Trigeminal sensory neuropathy in connective tissue disease

Trigeminal sensory neuropathy (TSN) may be a feature of connective tissue disorders and is frequently associated with progressive systemic sclerosis or mixed connective tissue disease (MCTD). [1,2] The pathogenesis of TSN is unknown, and there is limited information on the anatomic site of the pathologic process. Lecky et al [1] proposed lesions of both the Gasserian ganglion and the peripheral portions of the trigeminal nerve. We report a patient with TSN and MCTD where neuroimaging and electrophysiological studies suggested a lesion in the cisternal part of the nerve including the Gasserian ganglion. A 31-year-old man was well until August 1990, when he noticed bilateral burning sensations around his mouth followed by numbness of cheeks, tongue, and oral cavity and loss of taste. Neurologic examination showed a decreased perception of pinprick, light touch, and temperature in the territory of the second and third divisions of the trigeminal nerve on both sides. Taste was impaired. Cranial motor functions and reflexes were intact. Laboratory investigation included normal sedimentation rate, blood count, electrolytes, coagulation tests, and liver and muscle enzymes. The ANA titer was 1:640 with a speckled pattern; antibodies to both extractable nuclear antigens (ENA) and ribonucleoprotein (RNP) were negative. …