Bader, Sophie R.; Kothlow, Sonja; Trapp, Sascha; Schwarz, Susanne C. N.; Philipp, Hans-Christian; Weigend, Steffen; Sharifi, Ahmad R.; Preisinger, Rudolf; Schmahl, Wolfgang; Kaspers, Bernd; Matiasek, Kaspar
(28. January 2010):
Acute paretic syndrome in juvenile White
Leghorn chickens resembles late stages of acute
inflammatory demyelinating polyneuropathies in
In: Journal of Neuroinflammation, Vol. 7, No. 7
Background: Sudden limb paresis is a common problem in White Leghorn flocks, affecting about 1% of the
chicken population before achievement of sexual maturity. Previously, a similar clinical syndrome has been
reported as being caused by inflammatory demyelination of peripheral nerve fibres. Here, we investigated in detail
the immunopathology of this paretic syndrome and its possible resemblance to human neuropathies.
Methods: Neurologically affected chickens and control animals from one single flock underwent clinical and
neuropathological examination. Peripheral nervous system (PNS) alterations were characterised using standard
morphological techniques, including nerve fibre teasing and transmission electron microscopy. Infiltrating cells
were phenotyped immunohistologically and quantified by flow cytometry. The cytokine expression pattern was
assessed by quantitative real-time PCR (qRT-PCR). These investigations were accomplished by MHC genotyping and
a PCR screen for Marek’s disease virus (MDV).
Results: Spontaneous paresis of White Leghorns is caused by cell-mediated, inflammatory demyelination affecting
multiple cranial and spinal nerves and nerve roots with a proximodistal tapering. Clinical manifestation coincides
with the employment of humoral immune mechanisms, enrolling plasma cell recruitment, deposition of myelinbound
IgG and antibody-dependent macrophageal myelin-stripping. Disease development was significantly linked
to a 539 bp microsatellite in MHC locus LEI0258. An aetiological role for MDV was excluded.
Conclusions: The paretic phase of avian inflammatory demyelinating polyradiculoneuritis immunobiologically
resembles the late-acute disease stages of human acute inflammatory demyelinating polyneuropathy, and is
characterised by a Th1-to-Th2 shift.