2004 OPEN FORUM Abstracts
GUILLAIN-BARRE’SYNDROME AND PULMONARY INSUFFICIENCY IN THE YOUNGER PATIENT:
AN
ATYPICAL PRESENTATION
Larry
W. Kingrey, RRT; Robert H. Warren, MD -- Arkansas Children's
Hospital, Little Rock, AR
Introduction:
Guillain-Barre’ Syndrome (GBS) is an acute, demyelinating
polyneuropathy often triggered by a preceding bacterial or viral
infection. GBS is characterized by rapidly progressive ascending
symmetrical limb weakness and loss of tendon reflexes; often
resulting in paralysis of the legs, arms, respiratory muscles, and
face. Because of the potential for respiratory muscle involvement,
pulmonary function is carefully monitored, especially the forced
vital capacity (FVC). Intubation and mechanical ventilation may be
required due to declining FVC measurements or cardiovascular
instability. We report the case of a toddler in whom initial
presentation was atypical and challenges were faced in respiratory
status monitoring.
Case
Summary: A 2 y/o female was admitted with a 6-day history of
irritability with intermittent screaming, refusal to sleep in her
usual position of lying on her back and instead preferring to sleep
prone with her buttocks in the air, and progressive difficulty in
ambulation beginning with arching of the back and walking on tiptoes
to refusing to walk altogether. Physical exam also revealed sacral
pain with leg stiffness and unwillingness to flex her legs or move
her chin to her chest. Chest auscultation was clear. History included
up-to-date immunizations and mother’s recent recovery from
gastroenteritis. Over the next 5 days, diagnostic testing revealed
negative TB skin test and toxicology screens. Bone marrow biopsy, CT,
and MRI scans were normal. Following repeat lumbar punctures with
continued high protein levels of 246 and abnormal findings from a
nerve conduction study, a diagnosis of GBS was made. Intravenous IgG
(IVIG) was introduced. A respiratory care consult was ordered to
evaluate her respiratory status. Due to the patient’s age and
inability to cooperate, attempts to perform bedside pulmonary
function studies failed. Over the next 24 hours, she developed
tachycardia, tachypnea, O2 desaturations to 92%, left eye
pstosis, and drooling. With ABG analysis on room air revealing a pH
7.38, PaCO2 50, PaO2 79, HCO3 29, BE
+2.9, the patient was transferred to the ICU for closer monitoring.
Although BiPAP was considered, it was not necessary as respiratory
deterioration was avoided through bag-valve-mask hyperinflation
therapy of 10 breaths given with a peak inspiratory pressure of 25
cmH2O and pressure hold of 5-10 seconds. This therapy was
continued until discharge. After completion of IVIG, the patient
steadily improved and was discharged home after a 17-day hospital
stay.
Discussion:
Although GBS is readily diagnosed in adults who present with
typical features, diagnosis in young children can be delayed when
presenting neurological symptoms are generalized. The assessment of
ventilatory reserve through objective pulmonary function measurements
is often not possible in a young child who cannot perform the FVC
maneuver. The respiratory therapist, therefore, must depend on serial
observations, primarily of physical parameters, that indicate
pulmonary dysfunction. The determination that progressive pulmonary
dysfunction is occurring will allow for early intervention with
respiratory therapy modalities, with the great potential to prevent
frank respiratory failure and associated acute pulmonary events. This
case of GBS in a young child is a clear example of the need for
careful physical assessment of pulmonary function over time to
determine the potential for respiratory insufficiency and failure.
GUILLAIN-BARRE’SYNDROME AND PULMONARY INSUFFICIENCY IN THE YOUNGER PATIENT: AN ATYPICAL PRESENTATION