Recurrent Guillain-Barré Syndrome – Case Series
Correspondence Address: Source of Support: None, Conflict of Interest: None DOI: 10.4103/0028-3886.273649
Source of Support: None, Conflict of Interest: None
Keywords: Disability, Guillain-Barré syndrome, recurrent episode, variants
Guillain-Barré syndrome (GBS) is an autoimmune disorder of the peripheral nerves and their roots, which is presented as progressive muscle weakness and areflexia. Although the common feature of this inflammatory neuropathy is a monophasic course, in some GBS patients recurrence is noted. Recurrent GBS (RGBS) is observed in around 1–6% of patients with a history of GBS and could lead to the accumulation of the neurological deficit., Since there is still a lack of information about the main features of this rare disorder, the aim was to identify clinical features of RGBS in a large cohort of adult GBS patients.
During a 6-year period, 404 GBS patients were hospitalized in seven tertiary health care centers of three Balkan countries: Serbia, Bosnia and Herzegovina (Republic of Srpska), and Montenegro. The Ethical Board of the Neurology Clinic approved this study, and all patients signed the informed consent. The diagnosis of GBS was made if patients fulfilled the standard National Institute of Neurological Diseases and Stroke (NINDS) criteria. A definitive diagnosis of GBS subtypes was made according to the clinical and electrophysiological findings. Sociodemographic and clinical data (including age, gender, preceding factors, functional disability at nadir and on discharge, cerebrospinal fluid analysis, and therapeutic modalities) were obtained from medical records for actual and previous episodes. The degree of functional disability and the severity of each episode were graded using the GBS disability scale (GDS). The GDS ranges from 0 (no symptoms) to 6 (lethal outcome), where >3 was defined as a severe disability. RGBS was defined according to suggestions by Kuitwaard et al.
In this study, methods of descriptive statistics were used including mean, standard deviation, median, minimum and maximum.
Among 404 patients with GBS, 20 had a previous acute episode of polyradiculoneuritis. However, five patients were excluded after the follow-up period, since they were diagnosed with acute-onset chronic inflammatory demyelinating polyradiculoneuropathy and two patients were excluded due to their comorbidities. The final number of 13 (3.2%) RGBS patients were included.
Of our 13 patients, 10 were males (76.9%) and three were females (23.1%). All of them had two episodes of GBS. The mean age during the first GBS episode was 40.8 ± 22.5 years while during the second episode it was 53.6 ± 12.5 years. The mean interval between episodes was 12.9 ± 15.1 years. The features of 26 episodes of our 13 RGBS cases are summarized in [Table 1].
A preceding event appeared 10.0 ± 8.4 (median 8.5) days before the first GBS episode and 7.7 ± 4.3 (median 7) days before GBS recurrence (P > 0.05). In 15.4% of patients, the preceding event was the same before the first and the second attack.
The most common GBS variant was acute inflammatory demyelinating polyradiculoneuropathy (AIDP) both at the first and recurrent episodes (76.9% at the first and 84.6% in the second episode). Moreover, 23.1% of patients presented with a different variant during the second GBS attack while 76.9% had the same GBS variant during both attacks of the disease. The mean value of cerebrospinal fluid (CSF) proteins at the first GBS attack was 1.4 ± 0.6 g/L, while it was 1.7 ± 1.3 g/L during the disease recurrence (P > 0.05). The average pleocytosis value during the first GBS episode was 2.2 ± 2.2 mm 3, while during the recurrence of GBS it was 4.5 ± 7.6 mm 3 (P > 0.05). None of our RGBS patients had an autoimmune disease as a comorbid disorder.
The degree of functional disability of our patients was similar at both attacks, with GDS at nadir being 4.0 ± 0.6 (median 4) vs. 3.8 ± 1.2 (median 4) (P > 0.05) and at discharge 2.9 ± 0.8 (median 3) vs. 2.9 ± 0.9 (median 3) (P > 0.05), respectively. Severe functional disability was found in 84.6% of our patients at nadir during the first GBS attack and in 76.9% at nadir during recurrent GBS (P > 0.05). On discharge, severe disability was observed in 23.1% of patients both at the first and second GBS attack [Table 1].
Intravenous immunoglobulin therapy (IVIg) was directed in 38.5% of patients at the first and in 92.3% of patients during the second attack. Therapeutic plasma exchange (PLEx) was admitted in 23.1% of patients during the first attack and it was not administered during the second attack of GBS.
Although acute polyradiculoneuritis typically has a monophasic course, around 3% of our GBS patients experienced a recurrent variant of this rare disease. Similarly, previous literature data showed that RGBS occurred in 1–6% of patients with GBS.,, About three-quarters of our RGBS patients were males with a male to female ratio 3:1, and this ratio seems to be higher than in first-time GBS, suggesting that males with GBS might be at higher risk of recurrence., Mean age at the first GBS episode was about 40 years. The median duration between episodes of GBS in our cohort was 6 years, which fits in a wide range from 3.3–9.7 years showed in previous studies.,,
The cause of recurrence is still uncertain, especially since different preceding events can be present before attacks and different GBS variants can affect the same patient as shown in our cohort. Therefore, individual immunological differences and potential clustering of autoimmune disorders combined with genetic host factors , could modulate the well-known antiganglioside antibodies and molecular mimicry mechanisms.
The most common GBS variant in our cohort, both during the first and recurrent episodes was AIDP (77–85%). These findings are similar to earlier literature data. Previous studies indicate that patients who experienced RGBS had similar disease severity during different episodes. A similar finding was observed in our cohort. It is possible that our patients did not have more severe disability during the second GBS episode since they received IVIg/PLEx therapy more frequently during the recurrent episode.
Although GBS is generally a monophasic disease, around 3% of our patients with GBS have already had a episode of GBS. Almost one-quarter of our patients presented with a different variant during the second GBS attack. The degree of disability was similar during the first and the recurrent episode of the disease, but immunomodulatory therapy was more frequently applied during RGBS.
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Conflicts of interest
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