Pure Cholinergic or Adrenergic Disorders

Cholinergic dysautonomias

Associated with neuromuseular transmission defect Botulism (acute)

Lambert-Eaton myasthenic syndrome (chronic)

Not associated with neuromuscular transmission defect

Acute cholinergic dysautonomia

Chronic idiopathic anhidrosis

Adie's syndrome

Chagas' disease

Adrenergie dysautonomias

Pure adrenergic neuropathy

Dopamine-beta-hydroxylase deficiency children. Its clinical features include insensitivity to pain and temperature, absence of tears, hypoactive corneal and tendon reflexes, and absence of fungiform papillae of the tongue. Patients with familial dysautonomia have poor suck and feeding responses, esophageal reflux with vomiting and aspiration, uncoordinated swallowing, episodic hyperhidrosis, vasomotor instability, postural hypotension, hypertensive crises, supersensitivity to cholinergic and adrenergic agents, and absent histamine flare. 39]

Fabry's disease is an X-linked recessive disorder caused by a deficiency of alpha-galactosidase A (see Chapter 30 ). Its manifestations include painful distal peripheral neuropathy, a truncal reddish-purple macular papular rash, and angiectases of the skin, conjunctiva, nail bed, and oral mucosa, progressive renal disease, corneal opacities, and cerebrovascular accidents. The autonomic manifestations include hypohidrosis or anhidrosis, reduced saliva and tear formation, impaired histamine flare, and gastrointestinal dysmotility. y

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