Pharyngeal-Cervical-Brachial Variant of Guillain-Barré Syndrome Following Dengue Infection: A Rare Syndrome with Rare Association.

Pharyngeal-cervical-brachial (PCB) variant is a rare localized variant of Guillain-Barré syndrome that presents with a rapidly progressive oropharyngeal and cervicopharyngeal weakness associated with areflexia in the upper limb. Here, we are describing a case of a 20-year-old female who had a preceding history of fever with thrombocytopenia that was found to be dengue ELISA positive. This was followed by rapidly progressive weakness of cervical, bulbar, and bilateral facial muscles along with areflexia of upper limb. Nerve conduction study showed a reduced compound muscle action potential amplitude in the upper limb, and the CSF examination showed albuminocytological dissociation. The patient was given intravenous immunoglobulin, and she started improving within 3 days of commencing the treatment.

INTRODUCTION

Guillain–Barré syndrome (GBS) is an acute-onset, monophasic, immune-mediated polyneuropathy that often follows an antecedent infection. It is characterized by rapidly evolving ascending weakness, mild sensory loss, and hyporeflexia or areflexia, progressing to a nadir over up to 4 weeks. Acute inflammatory demyelinating polyradiculoneuropathy is the most widely recognized form of GBS, but other variants such as acute motor axonal neuropathy (AMAN), acute motor-sensory axonal neuropathy, and Miller Fisher syndrome are also well recognized. Pharyngeal-cervical-brachial (PCB) variant of GBS is rarely described in literature.

Here, we are describing this rare variant that was preceded by dengue fever. The patient was given intravenous immunoglobulin (IVIg), and she recovered completely. Our purpose is to describe a rare disease with rare association and excellent response to IVIg.

CASE REPORT

A 20-year-old female had a history of fever with thrombocytopenia which was subsequently found to dengue ELISA positive. She received symptomatic treatment and recovered in a few days. One week after subsidence of fever, the patient developed acute-onset dysphagia which was more for liquids than solid and was associated with nasal regurgitation. There was bilateral facial weakness so that the patient had difficulty in closing her eyes, exposing the teeth while brushing and there was accumulation of food particles between cheek and gums while eating. There was also difficulty in manipulating the food bolus inside the mouth and propelling it inside oropharynx. On examination, positive findings were bilateral involvement of cranial nerve 7 and 9–12 and areflexia of upper limb with normal reflexes in the lower limb. The brain and cervical spine magnetic resonance imaging (MRI) done to rule out any central cause was found to be normal. Blood counts, liver function tests, renal function tests, thyroid hormone levels, Serum Vitamin B12, and folic acid were all normal. Serum antinuclear antibody and anti-Ds DNA antibody levels were normal. CSF examination showed a protein level of 105 mg/dl, sugar 80 mg/dl, and no cells suggesting albuminocytological dissociation. Nerve conduction study showed reduced compound muscle action potential amplitude in bilateral upper limb nerves with normal conduction in the lower limb. Repetitive nerve stimulation test was done in the trapezius and abductor digiti minimi muscle showed no decremental response. Diagnosis of localized form of GBS was considered and the patient was started on IVIg at a dose of 2 g/kg given over a period of 5 days. The patient showed dramatic improvement within 3 days of initiating the treatment, and by the end of 1 week, the patient was almost free of all her symptoms.

DISCUSSION

In 1986, Ropper described three patients who developed rapidly progressive oropharyngeal, neck and shoulder weakness, in the absence of sensory disturbance, and with relative sparing of the lower limbs.[1] In a study done on 250 GBS patients, the incidence of PCB variant was 3%.[2] Patients with PCB variant of GBS typically present with rapidly progressive oropharyngeal and cervicobrachial weakness associated with areflexia in the upper limbs. Power in the lower limbs is usually preserved or only mildly affected, indicating that PCB represents a localized subtype of GBS.[3] In a study, it was found that antecedent upper respiratory tract infection and diarrhea occurred in 71% and 30% of patients, respectively, that was similar to GBS. About 31% of patients had serological evidence of Campylobacter jejuni infection, whereas cytomegalovirus (6%), Epstein–Barr virus (4%), Mycoplasma pneumoniae (3%), and Haemophilus influenzae (1%) were less common.[3] GBS is autoimmune in nature, and various antibodies had been described among different variants. The strongest association for PCB is the presence of IgG anti-GT1a antibodies, while other antibodies such as GM1b and GD1a had also been described.[2] Electrophysiologically, PCB is considered as a continuum of AMAN variant of GBS showing axonal conduction failure.[4] Very often, patients presenting with PCB are initially misdiagnosed as having brainstem stroke, myasthenia gravis, or botulism.

In our patient, there were typical signs and symptoms consistent with the diagnosis of PCB. Unusual thing was preceding a history of dengue fever that has not been described in the literature. To rule out any central cause, cranial and cervical MRI was done. There was no history of fluctuation, fatiguability, ptosis, or any evidence of decremental response on Repetitive nerve stimulation test (RNST) at 3 Hz excluding neuromuscular junction pathology like myasthenia gravis. Autoantibodies testing against specific neuronal gangliosides that were found to be associated with various subtypes of GBS was not done as a facility of this was not available at our hospital. We started treatment with IVIg, and the patient started recovering within 3 days of initiating treatment. By the end of 1 week, the patient was almost completely free of symptoms.

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 name and initial will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.