Postintravenous immunoglobulins posterior reversible encephalopathy syndrome in a patient with miller fisher variant of guillain–barre syndrome: A case report and literature review

Ans Alamami; Rabee Tawel; Farah Zahra; Mohamed Abelaty

doi:10.4103/LJMS.LJMS_103_20

CASE REPORT

Year : 2021 | Volume : 5 | Issue : 1 | Page : 31-33

Postintravenous immunoglobulins posterior reversible encephalopathy syndrome in a patient with miller fisher variant of guillain–barre syndrome: A case report and literature review

Ans Alamami¹, Rabee Tawel¹, Farah Zahra², Mohamed Abelaty¹
¹ Department of Critical Care, Hamad General Hospital, Hamad Medical Corporation, Doha, Qatar
² Department of Pharmacy, Alwakra Hospital, Hamad Medical Corporation, Doha, Qatar

Date of Submission	29-Nov-2020
Date of Acceptance	19-Mar-2021
Date of Web Publication	10-Apr-2021

Correspondence Address:
Dr. Ans Alamami
P.O. Box 3050, Al Rayan Street, Doha
Qatar

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/LJMS.LJMS_103_20

Abstract

Guillain–Barre syndrome (GBS) is an autoimmune disorder of the peripheral nerves manifested as inflammatory polyneuropathy of acute onset. The posterior reversible encephalopathy syndrome (PRES) is composed of gradual-onset neurological characteristics with unique radiological distribution (at the posterior brain region). Several etiologies, including hypertension, renal insufficiency, autoimmune diseases, medication use, and immunodeficiency, immunotherapy with intravenous immunoglobulin (IVIG) for various immune-related conditions, were associated with renal impairment, thrombotic manifestation, and reported PRES occurrence. Herein, we report a 73-year-old male who developed a clinical manifestation of PRES on top of IVIG therapy for Miller Fisher variant of GBS; therefore, PRES to be considered in a patient with GBS who treated with IVIG and developed alteration in the state of consciousness.

Keywords: Guillain–Barre syndrome, intravenous immunoglobulin, Miller Fisher syndrome, posterior reversible encephalopathy syndrome

How to cite this article:
Alamami A, Tawel R, Zahra F, Abelaty M. Postintravenous immunoglobulins posterior reversible encephalopathy syndrome in a patient with miller fisher variant of guillain–barre syndrome: A case report and literature review. Libyan J Med Sci 2021;5:31-3

How to cite this URL:
Alamami A, Tawel R, Zahra F, Abelaty M. Postintravenous immunoglobulins posterior reversible encephalopathy syndrome in a patient with miller fisher variant of guillain–barre syndrome: A case report and literature review. Libyan J Med Sci [serial online] 2021 [cited 2023 Mar 30];5:31-3. Available from: https://www.ljmsonline.com/text.asp?2021/5/1/31/313521

Introduction

Guillain–Barre syndrome (GBS) is an autoimmune disorder of the peripheral nerves manifested as inflammatory polyneuropathy of acute onset, the classical presentation of the GBS is ascending paralysis starting at the lower limb which progresses to involve multiple spinal levels depend on the severity of the disease variant. The underlying pathophysiology of the GBS involves inflammatory infiltrate with lymphocytes and monocytes around the myelin-producing cell (Schwann cell) ideally causing interruption of the nerve conduction, therefore, affecting the ability of the peripheral nerves to conduct a proper function.^[1] The variants of GBS depend on the predominant nerve type affected, which could be sensory, motor, or autonomic nerves. Typically, GBS is preceded by an infectious process, a well correlation was found and reported in several pieces of literature before, with Campylobacter jejuni infection. The diagnosis of GBS is mainly clinical diagnosis, with other diagnostic modality as supportive such as cerebrospinal fluid (CSF) analysis and nerve conduction study. Miller Fisher Syndrome is a GBS variant characterized by ataxia ophthalmoplegia and areflexia with specific positivity to anti-GQ1b antibody in 85%–90% of cases.

Posterior reversible encephalopathy syndrome (PRES) is a constellation of gradual-onset neurological manifestations with unique radiological distribution (posterior brain region).^[2] Among various etiologies including hypertension, renal insufficiency, autoimmune diseases, medication use, and immunodeficiency, the underlying pathophysiology is not yet well understood, as it includes interaction between hypertension, brain ischemia, disruption of the vascular endothelium inflammation, and subsequent edema. Its primary manifestations are headache, confusion, and seizures.^[3] Immunotherapy with intravenous immunoglobulin (IVIG) for various immune conditions has been associated with renal impairment, thrombotic manifestations, and reported PRES occurrence.^{[4],[5],[6],[7],[8]} It is unknown whether IVIG is directly associated with PRES development or the above mentioned is a side effect. We report a case developed a clinical manifestation of PRES on top of IVIG therapy for Miller Fisher variant of GBS.

Case Report

A 73-year-old male with a medical history of Type 2 diabetes and hypertension, who presented to the emergency department with chief complaints of bilateral lower limb weakness more on the right side, facial asymmetry, diplopia, and difficulty on swallowing of 3-day duration. The clinical examination revealed a reduced power and diminished reflexes in both lower limbs with reduced sensation up to the spinal level of T10; initially, cranial nerve examination was significant for binocular horizontal diplopia secondary to right abducent nerve palsy, and swallowing was impaired mainly for fluid. After the initial evaluation, there was a rapid deterioration of the patient's clinical condition with progressive respiratory failure requiring intubation and ventilatory support.

The initial basal blood laboratory evaluation of complete blood picture and metabolic balance was within normal, and a plain computed tomography scan head did not reveal any acute insult. A CSF analysis showed typical cytoalbumin dissociation with a high protein content of 6 mmol/l. A bilateral facial nerve sheath inflammation was aberrant in the contrast brain magnetic resonance imaging (MRI) [Figure 1], and a clinical diagnosis of GBS was concluded based on the clinical presentation and the CSF analysis supported later on by the nerve conduction study findings of decreased nerve conduction velocity. The management started with intravenous corticosteroids and IVIG administration of five doses (0.4 mg/kg/day for 5 days).

Figure 1: T1-weighted magnetic resonance imaging brain showing postcontrast enhancement of the bilateral facial nerve

Click here to view

On the 3^rd day of his IVIG course, the patient developed focal seizures involved mainly the left arm for few minutes aborted by intravenous lorazepam, 4 days after finishing the IVIG course the patient respiratory parameter improved, required minimal ventilatory support, unfortunately upon titrating the sedation down the patient did not show a significant improvement in his conscious level.

An electroencephalogram was done to rule out nonconvulsive seizures; meanwhile, the patient was off sedation, and it showed no seizure activity; moreover, it indicates the presence of an underlying nonspecific encephalopathic feature, a repeated brain MRI revealed a bilateral occipital, periventricular tiny enhancement consistent with PRES, the patient conscious level slowly improved in the consciousness level over the 1^st week after the completion of IVIG treatment course, but he was still requiring ventilatory support, the patient was started on plasmapheresis with six session in total, for which a significant improvement was achieved in terms of weaning from the ventilator support, limbs' power, swallowing ability, and extensive rehabilitation was conducted with impressive result eventually.

Discussion

The clinical features and the radiological findings of our patient matching the clinical description of Miller Fisher syndrome,^[8] which a severe variant of GBS characterized by a triad of areflexia, ophthalmoplegia, and ataxia.

The challenge we have encountered is related to the possible underlying etiology of the PRES, whether it is a consequence of IVIG treatment,^[4],[5],[6] especially there was a significant improvement in the conscious level after the IVIG course completion, a direct consequence of the GBS (fulminant GBS)^[9] in such a clinical scenario the state of conscious usually affected addition to absent brain stem reflex.

The clinical characteristics of PRES were initially described by Hinchey et al., i.e., neurological findings such as headache, confusion, visual alterations with a predominant postcirculatory abnormality on the brain image, a clinical presentation considered reversible upon treating the underlying pathology,^[3] with regard to the radiological characteristics of PRES, the brain MRI would reveal a unique vasogenic edema mainly at the postcerebral regions.^[10]

The IVIG treatment indicated for autoimmunity and immune deficiency was related to increased risk of thrombosis as previously described by Stetefeld et al.^[7] and with PRES^{[4],[5],[6],[7],[8]} as an independent factor; this finding developed mostly in patients with various risk factors that interfere with the cerebral endothelial state and cerebral perfusion pressure regulation. Among these risk factors, renal dysfunction, altered immunity, and medication use were observed in our case.

Conclusion

PRES should be considered in patients with GBS who treated with IVIG and developed new onset seizure with alterations in the state of consciousness.

Ethical approval

Patient consent has been obtained before the publication. Ethical approval was obtained from Medical Research Center, Hamad Medical Corporation, MRC-04–20–1039).

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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