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Table 189-2

Classification of Clinical Autonomic Disorders

I. Autonomic Disorders with Brain Involvement
  • Associated with multisystem degeneration
    1. Multisystem degeneration: autonomic failure clinically prominent
      • Multiple system atrophy (MSA)
      • Parkinson's disease with autonomic failure
      • Diffuse Lewy body disease with autonomic failure
    2. Multisystem degeneration: autonomic failure clinically not usually prominent
      • Parkinson's disease without autonomic failure
      • Other extrapyramidal disorders (inherited spinocerebellar atrophies, progressive supranuclear palsy, corticobasal degeneration, Machado-Joseph disease, fragile X syndrome [FXTAS])
  • Unassociated with multisystem degeneration (focal CNS disorders)
    1. Disorders mainly due to cerebral cortex involvement
      • Frontal cortex lesions causing urinary/bowel incontinence
      • Focal seizures (temporal lobe or anterior cingulate)
      • Cerebral infarction of the insula
    2. Disorders of the limbic and paralimbic circuits
      • Shapiro's syndrome (agenesis of corpus callosum, hyperhidrosis, hypothermia)
      • Autonomic seizures
      • Limbic encephalitis
    3. Disorders of the hypothalamus
      • Thiamine deficiency (Wernicke-Korsakoff syndrome)
      • Diencephalic syndrome
      • Neuroleptic malignant syndrome
      • Serotonin syndrome
      • Fatal familial insomnia
      • Antidiuretic hormone (ADH) syndromes (diabetes insipidus, inappropriate ADH secretion)
      • Disturbances of temperature regulation (hyperthermia, hypothermia)
      • Disturbances of sexual function
      • Disturbances of appetite
      • Disturbances of BP/HR and gastric function
      • Horner's syndrome
    4. Disorders of the brainstem and cerebellum
      • Posterior fossa tumors
      • Syringobulbia and Arnold-Chiari malformation
      • Disorders of BP control (hypertension, hypotension)
      • Cardiac arrhythmias
      • Central sleep apnea
      • Baroreflex failure
      • Horner's syndrome
      • Vertebrobasilar and lateral medullary (Wallenberg's) syndromes
      • Brainstem encephalitis
II. Autonomic Disorders with Spinal Cord Involvement
  • Traumatic quadriplegia
  • Syringomyelia
  • Subacute combined degeneration
  • Multiple sclerosis and neuromyelitis optica
  • Amyotrophic lateral sclerosis
  • Tetanus
  • Stiff-person syndrome
  • Spinal cord tumors
III. Autonomic Neuropathies
  • Acute/subacute autonomic neuropathies
    • Subacute autoimmune autonomic ganglionopathy (AAG)
    • Subacute paraneoplastic autonomic neuropathy
    • Guillain-Barré syndrome
    • Botulism
    • Porphyria
    • Drug induced autonomic neuropathies-stimulants, drug withdrawal, vasoconstrictor, vasodilators, beta-receptor antagonists, beta-agonists
    • Toxin-induced autonomic neuropathies
    • Subacute cholinergic neuropathy
  • Chronic peripheral autonomic neuropathies
    1. Distal small fiber neuropathy
    2. Combined sympathetic and parasympathetic failure
      • Amyloid
      • Diabetic autonomic neuropathy
      • AAG (paraneoplastic and idiopathic)
      • Sensory neuronopathy with autonomic failure
      • Familial dysautonomia (Riley-Day syndrome)
      • Diabetic, uremic, or nutritional deficiency
      • Geriatric dysautonomia (age >80 years)
    3. Disorders of orthostatic intolerance: reflex syncope; POTS; prolonged bed rest; space flight; chronic fatigue

Abbreviations: BP, blood pressure; CNS, central nervous system; HR, heart rate; POTS, postural orthostatic tachycardia syndrome.