CASE

An 11 year-old female presents with approximately 2 weeks of bilateral lower extremity pain that is localized to her knees and calves. The pain is worse with ambulation and not relieved with ibuprofen. She has difficulty ascending the stairs at school and recently started having trouble walking on flat ground. The patient also endorses neck, back, and shoulder pain that are less severe. She recalls an illness associated with vomiting and diarrhea that started approximately 1 week ago, which has still not resolved. She was seen by her PCP the day prior to presentation and had a negative Strep test. Review of systems is positive for diarrhea and headache.

Vital signs show a blood pressure of 146/91  and a heart rate of 104. She was afebrile. Physical exam showed diffuse tenderness in the bilateral paraspinal muscles of the cervical, thoracic, and lumbar spine, bilateral deltoids, bilateral posterior knees, and bilateral ankles. She had full passive range of motion, but decreased strength with shoulder abduction, elbow extension, knee extension, and hip flexion. Her neurologic exam revealed a waddling gait. Patellar deep tendon reflexes were diminished bilaterally and S1 reflexes were absent bilaterally. Initially, she was able to ambulate on her own, but while in the ED her exam quickly worsened to the point that she required assistance with ambulation. Sensation and finger-to-nose coordination were intact.

X-rays of the bilateral lower extremities showed no acute injuries or fractures. Lab work-up showed an elevated LDH, C3, and positive EBV IgG and IgM. A number of labs, including Lyme reflex, ASO HLA-B27, ANA did not result while the patient was in the emergency department. A lumbar puncture was performed, which showed a protein of 60 and normal cell count.

DIAGNOSIS

The ultimate diagnosis was unclear while the patient was in the emergency department, but considerations were reactive arthritis, myositis, Guillain-Barre syndrome (GBS), spinal cord injury, multiple sclerosis, juvenile arthritis, systemic lupus erythematous (SLE), Lyme disease, and vitamin deficiency.

It was presumed that the patient had GBS based on her clinical presentation and recent illness.

DISCUSSION

Epidemiology and prognosis

GBS is more commonly seen in adults than children, with an incidence of only 0.34-1.34 cases per 100,000 persons 18 years or younger.[1] Pediatric patients typically have a shorter clinical course and better prognosis than adults. Mortality is low (3-4%) and most pediatric patients (85-92%) have a complete or nearly complete recovery.[1]  

Types

There are several variations of GBS which are briefly compared below [1,2]:

Clinical presentation

A study of GBS in children has shown that most pediatric patients present with an unsteady gait (45%), neuropathic pain (34%), and an inability to walk (24%). About half of pediatric patients also present with autonomic dysfunction such as dysrhythmias, orthostatic hypotension, hypertension, paralytic ileus, bladder dysfunction, and abnormal sweating (Korinthenberg, 2007).

Diagnosis

The initial diagnosis of GBS in the emergency department is based on the clinical presentation and CSF analysis as other diagnostic modalities (electrodiagnostic studies, MRI, serum antibodies) are not available or feasible in the ED. CSF analysis will typically show albuminocytologic dissociation, which is an elevated CSF protein and normal CSF WBC count. However, this finding is only present in half to two-thirds of patients in the first week of symptoms. This finding increases to more than 75% of patients by the third week of symptoms (Yuki, 2012). Therefore, high clinical suspicion is important, especially in patients presenting early in the course.

Treatment

The main treatments of GBS, in addition to supportive care, include IVIG and plasma exchange. These treatments are typically reserved for children with more severe symptoms including progressive weakness, respiratory impairment, bulbar weakness, or inability to ambulate. IVIG is typically given over two or five days, although there may be a slightly lower chance of relapse with a 5 day course.[4]

Respiratory failure is responsible for most cases of death due to GBS, so it is important to closely monitor pulmonary function tests. Approximately 10 to 20 percent of children with GBS will require intubation.[5] Negative inspiratory force (NIF) is a bedside test that can measure respiratory muscle function and determine when respiratory support is needed. The NIF is a measurement of the inspiratory muscles and is measured by having a patient inhale maximally (Figure 1). If the NIF is less than -30cm H2O or declines >30% within 24 hours, the patient should be admitted to an ICU in preparation for possible intubation.[6] Young children may have difficulty performing this bedside test. If coaching does not achieve adequate effort, these patients should be closely monitored for signs of fatigue including increased respiratory rate, elevated pCO2, and accessory muscle use.[1]

Steroids have not been shown to be beneficial in GBS and are not recommended.[1]

CASE FOLLOW-UP

This patient was admitted to the hospital with GBS. Her hospital course was complicated by tachypnea and facial weakness. She was transferred to the PICU over concern for impending respiratory compromise and bulbar muscle involvement. She was treated with a 5 day course of intravenous immunoglobulin (IVIG). She required positive pressure ventilation, which was weaned off during the course of the hospitalization.  NIFs increased from 20-30% to 40%. Her infectious workup was positive for Enteropathogenic E. coli, subacute/chronic EBV, and acute Lyme disease. She regained some strength in her lower extremities but continued to have weakness in her upper extremities and bulbar muscles. She was discharged to a rehab facility for continued therapy.

TAKE-AWAYS

• GBS should be considered in any patient presenting with new neurologic complaints in the setting of a recent illness.

• NIF is a useful adjunct to determine if GBS patients have any signs of impending respiratory compromize or if they may benefit from respiratory support.

• In addition to supportive care, IVIG and plasma exchange are the mainstays of treatment.

Faculty Reviewer: Dr. Jane Preotle

REFERENCES

1. Ryan, M. (2019). Guillain-Barré syndrome in children: Epidemiology, clinical features, and diagnosis. UpToDate.  

2. Lin, J., Hsia, S. H., Wang, H. S., et al. (2012). Clinical variants of Guillain-Barré syndrome in children. Pediatric Neurology, 47(2), 91.

3. Yuki, N., Hartung, H. (2012). Guillain–Barré Syndrome. New England Journal of Medicine, 366, 2294-2304.

4. Korinthenberg, R., Schessl, J., Kirschner, J. (2007). Clinical presentation and course of childhood Guillain-Barré syndrome: a prospective multicentre study. Neuropediatrics, 38(1), 10. 

5. Hu, M., Chen, C., Lin, K., et al. (2012). Risk factors of respiratory failure in children with Guillain-Barré syndrome. Pediatric Neonatology, 53(5), 295-9.

6. Lawn, N., Fletcher, D., Henderson, R., et al. (2001). Anticipating mechanical ventilation in Guillain-Barré syndrome. Archives of Neurology, 58(6), 893.