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Chronic immune sensory polyradiculopathy (CISP): First juvenile case description.
Neurol Sci. 2020 Jul 10 [Online ahead of print]NS

Abstract

In its typical presentation, chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) occurs more often in old males as a progressive/recurrent motor and sensory nerve dysfunction with tendon areflexia. However, CIDP has also atypical clinical presentations, including pure sensory neuropathies, among which chronic immune sensory polyradiculopathy (CISP) accounts for only 0.5% of all CIDP, with no juvenile cases reported as yet. A 17-year-old girl presented for a progressive sensory ataxia and hands clumsiness. Diffuse tendon areflexia and hypokinaesthesia were observed. Motor and sensory nerve conduction studies were normal. F-waves were normal in median nerves and elongated in tibial nerves. H-reflex and somatosensory evoked potentials (SSEP) were absent. CSF normal cellularity with hyperproteinorrachia was found. Paraneoplastic, metabolic, and paraproteinemic neuropathies were excluded. A diagnosis of CISP has been made based on the presence of pure sensory symptoms in a polyneuropathic distribution, normal peripheral nerve conduction studies, and two supportive criteria (SSEP and CSF). Our paper describes the first CISP case in the pediatric age. We confirm SSEP and CSF as useful complementary tests for this diagnosis also at this age and suggest that clinicians should consider CISP in the spectrum of sporadic sensory ataxias of the pediatric age. We also suggest that in the presence of normal F-wave and peripheral motor nerve conduction, an absent H-reflex can further substantiate SSEPs in the diagnosis of CISP. Intravenous immunoglobulins were rapidly effective and safe.

Authors+Show Affiliations

Unit of Child Neuropsychiatry, University Hospital of Sassari, Department of Medical, Surgical and Experimental Sciences, University of Sassari, Viale San Pietro 42, 07100, Sassari, Italy. stefanos@uniss.it.Unit of Child Neuropsychiatry, University Hospital of Sassari, Department of Medical, Surgical and Experimental Sciences, University of Sassari, Viale San Pietro 42, 07100, Sassari, Italy.Unit of Child Neuropsychiatry, University Hospital of Sassari, Department of Medical, Surgical and Experimental Sciences, University of Sassari, Viale San Pietro 42, 07100, Sassari, Italy.Unit of Child Neuropsychiatry, University Hospital of Sassari, Department of Medical, Surgical and Experimental Sciences, University of Sassari, Viale San Pietro 42, 07100, Sassari, Italy.Humanitas Clinical and Research Institute, Milan, Italy.Humanitas Clinical and Research Institute, Milan, Italy.

Pub Type(s)

Letter

Language

eng

PubMed ID

32651857

Citation

Sotgiu, Stefano, et al. "Chronic Immune Sensory Polyradiculopathy (CISP): First Juvenile Case Description." Neurological Sciences : Official Journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology, 2020.
Sotgiu S, Minutolo A, Carta A, et al. Chronic immune sensory polyradiculopathy (CISP): First juvenile case description. Neurol Sci. 2020.
Sotgiu, S., Minutolo, A., Carta, A., Puseddu, G., Doneddu, P. E., & Nobile-Orazio, E. (2020). Chronic immune sensory polyradiculopathy (CISP): First juvenile case description. Neurological Sciences : Official Journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology. https://doi.org/10.1007/s10072-020-04588-y
Sotgiu S, et al. Chronic Immune Sensory Polyradiculopathy (CISP): First Juvenile Case Description. Neurol Sci. 2020 Jul 10; PubMed PMID: 32651857.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Chronic immune sensory polyradiculopathy (CISP): First juvenile case description. AU - Sotgiu,Stefano, AU - Minutolo,A, AU - Carta,A, AU - Puseddu,G, AU - Doneddu,P E, AU - Nobile-Orazio,E, Y1 - 2020/07/10/ PY - 2020/01/18/received PY - 2020/07/07/accepted PY - 2020/7/12/entrez KW - Atypical CIDP KW - CISP KW - Chronic immune polyradiculopathy KW - Electrodiagnostic criteria KW - Pediatric JF - Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology JO - Neurol. Sci. N2 - In its typical presentation, chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) occurs more often in old males as a progressive/recurrent motor and sensory nerve dysfunction with tendon areflexia. However, CIDP has also atypical clinical presentations, including pure sensory neuropathies, among which chronic immune sensory polyradiculopathy (CISP) accounts for only 0.5% of all CIDP, with no juvenile cases reported as yet. A 17-year-old girl presented for a progressive sensory ataxia and hands clumsiness. Diffuse tendon areflexia and hypokinaesthesia were observed. Motor and sensory nerve conduction studies were normal. F-waves were normal in median nerves and elongated in tibial nerves. H-reflex and somatosensory evoked potentials (SSEP) were absent. CSF normal cellularity with hyperproteinorrachia was found. Paraneoplastic, metabolic, and paraproteinemic neuropathies were excluded. A diagnosis of CISP has been made based on the presence of pure sensory symptoms in a polyneuropathic distribution, normal peripheral nerve conduction studies, and two supportive criteria (SSEP and CSF). Our paper describes the first CISP case in the pediatric age. We confirm SSEP and CSF as useful complementary tests for this diagnosis also at this age and suggest that clinicians should consider CISP in the spectrum of sporadic sensory ataxias of the pediatric age. We also suggest that in the presence of normal F-wave and peripheral motor nerve conduction, an absent H-reflex can further substantiate SSEPs in the diagnosis of CISP. Intravenous immunoglobulins were rapidly effective and safe. SN - 1590-3478 UR - https://www.unboundmedicine.com/medline/citation/32651857/Chronic_immune_sensory_polyradiculopathy_(CISP):_First_juvenile_case_description L2 - https://link.springer.com/10.1007/s10072-020-04588-y DB - PRIME DP - Unbound Medicine ER -
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