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Abstract
Objective: To define the peripheral neuropathy phenotypes associated with Castleman disease.
Methods: We conducted a retrospective chart review for patients with biopsy-proven Castleman disease evaluated between January 2003 and December 2014. Patients with associated peripheral neuropathy were identified and divided into 2 groups: those with Castleman disease without POEMS syndrome (CD-PN) and those with Castleman disease with POEMS syndrome (CD-POEMS). We used a cohort of patients with POEMS as controls. Clinical, electrodiagnostic, and laboratory characteristics were collected and compared among patient subgroups.
Results: There were 7 patients with CD-PN, 20 with CD-POEMS, and 122 with POEMS. Patients with CD-PN had the mildest neuropathy characterized by predominant sensory symptoms with no pain and mild distal sensory deficits (median Neuropathy Impairment Score of 7 points). Although both patients with CD-POEMS and patients with POEMS had a severe sensory and motor neuropathy, patients with CD-POEMS were less affected (median Neuropathy Impairment Score of 33 and 66 points, respectively). The degree of severity was also reflected on electrodiagnostic testing in which patients with CD-PN demonstrated a mild degree of axonal loss, followed by patients with CD-POEMS and then those with POEMS. Demyelinating features, defined by European Federation of Neurologic Societies/Peripheral Nerve Society criteria, were present in 43% of the CD-PN, 78% of the CD-POEMS, and 86% of the POEMS group.
Conclusion: There is a spectrum of demyelinating peripheral neuropathies associated with Castleman disease. CD-PN is sensory predominant and is the mildest phenotype, whereas CD-POEMS is a more severe sensory and motor neuropathy. Compared to the POEMS cohort, those with CD-POEMS neuropathy have a similar but less severe phenotype. Whether these patients respond differently to treatment deserves further study.
GLOSSARY
- CD-PN=
- Castleman disease with peripheral neuropathy only;
- CD-POEMS=
- Castleman disease with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes;
- IL-6=
- interleukin-6;
- NIS=
- Neuropathy Impairment Score;
- POEMS=
- polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes;
- PMTA=
- peroneal motor amplitude recording from the tibialis anterior muscle;
- TANES=
- tibialis anterior needle examination score;
- VEGF=
- vascular endothelial growth factor
Footnotes
Go to Neurology.org for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
Supplemental data at Neurology.org
- Received April 28, 2016.
- Accepted in final form September 8, 2016.
- © 2016 American Academy of Neurology
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