J Korean Med Sci.  2005 Jun;20(3):524-527. 10.3346/jkms.2005.20.3.524.

A Case of Femoral Compressive Neuropathy in AL Amyloidosis

Affiliations
  • 1Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea. leb7616@snu.ac.kr

Abstract

We describe a case of a 54-yr-old AL amyloidosis patient who developed femoral compressive neuropathy due to iliopsoas pseudohypertrophy. The patient, who presented with end stage renal disease, was referred to our clinic because of lower extremity weakness and polyarthritis. Finally, he was diagnosed as having kappa-AL amyloidosis, complicated by femoral compressive neuropathy, hypertrophic amyloid myopathy, amyloid arthropathy, carpal tunnel syndrome, and end stage renal disease. Femoral compressive neuropathy has never been reported in association with amyloid induced iliopsoas hypertrophic myopathy. This report expands the clinical spectrum of AL amyloidosis.

Keyword

Amyloidosis; Muscle Weakness; Femoral Neuropathy

MeSH Terms

Amyloidosis/*pathology
Fatal Outcome
Femoral Neuropathy/*pathology
Humans
Hypertrophy
Kidney Failure, Chronic/pathology
Male
Middle Aged
Nerve Compression Syndromes/*pathology
Psoas Muscles/pathology

Figure

  • Fig. 1 Immunofluorescent staining of the shoulder pad biopsy shows diffuse, strong deposits of kappa light chains (×200).

  • Fig. 2 Pelvis MRI. Enhanced T1-weighted (A) and T2-weighted (B) images, demonstrating pseudohypertrophied iliopsoas muscles (arrow heads) compressing femoral nerves (arrows).

  • Fig. 3 Endomysial and perimysial deposition of amyloid in the iliopsoas muscle. Congo red with light microscopy (A) and with polarizing microscopy (B) (×100).


Reference

1. Cohen AS. Amyloidosis. Bull Rheum Dis. 1991. 40:1–12.
2. Falk RH, Comenzo RL, Skinner M. The systemic amyloidoses. N Engl J Med. 1997. 337:898–909.
Article
3. Mandl LA, Folkerth RD, Pick MA, Weinblatt ME, Gravallese EM. Amyloid myopathy masquerading as polymyositis. J Rheumatol. 2000. 27:949–952.
4. Hull KM, Griffith L, Kuncl RW, Wigley FM. A deceptive case of amyloid myopathy: clinical and magnetic resonance imaging features. Arthritis Rheum. 2001. 44:1954–1958.
Article
5. Metzler JP, Fleckenstein JL, White CL 3rd, Haller RG, Frenkel EP, Greenlee RG Jr. MRI evaluation of amyloid myopathy. Skeletal Radiol. 1992. 21:463–465.
Article
6. Gertz MA, Kyle RA. Myopathy in primary systemic amyloidosis. J Neurol Neurosurg Psychiatry. 1996. 60:655–660.
Article
7. Fautrel B, Fermand JP, Sibilia J, Nochy D, Rousselin B, Ravaud P. Amyloid arthropathy in the course of multiple myeloma. J Rheumatol. 2002. 29:1473–1481.
8. Floege J, Ketteler M. Beta2-microglobulin-derived amyloidosis: an update. Kidney Int Suppl. 2001. 78:S164–S171.
Article
9. Lachmann HJ, Booth DR, Booth SE, Bybee A, Gilbertson JA, Gillmore JD, Pepys MB, Hawkins PN. Misdiagnosis of hereditary amyloidosis as AL (primary) amyloidosis. N Engl J Med. 2002. 346:1786–1791.
Article
10. Holmgren G, Ericzon BG, Groth CG, Steen L, Suhr O, Andersen O, Wallin BG, Seymour A, Richardson S, Hawkins PN. Clinical improvement and amyloid regression after liver transplantation in hereditary transthyretin amyloidosis. Lancet. 1993. 341:1113–1116.
Article
11. Yoon Y, Chang S, Ham D, Lee K, An B, Kwon O, Park E. A case of familial amyloid neuropathy presenting as autonomic failure. J Korean Neurol Assoc. 1995. 13:341–346.
12. Buxbaum JN, Jacobson DR. Beutler E, Lichtman MA, Coller BS, Kipps TJ, Seligsohn U, editors. The amyloidoses. Williams hematology. 2001. 6th edition. New York: McGraw-Hill;1305–1316.
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