Perinatal/Neonatal Case Presentation &&&&&&&&&&&&&& Transverse Myelitis: Unusual Sequelae of Neonatal Group B Streptococcus Disease

Michael S. Schimmel, MD mechanical ventilation and dopamine infusion. Intractable seizures Yechiel Schlesinger, MD required combined anticonvulsant medications (phenobarbital, Itai Berger, MD phenytoin, and continuous drip of lidocaine). Head ultrasonography Avraham Steinberg, MD (US), confirmed by computerized tomography (CT), noted brain Arthur I. Eidelman, MD edema and small cortical bleeding foci. At day 10 the baby was found to be paraplegic, i.e., completely flaccid with no spontaneous movements of lower extremities, a-reflexic, no motor response to pain stimulation, and with a sensory level at T10. Electromyography Group B streptococcus (GBS) meningitis is one of the most serious revealed no spontaneous activity in both lower extremities and very low complexes in response to stimulation. Nerve conduction velocity infections in the neonatal period. We wish to report a case of transverse (NCV) showed no motor response in the tibialis nerves and vastus myelitis following early onset form of GBS meningitis. The diagnosis and lateralis muscle. Sensory NCV showed severe deficit. Stimulation of the clinical approach will be discussed. ulnar and median nerves revealed normal responses. At 2 weeks, sleep Journal of Perinatology (2002) 22, 580 – 581 doi:10.1038/sj.jp.7210777 EEG showed alpha-like activity representing epileptogenic activity. Bladder studies were compatible with a neurogenic bladder, hypotonic type. Magnetic resonance image (MRI) of the spinal cord Group B streptococcus (GBS) is the most serious infection in the was normal. Due to this clinical presentation, a diagnosis of term infant and is characterized in its’ early-onset form primarily by transverse myelitis was made. Subsequent CT and ultrasound studies generalized sepsis. In contrast, meningitis is more common in the of the brain showed severe subcortical necrosis. late-onset form.1 We wish to describe a newborn that developed an Five months later, the baby was still completely paraplegic with unusual neurologic complication following early onset of GBS a sensory level at T10 and with a neurogenic bladder. Follow-up meningitis. spinal cord MRI was normal. However, EMG and NCV demonstrated severe motor and sensory axonal injury to both lower extremities, with no improvement compared with the initial CASE REPORT investigation. A baby boy was born to a gravida 4, para 3 healthy mother after 38 weeks of an uneventful pregnancy. Spontaneous rupture of DISCUSSION membranes occurred 6 hours before labor. Birth weight was 2310 g; head circumference was 32.5 cm. Initial physical examination was Transverse myelitis is a clinical diagnosis and was defined by 2 normal. At 48 hours, the baby was noted to be lethargic and a Fenichel as an acute demyelinating disorder of the spinal cord in lumbar puncture noted a CSF WBC count of 150/ml and a CSF which spinal cord imaging revealed a normal spinal cord and protein and sugar of 656 and 10 mg/dl, respectively. Both blood excluded acute cord compression. In the literature, there is a culture and CSF culture were positive for GBS. Treatment consisted of significant confusion between myelitis and myelopathy, the latter intravenous ampicillin and cefotaxime. Due to respiratory representing a situation with demonstrated spinal cord pathology. 3 deterioration and unstable blood pressure, the baby required Coker et al. reported four preterm infants with meningitis complicated by myelopathy (three were infected with E. coli and one with GBS). One infant had spinal cord atrophy seen in MRI confirming the diagnosis of myelopathy. One infant had extensive Department of Neonatology ( M.S.S., A.I.E. ), Shaare Zedek Medical Center, Jerusalem, Israel; Infectious Disease Unit ( Y.S. ), Shaare Zedek Medical Center, Jerusalem, Israel; Pediatric necrosis of the entire gray matter of the cervical spinal cord as seen at Neurology ( I.B., A.S. ), Shaare Zedek Medical Center, Jerusalem, Israel; School of Medicine autopsy. The other two infants had no spinal cord imaging studies ( M.S.S., A.I.E. ), Ben - Gurion University, Beer Sheva, Israel; and School of Medicine ( A.I.E., 4 A.S. ), Hebrew University, Jerusalem, Israel. and thus their exact diagnosis is unclear. Puvabanditsin et al. described a preterm infant with GBS meningitis who developed acute Address correspondence and reprint requests to Michael S. Schimmel, MD, Department of Neonatology, Shaare Zedek Medical Center, PO Box 3235, Jerusalem 91031, Israel. transverse myelitis with subsequent spinal cord cavitation. Thus, the

Journal of Perinatology 2002; 22:580 – 581 # 2002 Nature Publishing Group All rights reserved. 0743-8346/02 $25 580 www.nature.com / jp Transverse Myelitis, Sequelae of Neonatal GBS Disease, Neonatal GBS Disease Schimmel et al.

diagnosis of transverse meylitis in this case does not meet the However, the recently reported6 case of an oculomotor nerve criteria of Fenichel’s.2 Moffett and Berkowitz5 presented a term infant palsy following late onset of GBS at 25 days of life, which resolved with E. coli meningitis complicated by spinal cord dysfunction after 5 days of intravenous steroids suggests that steroid treatment resulting in quadriplegia and urinary retention. CT and MRI did not may be indicated when the specific diagnosis of myelitis is made. show any spinal cord abnormalities. Moffett’s description matches Thus, there is need for imaging of the spinal cord, including MRI, Fenichel’s definition of transverse myelitis following meningitis. before any steroid therapy is contemplated. Pending such a To the best of our knowledge this is the first reported case of a diagnosis of myelitis, therapy should be limited to appropriate GBS infection complicated by permanent neurologic damage antibiotics and maximum supportive care with an emphasis on secondary to transverse myelitis. The diagnosis was based on the maintaining blood pressure and perfusion so as to minimize any acute onset of paraplegia, decreased anal tone and a neurogenic possibility of ischemic damage to the spinal cord. bladder following GBS meningitis. Myelopathy was excluded, as repeated MRI failed to demonstrate a spinal cord lesion. Moffett and Berkowitz5 postulated that spinal cord vasculitis References secondary to an arteritis occurring in the acute phase of bacterial 1. Edwards ME, Baker CJ. Group B streptococcal infections. In: Remington JS, meningitis is the underlying etiology. Whereas an alternative Klein JO, editors. Infectious Diseases of the Fetus and Newborn Infant. 5th ed. mechanism may be of micro infarctions induced by micro-emboli Philadelphia, PA: WB Saunders; 2001. p. 1091–156. that obstruct the tiny vascular bed of the spinal cord, the technical 2. Fenichel GM. Clinical pediatric neurology. A signs and symptoms approach. limitations and resolution power of the currently available imaging 3rd ed. Philadelphia, PA: WB Saunders; 1997. techniques precludes making this diagnosis. 3. Coker SB, Muraskas JK, Thomas CT. Myelopathy secondary to neonatal bacterial meningitis. Pediatr Neurol 1994;10:259–61. The effectiveness of adjunctive treatment of antimicrobial therapy 4. Puvabanditsin S, Wojdylo EW, Kalavantavanich K. Group B streptococcal with steroids to reduce the sequelae in bacterial meningitis has meningitis, a case of transverse myelitis with spinal cord and posterior fossa 7 been studied in various clinical trails. Odio et al. noted a decrease in cysts. Pediatr Radiol 1997;27:317–8. hearing deficit after steroid therapy in patients with Haemophilus 5. Moffett KS, Berkowitz FE. Quadriplegia complicating Escherichia coli influenza type B meningitis in infants 6 weeks to 13 years. meningitis in newborn infant, case report and review of 22 cases of spinal Ciana et al.8 in his study in children living in developing countries cord dysfunction in patients with acute bacterial meningitis. Clin Infect Dis reported the same beneficial effect of steroids. 1997;25:211–4. As none of these clinical trials were performed in the neonatal 6. Murakami K. A case of late onset of group B streptococcus meningitis with period, the justification for routine use of steroid therapy as part of transient oculomotor nerve palsy. No to Hattatsu 1999;31:549–52. the management of early-onset neonatal bacterial meningitis is 7. Odio CM, Faingezicht I, Paris M, et al. The beneficial effects of early dexa- questionable. In fact a most recent textbook of pediatric infectious methasone administration in infants and children with bacterial meningitis. diseases explicitly states:9 ‘‘Despite the beneficial effects of N Engl J Med 1991;324:1525–31. 8. Ciana G, Parmar N, Antonio C, Pivetta S, Tamburlini G, Cuttini M. dexamethasone in several recent studies for the treatment of infants Effectiveness of adjunctive treatment with steroids in reducing short term and children with bacterial meningitis, no data are available for its mortality in a high risk population of children with bacterial meningitis. J Trop use in newborns; thus, utilization of steroids in neonatal meningitis Pediatr 1995;41:164–8. cannot be recommended at present.’’ This is particularly true, as 9. Saez-Llorens X, McCracken GH Jr. Perinatal bacterial diseases. In: Feigin RD, transverse myelitis following meningitis is an extremely uncommon Cherry JD, editors. Textbook of Pediatric Infectious Diseases. 4th ed. Phila- complication. delphia, PA: WB Saunders; 1998. p. 909.

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