Hereditary sensory and autonomic neuropathy: review and a case report with dental implications

Abstract

Hereditary sensory and autonomic neuropathy (HSAN) is a rare syndrome which is seen in early childhood. Five different types are described. Absence of pain and self-mutilation are characteristic findings of this syndrome. Teeth in the oral cavity can cause damage to the oral tissues and tongue. When it is diagnosed, there should be co-operation between dentist and neurologist. Using an oral shield prevents the biting and, thus, traumatization of the tissues can be prevented. A case report which is diagnosed as HSAN type 4 is presented and information submitted about its treatment.

Proposal for a standardized protocol for the systematic orofacial examination of patients with Hereditary Sensory Radicular Neuropathy

“…Histochemical and ultra‐structural examination of dental pulp tissue from the female patient demonstrated that nerve fibre morphology was normal, but the proportion of unmyelinated relative to myelinated fibres was lower than usually expected in normal dental pulp tissue (Akai & Wakisaka 1990). Similar biopsy results were found in the sural nerve of the same patient (Erdem et al. 2000).…”

“…The differential diagnosis may include assymbolia, hysteria and peripheral neuropathies (Person et al. 1977, Erdem et al. 2000).…”

Facial self-mutilation: an analysis of published cases

“…However, it must be kept in mind that some cases require more radical dental extractions,18 31 40 and lesions involving the floor of the mouth carry a potential for life-threatening cervical subcutaneous emphysema and pneumomediastinum 36…”

Neonatal sublingual traumatic ulceration – case report & review of the literature

“…Sublingual traumatic ulcers are often oral manifestations of an underlying neurologic or developmental disorder such as congenital insensitivity to pain with anhidrosis (CIPA) [hereditary sensory and autonomic neuropathy(HSAN) – type IV] (22, 23) and familial dysautonomia (FD) or Riley‐Day syndrome [HSAN type III] (24, 25). CIPA presents more frequently in boys than girls, and infants with this condition present commonly with episodes of high fever related to warm environmental temperatures as they do not perspire (23, 26).…”