[A case of slowly progressive "pharyngeal-cervical-brachial variant of Guillain-Barré syndrome (PCB)"].
A 69-year-old woman was admitted to our hospital because of slowly progressive weakness in the neck, shoulders, proximal arms, oropharyngeal muscles. From a viewpoint of clinical course and signs, it was suspected that the patient was suffered from motor neuron disease. However, serial electrophysiological studies showed the existence of local demyelination of the motor nerves. The immunoadsorption was then performed and marked recovery of symptoms was obtained. In this case, we could not detect any established anti-ganglioside antibodies which was related to pharyngeal-cervical-brachial variant of Guillain-Barré syndrome(PCB) or Guillain-Barré syndrome (GBS). It is suggested that unknown anti-ganglioside antibody may play an important role in cases of PCB. Despite of atypical slowliness of clinical progression and negative results of immunological studies, this patient is considered to be suffered from PCB, because of the results of electrophysiological studies and effectiveness of immunoadsorption therapy. Accordingly it may be important to take the possibility of PCB into diagnostic consideration, whenever the patient shows slowly progressive weakness in proximal arms, oropharyngeal muscles.
Kubo
J
J
Department of Neurology, Mihara Memorial Hospital, Instituete of Brain and Vessels, 366 Ohtamachi, Isesaki, Gunma 372-0006, Japan.
Hozumi
A
A
Mihara
B
B
Gomi
S
S
Hirata
K
K
D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000175:diagnosis; D001323:Autoantibodies / Q000032:analysis; D003937:Diagnosis, Differential; D005260:Female; D005732:Gangliosides / Q000276:immunology; D020275:Guillain-Barre Syndrome / Q000145:classification / Q000175:diagnosis* / Q000503:physiopathology; D006801:Humans; D009431:Neural Conduction
D001323:Autoantibodies; D005732:Gangliosides