TY - JOUR
T1 - Chronic progressive steroid responsive axonal polyneuropathy
T2 - A CIDP variant or a primary axonal disorder?
AU - Uncini, Antonino
AU - Sabatelli, Mario
AU - Mignogna, Teresa
AU - Lugaresi, Alessandra
AU - Liguori, Rocco
AU - Montagna, Pasquale
PY - 1996/3
Y1 - 1996/3
N2 - Five patients presented with chronic, progressive, predominantly motor polyneuropathy, CSF protein content was increased in 4 patients. Motor conduction velocities and EMG were consistent with axonal involvement. Sural nerve conductions were normal in all cases and sural nerve biopsy performed in 1 patient was normal. Serum antibodies to GM1, GD(1a), GD(1b), and GM2 were negative. All patients improved after steroid treatment and 3 completely recovered. Because of therapeutic implications it is important to differentiate these patients from those with chronic idiopathic axonal neuropathies. It is unclear whether this is a primary axonal, probably immune-mediated, polyneuropathy, or whether it represents one extreme of the chronic inflammatory demyelinating polyradiculoneuropathy spectrum characterized by severe axonal loss. We suggest that the term 'chronic inflammatory polyneuropathy,' encompassing cases from pure demyelinating to pure axonal neuropathies responsive to steroids, should be reinstated and that, like in Guillain-Barre syndrome, different subtypes should be individuated.
AB - Five patients presented with chronic, progressive, predominantly motor polyneuropathy, CSF protein content was increased in 4 patients. Motor conduction velocities and EMG were consistent with axonal involvement. Sural nerve conductions were normal in all cases and sural nerve biopsy performed in 1 patient was normal. Serum antibodies to GM1, GD(1a), GD(1b), and GM2 were negative. All patients improved after steroid treatment and 3 completely recovered. Because of therapeutic implications it is important to differentiate these patients from those with chronic idiopathic axonal neuropathies. It is unclear whether this is a primary axonal, probably immune-mediated, polyneuropathy, or whether it represents one extreme of the chronic inflammatory demyelinating polyradiculoneuropathy spectrum characterized by severe axonal loss. We suggest that the term 'chronic inflammatory polyneuropathy,' encompassing cases from pure demyelinating to pure axonal neuropathies responsive to steroids, should be reinstated and that, like in Guillain-Barre syndrome, different subtypes should be individuated.
KW - antiganglioside antibodies
KW - chronic axonal polyneuropathy
KW - chronic idiopathic neuropathy
KW - chronic inflammatory demyelinating polyradiculoneuropathy
KW - steroid treatment
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U2 - 10.1002/(SICI)1097-4598(199603)19:3<365::AID-MUS14>3.0.CO;2-R
DO - 10.1002/(SICI)1097-4598(199603)19:3<365::AID-MUS14>3.0.CO;2-R
M3 - Article
C2 - 8606703
AN - SCOPUS:0030042906
VL - 19
SP - 365
EP - 371
JO - Muscle and Nerve
JF - Muscle and Nerve
SN - 0148-639X
IS - 3
ER -