Late occurrence of chronic immune-mediated axonal polyneuropathy following bone marrow transplant for juvenile-onset α-mannosidosis

D. A. Mulrooney, S. M. Davies, D. Walk, L. R. Charnas

Research output: Contribution to journalArticle

5 Citations (Scopus)

Abstract

A 23-year-old woman with juvenile-onset α-mannosidosis developed an axonal polyneuropathy more than a year following successful unrelated donor (URD) BMT complicated by chronic graft-versus-host disease (GVHD). Progressive muscle weakness and paresthesias developed over at least 4 months, and made her nonambulatory. Nerve conduction and EMG studies demonstrated an axonal sensorimotor neuropathy. Cerebral spinal fluid (CSF) IgG was elevated with two peaks not identified in serum. Strength improved after a single course of plasma exchange and continued to improve over 12 months. The response to plasma exchange, elevated CSF IgG production, and evidence of a serum IgM peak suggest an immune-mediated mechanism. Chronic polyneuropathies following BMT are rare and are usually temporally related to GVHD or infection. This patient's disease was unusual because of its late occurrence and chronic onset in the face of resolved GVHD and in the absence of infection.

Original languageEnglish (US)
Pages (from-to)953-955
Number of pages3
JournalBone marrow transplantation
Volume32
Issue number9
DOIs
StatePublished - Nov 1 2003

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Mannosidase Deficiency Diseases
Polyneuropathies
Graft vs Host Disease
Plasma Exchange
Bone Marrow
Transplants
Immunoglobulin G
Unrelated Donors
Paresthesia
Neural Conduction
Muscle Weakness
Infection
Serum
Immunoglobulin M

Keywords

  • Acute motor axonal neuropathy
  • Polyneuropathy
  • α-mannosidosis

Cite this

Late occurrence of chronic immune-mediated axonal polyneuropathy following bone marrow transplant for juvenile-onset α-mannosidosis. / Mulrooney, D. A.; Davies, S. M.; Walk, D.; Charnas, L. R.

In: Bone marrow transplantation, Vol. 32, No. 9, 01.11.2003, p. 953-955.

Research output: Contribution to journalArticle

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