section name header

Table 186-2

Classification of Clinical Autonomic Disorders

  1. Autonomic disorders with brain involvement
    1. Associated with multisystem degeneration
      1. Multisystem degeneration: autonomic failure clinically prominent
        1. Multiple system atrophy
        2. Parkinson's disease with autonomic failure
        3. Diffuse Lewy body disease (some cases)
      2. Multisystem degeneration: autonomic failure clinically not usually prominent
        1. Parkinson's disease
        2. Other extrapyramidal disorders (inherited spinocerebellar atrophies, progressive supranuclear palsy, corticobasal degeneration, Machado-Joseph disease, FXTAS)
    2. Unassociated with multisystem degeneration (focal CNS disorders)
      1. Disorders mainly due to cerebral cortex involvement
        1. Frontal cortex lesions causing urinary/bowel incontinence
        2. Focal seizures (temporal lobe or anterior cingulate)
        3. Cerebral infarction of the insula
      2. Disorders of the limbic and paralimbic circuits
        1. Shapiro's syndrome (agenesis of corpus callosum, hyperhidrosis, hypothermia)
        2. Autonomic seizures
        3. Limbic encephalitis
      3. Disorders of the hypothalamus
        1. Thiamine deficiency (Wernicke-Korsakoff syndrome)
        2. Diencephalic syndrome
        3. Neuroleptic malignant syndrome
        4. Serotonin syndrome
        5. Fatal familial insomnia
        6. ADH syndromes (diabetes insipidus, inappropriate ADH secretion)
        7. Disturbances of temperature regulation (hyperthermia, hypothermia)
        8. Disturbances of sexual function
        9. Disturbances of appetite
        10. Disturbances of bp/HR and gastric function
        11. Horner's syndrome
      4. Disorders of the brainstem and cerebellum
        1. Posterior fossa tumors
        2. Syringobulbia and Arnold-Chiari malformation
        3. Disorders of bp control (hypertension, hypotension)
        4. Cardiac arrhythmias
        5. Central sleep apnea
        6. Baroreflex failure
        7. Horner's syndrome
        8. Vertebrobasilar and lateral medullary (Wallenberg's) syndromes
        9. Brainstem encephalitis
  2. Autonomic disorders with spinal cord involvement
    1. Traumatic quadriplegia
    2. Syringomyelia
    3. Subacute combined degeneration
    4. Multiple sclerosis and neuromyelitis optica
    5. Amyotrophic lateral sclerosis
    6. Tetanus
    7. Stiff-person syndrome
    8. Spinal cord tumors
  3. Autonomic neuropathies
    1. Acute/subacute autonomic neuropathies
      1. Subacute AAG
        1. Subacute paraneoplastic autonomic neuropathy
        2. Guillain-Barré syndrome
        3. Botulism
        4. Porphyria
        5. Drug-induced autonomic neuropathies-stimulants, drug withdrawal, vasoconstrictor, vasodilators, beta-receptor antagonists, beta-agonists
        6. Toxin-induced autonomic neuropathies
        7. Subacute cholinergic neuropathy
    2. Chronic peripheral autonomic neuropathies
      1. Distal small fiber neuropathy
      2. Combined sympathetic and parasympathetic failure
        1. Amyloid
        2. Diabetic autonomic neuropathy
        3. Autoimmune autonomic ganglionopathy (paraneoplastic and idiopathic)
        4. Sensory neuronopathy with autonomic failure
        5. Familial dysautonomia (Riley-Day syndrome)
        6. Diabetic, uremic, or nutritional deficiency
        7. Dysautonomia of old age
      3. Disorders of reduced orthostatic intolerance: reflex syncope, POTS, associated with prolonged bed rest, associated with space flight, chronic fatigue

Abbreviations: AAG, autoimmune autonomic ganglionopathy; ADH, antidiuretic hormone; FXTAS, fragile X-associated tremor/ataxia syndrome; HR, heart rate; POTS, postural orthostatic tachycardia syndrome.