Lateral horn cells in progressive autonomic failure

doi:10.1016/0022-510X(80)90064-7

Journal of the Neurological Sciences

Volume 46, Issue 3, June 1980, Pages 393-404

https://doi.org/10.1016/0022-510X(80)90064-7 Get rights and content

Abstract

Estimates of cell loss in the intermediolateral columns of the thoracic cord in cases of progressive autonomic failure were made by counting nerve cells in the lateral grey horns from 21 such cases, and comparing (a) with “normal” controls, (b) with 3 cases of uncomplicated Parkinson's disease and (c) with 3 cases of multiple system atrophy without overt autonomic failure. It was found that all cases of autonomic failure had suffered severe cell loss; on average three quarters of their lateral horn cells were lost. The cases of uncomplicated Parkinson's disease did not differ from the controls; but the cases of multiple system atrophy without autonomic disturbances had lost about half their lateral horn cells. These findings are tabulated, and discussed.

References (19)

D.J. Evans et al.
Idiopathic orthostatic hypotension
J. neurol. Sci.
(1972)
M. Gross et al.
Orthostatic hypotension in Parkinson's disease
Lancet
(1972)
E.G.S. Spokes et al.
Multiple system atrophy with autonomic failure — Clinical, histological and neurochemical observations on four cases
J. neurol. Sci.
(1979)
M.J. Aminoff et al.
Assessment of autonomic function in patients with a parkinsonian syndrome
Brit. med. J.
(1971)
R. Bannister et al.
Degenerative diseases of the nervous system associated with autonomic failure
Brain
(1972)
J.G. Graham et al.
Orthostatic hypotension and nicotine sensitivity in a case of multiple system atrophy
J. Neurol. Neurosurg. Psychiat.
(1969)
R.C. Hughes et al.
Primary neurogenic orthostatic hypotension
J. Neurol. Neurosurg. Psychiat.
(1970)
K. Jellinger et al.
Die ZNS-Veränderungen bei den olivo-ponto-cerebellren Atrophien
Z. Neurol.
(1971)
R.H. Johnson et al.
Autonomic failure with orthostatic hypotension due to intermediolateral column degeneration
Quart. J. Med.
(1966)

There are more references available in the full text version of this article.

Cited by (124)

Autonomic failure: Clinicopathologic, physiologic, and genetic aspects
2023, Handbook of Clinical Neurology
Over the past century, generations of neuroscientists, pathologists, and clinicians have elucidated the underlying causes of autonomic failure found in neurodegenerative, inherited, and antibody-mediated autoimmune disorders, each with pathognomonic clinicopathologic features. Autonomic failure affects central autonomic nervous system components in the α-synucleinopathy, multiple system atrophy, characterized clinically by levodopa-unresponsive parkinsonism or cerebellar ataxia, and pathologically by argyrophilic glial cytoplasmic inclusions (GCIs). Two other central neurodegenerative disorders, pure autonomic failure characterized clinically by deficits in norepinephrine synthesis and release from peripheral sympathetic nerve terminals; and Parkinson's disease, with early and widespread autonomic deficits independent of the loss of striatal dopamine terminals, both express Lewy pathology. The rare congenital disorder, hereditary sensory, and autonomic neuropathy type III (or Riley–Day, familial dysautonomia) causes life-threatening autonomic failure due to a genetic mutation that results in loss of functioning baroreceptors, effectively separating afferent mechanosensing neurons from the brain. Autoimmune autonomic ganglionopathy caused by autoantibodies targeting ganglionic α3-acetylcholine receptors instead presents with subacute isolated autonomic failure affecting sympathetic, parasympathetic, and enteric nervous system function in various combinations. This chapter is an overview of these major autonomic disorders with an emphasis on their historical background, neuropathological features, etiopathogenesis, diagnosis, and treatment.
Impaired peripheral vasoconstrictor response to orthostatic stress in patients with multiple system atrophy
2015, Parkinsonism and Related Disorders
Citation Excerpt :
Conversely, neurogenic OH is seen when peripheral vasoconstriction fails to occur under orthostatic stress [7]. This is thought to be related primarily to the loss of sympathetic preganglionic neurons in the intermediolateral column of the thoracolumbar spinal cord in patients with MSA [8–10]. Thus, it is assumed that many patients with MSA have impaired vasoconstrictor response to orthostatic stress, and these conditions increase the risk for OH.
Most patients with multiple system atrophy (MSA) develop autonomic dysfunction; however, orthostatic hypotension is not always present. Failure of the vasoconstrictor response is thought to be responsible for orthostatic hypotension, but the degree of impairment of this response in patients with MSA is unclear. We assessed autonomic function in patients with MSA by evaluating the vasoconstrictive response during a head-up tilt test and determining its relationship to orthostatic hypotension. As an additional examination, the efficacy of norepinephrine in treating orthostatic hypotension was also assessed.
The study included 82 patients with MSA and 28 controls. Measures of total peripheral resistance were obtained during a head-up tilt test. Norepinephrine was administered to the patients lacking a vasoconstrictive response to evaluate its ability to treat orthostatic hypotension.
At a 60° tilt, orthostatic hypotension occurred in 47.6% of the patients and 0% of controls. Reduction in total peripheral resistance from baseline at a 60° tilt was observed in 69.5% of the patients and 0% of controls. In patients with MSA, changes in systolic blood pressure from the baseline at a 60° tilt correlated positively with changes in the total peripheral resistance (r = 0.69, p < 0.0001). Norepinephrine prevented the reduction of total peripheral resistance and development of orthostatic hypotension.
A large number of patients with MSA with and without orthostatic hypotension have an impaired peripheral vasoconstrictive response, suggesting a high frequency of cardiovascular dysautonomia with an associated risk of developing orthostatic hypotension. A norepinephrine infusion was effective for treating orthostatic hypotension.
Central control of autonomic function and involvement in neurodegenerative disorders
2013, Handbook of Clinical Neurology
The central autonomic network includes the insular cortex, anterior cingulate cortex, amygdala, hypothalamus, periaqueductal gray, parabrachial nucleus, nucleus of the solitary tract, ventrolateral reticular formation of the medulla, and medullary raphe. These areas: are reciprocally interconnected; receive converging visceral and somatosensory information; generate stimulus-specific patterns of autonomic, endocrine, and motor responses; and are regulated according to the behavioral state, including the sleep–wake cycle. Several components of the central autonomic networks are affected in neurodegenerative disorders characterized by the presence of intracellular inclusions containing α-synuclein. These include multiple system atrophy (MSA), characterized by accumulation of glial cytoplasmic inclusions, and Lewy body disorders, including Parkinson disease (PD), dementia with Lewy bodies, and the so-called “pure” autonomic failure. In MSA, involvement of the rostral ventrolateral medulla may be primarily responsible for orthostatic hypotension; involvement in the pontine micturition area, sacral preganglionic nucleus, and Onuf nucleus is responsible for neurogenic bladder; and involvement of the pre-Bötzinger complex and medullary raphe may contribute to sleep-related respiratory abnormalities. In contrast, Lewy body disorders are characterized by early involvement of the enteric nervous system and cardiac sympathetic ganglia. The dorsal motor nucleus of the vagus is affected both in MSA and at early stages of PD.
Source and origin of nerve fibres immunoreactive for substance P and calcitonin gene-related peptide in the normal and chronically denervated superior cervical sympathetic ganglion of the rat
2013, Autonomic Neuroscience: Basic and Clinical
Citation Excerpt :
In MSA, the lesion resides within the central nervous system and involves the neural connexions responsible for baroreflex modulation of autonomic tone. There is loss of preganglionic neurones and consequent decentralization of ganglionic neurones (Low et al., 1978; Oppenheimer, 1980). MSA patients have normal or only slightly reduced supine plasma norepinephrine concentrations (Biaggioni et al., 1993, 1994) and intact noradrenergic innervation to the heart (Goldstein et al., 1997).
Immunohistochemical studies of sympathetic ganglia have indicated that the normal rat superior cervical ganglion contains both SP-IR and CGRP-IR fibres, and CGRP- and SP-immunoreactivity coexist in some fibres. In rat sympathetic ganglia decentralization by preganglionic denervation leads to intraganglionic increase of peptidergic fibres immunoreactive (IR) for substance P (SP) and calcitonin gene-related peptide. We explored the sources of SP- and CGRP-IR fibres in normal and in chronically decentralized rat SCGs.
The distribution of immunoreactivities for CGRP and SP was determined in SCGs of normal rats and of rats following preganglionic denervation followed by sensory denervation. Ganglia were studied after short-term (2–5 days) sensory denervation, and long-term (7–16 months) sympathetic denervation followed by short-term (2 days) sensory denervation. To explore for the production of SP and CGRP by intrinsic neurones within the ganglion, normal and chronically decentralized SCGs were examined following pretreatment by local in vivo application of colchicine. Normal and chronically decentralized ganglia were also injected with fluorescent tracer Fluorogold for retrograde tracing of extrinsic fibres back to their neurones of origin.
The observations suggest that in normal SCG in the rat the SP-IR and CGRP-IR nerve fibres are derived via direct links from vagus and glossopharyngeal nerves and the cervical plexus, or from nerve fibres running along the cervical sympathetic trunk, and the external carotid and the internal carotid nerves.
Sensory nerve inputs to the rat SCG following decentralization may contribute to the low levels of ganglionic activation observable in the autonomic failure of multiple system atrophy in man.
Exercise-induced hypotension in autonomic disorders
2012, Autonomic Neuroscience: Basic and Clinical
The autonomic nervous system closely integrates a range of vital processes, including, cardiovascular function. Physical activity, or exercise, requires a range of integrated autonomic and cardiovascular adjustments in order to maintain homeostasis. Pathological conditions that result in dysfunction of the autonomic nervous system, such as autonomic failure, can therefore jeopardize the control of blood pressure resulting in hypotension and have serious implications for health. Exercise induced hypotension, defined as a ≥ 10 mm Hg fall in systolic blood pressure during exercise, can be a significant symptom/event for autonomic failure, as well as other autonomic disorder, patients, including, Multiple System Atrophy and Spinal Cord Injury, and can reduce physical capacity, orthostatic tolerance, result in falls, complicate management and reduce quality of life. The likely mechanisms do not appear to be an altered cardiac output or active muscle vasodilation response to exercise, but rather, a lack of and/or a blunted increase in sympathetic nerve activity during exercise and/or excessive vasodilation in the splanchnic circulation. The severity of exercise induced hypotension seems to be higher during dynamic relative to static exercise. The possible management strategies for exercise induced hypotension include both non-pharmacological, e.g., reducing risk factors, and pharmacological measures, such as sympathomimetics, but studies on other pharmacological agents are limited.
REM behaviour disorder and neurodegenerative diseases
2011, Sleep Medicine
Rapid-eye movement (REM) sleep behaviour disorder (RBD) is an REM sleep parasomnia characterized by enactment of dream content during REM sleep associated with loss of muscle atonia. RBD can be either idiopathic or secondary to drugs or other diseases. The best recognized association is with neurodegenerative diseases, namely alpha-synucleinopathies. RBD may represent the first feature of neurodegeneration and can be considered an early marker of these disorders. This review describes the main clinical, pathogenetic, and therapeutic features of RBD, pointing to its association with neurodegenerative diseases and emphasizing the clinical and prognostic implications.

View all citing articles on Scopus

View full text

Lateral horn cells in progressive autonomic failure

Abstract

J. neurol. Sci.

Lancet

J. neurol. Sci.

Assessment of autonomic function in patients with a parkinsonian syndrome

Brit. med. J.

Degenerative diseases of the nervous system associated with autonomic failure

Brain

Orthostatic hypotension and nicotine sensitivity in a case of multiple system atrophy

J. Neurol. Neurosurg. Psychiat.

Primary neurogenic orthostatic hypotension

J. Neurol. Neurosurg. Psychiat.

Die ZNS-Veränderungen bei den olivo-ponto-cerebellren Atrophien

Z. Neurol.

Autonomic failure with orthostatic hypotension due to intermediolateral column degeneration

Quart. J. Med.