Case Report Guillain-Barre Syndrome in a Three-Month-Old Infant Wen-Kan Feng1 Kun-Long Hung2,3,* Chien-Hung Liu2 We report a three-month-old infant who suffered from progressive motor weakness within four days after fistulectomy for anal fistula. From the clinical presentations and the results of laboratory studies included spinal MR images and electrophysiological study, Guillain-Barre syndrome(GBS) was diagnosed. Immunotherapy with intravenous immunoglobulin was prescribed early after diagnosis ascertained. Following rehabilitation was performed that good outcome had been achieved. To our knowledge, this case might be the youngest patient of noncongenital Guillain-Barre syndrome that had been reported before. GBS in young infant is rare and clinical presentation is not distinct. It is very important that a infant with suspected GBS is managed as an emergency. Spinal MR imaging as a supplementary diagnostic modality is suggested. ( FJJM 2010; 8 (1) : 57-61 ) Key words: Guillain-Barré syndrome, infant, flaccid paralysis, areflexia, intravenous immunoglobulin INTRODUCTION Guillain-Barre syndrome (GBS) is the most common cause of flaccid paralysis in children and is characterized by various degrees of motor weakness, sensory abnormalities and autonomic dysfunction. Several clinical forms of GBS have been suggested based on clinical and electrodiagnostic feactures. In some forms, paralysis progresses very rapidly that lead to ventilatory failure, life-threatening arrhythmias and hypertension. Aspiration pneumonia due to in-coordinate swallowing movement is a hazard in young patients. The overall mortality in childhood GBS is about 5 %. Although GBS is far more common in adult, it occurs in children that the annual incidence is Department of Pediatrics, Sijhih Cathay General Hospital, Taipei1 Catholic University, School of Medicine, Taipei County, Taiwan3 Submitted March, 01, 2010; final version accepted March, 31, 2010. *Correspondence author: [email protected] 輔仁醫學期刊第 8 卷第 1 期 2010 about 0.1 cases per 100000(3), but infant as young as 3 months of age had never been reported. We reported a 3-month-old boy of GBS occuring soon after fistulectomy for anal abscess. According to the literature we reviewed, this might be the youngest case of non-congenital GBS that had been reported recently. CASE REPORT This 3-month-old boy had suffered from anal fistula with relapsing abscess formation since 15 days old. He admitted to our hospital for surgical intervention. Fistulectomy was performed smoothly under general anesthesia with intravenous Ketamin injection. However, weakness of both lower limbs Department of Pediatrics, Cathay General Hospital, Taipei2 Fu-Jen 57 Wen-Kan Feng Kun-Long Hung Chien-Hung Liu occurred on the 4th post-operative day. Under physical examination, the anterior fontanel was open, flat and normal-sized. Both lower limbs and right upper limb were flaccid. Muscle power of both lower limbs was zero and right upper limb was grade two plus. Deep tendon reflexes of both lower limbs were absent and decreased on right upper limb. The left upper limb was normal. No cranial nerve was involved. CSF study revealed cell count of 8 x 3/5, protein level 61 mg/dl, and glucose level 51 mg/dl. Microbiological evaluations included bacterial cultures from CSF, urine, blood and feces grew no aerobic or anaerobic pathogens. No virus was isolated from CSF, urine, throat swab and rectal swab. Serological studies included EBV IgM and CMV IgM were negative, antigen of influenza virus and parainfluenza virus were also negative. Chlamydia IgM antibody was positive. A significant abnormality of clinical neurophysiological studies was noted as shown in Table 1. The amplitude of the compound muscle action potential (CMAP) of bilateral peroneal nerves and left tibial nerve was decreased. There were also blockade of motor nerve conduction velocities (MNCV). The F-wave study remained normal. MRI study (Figure 1) of cervical-thoraciclumbar spine showed enhancement of ventral root at lumber spines. These findings are compatible with demyelinating polyneuropathy. Two successive high doses of intravenous immunoglobulin at 1g/kg/d infusion for two days were given at once, then physicotherapy started. Flaccidity of both lower limbs got little improvement and right upper limb got much improvement at discharge. He receives regular rehabilitation at OPD and is able to stand with support at the age 1 year and 3 month and walk with support six months later. DISCUSSION Gullian-Barre syndrome(GBS) is an acute, immune-mediateddemyelinatingpolyradiculoneuropathy. It is characterized by a classical triad of progressive motor weakness, areflexia and elevated cerebrospinal fluid (CSF) protein without pleocytosis. Severe GBS is associated with progressive motor disability leading to respiratory failure. GBS is known to be an immune-mediated disorder resulting from the autoimmune antibodies that cross-react with epitopes[1,2]on peripheral nerves that damages the nerve roots and axons. The autoantibodies may form in response to a variety of antigenic stimuli, such as bacterial and viral infections. GBS can affect all ages and the annual incidence Table 1. The amplitude of CMAP of bilateral Peroneal N. and left Tibial N. decreased. There were blockade of MNCV over Peroneal N. Nerve Conduction Study Nerve Latency(msec) Voltage(mV) Distance(cm) NCV F-Wave Proximal Proximal Proximal ~ Distal (m/sec) (msec) Distal Distal Distal Lt. Peroneal 5.4 3.2 0.3 3.8 8.5 2.5 38.6 Lt. Tibial 5.7 2.7 1.5 2.7 10 3 33.3 26.5 3.5 28.8 25.7 Rt. Peroneal Rt. Tibial 58 (Non- Pick Up) 6.1 2.1 0.6 0.5 11.5 Fu-Jen Journal of Medicine Vol.8 No.1 2010 GBS in young Infant Fig 1. Figure. Post-contrast(right) enhanced T1-FLAIR MRI of the lumber spine showed prominent enhancement of the ventral root(arrow) is steadily increased with age at 2 to 4 per 100,000 populations. GBS is less common in children that the annual incidence is about 0.1 case per 100,000 [3] . Except the neonatal GBS[4]that caused by blocking antibodies transmitted from mother with GBS transplacentally, this GBS patient as young as three months old in age is extremely rare. The diagnosis of a flaccid young infant of GBS should be differentiated to infant botulism, poliomyelitis, myasthenia gravis and infant muscular atrophy [5]. Failure to recognize the correct diagnosis and inappropriate treatment increased the morbidity. Although the diagnosis of GBS is clinical, electrophysiological studies are helpful. The most frequent findings on electrodiagnostic testing in GBS are proximal conduction block with increased distal latency and generalized slowing. Additionally, abnormal F wave studies were significantly characteristic in early GBS[6]. MRI is suggested as a supplementary diagnostic modality when the clinical and electrophysiological findings are equivocal. It revealed contrast enhancement in varying degrees in the spinal nerve roots surrounding the conus medullaris and extending the length of the cauda equine, indicating radicular inflammation, that anterior nerve roots enhanced more intensely 輔仁醫學期刊第 8 卷第 1 期 2010 than the posterior roots[7,8]. Recently, contrastenhanced spinal MR imaging becomes more important in diagnosing GBS in children, especially young infants. More than 70% of the patients had reported a preceding infection (usually virus-like respiratory or gastroenteric illness[9]confirms in GBS. Cytomegalovirus, EBV, mycoplasma pneumoniae and Campylobacter jejuni infection are particularly common in this syndrome[10]. Haemophilus influenzae infection was reported a high incidence of GBS by a Japanese group[10]. Other events as surgery, vaccination, malignancy and pregnancy are frequently associated with GBS. Our case experienced relapsing anal fistula with abscess and surgical intervention that might be the trigger factor. It is very important that a child with suspected GBS is managed as an emergency. So early diagnosis and treatment in GBS especially in young patients . Early treatment with intravenous immunoglobulin and methylprednisolone was emphasized for shorten the progressive course, presumably by limiting nerve damage, and regain independent walking[1, 7,11] . 59 Wen-Kan Feng Kun-Long Hung Chien-Hung Liu REFERENCES 1. 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Fu-Jen Journal of Medicine Vol.8 No.1 2010 GBS in young Infant 發生於三個月嬰兒之 Guillain-Barre syndrome 馮文侃 1 洪焜隆 2,3,* 劉建宏 2 我們報告一名三個月大發生 Guillain-Barre syndrome (GBS)的嬰孩，這可能是文獻發表最年輕的非先天性 GBS 病例，本報告分析臨床表現、檢查結果，包括脊椎 MRI 及電生理學檢查，對正確早期診斷 GBS 很有幫助，早期發現和及時使用靜脈免疫球蛋白治療，對病患的預後有很好的效果。(輔仁醫學期刊 2010；8 (1)：57-61) 關鍵字：Guillain-Barr 症候群，弛緩性麻痺，反射消失，嬰幼兒，免疫球蛋白汐止國泰綜合醫院小兒科 1 國泰綜合醫院小兒科 2 輔仁大學醫學系 3 投搞日期：2010 年 03 月 01 日；接受日期：2010 年 03 月 31 日 *通訊聯絡作者: 電子郵件: [email protected] 輔仁醫學期刊第 8 卷第 1 期 2010 61