Central nervous system vasculopathy in neonatal lupus erythematosus

ELSEVIER

Central Nervous System Vasculopathy in Neonatal Lupus Erythematosus F e r n a n d o C a b a f i a s , M D * , A d e l i n a Pellicer, M D * , E v a V a l v e r d e , M D * , C a r m e n M o r a l e s , M D t, a n d Jos~ Q u e r o , M D *

Central nervous system involvement in neonatal lupus erythematosus (NLE) has not been previously reported. We report four patients with NLE, all with complete congenital heart block and three with cerebral ultrasound and color Doppler flow imaging (CDFI) studies demonstrating evidence of associated vasculopathy in the gangliothalamic vasculature. CDFI confirmed blood flow through the affected vessels, indicating that blood flow was not compromised at this early stage. Short-term follow-up revealed no signs of progression of the vasculopathy, focal ischemia, gangliothalamic atrophy, or neurological impairment. Nevertheless, the implications of this finding with respect to the natural history of NLE remain to be defined, particularly in cases in which the disease develops into systemic lupus erythematosus later in life. Besides specific diagnostic studies for NLE, cerebral ultrasound, and CDFI studies are mandatory in all cases of complete congenital heart block, regardless of whether mothers are diagnosed as having connectivetissue disease or not. Neonates with signs of vasculopathy in the gangliothalamic region should be examined for NLE. Cabafias F, Pellicer A, Valverde E, Morales C, Quero J. Central nervous system vasculopathy in neonatal lupus erythematosus. Pediatr Neurol 1996; 15:124-126.

Introduction Neonatal lupus erythematosus (NLE) is a syndrome characterized by (1) complete congenital heart block (CHB), and (2) usually nonscarring, photodistributed papulosquamous eruption, or both. Nevertheless, multiple organ involvement is not common [1]. The syndrome is related to placental transmission of maternal autoantibodies [2,3]. Almost all newborns with "idiopathic" com-

From the *Division of Neonatology; Department of Pediatrics; and tDepartment of Pathology; La Paz Hospital; Aut6noma University of Madrid; Madrid, Spain.

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plete CHB and most newborns with a rash of NLE have antibodies against both SS-A/Ro and SS-B/La polypeptides [4]. The idea that these antibodies play a role in the production of NLE is supported by the observation that the duration of cutaneous disease in the infant parallels the duration of the maternally derived anti SS-A/Ro antibodies in the infant's serum [1]. Central nervous system (CNS) involvement in NLE has not been previously reported. We report four patients with NLE, three of them showing sonographic changes indicating vasculopathy in gangliothalamic vessels, as described previously [5]. This finding is relevant to the natural history of NLE and the patient's future neurological performance. The possible origin of this vascular lesion is discussed.

Patients Four patients had clinical and serological criteria of NLE (Table 1). Complete CHB was present in all four, all of whom underwent pacemaker implantation. In one patient (case 1), cutaneous disease and hematologic involvement coexisted with CHB. No mother had been diagnosed with systemic lupus erythematosus (SLE) or other connectivetissue disease before the current gestation, and all were asymptomatic and without treatment at the time. Therefore, connective-tissue disease was diagnosed on the basis of the fetal diagnosis of complete CHB. The serologic profiles of the mother-infant pairs are summarized in Table l. As part of the initial clinical evaluation, all infants underwent cerebral ultrasound scans (CUS) soon after birth. Hyperechogenic areas in the thalamic and/or basal ganglia vessels (HTBG) (Fig 1) as described previously in detail [5], were observed in three of the four patients with NLE (Table 1). These findings showed a linear and/or punctate shape and were visible in the first CUS in all cases. Color Doppler flow imaging (CDFI) revealed that these HTBG were located along the gangliothalamic vessels. CDFI confirmed blood flow through these vessels, and a blood flow velocity wave was recorded. The follow-up of these patients consisted of serial CUS and CDFI studies during hospitalization and after discharge (Table 1). In no case did the vasculopathy progress during follow-up. The neurological assessment at the time of sonographic evaluations, at birth, and during followup, was normal.

Communications should be addressed to: Dr. Cabafias; Division of Neonatology; La Paz Children's Hospital; Paseo de la Castellana 261; 28046 Madrid, Spain. Received December 27, 1995; accepted May 17, 1996.

© 1996 by Elsevier Science Inc. All rights reserved. PII S0887-8994(96)00159-2 • 0887-8994/96/$15.00

Table l.

Clinical data and CUS findings

Patient

Gestation (wk)/BW (gm)/Sex

1

35/2,284/F

ANA, SS-A/Ro, SS-B/La

36/2,550/M

CUS Findings

4

Clinical Findings

Antibodies Mother Newborn

Location of HTBG

Age at first CUS

Evolution

(days)

(mo)

CHB

Cutaneous disease

Thrombocytopenia

ANA, SS-AfRo, SS-B/La

Present

Present

Present

Th and BG bilateral

3

ANA, SS-A/Ro, SS-B/La

ANA, SS-A/Ro, SS-B/La

Present

Absent

Absent

Th and BG bilateral

3

36/2,016/M

ANA, SS-A/Ro

ANA, SS-A/Ro

Present

Absent

Absent

BG unilateral