Guillain Barre Syndrome Treatment Can You Get It Again

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An unusual case of recurrent Guillain-Barré syndrome with normal cerebrospinal fluid protein levels: a case study

• Rohitha Gamlathⁱ &
• Harith Wimalaratna¹

BMC Neurology book 16, Article number:161 (2016) Cite this commodity

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Abstract

Background

Guillain-Barré syndrome is an acquired polyradiculo-neuropathy, frequently preceded by an antecedent event. It is a monophasic disease but a recurrence charge per unit of 1–half dozen % is documented in a subset grouping of patients. Patients with Guillain-Barré syndrome show cerebrospinal fluid albuminocytologic dissociation. Normal cerebrospinal fluid protein levels during both initial and recurrent episodes of Guillain-Barré syndrome is a rare occurrence and has non been described earlier in the literature.

Case presentation

Twenty-v-yr-old Sri Lankan female with by history of consummate recovery following an astute inflammatory demyelinating polyneuropathy (AIDP) variant of Guillain-Barré syndrome 12 years dorsum presented with acute, ascending symmetrical flaccid quadriparasis extending to bulbar muscles, bilateral VII cranial nerves and respiratory compromise needing mechanical ventilation. Nerve conduction study revealed AIDP variant of Guillain-Barré syndrome. Cerebrospinal fluid analysis done after 2 weeks were normal during both episodes without albuminocytologic dissociation. She was treated with intravenous immunoglobulin resulting in a remarkable recovery. Both episodes had a complete clinical recovery in three and four months' fourth dimension respectively, rather a faster recovery than commonly expected.

Conclusion

Recurrence of Guillain-Barré syndrome can occur in a subset of patients with Guillain-Barré syndrome even after many years of asymptomatic period. Normal cerebrospinal fluid profile does not exclude Guillain-Barré syndrome and may occur in subsequent recurrences of Guillain-Barré syndrome arising the need for farther studies to identify the pathophysiology and the possibility of a different subtype of Guillain-Barré syndrome.

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Background

Guillain-Barré syndrome (GBS) is an caused heterogeneous group of disorders due to an immune-mediated inflammation and demyelination of the peripheral nervous system, following an antecedent affliction in 2 thirds of the patients, unremarkably an infection [ane–3]. Information technology is a medical emergency which normally presents with acute onset, apace progressive symmetrical ascending flaccid paralysis of the limbs with accompanying absent or diminished deep tendon reflexes. It is oft associated with sensory symptoms, cranial nerve interest, less ordinarily autonomic dysfunction and respiratory compromise.

GBS is a monophasic illness. Although rare, recurrence has been described following an asymptomatic menstruation of few months to years (iv months – x years) in 1–6 % of patients [3–6]. Recurrent GBS (RGBS) is characterized by 2 or more attacks of acute inflammatory demyelinating neuropathy with an onset to peak time of 4 weeks or less, and having complete or almost consummate recovery [three, v]. It is suggested by Kuitwaard et al. that there is a subset of patients with GBS who are susceptible for recurrence, characterized by younger historic period, milder grade of affliction and having Miller-Fisher variant of GBS [3]. Literature revealed that the patients with recurrence had similar but more severe symptoms and signs in subsequent episodes while having similar or unlike antecedent event [3, 6]. It is important to distinguish recurrent GBS from GBS with treatment related fluctuations (GBS-TRF) and chronic inflammatory demyelinating polyradiculo-neuropathy (CIDP) every bit the handling regimens are different. Cerebrospinal fluid (CSF) shows albuminocytologic dissociation in 82–xc % of the patients with GBS later on 10–fourteen days from onset of the illness [7]. Electrophysiological studies and CSF analysis are taken to aid clinical diagnosis of GBS simply normal CSF profile can be constitute in 10 % of GBS patients throughout the disease [8]. Therefore normal values cannot rule out GBS. 1000'maison et al. reported 12 cases of recurrent GBS (total of 32 episodes), where it was observed that all patients who were in the symptomatic phase of GBS and after 1 week of onset of disease showed CSF albuminocytologic dissociation. They observed normal CSF protein levels which was tested at the onset of disease (within 1 week) in 2 patients but had non identified a variant of RGBS with normal CSF protein levels during the symptomatic episodes, tested atleast i week later the onset of symptoms.

We present a rare case of RGBS presenting after 12 years, adding to the limited number of cases with a long asymptomatic interval. Such reported cases from South-Asia are rare. Apart from the young historic period at initial episode, she did not have other adventure factors for recurrence and had a rare findings of normal CSF protein concentrations on both presentations. Extensive literature survey including published instance reports and case series did not reveal a similar instance study of RGBS or occurrence with normal CSF poly peptide concentrations during both initial and recurrent presentations of GBS.

Instance presentation

A 25-yr-onetime Sri Lankan female presented with weakness of all four limbs in January 2014. She had a like affliction 12 years dorsum.

Beginning episode

In 2002, at the age of 13 years, patient had noticed tingling sensation of distal upper and lower limbs followed past weakness of lower limbs, involving both distal and proximal muscle groups. Weakness was progressive and ascending, involving both upper limbs and neck muscles by day six of the disease. There was no dysphagia, dysphonia, respiratory difficulty or bladder/bowel involvement. In that location was no significant medical history suggestive of preceding infection, toxin ingestion or like affliction in the past. On examination, there was flaccid quadriparesis (muscle ability course - 3/5) with areflexia in all iv limbs. There were no cranial nervus palsies or features suggestive of autonomic involvement. Sensory system examination was normal. Full blood count, blood picture, serum electrolytes, claret urea and liver enzymes profile were normal. Her erythrocyte sedimentation charge per unit was 13mm during 1^st hour. Nerve conduction studies performed on day 4 of the illness showed focal segmental demyelinating type sensory and motor neuropathy with conduction blocks (Table one), suggestive of astute inflammatory demyelinating polyneuropathy (AIDP). EMG studies were not performed during this episode. CSF analysis on day 14 of the disease revealed normal results with proteins – 30 thou/dl (normal 15–twoscore g/dl), white cells 2 with 100 % lymphocytes and normal CSF glucose levels compared to plasma values. She was treated with intravenous immunoglobulin for 5 consecutive days in addition to physiotherapy and discharged on day 18. She fabricated a complete recovery in 3 months based on normal tone, power and deep tendon reflexes on neurological examination. Follow-upward NCS was not performed later on clinical recovery.

Second episode

In 2014, patient readmitted with numbness and progressive ascending weakness of all four limbs for iii days duration and had developed poor cough response, dysphagia and difficulty in breathing during the post-obit two days. This episode was preceded past an upper respiratory tract infection ii weeks back. In that location was no similar illness noted in whatever of her family members. On examination, flaccid quadriparesis with generalized areflexia was noted with a muscle power of one/5 in lower limbs, 2/v in upper limbs and two/v in neck muscles. Weakness progressed to involve bilateral seventh cranial nerves and bulbar musculus without ophthalmoplegia. Balance of the neurological examination including sensory system and other organ system examination were normal except for a resting tachycardia of 130 beats/min without any significant claret force per unit area fluctuations. Respiratory charge per unit was thirty cycles/min with oxygen saturation of 92 % on air. Nerve conduction studies done on day ten of the illness revealed focal segmental demyelinating type sensory and motor neuropathy with prolonged distal motor latency, delayed F-wave and conduction blocks, concluding as AIDP variant of GBS (Table 1). Electromyogram done on solar day ten of the disease did not show whatever testify of denervation just noted fibrillation potentials of positive abrupt waves. CSF analysis on day 10 and a repeat written report on 24-hour interval 24 showed normal results without cyto-protein dissociation or pleocytosis (Table two) Full blood count, blood film, serum electrolytes, erythrocyte sedimentation charge per unit, claret urea and liver enzymes profile were normal. Serology for Mycoplasma, Campylobacter jejuni, cytomegalovirus, Epstein-Barr virus, hepatitis B & C and retroviral studies were negative. Autoimmune panel including anti-nuclear factor was normal. Due to the rapid progressive nature of weakness and respiratory distress, patient was mechanically ventilated for 12 days. Diagnosis of GBS was made and intravenous immunoglobulin 0.four thou/kg/day was administered for five days. In addition, she received physiotherapy to support her recover in motor office of limbs and speech therapy following extubation. She made a adept clinical recovery assessed subjectively equally well every bit objectively and was discharged home on 24-hour interval 28 with a muscle power of four/5 and deep tendon reflexes of +1.

Table one Nerve conduction written report during the initial presentation in 2002 (i^st episode) and recurrence (two^nd episode) of Guillain-Barre syndrome in 2014

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Table 2 Laboratory findings of Cerebrospinal fluid during the initial presentation in 2002 and recurrence of Guillain-Barré syndrome in 2014

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On follow-up, patient had normal neurological examination findings and subsequent nerve conduction study later four months revealed normal results (Table 1).

Conclusions

GBS is an astute, allowed mediated inflammatory polyradiculo-neuropathy involving the peripheral nervous system. Onset is preceded by an antecedent event in two thirds of the patients, normally an upper respiratory tract infection or a diarrheal illness [ane–iii], where the causative amanuensis is assumed to trigger an immune response against the gangliosides and glycolipids distributed along the myelin sheaths and peripheral nervous system. This results in marked inflammation of the peripheral nerves, resulting in demyelination and defective impulse propagation. It is a heterogeneous grouping of disorders which involves motor, sensory and autonomic nervous systems to varying degrees depending on the sub blazon; (1) Acute inflammatory demyelinating polyneuropathy, (2) Acute motor axonal neuropathy, (3) Astute motor sensory axonal neuropathy, (4) Miller Fisher syndrome, (5) Acute pan-autonomic neuropathy and (six) Pure sensory GBS.

GBS is a monophasic illness, with an annual incidence rate of i.2–three per 100 000 population [9]. Nonetheless, recurrence of GBS is observed in 1–6 % of patients, where information technology is defined every bit 2 or more attacks of acute inflammatory demyelinating neuropathy with an onset to peak time of 4 weeks or less having consummate or most complete recovery [3–6]. The fourth dimension lag between 2 episodes of GBS was 4 months to 10 years in a written report done by Das et al. and a mean of 7 years with a range from 2 months to 37 years was described by Kuitwaard et al. [3, 5]. Patients tend to get like clinical presentations and shorter intervals in betwixt subsequent episodes of GBS [3]. Results of the written report by Kuitwaard likewise found that RGBS patients were younger, with milder disease and had Miller-Fisher variant of GBS at the initial episode. Patients with above characteristics on initial presentation of GBS are more than prone for recurrences [iii]. They as well identified that at that place are like presentations merely more than severe clinical deficit and residual effects with each recurrence [iii, half dozen]. However, in that location is express literature addressing why just a certain subset of patients with GBS become recurrences of the disease. The indexed case, although young at presentation, patient had a more alarming disease initially with poor neck muscle ability and limb power and did not take Miller-Fisher variant. This shows a deviation from the classically identified features favoring a recurrence of GBS. The fourth dimension gap between the episodes was 12 years. During the episode of recurrence, she had rapid development of more astringent disabling illness involving cranial nerves and respiratory compromise needing mechanical ventilation. Both episodes had AIDP variant of GBS with similar initial presentations.

The RGBS patients with similar presentations during the subsequent episodes had different antecedent infections and this may point towards immunogenic and host factors every bit major determinants of the disease [three, five, 6]. Nevertheless, verbal mechanism past which similar clinical manifestations occur during recurrence is non established. Our patient also had an upper respiratory tract infection preceding the recurrence of GBS but did not have an event during the initial episode.

It is important to distinguish RGBS from two clinical entities; (one) GBS with treatment related fluctuations (GBS-TRF), (ii) Chronic inflammatory demyelinating polyneuropathy (CIDP). GBS-TRF which occurs in 6–xvi % of patients with GBS is divers as pregnant deterioration inside ii months later disease onset following post treatment comeback or stabilization [3]. Repeating immunoglobulin or plasmaparesis in such patients will improve the result [10]. Since our patient had a long asymptomatic period, GBS-TRF is less likely simply CIDP comes every bit a differential diagnosis. CIDP is suspected when progression of weakness lasts more than than viii weeks followed by a chronic course only it tin be of steadily progressive, relapsing remitting or monophasic. The handling differs every bit CIDP can be treated with either immunoglobulin or immunosuppressive therapy with a subsequent maintenance immunosuppressive drug treatment whereas GBS and GBS-TRF do not show a response to immunosuppressant therapy but has good response to immunoglobulin or plasmaparesis. GBS is a more likely diagnosis in our patient as there was a rapid onset of symptoms, subsequent consummate or near complete recovery, high incidence of an ancestor disease, normal CSF protein levels at the onset of a recurrence.

Diagnosis of GBS is mainly clinical and supported by testify from electrophysiological studies and CSF analysis. Characteristically CSF has high poly peptide levels with normal cell counts and sugar levels. Nerve roots that exit from the spinal string traverse through CSF and when nerve roots are inflamed in GBS, proteins leak in to the CSF. Since the inflammation is confined to the nerve roots, pregnant numbers of inflammatory cells are not seen. Although normal CSF findings are seen during the first week of affliction, albuminocytologic dissociation is seen in 82–90 % of the patients with GBS past the end of 2d week of the affliction [7]. Chiliad'maison et al. had also observed normal CSF proteins at the onset of recurrent GBS episodes but likewise noted that it was elevated when measured after 1 week and when the patient is symptomatic [4]. x % of patients with typical GBS may have normal CSF findings though out the grade of affliction [vii, viii, xi] merely the pathophysiological mechanism leading to normal values is not well understood. Medical literature on above area is not extensive. The index case also had normal levels of CSF full proteins (performed after 2 weeks of onset of affliction) during both episodes of GBS, which is uncommon and non described in previously reported cases of RGBS.

Study done by Gonzalez-Quevedo et al. revealed raised CSF full proteins correlated with the degree of inflammation at the nerve roots and higher level of CSF proteins were related to clinical severity [12]. Corston et al. studied amino acids in CSF of GBS patients quantitatively equally well equally qualitatively. Of the 12 patients studied, 4 had normal CSF protein levels (<twoscore grand/dL) and in 3 of them repeat CSF assay confirmed normal CSF protein levels. Assay revealed that 12 amino acids (Eg - ornithine, lysine, arginine, glycine, alanine plus citrulline, leucine, tyrosine and phenylalanine) were raised in patients with high CSF proteins while half dozen of the amino acids (alanine plus citrulline, 2 amino-butyric acid, leucin, ornithine and lycine) were raised to a higher place normal reference range in patients who had normal CSF total proteins [xiii]. Three amino acids (phosphoethanolamine, serine and glutamic acrid) in the CSF of GBS patients showed reduced concentrations. This written report highlights the fact that even when the total poly peptide content in CSF is normal, patients with GBS have alteration of specific amino acids in CSF. Our patient had a severe disease with respiratory compromise during the recurrence but in contrast to the study by Gonzalez-Quevedo et al. she had normal CSF protein levels. Due to the unavailability of the laboratory facilities, we could not perform the CSF amino acid analysis in our patient.

Alphabetize patient had a recurrence of GBS afterward a long asymptomatic period of 12 years as supported by astute onset, rapid progression, disability peaked within ii weeks followed by complete subsequent recovery post-obit handling, presence of antecedent infection and supportive electrophysiological prove. She also had more severe disease during the recurrence. CSF findings were not characteristic and were normal during both episodes occurring 12 years apart, which is a rare finding and not described before in patients with RGBS. Mechanism backside normal level of CSF proteins during recurrent episodes remains unclear.

Recurrence of GBS is rare but tin can occur later many years of asymptomatic period and is associated with more astringent clinical manifestations. In such a presentation, it is of import to distinguish GBS from CIDP as the treatment modalities are different. Normal CSF total poly peptide levels tested after 1 week after the onset of disease can occur in initial and recurrent episodes of GBS, where the mechanism is not fully understood. Research on recurrent GBS is needed to evaluate the college probability of certain subgroup of patients with GBS for recurrences, to identify the occurrence and pathophysiology of normal CSF profile and the possibility of a different subtype in recurrent GBS patients.

Abbreviations

AIDP:

Acute inflammatory demyelinating polyneuropathy

CIDP:

Chronic inflammatory demyelinating polyneuropathy

CSF:

Cerebrospinal fluid

GBS:

Guillain-Barré syndrome

GBS-TRF:

Guillain-Barré syndrome with treatment related fluctuations

RGBS:

Recurrent Guillain-Barré syndrome

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Acknowledgement

The authors acknowledge the contribution of Dr. W. Dharmakeerthi, Consultant Neuro-electrophysiologist, Teaching Hospital, Kandy, Sri Lanka, staff of biochemistry laboratory, medical intensive care unit and medical ward in Teaching Hospital, Kandy, Sri Lanka for the support provided in the process of diagnosis and management of this patient.

Funding

None of the authors have received any financial assistance for this manuscript.

Availability of data and cloth

Details of the patient is bachelor in the hospital notes for the Editor-in-primary of this periodical for review.

Authors' contributions

HW made the clinical diagnosis, fabricated clinical decisions in management and supervised the manuscript drafting. SSCG drafted the first manuscript and reviewed the literature. HW, RG and SSCG were involved in straight direction of the patient. All authors read and canonical the final manuscript.

Authors' information

HW (MBBS, MD, FRCP(Edin), FRCP(Lond), FCCP) is a Consultant Physician. RG (MBBS, MD) is a Senior Registrar in Medicine. SSCG (MBBS) is a Registrar in Medicine. All authors are attached to Didactics Hospital, Kandy, Sri Lanka.

Competing interests

The authors declare that they have no competing interests.

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Written informed consent was obtained from the patient for publication of this case report. A copy of the written consent is available for review past the Editor-in-chief of this journal.

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1. Teaching Hospital, Kandy, Sri Lanka

   Sonali Sihindi Chapa Gunatilake, Rohitha Gamlath & Harith Wimalaratna

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Correspondence to Sonali Sihindi Chapa Gunatilake.

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Gunatilake, S.S.C., Gamlath, R. & Wimalaratna, H. An unusual case of recurrent Guillain-Barré syndrome with normal cerebrospinal fluid poly peptide levels: a case report. BMC Neurol 16, 161 (2016). https://doi.org/10.1186/s12883-016-0687-z

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• Received: 02 Apr 2016

• Accustomed: 29 August 2016

• Published: 05 September 2016

• DOI : https://doi.org/10.1186/s12883-016-0687-z

Keywords

• Guillain-Barré syndrome
• Albuminocytologic dissociation
• Acute inflammatory demyelinating polyneuropathy
• Cerebrospinal fluid
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