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A Case of Bickerstaff S Brainstem Encephalitis in Childhood '

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A Case of Bickerstaff S Brainstem Encephalitis in Childhood ' View metadata, citation and similar papers at core.ac.uk brought to you by CORE provided by Directory of Open Access Journals Korean Journal of Pediatrics Vol. 53, No. 4, 2010 DOI : 10.3345/kjp.2010.53.4.607 Case report 1)jtj A case of Bickerstaff’s brainstem encephalitis in childhood Ji Youn Kim, M.D., Young Ok Kim, M.D., Young Jun Son, M.D. and Young Jong Woo, M.D. Department of Pediatrics, Chonnam National University Medical School, Gwangju, Korea = Abstract = Bickerstaff's brainstem encephalitis (BBE) is a rare disease diagnosed by specific clinical features such as 'progressive, relatively symmetric external ophthalmoplegia and ataxia by 4 weeks' and 'disturbance of consciousness or hyperreflexia' after the exclusion of other diseases involving the brain stem. Anti-ganglioside antibodies (GM, GD and GQ) in the serum or cerebrospinal fluid (CSF) are sometimes informative for the diagnosis of BBE because of the rarity of positive findings in other diagnositic methods: brain magnetic resonance imaging (MRI), routine CSF examination, motor nerve conduction study, and needle electromyography. We report a rare case of childhood BBE with elevated anti-GM1 antibodies in the serum, who had specific clinical symptoms such as a cranial polyneuropathy presenting as ophthalmoplegia, dysarthria, dysphagia, and facial weakness; progressive motor weakness; altered mental status; and ataxia. However, the brain MRI, routine CSF examination, nerve conduction studies, electromyography, somatosensory evoked potentials, and brainstem auditory evoked potentials were normal. BBE was suspected and the patient was successfully treated with intravenous immunoglobulins. (Korean J Pediatr 2010;53:607-611) Key Words : Encephalitis, Brain stem, Child bulins (IVIG) for the treatment of BBE suggests an auto- Introduction immune etiology5, 6). There have been several reports of BBE associated with antiganglioside antibodies. Anti-GQ1b Bickerstaff's brain stem encephalitis (BBE) is characte- antibodies are frequently detected in as much as among 66 rized by the acute onset of external ophthalmoplegia, ata- % of patients with BBE4, 7). Other antibodies that may be xia, altered consciousness, and hyperreflexia after the positive in patients with BBE are: anti-GM1, anti GD1a, exclusion for other diseases involving the brain stem1-3). and anti-Ga1NAc-Gd1a8). We report a case of BBE with In addition, the neurological signs that suggest this abnor- elevated anti-GM1 antibodies in the serum and CSF in mality of the central nerves system presents with: abnor- addition to specific clinical symptoms: a cranial polyneuro- malities of the pupils, facial weakness, bulbar palsy, dy- pathy presenting as ophthalmoplegia, dysarthria, dyspha- sesthesia, limb weakness, positive Babinski sign, nystag- gia, and facial weakness; progressive motor weakness; al- mus, and blepharotosis4). tered mental status; and ataxia. Most cases have a history of a prior infection such as Campylobacter jejuni that can be diagnosed by serum IgM, Case report IgG, and IgA antibody titers to C. jejuni1-3), an autoim- mune mechanism produced by microbial infection may A previously healthy girl, 9 years 6 months of age, pre- trigger the pathogenesis of BBE4). The successful use of sented to the Chonnam National University Hospital plasmapheresis, steroid treatment, intravenous immunoglo- (CNUH) due to double vision, slurred speech and intermit- tent ataxia following a fever that was documented once Received : 25 September 2009, Revised : 5 January 2010 three days before admission. There were no other asso- Accepted : 2 March 2010 ciated symptoms such as diarrhea, cough, coryza or sore Address for correspondence : Young Ok Kim, M.D. Department of Pediatrics, Chonnam National University Hospital throat. On the day following admission her both eyelids 8 Hakdong, Gwangju 501-757, Korea became ptotic and the eyes were totally opthalmoplegic. Tel : +82.62-220-6646, Fax : +82.62-222-6103 E-mail : [email protected] Both pupils were dilated and fixed without light reflexes. - 607 - JY Kim, YO Kim, YJ Son, et al. Hospital day 1st 2nd 3rd 5th 6th 7th 8th 10th 11th 12th 13th 19th 20th 23th 24th 25th 37th Mental status Alert Drowsy/Stuporous Alert G5/G5 G5/G5 G3/G3 Muscle strength G3/G3 G2/G2 G4/G4 G5/G5 Upper (R/L) G5/G5 G3/G3 Lower (R/L) G3/G3 G2/G2 G4/G4 G5/G5 Bulbar palsy Dysarthria Dysarthria, dysphagia, facial palsy, respiratory failure Mild facial palsy Limited gaze (+) Ocular movement Intact Total ophthalmoplegia (external and internal) with ptosis DTR (R/L) ++/++ +/+ ++/++ IVIG Treatment MethyIPRD Ventilator care Fig. 1. Patient hospital course. Abbreviations : R, right; L, left; DTR, deep tendon reflex; G, grade; IVIG, intravenous immunoglobulin; MethylPRD, methylprednisolone. Fig. 2. Brain magnetic resonance imaging scan obtained on day 2 shows no abnormal intracra- nial findings in T1 (A) and T2 (B) weighted images. The patient could not wrinkle her forehead or push her pH and bicarbonate), urine analysis and CSF examination. tongue out. In addition, she was unable to swallow. The Additional studies revealed increased levels of IgM and finger-to-nose and heel-to-knee maneuvers were ab- IgG anti-GM1 antibodies, both in CSF and serum. IgM 3 normal bilaterally. The muscle strength in the extremities EU/mL and IgG 3-6 EU/mL (normal, 0 EU/mL), respec- was reduced in the lower to upper limbs and was grade tively, in the CSF; and IgM 20.32 EU/mL and IgG 32.57 2-3 on the Medical Research Council Scale. Deep tendon EU/mL (normal, <20 EU/mL), respectively, in the serum. reflexes were brisk but sensation was intact. The patient In addition, there was transient elevation of antistrep- gradually became drowsy and stuporous. The patient rapi- tolysin O (ASO) (845 IU/mL on admission and 438 IU on dly deteriorated over the first two weeks and developed 11th day) in the serum and transient positive mycoplasma transient malignant hypertension with seizures on 7th day. antibodies (1:80) associated with a positive cold agglutinin However, by one month gradual improvement was noted test (1:128) in the blood. No abnormal findings were re- (Fig. 1). vealed on repeat CSF examination on day 5: stain, culture, The routine tests on admission showed no abnormalities oligoclonal bands, myelin basic protein, IgG and albumin in the complete blood count, blood chemistry (including (including Ig G index), mycoplasma PCR and herpes sim- plasma glucose, ammonia, electrolytes, C-reactive protein, plex virus (HSV) PCR. The plasma renin activity (PRA, - 608 - A case of Bickerstaff’s brainstem encephalitis in childhood 10.5 ng/mL/hr) was transiently increased with the deve- outpatient clinic with mild limitation of the right lateral gaze lopment of malignant hypertension. However, during follow and facial expression. However, all symptoms completely -up, the PRA and aldosterone levels returned to normal resolved by about three months after discharge. (1.3 ng/mL/hr and <1.0 ng/dL, respectively). The brain magnetic resonance imaging (MRI) on admission showed Discussion no abnormalities (Fig. 2). The electroencephalogram was normal on admission but showed irregular high amplitude Bickerstaff and Cloake1) reported three cases of drowsi- delta wave slow activity during deterioration of the pa- ness, ophthalmoplegia and ataxia in 1951, and proposed that tient’s mental status. However, there were no epileptiform the lesion responsible for these clinical signs was in the discharges postictally on day 7 when malignant hyperten- midbrain. Bickerstaff9) reviewed this syndrome for the sion was noted (Fig.3). The nerve conduction velocity, handbook of Clinical Neurology under the title of 'brainstem electromyography, somatosensory evoked potentials, brain encephalitis. The diagnostic criteria for BBE include: (1) stem auditory evoked potentials and visual evoked poten- progressive, relatively symmetric ophthalmoplegia and tials evaluated during admission in 1 month showed no ataxia by four weeks, (2) either altered consciousness abnormal findings. (coma, semicoma or stupor) or pyramidal signs (hyperre- The patient was diagnosed with brainstem encephalitis flexia or pathological reflexes), and (3) limb strength of 5 or with a bilateral cranial polyneuropathy including the cranial 4 on the Medical Research Council scale10). The clinical second, third, fourth, sixth, seventh, ninth, and twelfth symptoms of BBE are similar to those of the Fisher syn- nerves. We treated the patient with intravenous immuno- drome (FS); the common features are ophthalmoplegia, globulins (400 mg/kg for 5 days) and methylprednisolone ataxia and CSF albumin-cytological dissociation10). How- (for the first 3 days). On day 5 she required the assisted ever, the diagnosis of BBE must include alteration of con- ventilation until day 18 due to respiratory failure; however, sciousness or pyramidal signs, which reflect a serious by day 18 she could be weaned from this respiratory brainstem lesion; the FS does not include these charac- support. The extraocular nerve palsies, motor weakness teristics10). and deep tendon reflexes gradually improved over the next The etiology of BBE remains unknown. However, one three weeks. The deep tendon reflexes were weakly widely accepted hypothesis suggests an infectious agent present in all four extremities. The patient was discharged triggers an immunological response3, 11-15). Bickerstaff and after 37 days of admission and was followed in the Cloake1) speculated that the etiology of BBE is similar to Fig. 3. The postictal electroencephalography
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