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Thirteen patients,11 women and 2 men,developed sensory and autonomic neuronopathies in association with features of primary Sjogren's syndrome. In 11,Sjogren's syndrome had not been previously diagnosed at the time of neurological presentation.All had prominent loss of kinesthesia and proprioception.Pain and thermal sensibility were less severely affected. Most had evidence of autonomic insufficiency.In some this was severe,with Adie's pupils,fixed tachycardia,and orthostatic hypotension.The course ranged from an abrupt,devastating onset to indolent progression over years.Stabilization or functional improvement occurred in 6 patients,2 of whom received no drug therapy.Sensory nerve conduction studies and examination of nerve biopsy specimens demonstrated a wide spectrum in the severity of loss of large myelinated fibers.The cutaneous nerves of 6 patients had perivascular mononuclear infiltrates without necrotizing arteritis.Examination of biopsy specimens of dorsal root ganglia in 3 patients revealed lymphocytic(t-cell)infiltration in the dorsal roots and ganglia,with focal clusters around neurons.In the more mildly affected ganglia,individual sensory neurons were undergoing degeneration.In the most advanced case,very few neurons remained.The possibility of Sjogren's syndrome should be considered in patients,especially women,who develop acute,subacute,or chronic sensory and autonomic neuropathies,with ataxia and kinesthetic loss. |
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ataxia
ataxic gait
autonomic dysfunction
ganglionitis
nerve biopsy
neuronopathy,sensory
neuropathy
neuropathy,ataxic
neuropathy,sensory
position sensation
position sensation,abnormal
sensory ganglia
sensory ganglia,abnormal
Sjogren's syndrome
Sjogren's syndrome,neurologic manifestations of
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