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Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired immune-mediated inflammatory disease, which may have a chronic progressive or recurrent process
.
Diagnosis depends on a combination of clinical and neurophysiological characteristics
Some studies have also analyzed a single IgG band in cerebrospinal fluid, which is more common in central nervous system inflammatory diseases, and may become OCB during follow-up, especially in multiple sclerosis
.
A single IgG band in cerebrospinal fluid has also been reported in a small number of patients with peripheral nervous system diseases
The purpose of the study is to investigate the cerebrospinal fluid characteristics of continuous CIDP patients observed at the time of diagnosis, including currently established laboratory parameters and less investigated OCB, to determine characteristics that may help diagnosis and/or prognosis
From September 2004 to September 2019, 48 patients (34 men, average age 59.
4±15.
5 years, range 16-83 years) were evaluated at the Padua Neuropathy Center, according to the European Federation of Neurological Societies / Peripheral The neurological society guidelines diagnosed clear CIDP, and 2 patients were considered
.
Of these patients, 33 had typical CIDP and 15 atypical variants (4 cases of multifocal acquired demyelinating sensory and motor (MADSAM) neuropathy, 3 cases of pure motor CIDP, 3 cases of pure sensory CIDP, and 3 cases) Distal acquired demyelinating symmetric neuropathy and 2 cases of focal CIDP)
After written informed consent, before starting any treatment, cerebrospinal fluid analysis was performed on first admission
.
As a control, we considered 86 consecutive patients, 32 GBS patients, 18 anti-MAG antibody neuropathy, 4 multifocal motor neuropathy, and 32 other NINP patients (19 axonal idiopathic neuropathy, 5 Anti-Hu antibody-negative sensory neuropathy, 1 case of hereditary neuropathy, 1 case of diabetic neuropathy, 1 case of schwannomatosis, 1 case of small fiber neuropathy, 4 cases of other demyelinating neuropathy; average age at recruitment They were 61.
CSF was collected by non-traumatic lumbar puncture in the sitting position
.
Routine examinations of paired CSF and serum samples include: CSF red blood cell count, CSF white blood cell and differentiated cell count, CSF total protein concentration, albumin and IgG determination, and search for OCBs, using agarose isoelectric focusing (IEF) method, and then transfer to nitrification On the cellulose membrane, IgG is specifically immunofixed and amplified with avidin-biotin and peroxidase staining
Isoelectric focusing results of matched serum and cerebrospinal fluid specimens in two CIDP patients
The interpretation of the OCBs model is based on the current guidelines for the study of cerebrospinal fluid in neuroinflammatory diseases of the central nervous system
.
For the purpose of this study, the following pathological patterns were considered: (a) CSF restricted OCBs (pattern 2: presence of intrathecal IgG synthesis) (b) the same OCB in serum and cerebrospinal fluid (pattern 4: mirror mode, whole body OCB production) , No intrathecal IgG synthesis) and (c) identification of the same OCB in serum and cerebrospinal fluid, with regular and periodic intervals, with reduced intensity (pattern 5: paraprotein pattern, presence of monoclonal gammopathy)
In addition, CIDP group (26/48 cases, 54.
2%, p=0.
0009), GBS group (23/32 cases, 71.
9%, p<0.
0001) and anti-MAG group (10/18 cases, 55.
5%, p=0.
0101) The increase rate of cerebrospinal fluid protein was higher than that of the NINPs group (5/32 cases, 15.
6%)
.
The cerebrospinal fluid albumin concentration of CIDP and GBS patients was significantly increased (p=0.
Compared with NINP, CIDP, GBS and anti-MAG antibody neuropathy have significantly increased CSF protein and blood-spinal nerve root barrier damage
.
Intrathecal humoral immune response is rare in CIDP patients
.
Systemic oligoclonal activation is more frequent, but there is no significant difference from the control group
.
Ruiz M , Puthenparampil M , Campagnolo M , et alOligoclonal IgG bands in chronic inflammatory polyradiculoneuropathies
Journal of Neurology, Neurosurgery & Psychiatry Published Online First: 13 April 2021.
doi: 10.
1136/jnnp-2020-325868
doi: 10.
1136/jnnp-2020-325868
Isoelectric focusing results of matched serum and cerebrospinal fluid specimens in two CIDP patients
The interpretation of the OCBs model is based on the current guidelines for the study of cerebrospinal fluid in neuroinflammatory diseases of the central nervous system
.
For the purpose of this study, the following pathological patterns were considered: (a) CSF restricted OCBs (pattern 2: presence of intrathecal IgG synthesis) (b) the same OCB in serum and cerebrospinal fluid (pattern 4: mirror mode, whole body OCB production) , No intrathecal IgG synthesis) and (c) identification of the same OCB in serum and cerebrospinal fluid, with regular and periodic intervals, with reduced intensity (pattern 5: paraprotein pattern, presence of monoclonal gammopathy)
.
At the time of spinal cord puncture, the average duration of CIDP patients was 2.
9±4.
6 years, the median INCAT disability score was 2.
0 (range 0-7), and the median I-RODS was 14 (average 16.
1, range 2-38)
.
Compared with the NINPs group, the CSF protein concentration of the CIDP group and the NINPs group both increased (35.
1±10.
0) mg/dL vs 63.
4±58.
2 mg/dL, p=0.
032) and GBS (76.
8±44.
5) mg/dL, p =0.
0013)
.
In addition, CIDP group (26/48 cases, 54.
2%, p=0.
0009), GBS group (23/32 cases, 71.
9%, p<0.
0001) and anti-MAG group (10/18 cases, 55.
5%, p=0.
0101) The increase rate of cerebrospinal fluid protein was higher than that of the NINPs group (5/32 cases, 15.
6%)
.
The cerebrospinal fluid albumin concentration of CIDP and GBS patients was significantly increased (p=0.
044) p<0.
0001))
.
CIDP patients (18 mild, 6 moderate, 2 severe), GBS patients (10 mild, 13 moderate, 1 severe), NINPs (15.
6%) and anti-MAG antibody neuropathy patients B-SNR-B injury occurred in 26/48 (54.
2%) (8 cases of mild and 4 cases of moderate)
.
In terms of CSF protein concentration (p=0.
27), QALB (p=0.
13) and QALB ratio (p=0.
29), and BNB injury frequency (p=0.
77), no differences were observed between typical and atypical CIDP
.
A strong correlation between CSF protein concentration and QALB ratio was observed in all groups: CIDP (r:0.
89, r2:0.
79, p<0.
0001), GBS (r:0.
71, r2:0.
50, p<0.
0001), anti- MAG antibody neuropathy (r:0.
92, r2:0.
84, p<0.
0001) and NINPs (r:0.
82, r2:0.
68, p<0.
0001)
.
Compared with NINP, CIDP, GBS and anti-MAG antibody neuropathy have significantly increased CSF protein and blood-spinal nerve root barrier damage
.
Intrathecal humoral immune response is rare in CIDP patients
.
Systemic oligoclonal activation is more frequent, but there is no significant difference from the control group
.
Ruiz M , Puthenparampil M , Campagnolo M , et alOligoclonal IgG bands in chronic inflammatory polyradiculoneuropathies
Journal of Neurology, Neurosurgery & Psychiatry Published Online First: 13 April 2021.
doi: 10.
1136/jnnp-2020-325868
doi: 10.
1136/jnnp-2020-325868
Isoelectric focusing results of matched serum and cerebrospinal fluid specimens in two CIDP patients
The interpretation of the OCBs model is based on the current guidelines for the study of cerebrospinal fluid in neuroinflammatory diseases of the central nervous system
.
For the purpose of this study, the following pathological patterns were considered: (a) CSF restricted OCBs (pattern 2: presence of intrathecal IgG synthesis) (b) the same OCB in serum and cerebrospinal fluid (pattern 4: mirror mode, whole body OCB production) , No intrathecal IgG synthesis) and (c) identification of the same OCB in serum and cerebrospinal fluid, with regular and periodic intervals, with reduced intensity (pattern 5: paraprotein pattern, presence of monoclonal gammopathy)
.
At the time of spinal cord puncture, the average duration of CIDP patients was 2.
9±4.
6 years, the median INCAT disability score was 2.
0 (range 0-7), and the median I-RODS was 14 (average 16.
1, range 2-38)
.
Compared with the NINPs group, the CSF protein concentration of the CIDP group and the NINPs group both increased (35.
1±10.
0) mg/dL vs 63.
4±58.
2 mg/dL, p=0.
032) and GBS (76.
8±44.
5) mg/dL, p =0.
0013)
.
In addition, CIDP group (26/48 cases, 54.
2%, p=0.
0009), GBS group (23/32 cases, 71.
9%, p<0.
0001) and anti-MAG group (10/18 cases, 55.
5%, p=0.
0101) The increase rate of cerebrospinal fluid protein was higher than that of the NINPs group (5/32 cases, 15.
6%)
.
The cerebrospinal fluid albumin concentration of CIDP and GBS patients was significantly increased (p=0.
044) p<0.
0001))
.
CIDP patients (18 mild, 6 moderate, 2 severe), GBS patients (10 mild, 13 moderate, 1 severe), NINPs (15.
6%) and anti-MAG antibody neuropathy patients B-SNR-B injury occurred in 26/48 (54.
2%) (8 cases of mild and 4 cases of moderate)
.
In terms of CSF protein concentration (p=0.
27), QALB (p=0.
13) and QALB ratio (p=0.
29), and BNB injury frequency (p=0.
77), no differences were observed between typical and atypical CIDP
.
A strong correlation between CSF protein concentration and QALB ratio was observed in all groups: CIDP (r:0.
89, r2:0.
79, p<0.
0001), GBS (r:0.
71, r2:0.
50, p<0.
0001), anti- MAG antibody neuropathy (r:0.
92, r2:0.
84, p<0.
0001) and NINPs (r:0.
82, r2:0.
68, p<0.
0001)
.
Compared with NINP, CIDP, GBS and anti-MAG antibody neuropathy have significantly increased CSF protein and blood-spinal nerve root barrier damage
.
Intrathecal humoral immune response is rare in CIDP patients
.
Systemic oligoclonal activation is more frequent, but there is no significant difference from the control group
.
.
Intrathecal humoral immune response is rare in CIDP patients
.
Systemic oligoclonal activation is more frequent, but there is no significant difference from the control group
.
Ruiz M , Puthenparampil M , Campagnolo M Ruiz M Ruiz Puthenparampil M Puthenparampil Campagnolo M Campagnolo , et al Oligoclonal IgG bands in chronic inflammatory polyradiculoneuropathies Journal of Neurology, Neurosurgery & Psychiatry Published Online First: 13 April 2021.
Published Online First: doi: 10.
1136/ jnnp-2020-325868 doi: leave a message here