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Journal of Child Neurology
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Nonprogressive Type II Hereditary Sensory Autonomic Neuropathy: A Homogeneous Clinicopathologic Entity

Gerard Ferrière, MD

Unité de Neurologie du Developpement, Service de Neurologie Pédiatrique, Université Catholique de Louvain, Medical School, Brussels, Belgium

Francesco Guzzetta, MD

Istituto de Neuropsichiatria Infantile, Medical School, Università di Messina, Messina, Italy

Sophie Kulakowski, MD

Institut Medico-Chirurgical "Les Petites Abeilles", Vlezembeek, Brussels, Belgium

Philippe Evrard, MD

Unité de Neurologie du Developpement, Service de Neurologie Pédiatrique, Université Catholique de Louvain, Medical School, Brussels, Belgium

Two different clinical subtypes were previously identified within hereditary sensory autonomic neuropathy (HSAN) type II: a stable congenital form and a progressive one. This paper discusses two clinicopathologic cases of nonprogressive HSAN type II with morphometric correlations. In addition, a retrospective literature search was carried out to locate other cases where an accurate histologic examination, including ultrastructural features, was available in order to relate clinical and pathologic aspects of the disease. The combined data support the individualization of this neuropathic form as a homogeneous disease, as has been suggested during the last century and underline the clinical importance of this concept for the prognosis and investigation of sensory, auditory, autonomic, and motor functions in children with sensory neuropathies. (J Child Neurol 1992;7:364-370).

Journal of Child Neurology, Vol. 7, No. 4, 364-370 (1992)
DOI: 10.1177/088307389200700406
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