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Case report

A young male with an unusual presentation of Guillain-Barré syndrome (GBS) mimicking stroke: a case report

A young male with an unusual presentation of Guillain-Barré syndrome (GBS) mimicking stroke: a case report

Mohamed Sheikh Hassan1,&, Nor Osman2, Bakar Ali3

 

1Department of Neurology, Mogadishu Somali Turkish Training and Research Hospital, Mogadishu, Somalia

 

 

&Corresponding author
Mohamed Sheikh Hassan, Department of Neurology, Mogadishu Somali Turkish Training and Research Hospital, Mogadishu, Somalia

 

 

Abstract

Guillain-Barré syndrome (GBS) is an immune mediated polyneuropathy. Multiple clinical variants of GBS have been identified. Acute Motor Axonal Neuropathy (AMAN) is one of these, and it is distinguished by selective involvement of motor nerves and axonal involvement with sensory sparing in electrophysiological studies. Here, we report a case of a 33-year-old male patient who was referred to our neurology polyclinic following a 5 days history of quadriplegia that initially started as right hemiplegia (right side weakness) and 2 days later progressed into quadriplegia, which later progressed into asymmetric cranial nerve involvement. The patient initially presented to the emergency room of another hospital with hemiparesis, mimicking a cerebrovascular event. Based on the neurological examination, imaging findings, cerebrospinal fluid (CSF) analysis, and nerve conduction studies, a diagnosis of AMAN (a variant of GBS) was made. The patient was admitted and treated with intravenous immunoglobulin (IVIG) for 7 days. After some clinical improvement, he was discharged and transferred to neurologic rehabilitation. After 6 months of physiotherapy, the patient's condition had improved massively and was back to normal functioning. Although the usual presentation of GBS is that of progressive ascending weakness, clinical variations exist (including asymmetric hemiplegic presentation), which may challenge the diagnosis. The purpose of this case is to raise awareness of GBS clinical heterogeneity in order to promote early correct diagnosis of this clinical variety.

 

 

Introduction    Down

Guillain-Barré syndrome (GBS) is assumed to be autoimmune and operated by a preceding infection, most often respiratory or gastrointestinal infections. Generally, infections by microorganisms such as Campylobacter jejuni, CMV, Mycoplasma pneumonia, or influenza virus exist several weeks prior to approximately two thirds of GBS cases [1]. A small percentage of patients develop GBS after immunization with quadrivalent meningococcal polysaccharide conjugate vaccine (MCV4), following surgery or trauma, and bone-marrow transplantation. GBS has also been linked to other autoimmune diseases and malignancies such as systemic lupus erythematosus, and Hodgkin's lymphoma [2]. Although the classic description of GBS is that of a demyelinating neuropathy with ascending weakness, many clinical variants have been well documented in the medical literature, and variants involving the cranial nerves or pure motor involvement and axonal injury have also been described [3]. Here we present a rare case of GBS presenting as hemiparesis mimicking a stroke.

 

 

Patient and observation Up    Down

Patient information: a 33-year-old male patient presented with sudden onset hemiplegia progressing to quadriplegia with cranial nerve involvement. He had no significant past medical/surgical history and family history.

 

Clinical findings: the patient´s symptoms started with a generalized moderate intensity headache, and 5 days later, progressive weakness that initially started as right side of the body weakness (hemiplegia) and progressed two days later to quadriplegia. With a clinical suspicion of stroke, the patient was first evaluated at a local hospital. A brain MRI including diffusion sequence was performed, but no specific findings were obtained. After that, the patient was referred to our hospital. At presentation to our hospital, the patient was quadriplegic. There was no history of diarrhea or respiratory infection prior to the onset of weakness. A physical examination revealed a slightly overweight male conscious with normal vital signs. His higher cortical functions were intact. Motor examination revealed normal bulk with hypotonia in all four extremities, with a power of 1/5 in both upper and lower extremities (both proximally and distally). He had generalized areflexia with bilateral mute planter response. At the time of admission, there was no facial asymmetry; likewise, other cranial nerves were intact and symmetric. A cerebellar examination wasn´t possible because of the patient´s quadriparesis. The chest was clear to auscultation. Examination of the cardiovascular and gastrointestinal systems was unremarkable. Laboratory investigations showed a normal complete blood picture and a normal metabolic panel.

 

Diagnostic assessment: brain MRI with diffusion sequence and cervical MRI were normal. Cerebrospinal fluid analysis showed cytoprotein dissociation with 5 cells, 131 mg/dl protein, and a normal glucose of 68 mg/dl (serum blood sugar of 117 mg/dl). Nerve conduction studies were suggestive of a demyelinating polyneuropathy with secondary axonal degeneration. Three days after admission, the patient developed right-side facial asymmetry (peripheral facial palsy). During the next two days, the facial asymmetry progressed into diplegia (bilateral facial palsy) and mild dysphagia. On the basis of the above clinical, laboratory, and radiological findings, a diagnosis of Acute Motor Axonal Neuropathy (AMAN), a variant of GBS was made.

 

Therapeutic intervention: after the diagnosis, IVIG was started along with other supportive treatments. After 7 sessions of IVIG treatment, there wasn´t much improvement in terms of muscle strength. However, facial palsy and dysphagia improved, and he was discharged after 7 sessions of IVIG and referred for neurologic rehabilitation.

 

Follow-up and outcomes: the patient was followed up in our neurology outpatient clinic. After 6 months of physiotherapy, the patient´s condition improved massively, muscle strength was back to normal (5/5) in both upper and lower extremities (proximally and distally) and he was back to normal functioning.

 

 

Discussion Up    Down

Guillain-Barré syndrome (GBS) is described as an acute inflammatory polyneuropathy characterized by rapidly evolving ascending weakness, mild sensory loss, and hypo- or areflexia, progressing to a nadir over up to four weeks. Besides the classic presentation of GBS, clinical variants are based on the types of nerve fibers involved (motor, sensory, sensory-motor, cranial or autonomic). Although the classic description of GBS is that of a demyelinating neuropathy with ascending weakness, yet many clinical variants have been reported in the medical literature, and variants involving the cranial nerves or pure motor involvement and axonal injury are not uncommon [3,4]. In the present case, the patient first presented as hemiplegia, simulating a cerebrovascular event. After being excluded through brain MRI with diffusion sequence, and the progression to quadriplegia mandated further evaluation and investigation to be performed which eventually led to the unexpected diagnosis of AMAN. In about 75% of cases, it is preceded by 1-2 weeks of acute illness, mostly respiratory or gastrointestinal infections. Camplylobacter jejuni(C. jejuni) infection is, overall, the most common antecedent infection and has been reported in up to 32% of cases [5]. However, our patient had no history of gastrointestinal/respiratory infections or any other infections prior to the weakness.

 

Acute motor axonal neuropathy (AMAN) is distinguished from acute inflammatory demyelinating polyneuropathy (AIDP) by its involvement of exclusively motor nerves and an electrophysiological pattern indicating axonal involvement. It is usually associated with a preceding Campylobacter jejuni infection. The clinical features and recovery are very similar to those of AIDP. However, more AMAN patients require assisted ventilation because of impending respiratory failure [6]. Although our case purely involved motor nerves and had axonal involvement on electromyography (EMG) studies, our patient initially presented as an asymmetrical pattern (right hemiplegia) with no involvement of respiratory muscles both on admission and next follow ups. Guillain-Barré syndrome is a clinical diagnosis, but additional investigations can be helpful or even needed for confirmation. Examination of the cerebrospinal fluid (CSF) is important, especially to exclude other causes of weakness associated with an increase in CSF cell count. Imaging studies, such as computed tomography (CT) and magnetic resonance imaging (MRI) scanning of the brain and spine, may be more helpful in excluding other diagnoses, such as mechanical causes of myelopathy, than in assisting in the diagnosis of GBS [7].

 

In our case, the progression from hemiplegia to quadriplegia in a matter of days, along with intact cranial nerves and high cortical functions, led to the suspicion of GBS. With the help of the nerve conduction study (which showed demyelinating polyneuropathy with secondary axonal degeneration), brain and spine imaging (which were negative) and CSF analysis (showing cytoprotein dissociation with 5 cells, 131 mg/dl protein and normal glucose level), the diagnosis was shifted to AMAN. Treatment of GBS though challenging, is mainly supportive and is centered on the anticipation and prevention of various complications. Immunomodulation with plasmapheresis and IV immunoglobulin (IVIG) helps to shorten the course of the disease. Patients with more severe disease and rapid progression of illness require extensive and prolonged rehabilitation and also show delayed and incomplete recovery [8]. In our case, the patient was admitted to the neurology inpatient ward and IVIG was started along with other supportive treatment. After 7 sessions of IVIG treatment, there wasn´t much improvement in terms of muscle strength. However, clinical progression disappeared and he was referred for neurologic rehabilitation. He was followed up in neurology outpatient clinic. After six months, he recovered massively and was back to normal functioning.

 

 

Conclusion Up    Down

Although the usual presentation of GBS is progressive ascending weakness, clinical variations exist (including asymmetric hemiplegic presentation), which may be challenging for diagnosis. The purpose of this case is to raise awareness of GBS clinical heterogeneity in order to promote early correct diagnosis of this clinical variety.

 

 

Competing interests Up    Down

The authors declare no competing interests.

 

 

Authors' contributions Up    Down

All authors have contributed to the manuscript. They read and agreed to the final manuscript.

 

 

References Up    Down

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