Utility of somatosensory evoked potentials in the assessment of response to IVIG in a long-lasting case of chronic immune sensory polyradiculopathyReport as inadecuate




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BMC Neurology

, 17:127

Neuromuscular disorders and peripheral neurology

Abstract

BackgroundChronic immune sensory polyradiculopathy CISP identifies a progressive acquired peripheral dysimmune neuropathy recognized as a chronic inflammatory demyelinating polyradiculoneuropathy CIDP variant. We describe a young woman with a thirteen-year history of CISP with a belated variable response to intravenous immunoglobulin IVIG and an almost erratic anticipation of symptoms between IVIG cycles. The association of IVIG and corticosteroids, immunosuppressants, plasmapheresis, did not lead to clinical improvement and was characterized by significant side effects. We evaluated a combined clinical and somatosensory evoked potentials SSEPs approach aimed to identify possible predictive parameters concerning the effect and duration of each IVIG administration. Neurologic disability was evaluated using INCAT - Overall Disability Sum Score INCAT-ODSS.

Case presentationA 30-year-old woman presented on 2004 for the subacute onset of asymmetric paresthesias in the lower limbs over the previous six months. The symptoms had been relapsing-remitting during the first four months, followed by a slow progression, resulting in limbs ataxia and a progressive gait disturbance requiring Canadian crutches. Motor and sensory nerve conduction studies and electromyographic evaluation were into normal limits. Median SSEPs were normal, while tibial SSEPs were characterised by the bilateral absence of both lumbar and cortical responses. Cerebrospinal fluid detected an increased protein concentration, while spinal MRI showed a pronounced thickening of the sacral nerve roots, together with a tube-shaped enlargement. These findings led to the diagnosis of CISP and the patient was treated with IVIG reaching a stable remission over the following 9 years. In early 2014, the patient began to show a variable response to treatment with erratic anticipation of sensory disturbances, and a more pronounced walking disability: corticosteroids, plasmapheresis, mycophenolate mofetil and cyclophosphamide were uneffective and burdened by relevant side effects. To better assess the response to IVIG in terms of time-effect, consistency and duration, we have combined a scheduled clinical and SSEPs evaluation during and after each IVIG cycle.

ConclusionsThe correlation between the neurophysiological data and the INCAT-ODSS scores has allowed the modulation of IVIG cycles with a significant reduction of the clinical fluctuations and disability. SSEPs may therefore represent an useful and recommended additional aid for the treatment schedule of this rare clinical form.

KeywordsChronic immune sensory polyradiculopathy Somatosensory evoked potentials Chronic inflammatory demyelinating polyneuropathy Intravenous immunoglobulin INCAT - Overall disability sum score AbbreviationsCIDPChronic inflammatory demyelinating polyradiculoneuropathy

CISPChronic immune sensory polyradiculopathy

CSFCerebrospinal fluid

EFNS-PNSEuropean Federation of Neurological Societies-Peripheral Nerve Society

INCAT-ODSSInflammatory Neuropathy Cause and Treatment - Overall Disability Sum Score

IVIGIntravenous immunoglobulin

MRIMagnetic resonance imaging

NVNormal value

SDsStandard Deviations

SPSSStatistical Package for Social Science

SSEPsSomatosensory evoked potentials

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Author: Angelo Maurizio Clerici - Eduardo Nobile-Orazio - Marco Mauri - Federico Sergio Squellati - Giorgio Giovanni Bono

Source: https://link.springer.com/



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