%0 Journal Article
%T Intravenous Immunoglobulin and Mycophenolate Mofetil for Long-Standing Sensory Neuronopathy in Sj£¿gren's Syndrome
%A Maria Giovanna Danieli
%A Lucia Pettinari
%A Ramona Morariu
%A Fernando Monteforte
%A Francesco Logullo
%J Case Reports in Immunology
%D 2012
%I Hindawi Publishing Corporation
%R 10.1155/2012/186320
%X Sensory neuronopathy is described in association with the Sj£¿gren's syndrome (SS). We studied a 55-year-old woman with a 4-year history of progressive asymmetric numbness, distal tingling, and burning sensation in upper and lower limbs. In a few months, she developed ataxia with increased hypoanaesthesia. Electrodiagnostic tests revealed undetectable distal and proximal sensory nerve action potential in upper and lower limbs. Cervical spine magnetic resonance showed a signal hyperintensity of posterior columns. Previous treatment with high-dose glucocorticoids and azathioprine was ineffective. A combined treatment with intravenous immunoglobulin and mycophenolate mofetil was followed by a progressive and persistent improvement. This case documented the efficacy and the safety of the coadministration of intravenous immunoglobulin and mycophenolate mofetil in sensory neuronopathy associated with SS refractory to conventional immunosuppressive therapy. 1. Introduction Central nervous system involvement in Sj£¿gren¡¯s syndrome (SS) is rarely reported and may be severe and varied [1]. Sensory neuronopathy (or sensory ganglionopathy, SN) is a distinctive neuropathy of SS, accounting for 15¨C20% of all neuropathies seen in this condition [2]. A sensory neuropathy is often the presenting feature of SS, and, therefore, a high index of suspicion is required, particularly in female patients with non-length-dependent, painful, or ataxic sensory neuropathy or those with trigeminal sensory and autonomic involvement [3]. At the onset of SN, numbness, tingling, burning, and pain sensations are reported in all limbs, usually with asymmetric distribution. With the disease progression, the sensory disturbances can involve the trunk, the face or they develop into a symmetric way. On examination, degeneration of large sensory neurons leads to gait ataxia, proprioceptive sensory loss, and widespread deep tendon areflexia [3]. When smaller sensory neurons are affected, deficits are those of hypoesthesia to pain and thermal stimuli with hyperacute pain. Autonomic nervous system involvement may cause fixed tachycardia, orthostatic hypotension, and gastrointestinal pseudo-obstruction. The response to treatment is usually poor, even with glucocorticoids, immunosuppressants, and plasmapheresis [3]. Here we report the case of a woman with primary SS who presented with SN that was successfully managed with intravenous immunoglobulin and mycophenolate mofetil coadministration. 2. Case Report In 2001, a 55-year-old woman presented with progressive asymmetric numbness distal tingling and
%U http://www.hindawi.com/journals/crii/2012/186320/