e u r o p e a n j o u r n a l o f p a e d i a t r i c n e u r o l o g y 1 8 ( 2 0 1 4 ) 4 4 4 e4 4 8
Official Journal of the European Paediatric Neurology Society
Case study
CNS vasculitis and stroke in neonatal lupus erythematosus: A case report and review of literature Arushi G. Saini a, Naveen Sankhyan a, Sagar Bhattad a, Sameer Vyas b, Biman Saikia c, Pratibha Singhi a,* a
Department of Pediatrics, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh 160012, India b Department of Radiodiagnosis, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh 160012, India c Department of Immunopathology, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh 160012, India
article info
abstract
Article history:
Neonatal lupus erythematosus refers to the clinical spectrum of cardiac, cutaneous and
Received 3 September 2013
other systemic abnormalities in neonates born to mothers with autoantibodies against Ro/
Received in revised form
SSA and La/SSB antigens. Isolated central nervous system involvement is very rare and has
12 January 2014
been described as transient vasculopathy only. We describe a 2-months-old girl who pre-
Accepted 19 January 2014
sented with acute ischemic stroke secondary to central nervous system vasculitis without any cardiac, cutaneous or hematological manifestations. The mother was pauci-symp-
Keywords:
tomatic with raised anti-Ro autoantibody titers; the baby was positive for autoantibodies
Neonatal lupus
against Ro-antigen. Angiography confirmed vasculitis in cerebral vasculature. Our case
Vasculitis
highlights that neonatal lupus erythematosus can present with isolated nervous system
Stroke
manifestations and the vascular damage can be permanent in the form of vasculitis. Early
Congenital heart block
recognition will help pediatricians identify such possible permanent complications in
Vasculopathy
newborns with neonatal lupus erythematosus. A review of previously reported central nervous system manifestations of neonatal lupus is also presented. ª 2014 Published by Elsevier Ltd on behalf of European Paediatric Neurology Society.
1.
Introduction
Neonatal lupus erythematosus (NLE) is a passively acquired, autoimmune disease of the developing fetus and neonate defined by characteristic clinical features in the presence of
specific maternal anti-Ro/SSA or anti-La/SSB antibodies. The most common clinical manifestations are fetal-onset permanent cardiac conduction defects; transient, non-scarring papulo-squamous rash; transient hepatic dysfunction and variable cytopenias. To the best of our knowledge, acute, ischemic stroke in infancy has not been described with this
* Corresponding author. Advanced Pediatric Centre, PGIMER, Chandigarh 160012, India. Tel.: þ91 172 5304x275, þ91 172 6699x274; fax: þ91 172 4401x274, þ91 172 5078x274. E-mail address: [email protected] (P. Singhi). 1090-3798/$ e see front matter ª 2014 Published by Elsevier Ltd on behalf of European Paediatric Neurology Society. http://dx.doi.org/10.1016/j.ejpn.2014.01.007
e u r o p e a n j o u r n a l o f p a e d i a t r i c n e u r o l o g y 1 8 ( 2 0 1 4 ) 4 4 4 e4 4 8
condition. The central nervous system (CNS) vasculopathy described till now has been found to be particularly nonprogressive or transient. We describe a 2-months-old girl with isolated CNS manifestation of NLE in the form of cerebral vasculitis resulting in acute, arterial ischemic stroke with permanent neurological deficits in early infancy. Our case highlights the unique aspects of the disease process that may help pediatricians improve patient care and timely recognize the possible permanent complications such as CNS vasculitis in newborns or young infants with NLE.
2.
Case study
A previously well, two-months-old girl presented with a history of low grade, intermittent fever for the past one week. On day five of fever, she developed lethargy, poor feeding, rapid breathing and three episodes of left-sided clonic seizures. There was no history of cyanosis, cough, altered sensorium, rash, icterus, bleeding from any site, or trauma. Her fever and other systemic symptoms subsided with intravenous antibiotics and anticonvulsant (phenytoin 8 mg/kg/day and phenobarbitone 5 mg/kg/day) therapy. However, on day 14 of the illness, she had a recurrence of focal seizures and was then referred to our centre for further care. She was born to non-consanguineous parents by term elective caesariansection (for breech position). Birth weight was 2.7 kg and the perinatal period was uneventful. Prior to the illness, the child had attained social smile, face-regard, partial neck-control and turning of head to sound. The mother had a past history of fever and joint pains lasting for a week, one-year ago. She also complained of occasional joint stiffness of the hands in the morning. There was no history of drug intake, photosensitive rash, joint swelling, oral ulcers, hair loss or weight loss in the mother. On examination the baby’s weight was 2.8 kg (
Official Journal of the European Paediatric Neurology Society
Case study
CNS vasculitis and stroke in neonatal lupus erythematosus: A case report and review of literature Arushi G. Saini a, Naveen Sankhyan a, Sagar Bhattad a, Sameer Vyas b, Biman Saikia c, Pratibha Singhi a,* a
Department of Pediatrics, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh 160012, India b Department of Radiodiagnosis, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh 160012, India c Department of Immunopathology, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh 160012, India
article info
abstract
Article history:
Neonatal lupus erythematosus refers to the clinical spectrum of cardiac, cutaneous and
Received 3 September 2013
other systemic abnormalities in neonates born to mothers with autoantibodies against Ro/
Received in revised form
SSA and La/SSB antigens. Isolated central nervous system involvement is very rare and has
12 January 2014
been described as transient vasculopathy only. We describe a 2-months-old girl who pre-
Accepted 19 January 2014
sented with acute ischemic stroke secondary to central nervous system vasculitis without any cardiac, cutaneous or hematological manifestations. The mother was pauci-symp-
Keywords:
tomatic with raised anti-Ro autoantibody titers; the baby was positive for autoantibodies
Neonatal lupus
against Ro-antigen. Angiography confirmed vasculitis in cerebral vasculature. Our case
Vasculitis
highlights that neonatal lupus erythematosus can present with isolated nervous system
Stroke
manifestations and the vascular damage can be permanent in the form of vasculitis. Early
Congenital heart block
recognition will help pediatricians identify such possible permanent complications in
Vasculopathy
newborns with neonatal lupus erythematosus. A review of previously reported central nervous system manifestations of neonatal lupus is also presented. ª 2014 Published by Elsevier Ltd on behalf of European Paediatric Neurology Society.
1.
Introduction
Neonatal lupus erythematosus (NLE) is a passively acquired, autoimmune disease of the developing fetus and neonate defined by characteristic clinical features in the presence of
specific maternal anti-Ro/SSA or anti-La/SSB antibodies. The most common clinical manifestations are fetal-onset permanent cardiac conduction defects; transient, non-scarring papulo-squamous rash; transient hepatic dysfunction and variable cytopenias. To the best of our knowledge, acute, ischemic stroke in infancy has not been described with this
* Corresponding author. Advanced Pediatric Centre, PGIMER, Chandigarh 160012, India. Tel.: þ91 172 5304x275, þ91 172 6699x274; fax: þ91 172 4401x274, þ91 172 5078x274. E-mail address: [email protected] (P. Singhi). 1090-3798/$ e see front matter ª 2014 Published by Elsevier Ltd on behalf of European Paediatric Neurology Society. http://dx.doi.org/10.1016/j.ejpn.2014.01.007
e u r o p e a n j o u r n a l o f p a e d i a t r i c n e u r o l o g y 1 8 ( 2 0 1 4 ) 4 4 4 e4 4 8
condition. The central nervous system (CNS) vasculopathy described till now has been found to be particularly nonprogressive or transient. We describe a 2-months-old girl with isolated CNS manifestation of NLE in the form of cerebral vasculitis resulting in acute, arterial ischemic stroke with permanent neurological deficits in early infancy. Our case highlights the unique aspects of the disease process that may help pediatricians improve patient care and timely recognize the possible permanent complications such as CNS vasculitis in newborns or young infants with NLE.
2.
Case study
A previously well, two-months-old girl presented with a history of low grade, intermittent fever for the past one week. On day five of fever, she developed lethargy, poor feeding, rapid breathing and three episodes of left-sided clonic seizures. There was no history of cyanosis, cough, altered sensorium, rash, icterus, bleeding from any site, or trauma. Her fever and other systemic symptoms subsided with intravenous antibiotics and anticonvulsant (phenytoin 8 mg/kg/day and phenobarbitone 5 mg/kg/day) therapy. However, on day 14 of the illness, she had a recurrence of focal seizures and was then referred to our centre for further care. She was born to non-consanguineous parents by term elective caesariansection (for breech position). Birth weight was 2.7 kg and the perinatal period was uneventful. Prior to the illness, the child had attained social smile, face-regard, partial neck-control and turning of head to sound. The mother had a past history of fever and joint pains lasting for a week, one-year ago. She also complained of occasional joint stiffness of the hands in the morning. There was no history of drug intake, photosensitive rash, joint swelling, oral ulcers, hair loss or weight loss in the mother. On examination the baby’s weight was 2.8 kg (
