[Trigeminus neuropathy as a rare main symptom of progressive systemic sclerosis]

In addition to the habitual skin manifestations, the following forms of organic involvement were found (with decreasing frequency) in 33 patients with progressive systemic sclerosis treated from 1972-1986 at the Clinic of Rheumatology, University Hospital of Zürich: blood vessels 29, lungs 18, digestive system 11, joints 11, bones 8, lacrimal and salivary glands 7, musculature 3, heart 3, kidneys 2, cranial nerves 1 and peripheral neuropathy 1.-A 65-year-old patient with classic progressive systemic sclerosis and visceral involvement of the esophagus and the lungs showed, as a principal sign, unilateral neuropathy of the trigeminal nerve involving all three branches as well as absent corneal reflex. Electromyographic examination afforded evidence of a sensory neuropathy of the trigeminal nerve. Chemical laboratory tests revealed a typical high titre of antinuclear antibodies. In addition to autoimmunological causes, proliferation of fibrous structures of the perineurium and vascular lesions are discussed in connection with these complications.-The subsequent history of 37 patients with localized scleroderma and 4 patients suffering from progressive systemic sclerosis, treated at the Clinic of Dermatology, University Hospital of Zürich from 1980-1985 provided no evidence of a cranial nerve involvement. The fact that during the period 1975 to 1985 only one patient treated at the Clinic of Neurology of Zürich University Hospital showed signs of disturbances of pupil motility confirms the very rare involvement of the nervous system in progressive systemic sclerosis.