[3 cases of rare peripheral neuropathies associated with primary Gougerot-Sjögren syndrome]

Abstract

Several forms of peripheral neuropathy occur in Sjögren's syndrome (dryness of eyes, mouth and other mucous membranes). Symmetrical sensorimotor polyneuropathy occurs most frequently followed by sensory neuropathy. Pure sensory neuronopathy, trigeminal sensory neuropathy and autonomic neuropathy are also common. We report three rare forms of peripheral neuropathy related to Sjögren's syndrome and analyze their clinical course and response to therapy. There were a case of mononeuropathy multiplex (MM), a case of recurrent cranial polyneuropathy (RCP) and a case of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). The patients were females, 70, 63 and 81 years old respectively. All of them fulfilled the criteria of Sjögren's syndrome, without clinical or serological abnormalities of systemic disease. Only one patient presented with positive antinuclear antibodies. In two patients the onset of neuropathy occurred before the sicca syndrome long. The sural biopsy of the patient with MM exhibited vasculitis of the small vessels and a mild sensory polyneuropathy was superimposed. The patient with RCP had 8 episodes of isolated third nerve palsy and 5 episodes of multiple cranial neuropathy. The patient with CIDP responded poorly to treatment while in the patients with MM and CRP the response to corticotherapy was good. In conclusion, Sjögren's syndrome must be considered in neuropathies of unknown cause not only when they are sensory, autonomic or trigeminal but also when they are recurrent suggesting an ischemic mechanism. Even a CIDP requires a search for Sjögren's syndrome before being considered idiopathic.