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Abstract
Clinical, electrophysiological and pathological findings in 23 patients with subacute
and relapsing idiopathic demyelinating polyneuropathies are described. In 17 patients
with relapsing polyneuropathy, the neurological illness was unaccompanied by any systemic
disturbances. The term preferred for the neuropathy in this group of patients is chronic
relapsing polyneuritis. The findings in this group suggest that the common form of
this syndrome is due to a single disease entity. Chronic relapsing polyneuritis differs
from acute idiopathic polyneuritis chiefly in regard to the rate of evolution and
the severity of the initial episode of polyneuropathy. If these two polyneuropathies
have the same pathogenesis, the factor which determines whether the disease is acute
and self-limiting or chronically relapsing is often present at the time of onset of
the disease. The relationship of chronic relapsing polyneuritis to relapsing hypertrophic
polyneuropathy and progressive hypertrophic polyneuropathy is also discussed and it
is concluded that these diseases may constitute a spectrum of pathogenetically related
disorders. In chronic relapsing polyneuritis, as in other demyelinating polyneuropathies,
a marked segmental reduction in axon diameter accompanies demyelination. This corresponds
to a more than 50% reduction in the volume of the affected region of the axon and
it is associated with increased packing of axoplasmic organelles and wrinkling of
the axolemma. It is suggested that in the normal myelinated nerve fibre, the Schwann
cell and myelin sheath maintain fluid locally within the axon.
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Article info
Publication history
Received:
July 25,
1975
Footnotes
☆This study was supported by the Wellcome Trust and the National Health and Medical Research Council of Australia.
Identification
Copyright
© 1976 Published by Elsevier Inc.