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SENSORY GANGLIONOPATHY ASSOCIATED WITH SJÖGREN'S SYNDROME PRESENTING WITH DYSPHAGIA
Author(s) -
Scoditti U.,
Palomba V.,
Marbini A.,
Pavesi G.,
Gemignani F.
Publication year - 2000
Publication title -
journal of the peripheral nervous system
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1
H-Index - 67
eISSN - 1529-8027
pISSN - 1085-9489
DOI - 10.1046/j.1529-8027.2000.00513-57.x
Subject(s) - medicine , corneal reflex , dysphagia , sensory system , neurological examination , reflex , weakness , sensory loss , pathology , dermatology , anatomy , surgery , anesthesia , psychology , neuroscience
Sensory ganglionopathy is a sensory neuropathy characterized by inflammatory infiltration of the sensory ganglia, including trigeminal ganglia, in association with Sjögren's syndrome, or idiopathic. We report a patient who had a peculiar presentation with dysphagia. A 76‐year‐old man presented with a 1‐year history of dysphagia and weight loss, and facial dysesthesias. A diagnosis of amyotrophic lateral sclerosis was initially suspected, because of muscular atrophy and bulbar signs, however EMG showed only mild neurogenic changes, excluding signs of motor neuron involvement. Neurological examination showed absent corneal reflexes, moderate muscular wasting and mild weakness in the extremities, absent deep tendon reflexes in the legs, and sensory loss over the face and extremities. Electroneurographic study demonstrated absent blink reflex, and severely decreased or absent sensory action potentials in the limb nerves. Laboratory investigations, including CSF examination and search for anti‐neural antibodies, were negative. A sicca syndrome was demonstrated by Schirmer test and salivary gland scintigraphy, and lower lip biopsy showed focal sialoadenitis according to the criteria of Greenspan et al (1974). A pure sensory or mainly sensory neuropathy with prominent trigeminal involvement is quite typical in the course of collagen diseases, and in particular in Sjögren's syndrome, supported by T cell infiltration in the dorsal root ganglia. In our case, the presence of sicca syndrome and focal sialoadenitis was consistent with the diagnosis of Sjögren's syndrome. We suggest that dysphagia, an atypical symptom in sensory ganglionopathy, may be related to involvement of the sensory ganglia of the glossopharyngeal and/or vagus nerve.