Quantitative studies of sympathetic ganglia and spinal cord intermedio-lateral gray columns in familial dysautonomia☆
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Cited by (111)
Autonomic failure: Clinicopathologic, physiologic, and genetic aspects
2023, Handbook of Clinical NeurologyFamilial dysautonomia: History, genotype, phenotype and translational research
2017, Progress in NeurobiologyCitation Excerpt :There is scant neuropathological data on efferent sympathetic noradrenergic neurons in FD. There are fewer preganglionic sympathetic neurons in the intermediolateral cell column of the spinal cord (Pearson and Pytel, 1978b), the sympathetic ganglia are reduced to one third the size of normal (Dyck et al., 1978; Pearson and Pytel, 1978b) and sympathetic nerve terminals (in renal biopsy samples) could not be identified (Pearson et al., 1975, 1980). The remaining sympathetic neurons, while reduced in number, are functionally active and are capable of releasing catecholamines and raising blood pressure excessively (Figs. 5 and 7).
Brainstem reflexes in patients with familial dysautonomia
2015, Clinical NeurophysiologyCitation Excerpt :The similar proportion of amplitude decrement for the early and late components, approximately 50%, indicates that both peripheral trigeminal afferents and central pathways within the brainstem are affected in a similar proportion, as occurred with the latencies of these responses. This is in line with pathology samples in FD showing involvement of brainstem reticular formation (Cohen and Solomon, 1955; Brown et al., 1964) and marked reduction in the number of sensory neurons in the trigeminal ganglia (Brown et al., 1964; Aguayo et al., 1971; Pearson et al., 1971, 1975; Pearson and Pytel, 1978b), as well as the decreased sensory nerve action potentials in the limbs (Hilz and Axelrod, 2000). Impairment of the efferent neurons, as suggested by the slightly decreased orbicularis oculi CMAP and masseter EMG amplitudes, is unlikely to fully explain the marked decrease in reflex amplitudes.
Hereditary Sensory and Autonomic Neuropathies
2015, Neuromuscular Disorders of Infancy, Childhood, and Adolescence: A Clinician's ApproachAutonomic and Sensory Disorders
2013, Emery and Rimoin's Principles and Practice of Medical GeneticsFamilial dysautonomia (Riley-Day syndrome): When baroreceptor feedback fails
2012, Autonomic Neuroscience: Basic and ClinicalCitation Excerpt :Even trivial emotions (phone calls, action sequences in films) provoke dramatic increases in blood pressure and heart rate. Despite neuropathological evidence that the sympathetic ganglia are reduced in size (Dyck et al., 1978; Pearson and Pytel, 1978), plasma norepinephrine levels do increase with emotional arousal (Norcliffe-Kaufmann et al., 2010). This suggests that the fewer post-ganglionic sympathetic nerves that do remain (Pearson et al., 1979) are physiologically active and their effects are magnified by denervation supersensitivity.
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This research was funded solely by the members of the Dysautonomia Foundation Inc. to whom the authors wish to express their thanks.