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  • Review Article
  • Published:

The clinical approach to autonomic failure in neurological disorders

Nature Reviews Neurology volume 10, pages 396–407 (2014)Cite this article

Subjects

Key Points

  • Autonomic failure can occur in isolation, in association with peripheral neuropathy, or as a manifestation of a neurodegenerative disorder

  • Disorders associated with autonomic failure can be classified according to the type and severity of autonomic manifestations, associated neurological symptoms, and temporal profile

  • Acute or subacute autonomic failure suggests an autoimmune autonomic ganglionopathy, which may be paraneoplastic

  • In patients with parkinsonism and autonomic failure, early onset and progression of orthostatic hypotension or urogenital dysfunction, urinary incontinence, generalized anhidrosis, and/or laryngeal stridor are highly suggestive of multiple system atrophy

  • Autonomic failure can occur with any type of diabetic or amyloid neuropathy

  • Important causes of painful neuropathy associated with autonomic failure include Sjögren syndrome, HIV infections, Fabry disease, and sodium channelopathies

Abstract

Central or peripheral neurological disorders can manifest with autonomic failure or autonomic hyperactivity, which may affect the sympathetic, parasympathetic and/or enteric nervous systems. Disorders causing autonomic failure can be classified according to the presence or absence of associated neurological manifestations, such as peripheral neuropathy or parkinsonism, and their temporal profile (acute or subacute, chronic progressive, static, or episodic). A systematic approach allows focused evaluation to detect treatable, potentially disabling or life-threatening conditions. Subacute isolated autonomic failure affecting sympathetic, parasympathetic and enteric nervous system function, in various combinations, occurs in autoimmune autonomic ganglionopathy, which might be the first manifestation of an underlying neoplasm. Autonomic failure can be an important feature of several types of peripheral neuropathy, including sensorimotor peripheral neuropathies, sensory ganglionopathy, and distal painful peripheral neuropathies. Progressive autonomic failure occurs in neurodegenerative synucleinopathies such as multiple system atrophy and Lewy body disorders. Autonomic failure may also occur in hereditary leukoencephalopathies or prion disorders. This Review outlines the clinical approach to patients with generalized autonomic failure, focusing predominantly on classification and diagnosis, but also touching briefly on treatment and management.

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Figure 1: Evaluating the main causes of autonomic failure.

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  1. Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, 55905, MN, USA

    Eduardo E. Benarroch

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Benarroch, E. The clinical approach to autonomic failure in neurological disorders. Nat Rev Neurol 10, 396–407 (2014). https://doi.org/10.1038/nrneurol.2014.88

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