Treatment-related fluctuations in Guillain-Barré syndrome​: clinical features and predictors of recurrence

ALESSANDRO, Lucas; CASTIGLIONE, Juan Ignacio; BRAND, Patricio; BRUNO, Veronica; BARROSO, Fabio

doi:10.1590/0004-282X-ANP-2021-0226

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Article • Arq. Neuro-Psiquiatr. 80 (5) • May 2022 • https://doi.org/10.1590/0004-282X-ANP-2021-0226 copy

Treatment-related fluctuations in Guillain-Barré syndrome: clinical features and predictors of recurrence

Fluctuaciones relacionadas al tratamiento en el Síndrome de Guillain-Barré: características clínicas y predictores de recurrencia

Authorship SCIMAGO INSTITUTIONS RANKINGS

ABSTRACT

Background:

A treatment-related fluctuation (TRF) in a patient with Guillain-Barré syndrome (GBS) is defined as clinical deterioration within two months of symptom onset following previous stabilization or improvements with treatment.

Objective:

To investigate the clinical characteristics and factors that could increase the risk of relapse of GBS in patients with and without TRFs.

Methods:

Retrospective review of medical records of patients (>18 years) with GBS evaluated between January/2006 and July/2019. Demographic and clinical characteristics, ancillary studies, treatment received, and the clinical course of patients with and without TRFs were analyzed.

Results:

Overall, 124 cases of GBS were included; seven (5.6%) presented TRFs. GBS-TRF cases were triggered more frequently by infectious mononucleosis (28.57 vs. 8.55%; p=0.01). GBS-TRF were initially treated with plasmapheresis more frequently than those without TRF (14.29 vs. 1.70%; p=0.0349). Combined treatment (71.43 vs. 4.27%; p<0.001) and corticosteroids (42.86 vs. 1.71%; p<0.001) were more commonly used in the GBS-TRF group. GBS-TRF patients presented a higher median initial disability score (4 vs. 2; p=0.01).

Conclusions:

Patients with GBS triggered by infectious mononucleosis and a high degree of initial disability have higher chances of developing TRFs. Although patients with TRF were treated with plasmapheresis more often, the total number was too low to suggest a link between plasma exchange and TRF.

Keywords:
Cytomegalovirus; Herpesvirus 4, Human; Guillain-Barré Syndrome; Infectious Mononucleosis

	Total (n=124)	GBS without TRF (n=117)	GBS with TRF (n=7)	p-value
Demographic data
Age in years; median (range)	48 (21-86)	48 (21-86)	53 (32-81)	0.51
Men; n (%)	71 (57.26)	67 (57.26)	4 (57.14)	0.99
Diabetes mellitus; n (%)	11 (8.87)	9 (7.69)	2 (28.57)	0.06
Autoimmune disease; n (%)	28 (22.58)	27(23.08)	1 (14.29)	0.59
Facial paralysis; n (%)	8 (6.45)	7 (5.98)	1 (14.29)	0.38
Oncological disease; n (%)	10 (8.06)	9 (7.69)	1 (14.29)	0.53
HIV; n (%)	5 (4.07)	5 (4.31)	0	0.57
Triggers; n (%)	86 (69.35)	82 (70.08)	4 (57.14)	0.47
Respiratory infection; n (%)	41(33.06)	40 (34.19)	1 (14.29)	0.27
Acute diarrhea; n (%)	31 (25.00)	30 (25.64)	1 (14.29)	0.50
Infectious mononucleosis; n (%)	8 (6.45)	6 (5.12)	2 (28.57)	0.01*
Vaccination; n (%)	2 (1.61)	2 (1.71)	0	0.72
Others; n (%)	4 (3.22)	4 (3.41)	0	0.62
Time from trigger to the first symptom; median days (range)	10 (1-30)	10 (1-30)	5 (3-10)	0.10
Time from trigger to weakness; median days (range)	11(1-40)	11(1-40)	7(3-37)	0.48
Clinical manifestations
Neuropathic pain; n (%)	78 (62.9)	71 (60.68)	7 (100)	0.03*
Neck MRC; median (range)	5 (0-5)	5	4	0.86
Worst MRC-Sum Score; median(range)	51 (0-60)	51	50	0.78
Sensory disturbances; n (%)	110 (88.71)	104 (88.89)	6 (85.71)	0.79
Paresthesia; n (%)	94 (77.69)	87 (76.32)	7 (100)	0.14
Cutaneous sensory; n (%)	50 (41.32)	46 (40.35)	4 (57.14)	0.38
Proprioceptive sensory; n (%)	44 (36.36)	42 (36.84)	2 (28.57)	0.66
Sensory ataxia; n (%)	25 (20.16)	23 (19.66)	2 (28.57)	0.56
Osteotendinous reflexes
Generalized areflexia; n (%)	66 (53.23)	61 (52.14)	5 (71.43)	0.32
Hyporeflexia/partial areflexia; n (%)	50 (40.32)	48 (41.03)	2 (28.57)	0.51
Normal; n (%)	8(6.45)61	8(6.48)	0	0.47
Autonomic dysfunctions; n (%)	43 (34.68)	40 (34.19)	3 (42.86)	0.64
Severe; n (%)	21 (16.94)	20 (17.09)	1 (14.29)	0.84
HR abnormality; n (%)	30 (24.19)	28 (23.93)	2 (28.57)	0.78
BP changes; n (%)	26 (20.97)	23 (19.66)	3 (42.86)	0.14
Gastrointestinal disturbances; n (%)	18 (14.52)	15 (12.82)	3 (42.86)	0.02*
Genitourinary disturbances; n (%)	15 (12.10)	14 (11.97)	1 (14.29)	0.85
Temperature dysregulation; n (%)	10 (8.06)	8 (6.84)	2 (28.57)	0.04*
CN involvement; n (%)	60 (48.39)	57 (48.72)	3 (42.86)	0.76
Ocular-motility; n (%)	11 (8.87)	10 (8.55)	1 (14.29)	0.60
Facial; n (%)	54 (43.55)	52 (44.44)	2 (28.57)	0.41
Bilateral facial; n (%)	30 (24.19)	28 (23.93)	2 (28.57)	0.78
Lower CN; n (%)	10 (8.06)	9 (7.69)	1 (14.29)	0.53
Multiple CN involvement; n (%)	15 (12.12)	14 (11.97)	1 (14.29)	0.85
CSF and electrophysiological variant characteristics
Albuminocytologic dissociation; n (%)	85 (69.67)	79 (68.70)	6 (85.71)	0.34
CSF protein level; mg/dL median (range)	79.9 (38.6-605)	79.9 (38.6-605)	90.94 (60-176)	0.47
Time between symptom onset and LP in days; median (range)	7 (1-45)	7 (1-45)	7(2-30)	0.60
EMG classification
AMAN; n (%)	4 (3.23)	4 (3.23)	0	0.62
AMSAN; n (%)	7 (5.65)	6 (5.13)	1 (14.29)	0.30
AIDP; n (%)	89 (71.77)	83 (70.94)	6 (85.71)	0.40
Unclassified/Normal; n (%)	18 (14.52)	18 (15.38)	0	0.26
Miller Fisher; n (%)	6 (4.84)	6 (5.13)	0	0.54
Treatment and evolution
No treatment requirements; n (%)	9 (7.26)	9 (7.69)	0	0.44
Treatment establishment time in days; median in days (range)	7 (1-45)	7 (1-45)	7(2-30)	0.64
Initial treatment
Immunoglobulin; n (%)	112 (90.32)	106 (90.59)	6 (85.71)	0.67
Plasmapheresis; n (%)	3 (2.41)	2 (1.70)	1 (14.29)	0.0349*
Combined treatment; n (%)	10 (8.06)	5 (4.27)	5 (71.43)	<0.001*
Corticosteroids; n (%)	5 (4.03)	2 (1.71)	3 (42.86)	<0.001*
Admission at ICU; n (%)	18 (14.52)	16 (13.68)	2 (28.57)	0.27
Days in ICU; median (range)	0 (0-120)	0 (0-120)	0 (0-10)	0.38
Orotracheal intubation; n (%)	10 (8.06)	9 (7.69)	1 (14.29)	0.53
Non-invasive ventilation; n (%)	5 (4.07)	5 (4.31)	0	0.57
Hemodynamic support; n (%)	6 (4.84)	5 (4.27)	1 (14.29)	0.23
Baseline GBS disability score; median (range)	2 (1-5)	2	4	0.01*
GBS disability score after 6 months; median (range)	0 (0-4)	0	1	0.08
GBS disability score after 1 year; median (range)	0 (0-4)	0	0	0.86
MRC-Sum Score at discharge; median (range)	56 (19-60)	56	53	0.84
Final MRC-Sum Score; median (range)	60 (20-60)	60	60	0.70
Mortality; n (%)	1 (0.81)	1 (0.85)	0	0.80
Follow-up in months; median (range)	36 (6-156)	36 (6-132)	27 (6-156)	0.47

N°/Age/Sex	T₁ (days)	Initial treatment	MRC₁/D₁	T_R (days)	T₂ (days)	Relapse treatment	Total n° of TRF	TT (days)	MRC₂/D₂	MRC₃/D₃
1/33/M	4	IVIg (2 g/kg)	45/4	4	16	1st relapse: IVIg (2 g/kg) 2nd relapse: corticosteroids	2	60	60/1	60/0
2/32/M	9	IVIg (2 g/kg)	52/2	14	2	IVIg (2 g/kg)	1	35	60/0	60/0
3/71/F	7	IVIg (2 g/kg)	58/3	30	20	IVIg (1 g/kg)	1	60	59/2	60/1
4/81/F	14	IVIg (2 g/kg)	52/4	2	25	PE (5 sessions)	1	44	53/ 2	-
5/53/M	2	PE (7 sessions)	39/5	15	23	IVIg (2 g/kg)	1	38	54/ 2	60/0
6/57/F	30	IVIg (2 g/kg)	50/3	4	8	IVIg (2 g/kg)+corticosteroids	1	54	60/1	60/1
7/46/M	3	IVIg (2 g/kg)	50/4	9	8	IVIg (2 g/kg)+corticosteroids (4 months)	1	30	58/1	60/0

Academia Brasileira de Neurologia - ABNEURO R. Vergueiro, 1353 sl.1404 - Ed. Top Towers Offices Torre Norte, 04101-000 São Paulo SP Brazil, Tel.: +55 11 5084-9463 | +55 11 5083-3876 - São Paulo - SP - Brazil
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[1] Correspondence: Lucas Alessandro; Email: lalessandro@fleni.org.ar.

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Treatment-related fluctuations in Guillain-Barré syndrome​: clinical features and predictors of recurrence

Fluctuaciones relacionadas al tratamiento en el Síndrome de Guillain-Barré: características clínicas y predictores de recurrencia

ABSTRACT

Background:

Objective:

Methods:

Results:

Conclusions:

Treatment-related fluctuations in Guillain-Barré syndrome: clinical features and predictors of recurrence