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Neurology. 2016 Dec 6;87(23):2457-2462. Epub 2016 Nov 2.

Clinical spectrum of Castleman disease-associated neuropathy.

Author information

1
From the Departments of Neurology (E.N., M.L.M.), Internal Medicine (A.D.), and Medicine (J.M.), Mayo Clinic, Rochester, MN.
2
From the Departments of Neurology (E.N., M.L.M.), Internal Medicine (A.D.), and Medicine (J.M.), Mayo Clinic, Rochester, MN. mauermann.michelle@mayo.edu.

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.

PMID:
27807187
PMCID:
PMC5177676
DOI:
10.1212/WNL.0000000000003405
[Indexed for MEDLINE]
Free PMC Article

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