Pure Cholinergic or Adrenergic Disorders

Cholinergic Dysautonomias. Acute or chronic cholinergic dysautonomias may occur in isolation or in patients with impaired neuromuscular transmission. In the Lambert-Eaton myasthenic syndrome (see Ch§p!ei.50. ) and in botulism (see Chapter39 ), acetylcholine release from motor as well as autonomic neurons fails. Thus, neuromuscular and autonomic cholinergic neurotransmission is impaired, and muscle weakness, reflex loss, and autonomic failure result. y

Chronic disorders that affect cholinergic function without neuromuscular impairment include Adie's syndrome, chronic idiopathic anhidrosis, Ross's syndrome (a combination of Adie's pupils and segmental anhidrosis), and Chagas' disease, a disease caused by Trypanosoma cruzi that affects the neurons of the parasympathetic ganglion and produces megaesophagus, megacolon, and cardiomyopathy (see Ch.a.P.t®I..4.2 ).

Pure Adrenergic Neuropathy. Dopamine-beta hydroxylase deficiency is a hereditary disease characterized by an inability to convert dopamine (DA) to NE. This disorder is characterized by severe orthostatic hypotension accompanied by ptosis, ejaculatory failure, nocturia, nasal congestion, and hyperextensible joints. Typically, the serum NE/dA ratio is 0.1 (normal 10). y

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