PMID- 19715171
OWN - NLM
STAT- MEDLINE
DCOM- 20091109
LR  - 20190917
IS  - 0009-918X (Print)
IS  - 0009-918X (Linking)
VI  - 49
IP  - 7
DP  - 2009 Jul
TI  - [Anti-MuSK antibody-positive myasthenia gravis with nephrotic syndrome: a case 
      report].
PG  - 424-7
AB  - A 40-year-old man was admitted to our hospital because of dysarthria, difficulty 
      swallowing, double vision and weakness of both upper extremities. There were no 
      detectable anti-AChR antibodies. He was diagnosed with seronegative myasthenia 
      gravis (SNMG) based on a positive edrophonium test and positive waning on 
      repetitive stimulation. Thereafter serological examination detected 
      anti-muscle-specific kinase (MuSK) antibodies and he was diagnosed with anti-MuSK 
      antibody-positive MG. Three years after the onset, the patient developed rapidly 
      progressing respiratory failure and fever. He was diagnosed with aspiration 
      pneumonia caused by swallowing difficulty. He was treated with mechanical 
      ventilation, plasma exchange and antibiotics. Laboratory tests on admission also 
      demonstrated nephrotic syndrome. Renal biopsy specimens showed diffuse thickening 
      of the basement membrane by PAS and PAM stain, and granular immunofluorescent 
      deposits of IgG4 along the glomerular capillary walls. Therefore, he was also 
      diagnosed with membranous nephropathy in addition to anti-MuSK antibody-positive 
      MG. MG is sometimes complicated with nephrotic syndrome, however there has been 
      no report of anti-MuSK-antibody positive MG complicated with nephrotic syndrome. 
      It has been reported that anti-MuSK-antibodies are IgG4 and that membranous 
      nephropathy is suggested to be an IgG4 mediated disease. Our findings suggest 
      that IgG4 may play an important role in the pathogenesis of our patient.
FAU - Shioyama, Mitsuaki
AU  - Shioyama M
AD  - Department of Internal Medicine, Shinosaka Hospital.
FAU - Ueda, Haruo
AU  - Ueda H
FAU - Aomatsu, Hiromi
AU  - Aomatsu H
FAU - Mitui, Yoshiyuki
AU  - Mitui Y
FAU - Naiki, Yoshito
AU  - Naiki Y
FAU - Samejima, Kenichi
AU  - Samejima K
FAU - Funauchi, Masanori
AU  - Funauchi M
FAU - Shiraishi, Hirokazu
AU  - Shiraishi H
FAU - Motomura, Masakatsu
AU  - Motomura M
FAU - Kusunoki, Susumu
AU  - Kusunoki S
LA  - jpn
PT  - Case Reports
PT  - English Abstract
PT  - Journal Article
PL  - Japan
TA  - Rinsho Shinkeigaku
JT  - Rinsho shinkeigaku = Clinical neurology
JID - 0417466
RN  - 0 (Autoantibodies)
RN  - 0 (Receptors, Cholinergic)
RN  - EC 2.7.10.1 (MUSK protein, human)
RN  - EC 2.7.10.1 (Receptor Protein-Tyrosine Kinases)
SB  - IM
MH  - Adult
MH  - Autoantibodies/blood
MH  - Humans
MH  - Male
MH  - Myasthenia Gravis/*complications/*immunology
MH  - Nephrotic Syndrome/*complications
MH  - Receptor Protein-Tyrosine Kinases/*immunology
MH  - Receptors, Cholinergic/*immunology
EDAT- 2009/09/01 06:00
MHDA- 2009/11/10 06:00
CRDT- 2009/09/01 09:00
PHST- 2009/09/01 09:00 [entrez]
PHST- 2009/09/01 06:00 [pubmed]
PHST- 2009/11/10 06:00 [medline]
AID - 10.5692/clinicalneurol.49.424 [doi]
PST - ppublish
SO  - Rinsho Shinkeigaku. 2009 Jul;49(7):424-7. doi: 10.5692/clinicalneurol.49.424.