A Clinicopathological Study of the Guillain-Barre Syndrome: Nine Cases and Literature Review

Abstract

The postmortem findings are reported from 9 cases of the Guillain-Barré syndrome with survival between 10 days and 1 yr. In 8 cases there was multifocal loss of myelin throughout the peripheral nervous system with relative preservation of axons. In 1 case there was predominant loss of axons. Inflammatory mononuclear cell infiltration was present in the peripheral nervous system of all cases except 1 case surviving a year. The extent and severity of cell infiltration was variable, usually being less prominent than in previous reports, and sometimes sparing nerves in which myelin destruction was severe. Vesicular dissolution of myelin noted by electron microscopy was considered to be a postmortem artefact. In cases examined within 30 days after the onset, immunohistochemical studies with monoclonal antibodies identified more leucocytes (PD7/2B11+) and T cells (UCHL1+) in the endoneurium than in cases examined later or control cases. These findings and recent single case reports indicate that the pathology of the Guillian-Barré syndrome is variable. This variability may reflect differences in pathogenesis, with greater cell-mediated immunity in some cases and greater antibody targeted macrophage-mediated demyelination in others.

Muscle Pathology in the Early Stage of Guillain-Barré Syndrome

“…Although numerous pathological studies have been performed on nervous tissue in GBS [6][7][8][9][10], very few detailed skeletal muscle pathologies in this disorder have been studied. We performed light and electron microscopic examinations of muscles from patients with the early course of GBS and from controls, and found some striking findings not previously reported in GBS or not commonly found in neurogenic atrophy.…”

Guillain-Barré Syndrome and Related Disorders

“…Pathological material from patients with GBS shows a similar accumulation of lymphocytes and macrophages in a perivascular distribution scattered throughout the peripheral nervous system with a predilection for spinal roots [39][40][41] . Macrophages and T cells express major histocompatibility (MHC) class II antigens which are upregulated on Schwann cells in the region of in fl ammatory lesions in patients with GBS.…”

“…In classical pathological studies of GBS, demyelination was most prominent adjacent to regions of intense perivenular in fl ammation [ 39,40 ] . Pathological material from patients with GBS shows a similar accumulation of lymphocytes and macrophages in a perivascular distribution scattered throughout the peripheral nervous system with a predilection for spinal roots [39][40][41] .…”

Guillain-Barré as an Autoimmune Disease

“…Several other observations regarding the association of autoantibodies and GBS are noteworthy. (I) Some authors described deposits of IgM and IgG in peripheral nerve tis sue fibers [11,38], while others [39,40] failed to observe similar findings. (2) No class switching from IgM to IgG was observed [29] in myelin autoantibodies, to mark the possible generation o f an immunologic memory.…”