Pure sensory chronic inflammatory polyneuropathy: rapid deterioration after steroid treatment

Chroni, Elisabeth; Veltsista, Dimitra; Gavanozi, Evangelia; Vlachou, Tavitha; Polychronopoulos, Panagiotis; Papathanasopoulos, Panagiotis

doi:10.1186/s12883-015-0291-7

Cited by 9 publications

(7 citation statements)

References 25 publications

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“…Consider also that we often modulate “empirically” the IVIG cycles following the criterion of the clinical worsening, as well as the choice of other therapeutic measures (corticosteroids, immunosuppressants, plasmapheresis) [22–24]. …”

Section: Discussionmentioning

confidence: 99%

“…In the present case, considering clinical fluctuations and the erratic response to IVIG treatment, we have planned a neurophysiological approach (SSEPs) together with the daily ODSS record, not only for a diagnostic confirmation, but also to search possible correlations between neurophysiological and clinical data potentially exploitable for therapeutic purposes. Consider also that we often modulate “empirically” the IVIG cycles following the criterion of the clinical worsening, as well as the choice of other therapeutic measures (corticosteroids, immunosuppressants, plasmapheresis) [ 22 – 24 ].…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2017

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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.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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

et al. 2017

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“…There is no known method for identifying people with CIDP who will deteriorate. Deterioration has been considered more likely in pure motor CIDP (Donaghy 1994), but also occurs in pure sensory CIDP (Chroni 2015;Rajabally 2012). The likelihood of response to steroids in various atypical forms of CIDP is also uncertain.…”

Section: Librarymentioning

confidence: 99%

Corticosteroids for chronic inflammatory demyelinating polyradiculoneuropathy

Hughes

Mehndiratta

Rajabally

2017

Cochrane Database of Systematic Reviews

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“…About 90% of patients are reported to respond to IVIG or steroids in most series with only very few patients requiring PLEX or alternate immunological agents ( 5 , 31 – 33 ). There are rare reports of a patient deteriorating with one of the first-line agents but then responding to another agent, such as rituximab ( 34 – 36 ). In general, the treatment and expected response of pure sensory CIDP are similar to typical CIDP.…”

Section: Pure Sensory Cidpmentioning

confidence: 99%

Treatment Approaches for Atypical CIDP

2021

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The variants of chronic inflammatory demyelinating polyneuropathy (CIDP) differ not just in their clinical, pathological and electrophysiological characteristics, but often in their indifferent response to conventional immunosuppressive agents which are effective in typical CIDP. High quality evidence is lacking as far as the management of these atypical variants is concerned. In this review, we summarize the treatment approaches to each of these CIDP variants based on existing data. Distal acquired demyelinating symmetric polyneuropathy (DADS) has the phenotype of a symmetric, demyelinating sensory, length-dependent polyneuropathy and is frequently associated with paraproteinemia and anti myelin associated glycoprotein (MAG) antibodies. While the management of idiopathic DADS (DADS-I) is the same as CIDP, DADS-M responds suboptimally and has a favorable response to rituximab. Multifocal acquired demyelinating sensory and motor neuropathy (MADSAM) manifests as a chronic progressive demyelinating mononeuropathy multiplex which can evolve to a confluent pattern indistinguishable from CIDP. Evidence favors treating MADSAM with conventional immunomodulatory therapy (IMT), but this disorder responds less favorably than CIDP. Some patients present with purely sensory symptoms, known as pure sensory CIDP or chronic inflammatory sensory polyradiculoneuropathy (CISP), the latter localizing to a pre-ganglionic pathology. Both respond well to first line IMT, particularly to intravenous immunoglobulin (IVIG), but patients relapse without maintenance therapy. Pure motor CIDP resembles multifocal motor neuropathy with conduction block (MMNCB), but the previously reported worsening status after steroid treatment was not reproduced in recent studies, and IVIG remains the first-line therapy. Some focal forms of CIDP defy exact classification, but respond well to first-line IMT including IVIG. Overall, atypical CIDP responds to treatment with first-line IMT, but has a suboptimal response compared to CIDP. There is evidence for effectiveness with agents such as rituximab, especially in DADS-M, and this medication can also be used in cases refractory to conventional IMTs. Rituximab is also effective in CIDP with IgG4 antibodies which has distinct clinical features and is mostly refractory to first-line IMT.

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Pure sensory chronic inflammatory polyneuropathy: rapid deterioration after steroid treatment

Cited by 9 publications

References 25 publications

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

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

Corticosteroids for chronic inflammatory demyelinating polyradiculoneuropathy

Treatment Approaches for Atypical CIDP

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