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Autonomic dysfunction in chronic inflammatory demyelinating polyradiculoneuropathy.

J J Figueroa1, P J B Dyck, R S Laughlin

  • 1Department of Neurology, Mayo Clinic, Rochester, MN, USA.

Neurology
|February 24, 2012
PubMed
Summary
This summary is machine-generated.

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) often presents with mild autonomic deficits, primarily affecting sudomotor and cardiovagal functions. These autonomic issues in CIDP are typically mild and suggest distal postganglionic axon damage.

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Area of Science:

  • Neurology
  • Autonomic Neuroscience

Background:

  • Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a neurological disorder affecting peripheral nerves.
  • Autonomic nervous system dysfunction (dysautonomia) can occur in CIDP but has not been well quantified.
  • The Composite Autonomic Severity Score (CASS) is a validated tool for measuring autonomic function.

Purpose of the Study:

  • To quantify autonomic deficits in patients with CIDP using the CASS.
  • To characterize the pattern and severity of dysautonomia in CIDP.
  • To explore the relationship between somatic and autonomic deficits in CIDP.

Main Methods:

  • Retrospective analysis of autonomic function in 47 CIDP patients.
  • Utilized the Composite Autonomic Severity Score (CASS) ranging from 0 to 10.
  • Assessed neurologic deficits with Neuropathy Impairment Score (NIS) and small fiber involvement with quantitative sensory testing.

Main Results:

  • 47% of CIDP patients (22/47) exhibited autonomic deficits (CASS ≥1), but these were mild (mean CASS 0.8 ± 0.9).
  • Deficits were predominantly sudomotor (34%) and cardiovagal (21%), with relative adrenergic sparing (9%).
  • Sudomotor impairment was mainly distal and postganglionic; somatic deficits did not predict autonomic involvement.

Conclusions:

  • Autonomic involvement in classic CIDP is generally mild, cholinergic, and predominantly sudomotor.
  • The findings suggest distal postganglionic axon lesions as the primary cause of sudomotor impairment in CIDP.
  • Extensive or severe autonomic dysfunction (CASS ≥4) in suspected CIDP warrants consideration of alternative diagnoses.