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Three Families With Polyneuropathy Associated With Monoclonal Gammopathy
Sanne M. Manschot, MD;
Nicolette C. Notermans, MD;
Leonard H. van den Berg, MD;
Jan J. G. M. Verschuuren, MD;
Henk M. Lokhorst, MD
Arch Neurol. 2000;57:740-742.
Objective To report familial occurrence of polyneuropathy associated with monoclonal gammopathy.
Design Case reports.
Patients We describe 6 patients (3 pairs) with a polyneuropathy associated with IgM monoclonal gammopathy. Four of the 6 patients had a demyelinating polyneuropathy on neurophysiological examination. Three patients had elevated antibodies against myelin-associated glycoprotein. No duplication on chromosome 17 or a mutation on chromosome 1 was found in any family.
Conclusion Familial occurrence of polyneuropathy without the presence of hereditary motor and sensory neuropathy type I is a reason to search for the presence of monoclonal gammopathy.
From the Department of Neuromuscular Disorders, Rudolf Magnus Institute of Neurosciences, Utrecht (Drs Manschot, Notermans, and van den Berg), the Department of Neurology, Leiden University Medical Centre, Leiden (Dr Verschuuren), and the Department of Haematology, University Hospital Utrecht (Dr Lokhorst), the Netherlands.
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