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New autonomic and sensory neuropathy with loss of adrenergic sympathetic function and sensory neuropeptides. 1991

P Anand, and P Rudge, and C J Mathias, and D R Springall, and M A Ghatei, and M Naher-Noe, and M Sharief, and V P Misra, and J M Polak, and S R Bloom
Department of Neurology, National Hospital for Neurology and Neurosurgery, London, UK.

A 30-year-old woman with longstanding dizziness was found to have a severe postural fall in blood pressure and a reduced skin axon-reflex flare response. Autonomic tests indicated selective impairment of adrenergic sympathetic function. Plasma noradrenaline, adrenaline, dopamine, and dopamine beta hydroxylase were undetectable. Skin biopsy specimens showed loss of tyrosine hydroxylase and neuropeptide Y (markers of adrenergic sympathetic fibres) and of substance P and calcitonin gene-related peptide (sensory neuropeptides). A sural nerve biopsy specimen showed severe depletion of unmyelinated fibres. The constellation of losses were compatible with nerve growth factor (NGF) deprivation, which was confirmed on assay. This new syndrome may be explained by loss of trophic action of NGF.

UI MeSH Term Description Entries
D009414 Nerve Growth Factors Factors which enhance the growth potentialities of sensory and sympathetic nerve cells. Neurite Outgrowth Factor,Neurite Outgrowth Factors,Neuronal Growth-Associated Protein,Neuronotrophic Factor,Neurotrophic Factor,Neurotrophic Factors,Neurotrophin,Neurotrophins,Growth-Associated Proteins, Neuronal,Neuronal Growth-Associated Proteins,Neuronotrophic Factors,Neurotrophic Protein,Neurotrophic Proteins,Proteins, Neuronal Growth-Associated,Factor, Neurite Outgrowth,Factor, Neuronotrophic,Factor, Neurotrophic,Factors, Nerve Growth,Factors, Neurite Outgrowth,Factors, Neuronotrophic,Factors, Neurotrophic,Growth Associated Proteins, Neuronal,Growth-Associated Protein, Neuronal,Neuronal Growth Associated Protein,Neuronal Growth Associated Proteins,Outgrowth Factor, Neurite,Outgrowth Factors, Neurite,Protein, Neuronal Growth-Associated
D010523 Peripheral Nervous System Diseases Diseases of the peripheral nerves external to the brain and spinal cord, which includes diseases of the nerve roots, ganglia, plexi, autonomic nerves, sensory nerves, and motor nerves. Peripheral Nerve Diseases,Peripheral Neuropathies,PNS (Peripheral Nervous System) Diseases,PNS Diseases,Peripheral Nervous System Disease,Peripheral Nervous System Disorders,Nerve Disease, Peripheral,Nerve Diseases, Peripheral,Neuropathy, Peripheral,PNS Disease,Peripheral Nerve Disease,Peripheral Neuropathy
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000320 Adrenergic Fibers Nerve fibers liberating catecholamines at a synapse after an impulse. Sympathetic Fibers,Adrenergic Fiber,Fiber, Adrenergic,Fiber, Sympathetic,Fibers, Adrenergic,Fibers, Sympathetic,Sympathetic Fiber
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D001342 Autonomic Nervous System Diseases Diseases of the AUTONOMIC NERVOUS SYSTEM, including sympathetic, parasympathetic, and enteric nervous systems. Autonomic Disorders,Central Autonomic Nervous System Diseases,Disorders of the Autonomic Nervous System,Dysautonomia,Nervous System Diseases, Autonomic,Nervous System Diseases, Parasympathetic,Nervous System Diseases, Sympathetic,Non-Familial Dysautonomia,Parasympathetic Nervous System Diseases,Peripheral Autonomic Nervous System Diseases,Sympathetic Nervous System Diseases,ANS (Autonomic Nervous System) Diseases,ANS Diseases,Autonomic Central Nervous System Diseases,Autonomic Diseases,Autonomic Nervous System Disorders,Autonomic Peripheral Nervous System Diseases,Segmental Autonomic Dysfunction,ANS Disease,Autonomic Disease,Autonomic Disorder,Autonomic Dysfunction, Segmental,Autonomic Dysfunctions, Segmental,Disorder, Autonomic,Dysautonomia, Non-Familial,Dysautonomias,Non Familial Dysautonomia,Non-Familial Dysautonomias,Segmental Autonomic Dysfunctions
D013497 Sural Nerve A branch of the tibial nerve which supplies sensory innervation to parts of the lower leg and foot. Nerve, Sural,Nerves, Sural,Sural Nerves
D013577 Syndrome A characteristic symptom complex. Symptom Cluster,Cluster, Symptom,Clusters, Symptom,Symptom Clusters,Syndromes

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