Keratopathy as an indication of multifocal congenital sensitive polyneuropathy

Patient. A 63-year-old female with bilateral recurrent corneal ulcerations for 10 years, suffered from vascularisation of the cornea and absence of co rneal sensitivity. Other symptoms were multifocal hypoaesthesia with hypalg esia of hands and legs, generalised lack of deep tendon reflexes, absence o f somato-sensory evoked potentials (SSEP) and of sensory nerve action poten tials (SNAP) in these regions. A sural biopsy demonstrated extreme lack of myelinated fibres. Acquired causes for polyneuropathy were excluded. Therapy. Subsequent to local ocular treatment we carried out a perforating corneal transplantation of the left eye because of corneal scars. This had to be repeated 2 years later because of vascularisation of the transplant. The visual function of the left eye could be stabilised at values between 0 .2 and 1/50. Conclusion. Anamnesis and clinical symptoms of the patient are compatible w ith the diagnosis of hereditary sensory neuropathy type II (HSN II) affecti ng the trigeminal nerves. In patients with neuropathy and impaired corneal sensitivity, a favourable prognosis may be achieved by a corneal transplant .