DOI: https://doi.org/10.21203/rs.3.rs-2382481/v1
Guillain-Barré syndrome is a type of immune-mediated acute polyradiculoneuritis that is typically preceded by an unspecified infection. A 6-year-old black male preliminary school student who admitted to the pediatric ward with generalized body weakness comprising both lower and upper extremities (unable to move legs and hands as a usual motor movement), not walking alone without caregiver assistance, being unable to swallow food, blurred vision, tingling in the feet and hands, an irregular heartbeat, and severe muscular pain. The patient had quadriparesis, or muscle weakness, in all four limbs (both legs and arms). Motor examination revealed decreased muscle strength in all limbs, with a Medical Research Council score of 2/5 in the right arm of the upper extremities and 1/5 in the right leg of the lower extremities, and 1/5 in the left leg of the lower extremities and 2/5 in the left arm of the upper extremities. According to the clinical signs of Guillain-Barré syndrome, he received 400 mg/kg of intravenous immunoglobulin every day for five days.
Vaccine-associated Guillain–Barré syndrome (GBS) is defined as the onset of GBS symptoms within a six-week period after receiving a vaccine, as demonstrated by the Vaccine Adverse Event Reporting System [1]. Guillain–Barré syndrome is an acute immune-mediated disorder that affects the peripheral nervous system, resulting in flaccid paralysis and polyradiculoneuritis [2, 3]. Progressive motor weakness, areflexia, and albuminocytologic dissociation are the classical triad characterizations of Guillain–Barré syndrome [4]. Guillain-Barré syndrome is uncommon in children because it is associated with unilateral peripheral facial nerve palsy [5]. The incidence of Guillain–Barré syndrome is 0.5–2 per 100, 000 pediatric patients younger than eighteen years [6]. The common signs and symptoms of Guillain–Barré syndrome are progressive, ascending, symmetrical flaccid limb paralysis, along with areflexia or hyporeflexia, and with or without cranial nerve problems [7]. The typical etiology of Guillain–Barré syndrome is an infectious disease, commonly caused by Campylobacter jejuni. Vaccines and vaccinations like a meningococcal vaccine, a poliovirus vaccine, an influenza vaccine, and a rabies vaccine are rarely associated with Guillain–Barré syndrome [8]. Early administration of intravenous immunoglobulins or plasma exchange has been shown to be beneficial and necessary, particularly in individuals with fast-progressing weakness [9]. This case report demonstrates the rare case of inactivated poliovirus vaccine-induced Guillain–Barré syndrome in middle age childhood.
A 6-year-old black male elementary school student was admitted to the pediatric ward on June 1, 2022, with a symptom of generalized body weakness (bilateral upper and lower extremity paralysis) 14 days earlier. The patient was given the inactivated poliovirus vaccine at the ages of two months, four months, 14 months, and eventually at the age of five years. Four weeks ago, the patient received the final inactivated poliovirus vaccination. He had no prior history of a fall, travel, urinary tract infections, bowel or urine incontinence, upper respiratory tract infections, or trauma. The patient had not provided any previous medical or medication histories. He had also no medical or medication histories in his family. He was in good health two weeks before his hospitalization and was attending school normally with no health issues.
Up on admission, he presented with generalized body weakness comprising both lower and upper extremities (unable to move legs and hands as a usual motor movement), not walking alone without caregiver assistance, being unable to swallow food, having eyesight problems, having tingling in the feet and hands, having an irregular heartbeat, and experiencing significant muscular discomfort. The patient had quadriparesis, or muscle weakness, in all four limbs (both legs and arms). His vital signs showed a heart rate of 98 beats per minute, a respiratory rate of 18 breaths per minute, a body temperature of 37.8 degrees celsius, and a saturation oxygen level of 96% on room air.
His physical examination revealed that his skin test, ear, lung, and abdominal examinations were all stable or normal. His cardiovascular, dermatologic, and respiratory systems were also normal. To assess the level of consciousness of patient the Glasgow Coma Scale, revealed eye opening reaction was 1/4 (no response), the motor response was 3/6 (abnormal flexion), and the verbal response was 2/5 (incomprehensible sounds). On a Glasgow Coma scale, his overall score was 6 (1/4 + 3/6 + 2/5). According to his Glasgow Coma Scale, he had severe brain damage and was comatose or partially unresponsive. His motor examination indicated reduced muscular strength in all limbs, with a Medical Research Council score of 2/5 in the right arm upper limbs and 1/5 in the right leg of the lower limbs, and 1/5 in the left leg of the lower extremities and 2/5 in the left arm of the upper limbs.
The assessment of the Erasmus Guillain Barré syndrome Respiratory Insufficiency Score revealed that the time lapse between the onset of weakness and the patient's hospital admission was greater than 7 days and scored as 0, facial and/or bulbar weakness at admission was absent and scored as 0, and the Medical Research Council sum score at admission was 34 out of 60 and scored as 2. This patient's Erasmus Guillain Barré Syndrome Respiratory Insufficiency Score was a perfect 2 (0 + 0 + 2). With a Respiratory Insufficiency Score of 0–2 for Erasmus Guillain Barré syndrome, this patient does not require mechanical ventilation.
The laboratory examination shown white blood cell count of 11,200 cells/mm3 (normal value: 4,500 − 11,000 cells/mm3), erythrocyte sedimentation rate of 54 mm/hour (normal value: 0–20 mm/hr), neutrophils of 73% (normal value: 55–70%), lymphocytes of 21% (normal value: 20–40%), serum glucose of 131 mg/dL (normal value: 70–110 mg/dL), serum creatinine of 0.5 mg/dL (normal value: 0.7–1.3 mg/dL), blood urea nitrogen of 33 mg/dL (normal value: 6–20 mg/dL), hemoglobin of 14.8 g/dL (normal value: 13.8–17.2 mg/dL), hematocrit of 43% (normal value: 41–50%), alanine aminotransferase of 42 units/L (normal value: 0–35 units/L), and aspartate aminotransferase of 50 units/L (normal value: 0–35 units/L). The cerebrospinal fluid examination revealed yellow fluid, albuminocytological dissociation with 1.43 g/L proteins, a normal cerebrospinal fluid/serum albumin ratio, and no oligoclonal bands. The presence of albumin-cytological separation supports the diagnosis of Guillain Barré syndrome.
When the patient was admitted, the physician prescribed dexamethasone 8 mg/ 2 ml intravenously twice daily for five days, but the pediatric specialist stopped after only he taken one injection because dexamethasone is not recommended for Guillain-Barre syndrome. On the same day, an oral suspension of ibuprofen 100 mg/5 ml was started, 10 ml three times a day for five days. After cerebrospinal fluid findings supported the diagnosis of Guillain Barré syndrome, he underwent intravenous immunoglobulin 400 mg/kg every day for five days. The patient's caregivers counseled as they made gentle massages, changed the patient’s position frequently, and moved upper and lower extremities commonly. He advised conducting motor stimulation exercises to restore muscle tone, as well as face mobility and paralysis training. The patient was advised not to climb ladders because they were afraid of falling and to avoid rigorous physical activities such as anerobic and aerobic sports.
After thirteen days of pharmacological and non-pharmacological treatment, he partially recovered from paralysis and started walking alone, slowly. The patient was admitted back home with marked improvement on July 16, 2022. His functional motor impairments upon discharge could be examined using the Guillain-Barre syndrome disability scale, and his Hughes disability score revealed that he had a grade 2 Hughes score (able to walk ten meters or more without assistance but unable to run).
Guillain–Barré syndrome is an acute immune-mediated polyradiculoneuritis that affects motor, sensory, and autonomic nerves and presents with a wide range of neurological manifestations, such as progressive flaccid paralysis [10]. In this case, the patient presented with generalized body weakness comprising both lower and upper extremities (unable to move legs and hands as a usual motor movement), not walking alone without caregiver assistance, being unable to swallow food, and severe pain. In Guillain–Barré syndrome, various laboratory investigations comprise white blood cells, neutrophils, erythrocyte sedimentation rate, serum glucose, cerebrospinal fluid, alanine aminotransferase, and aspartate aminotransferase, which were elevated. In this case, cerebrospinal fluid testing revealed yellow-colored fluid, and albuminocytological dissociation with 1.43 g/L proteins, a normal cerebrospinal fluid/serum albumin ratio, and a lack of oligoclonal bands.
The Glasgow coma scale is divided into three categories to assess level of consciousness: A Glasgow coma score of eight or less on the Glasgow coma scale indicates severe brain injury. Class II: Glasgow coma scale 9–12, which revealed moderate brain injury. Class III: Glasgow coma scale 13–15, which showed mild/no brain injury. In this case report, the patient's Glasgow coma scale score was 6, indicating severe brain injury and partial unresponsiveness, or comatoseness. Mechanical ventilation in Guillain-Barré syndrome was evaluated by Erasmus GBS Respiratory Insufficiency. A score of 0–2 for Erasmus GBS Respiratory Insufficiency suggests minimal risk, 3–4 for moderate risk, and greater than or equal to 5 for severe risk for mechanical ventilation [11]. The patient in this study has a respiratory insufficiency score of 2 from Erasmus GBS and does not require mechanical ventilation.
The disability score for Guillain-Barré syndrome (Hughes disability score) was graded as 0 in a healthy state, 1 in a minor symptom state and capable of running, 2 in those able to walk ten meters or more without assistance but unable to run, 3 in those able to walk ten meters across an open space with help, 4 in those bedridden or chairbound, 5 in those requiring assisted ventilation for at least part of the day, and 6 in patients who died [11]. In this study, the patient's Guillain-Barré syndrome disability score was 2, indicating that the patient could walk ten meters or more without help but could not run.
The immune response in Guillain-Barré syndrome damages either myelin (acute inflammatory demyelinating polyradiculoneuritis) or the axon (acute motor axonal neuropathy, as well as acute motor and sensory axonal neuritis [13]. Acute inflammatory demyelinating polyradiculoneuritis; which is a motor sensory demyelinating disorder; acute motor axonal neuropathy, and acute motor and sensory axonal neuropathy are the most common causes of Guillain–Barré syndrome. Some rare causes of Guillain–Barré syndrome comprise Miller Fisher Syndrome, paraparesis Guillain–Barré syndrome, pharyngeal-cervical-brachial weakness, and bilateral facial palsy with paresthesia, Bickerstaff brainstem encephalitis, which can overlap with Miller Fisher Syndrome, polyneuritis cranialis, and acute autonomic neuritis [14–17]. In this study, the patient was diagnosed with acute inflammatory demyelinating polyradiculoneuritis subtype of Guillain-Barré syndrome.
The management of Guillain-Barre syndrome is based on whether the patient has mildly acute, severely acute, or chronic problems [18]. Early commencement of intravenous immunoglobulins or plasma exchange has been shown to be beneficial and critical, particularly in individuals with quickly progressing weakness [19]. Dexamethasone is not recommended for Guillain-Barre syndrome because it suppresses the recruitment of scavengers, which are beneficial for nerve degeneration, resulting in a delay in clinical recovery in GBS [20]. On admission, ibuprofen 100 mg/5 mL oral suspension was initiated, 10 mL three times a day for five days. Ibuprofen prevents inflammation caused by Guillain-Barré syndrome by lowering prostaglandin release and also alleviates pain due to injured large myelinated sensory fibers [21]. Simultaneously, intravenous immunoglobulin (400 mg/kg per day for 5 days) was administered to him to combat the Guillain-Barré syndrome antibodies responsible for nerve damage [22, 23].
The study used face-to-face communication with the patient and was devoid of selection bias, response bias, and information bias. The study was conducted through direct observation; there is no feedback barrier between the investigator and the responder.
There was no follow-up after the patient was discharged to determine whether he was completely recovered and restored to his normal motor strength or not. The patient in this study refused to allow his body to publish on this article, and he only permitted to publish his other information for scientific discussion. The study was not based on systematic investigations to identify the predictors of Guillain-Barré syndrome.
Guillain-Barré syndrome is a heterogeneous disorder causing muscle weakness, sensory change, dysautonomia, and frequently cranial neuritis. Guillain-Barré syndrome perhaps happened spontaneously after bacterial or viral infections, and it has also been correlated with several vaccines. The patient's motor assessment in this study revealed decreasing muscular strength in all limbs with a Medical Research Council score. In this study, the patient received 400 mg/kg per day of intravenous immunoglobulin for five days due to clinical signs of Guillain-Barré syndrome.
Consent for publication
Written informed consent was obtained from the patient for publication of this case report.
Funding
None
Conflict of interest
The author has no financial or proprietary interest in any of the materials discussed in this article.
Acknowledgments
The author is grateful to the study participant for his willingness to respond to all questions.