Guillain–Barré syndrome with hyperreflexia and bilateral papillitis in a child

Abstract

Guillain–Barré syndrome (GBS) is an acute inflammatory polyneuropathy characterized by rapidly progressive symmetric weakness, and areflexia. Areflexia is necessary for the diagnosis of GBS. However, recently there have been studies of hyperreflexia with axonal neuropathy form of GBS. We report a 14-year-old boy with GBS, who presented with hyperreflexia and bilateral papillitis. To the best of our knowledge, this is the first pediatric patient presenting with papillitis and hyperreflexia with acute motor and sensory axonal neuropathy form of GBS.

Introduction

Guillain–Barré syndrome (GBS) is an acute inflammatory polyneuropathy most commonly characterized by rapidly progressive, essentially symmetric weakness and hyporeflexia or areflexia. Various subtypes have been described based on clinical, electrodiagnostic, and pathologic criteria. The most common underlying subtypes of the syndrome are acute inflammatory demyelinating polyradiculoneuropathy (AIDP). Another subtypes are acute motor axonal neuropathy (AMAN), and acute motor and sensory axonal neuropathy (AMSAN).[1]

GBS is recognized as a heterogeneous disorder with various clinical manifestations.[2,3,4,5] In literature, rarely there has been optic nerve involvement and hyperreflexia in patients with GBS.[6] Here, we describe a patient with AMSAN form of GBS, who presented with hyperreflexia and bilateral papillitis.

Case Report

A 14-year-old male patient was admitted to our hospital with sudden onset loss of vision, numbness, and weakness of the legs and the arms. Ten days before to the onset of his symptoms, he had upper respiratory tract infection. His first symptoms were bilaterally visual loss since 5 days on admission. Three days after visual symptoms, numbness, and mild weakness in his arms and legs were revealed. There was no evidence of gastrointestinal infection, history of consanguinity, and neurologic disease in the family.

On examination, he appeared to be blind, was unable to see or fixate on any target and could not see colors. The ophthalmologic exam revealed bilateral papillitis. Other cranial nerves were intact, and his muscle strength was graded as 4/5 in the bilaterally distal muscles including the arms and legs symmetrically. Furthermore, he had finger extensor weakness in the upper extremities. Deep tendon reflexes were brisk in all extremities without spasticity and Babinski sign was bilaterally negative. Hyperesthesia was determined in distal parts of the extremities. He was able to walk alone. The remainder of the physical and neurological examination was normal.

On laboratory examination, routine hematological, biochemical analyzes, and the urine and stool analysis were normal. Serologic tests for toxoplasmosis, rubella, cytomegalovirus, herpes simplex virus, Epstein–Barr virus, brucella, Salmonella, hepatitis A, B, HIV, Borrelia burgdorferi, and Mycoplasma pneumoniae were negative. The antibodies against gangliosides (GM1, GQ1B, GD1B, GT1B, GD1a, GM3, and GM2) for Campylobacter jejuni were negative. A magnetic resonance imaging of his spinal cord and brain was normal. The visual evoked potential could not be obtained. Because he was diagnosed as papillitis, a course of intravenous methylprednisolone pulse therapy (1 g/day for 3 days) was given and followed by orally 2 g/kg tapering in 14 days. Five days after admission to our hospital, the nerve conduction studies revealed AMSAN form [Table 1]. Lumbar puncture was performed and opening pressure was 18 cm H₂O with no cells, glucose 56 mg/dL (normal, 40–70 mg/dL), and protein 78 mg/dL (normal, 10–50 mg/dL). We diagnosed as AMSAN form of GBS, and intravenous immunoglobulins treatment was immediately started (0.4 g/kg/day over 5 days). After intravenous methylprednisolone pulse therapy and immunoglobulin treatment, his complaints disappeared over the following weeks.

Table 1.

Result of nerve conduction study

Discussion

GBS is a common cause of acute peripheral neuropathy and is characterized by hyporeflexia or areflexia. Recently, there have been several descriptions of reflex preservation and hyperreflexia in axonal GBS.[4,5,6] Hyperreflexia developed during the early phase of recovery of AMAN form. In the literature, moreover, 48% of Chinese and 33% of Japanese patients with GBS showed hyperreflexia.[7,8] Those patients with AMAN subtype of GBS and hyperreflexia have been reported following C. jejuni infection.

The mechanism that causes hyperreflexia in GBS is unknown. Hyperreflexia has been reported to be an associated finding of spinal inhibitor intermediate neuronal or upper motor neuronal dysfunction.[9] Because hyperreflexia was found only in patients with anti-GM1 antibodies, they speculate a possible role of this antibody. Anti-GM1 positive serum has been reported to cause injury in nerve roots and central axons of the spinal cord on injection into the subarachnoid space.[10] Inflammation in the spinal roots may lead to local dysfunction of the blood-central nervous system barrier and allow anti-GM1 antibodies to bind with the neural structures in the spinal cord.[11] Our patient was diagnosed with AMSAN subtype of GBS. Furthermore, we found that the antibodies against gangliosides were absent. There is only one patient report of AMSAN form with hyperreflexia in literature. Tosun et al.[5] reported a 12-year-old girl with hyperactive deep tendon reflexes with AMSAN form without GD1a-antibody. The authors could not explain the condition in the patient.

In GBS, optic nerve involvement is mostly seen in AIDP patients.[12,13] However, only one patient has been described with the axonal form and optic nerve involvement. Neuwirth et al.[6] reported an adult patient with AMAN form GBS who presented with hyperreflexia and papillitis, as in our patient. However, we could not detected GD1a ganglioside antibodies in our patient with AMAN form GBS.

To the best of our knowledge, this is the first described case without GD1a-associated AMSAN form with hyperreflexia and papillitis.

Conclusion

Hyperactive deep tendon reflexes are rare in GBS but does not exclude the diagnosis. Therefore, the nerve conduction studies should be performed for evaluation to confirm the diagnosis of GBS.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.