases↗