Hereditary sensory and autonomic neuropathies — classification and clinical characteristics

  • D. Burg
  • G. Burg
  • D. Pongratz
Conference paper


Hereditary sensory and hereditary sensory and autonomic neuropathies (HSN or HSAN) are very rare conditions and inhomogeneous with respect to genetic and clinical features. Various disease entities have to be differentiated.

The most common disorder of this group of hereditary peripheral neuropathies is HSN type I with a great variability of age of onset and impairment of pain and temperature sensation as the main and earliest clinical signs. However, the diagnosis is usually not made before cutaneous alterations occur.

HSN II occurs in early childhood and reveals affection of all sensory qualities, mostly accompanied by prominent cutaneous and osseous alterations depending on the stress to which the denervated structures are subjected.

Whereas in HS(A)N I and II the autonomic denervation essentially affects skin structures, HSAN III is characterized by additional autonomic disturbances such as the regulation of body temperature, of heart and respiration, the intestinal tract and further more. Lack of tears in crying is suggested to be an obligate symptom.

In HSAN IV anhidrosis, episodes of fever, analgesia and impaired temperature sensation are crucial symptoms.

The term “hereditary sensory neuropathies” denotes a group of genetically determined neuropathies whose pathogenesis is still unclear. The most important clinical features are sensory and autonomic disturbances of variable predominance. The leading symptoms are marked impairment of pain and temperature sensation, particularly in fingers and toes, and a tendency to develop ulcerations and bony degeneration. More or less severe additional symptoms of peripheral or central origin that affect other sensory modalities or motor function contribute to further differentiation.


Autonomic Neuropathy Sensory Neuropathy Sensory Impairment Familial Dysautonomia Temperature Perception 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


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Copyright information

© Springer-Verlag Wien 1995

Authors and Affiliations

  • D. Burg
    • 1
  • G. Burg
    • 2
  • D. Pongratz
    • 3
  1. 1.Friedrich Baur-Institute of the Department of Internal MedicineUniversity of MunichMunichGermany
  2. 2.University of ZurichZurichSwitzerland
  3. 3.Friedrich-Baur InstituteGermany

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