TY - JOUR
T1 - Fisher-pharyngeal-cervical-brachial overlap syndrome with novel ganglioside antibodies
AU - Pedavally, Swetha
AU - Hernández, Zulma M.
AU - Zeidman, Lawrence A.
N1 - Publisher Copyright:
Copyright © 2018 Wolters Kluwer Health, Inc. All rights reserved.
PY - 2018
Y1 - 2018
N2 - Several variants of Guillain-Barré syndrome have been described. The Fisher syndrome (FS) presents with ataxia, areflexia, and ophthalmoparesis. The pharyngeal-cervical-brachial (PCB) variant presents with bulbar weakness, along with arm and neck weakness. The 2 variant syndromes can overlap. Both the isolated and overlap syndromes respond to immunomodulatory treatment, thus are important to recognize clinically. Ganglioside antibodies are detectable in the variant syndromes and may aid in their diagnosis. The FS typically is associated with anti-GQ1b antibodies, and PCB is typically associated with anti-GT1a antibodies, whereas the overlap syndrome may have both ganglioside antibody subtypes. We present a case of overlap FS-PCB syndrome with a novel ganglioside antibody profile of GM1 and GD1b antibodies, which typically are associated with other variant syndromes. This case suggests the need for all ganglioside antibodies to be tested in suspected variant Guillain-Barré syndromes. The antibodies may prove especially useful in cases in which the clinical diagnosis is ambiguous.
AB - Several variants of Guillain-Barré syndrome have been described. The Fisher syndrome (FS) presents with ataxia, areflexia, and ophthalmoparesis. The pharyngeal-cervical-brachial (PCB) variant presents with bulbar weakness, along with arm and neck weakness. The 2 variant syndromes can overlap. Both the isolated and overlap syndromes respond to immunomodulatory treatment, thus are important to recognize clinically. Ganglioside antibodies are detectable in the variant syndromes and may aid in their diagnosis. The FS typically is associated with anti-GQ1b antibodies, and PCB is typically associated with anti-GT1a antibodies, whereas the overlap syndrome may have both ganglioside antibody subtypes. We present a case of overlap FS-PCB syndrome with a novel ganglioside antibody profile of GM1 and GD1b antibodies, which typically are associated with other variant syndromes. This case suggests the need for all ganglioside antibodies to be tested in suspected variant Guillain-Barré syndromes. The antibodies may prove especially useful in cases in which the clinical diagnosis is ambiguous.
KW - Anti-GM1 IGM
KW - Fisher syndrome
KW - Guillain- Barré syndrome
KW - Intravenous immunoglobulin
KW - Miller Fisher syndrome
KW - Pharyngeal-cervical-brachial variant Guillain-Barré syndrome
KW - anti-GD1b IGM
UR - http://www.scopus.com/inward/record.url?scp=85051834390&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85051834390&partnerID=8YFLogxK
U2 - 10.1097/CND.0000000000000205
DO - 10.1097/CND.0000000000000205
M3 - Article
C2 - 29794578
AN - SCOPUS:85051834390
SN - 1522-0443
VL - 19
SP - 224
EP - 227
JO - Journal of Clinical Neuromuscular Disease
JF - Journal of Clinical Neuromuscular Disease
IS - 4
ER -