Myeloma-associated Amyloidosis Presenting as Orthostatic Intolerance

Heejeong Jeong; Wonsik Nam; Seungnam Son; Soo-Kyung Kim; Heeyoung Kang; Nack-Cheon Choi; Oh-Young Kwon; ByeongHoon Lim; Ki-Jong Park

doi:10.14253/KJCN.2015.17.1.24

Journal List > Korean J Clin Neurophysiol > v.17(1) > 1084153

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Jeong, Nam, Son, Kim, Kang, Choi, Kwon, Lim, and Park: Myeloma-associated Amyloidosis Presenting as Orthostatic Intolerance

Original Article

Korean J Community Nutr 2015;17(1):24-27.

Published online: 18 December 2015

DOI: https://doi.org/10.14253/KJCN.2015.17.1.24

Myeloma-associated Amyloidosis Presenting as Orthostatic Intolerance

Heejeong Jeong, Wonsik Nam, Seungnam Son, Soo-Kyung Kim, Heeyoung Kang, Nack-Cheon Choi, Oh-Young Kwon, ByeongHoon Lim, Ki-Jong Park

Department of Neurology, Institute of Health Science, Gyeongsang National University School of Medicine, Jinju, Korea

Address for correspondence; Ki-Jong Park Department of Neurology, Institute of Health Science, Gyeongsang National University School of Medicine, 79 Gangnam-ro, Jinju 660-702, Korea Tel: +82-55-750-8077 Fax: +82-55-755-1709 E-mail: pkjong@gnu.ac.kr

Received 21 October 2014 Revised 23 January 2015 Accepted 6 March 2015

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Amyloidosis is a systemic disorder associated with clonal plasma cell dyscrasia. Nephrotic syndrome, congestive heart failure, autonomic and peripheral neuropathy is often associated features in amyloidosis. Early diagnosis is most important because of different prognosis by stage. The diagnosis can be delayed since symptoms of amyloidosis may vary or nonspecific. We describe a patient of myeloma-associated amyloidosis, who showed orthostatic intolerance as the first symptom of the disease. (Korean J Clin Neurophysiol 2015;17:24-27)

Keywords: Orthostatic intolerance, Amyloidosis, Neuropathy

REFERENCES

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Fig. 1.

(A) Head-up tilt table test shows marked orthostatic hypotension. (B) Quantitative sudomotor axon reflex test (QSART) shows global postganglionic sudomotor dysfunction.

Table 1.

Autonomic function test. There are diffuse postganglionic sudomotor impairment, cardiovagal and cardiovascular adrenergic impairment. These findings suggest global autonomic neuropathy

		Nnormal value
Cardiovagal function
1. Heart rate difference	1.23	≥ 7
2. I:E ratio	1.01	≥ 1.05
3. Valsalva ratio	1.04	≥ 1.29
Cardiovascular adrenergic function
1. BP recovery time	20 seconds
2. Head-up tilt test
Supine position	124/70 mmHg
Erect position_1min	70/42 mmHg
Erect position_3min	68/44 mmHg
Sudomotor function (volumme)
1. Forearm	0.27	≥ 0.73
2. Proximal leg	0.06	≥ 0.87
3. Distal leg	0.23	≥ 0.84
4. Foot	0.09	≥ 0.3

Fig. 2.

Duodenal mucosa biopsy finding with Congo-red staining shows amyloid positive (A), apple-green birefringence under polarized light microscope (B) (Congo-red stain, ×40).

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