Basal ganglia infarcts with calcifications in children

EUROPEAN JOURNAL OF RADIOLOGY

:g ELSEVIER

European Journal of Radiology 20 (1995) 48-51

Basal ganglia infarcts with calcifications in children T a m e r K a y a *a, R a g i p O z k a n , N u r g / i l B o z o ~ l u a, B a k i A d a p i n a r a, A y t e n Y a k u t b ""

a

aDepartment of Radiology, Faculty of Medicine, Osmangazi University. Eski~ehir, Turkey bDepartment of Pediatric Neurology, Faculty of Medicine, Osmangazi University, Eski~ehir, Turkey Received 29 November 1994; revision received 14 January 1995; accepted 20 January 1995

Keywords: Basal ganglia, infarcts; Basal ganglia, calcifications; Brain, basal ganglia

1. Introduction

The incidence of ischemic strokes in children is given as six cases in 1 000 000 children per year when heart disease, trauma and perinatal complications have been excluded [1]. Basal ganglia infarcts (BGI) are extremely rare in ischemic stroke [2]. On the other hand the incidence of diffuse calcifications of basal ganglia (CBG) is reported as 43/18 000 [3]. The present report describes four children with BGI and CBG. The association of these two unusual abnormalities is discussed. 2. Methods

Four children (age 9 months to 3.5 years) with acute onset of hemiplegia were found to have BGI and CBG on computed tomography (CT). Two underwent magne-

* Corresponding author, Osmangazi Universitesi Tip Fakfiltesi, Radyoloji Anabilim Dali, 26480 Eski~ehir, Turkey. Tel.: +90 222 2390087, 2392979/4017; Fax: +90 222 2391477.

tic resonance imaging (MRI). All CT examinations were performed using a third generation CT scanner (Toshiba TCT 600) with contiguous 10-mm thick slices. MRI was performed on a 0.5 Tesla MR unit (Philips Gyroscan) using spin echo pulse sequences. Tl-weighted, T2weighted and proton density weighted images were obtained in axial, coronal and sagittal planes. All slices were contiguous and 10 mm thick. 3. Case reports

The clinical and neuroradiologic findings are summarised in Table 1. All patients had BGI (Figs. la, 2a, 3a, 4a) and slightly asymmetric punctate CBG (Figs. lc, 2c, 3b, 4b) demonstrated on CT. MRI confirmed CT findings in patient 1 (Fig. 1b). In patient 2, MRI showed BGI more definitively (Fig. 2b). Birth history, laboratory studies (hemogram, erythrocyte sedimentation rate, serum electrolytes, blood urea nitrogen, creatinine, glucose) and echocardiographic examinations were normal in all patients. In patient 1, follow-up neurologic examination was normal, except minimal paresis after 1 year.

Table 1 Clinical and neuroradiologic findings in patients with basal ganglia calcification Patient No.

Age

Sex

Neurological examination

Course of illness

1 2 3 4

9 months 3.5 years 9.5 months 9 months

M M F M

Left hemiplegia Right hemiplegia Right hemiplegia Right hemiplegia

Gradually Gradually Gradually Gradually

improved improved improved improved

NL, nucleus lentiformis; CR, corona radiata. 0720-048X/95/$09.50 © 1995 Elsevier Science Ireland Ltd. All rights reserved SSDI 0720-048X(95)00607-R

Localisation of BGI over over over over

3 weeks 2 weeks 3 weeks 2 weeks

Right NL + CR Left NL + CR Left NL + CR Left NL

T. Kaya et al./ European Journal of Radiology 20 (1995) 48-51

a

b

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c

Fig. I. Patient 1. (a) Unenhanced CT shows a well-defined infarct in the right nucleus lentiformis and corona radiata. (b) T2-weighted axial MRI image shows hyperintense lesion interpreted as infarct. (c) Bilateral slightly asymmetric punctate CBG.

Patients 2, 3 and 4 had completely normal neurologic findings at the follow-up visits. 4. Discussion

Ischemic strokes can be seen secondary to a number of conditions such as: congenital and acquired heart disease, local and systemic infections, head and neck trauma, arteritis (Moya-Moya, Takayashu, collagen diseases), iatrogenic (angiography, radiotherapy, umbilical vein catheters), hematologic disease (sickle cell disease, thrombocytosis, polycythemia, intravascular

a

b

coagulopathy), metabolic disorders (homocystinuria, diabetes, hyperlipidemia, dehydration, Fabry's disease), drug abuse (LSD, amphetamines, cannabis, contaminants), abnormal vessels, aminoacidopathies, neurofibromatosis and idiopathic cases [4-6]. Etiological and predisposing factors of ischemic stroke are dramatically different in the pediatric and the adult populations. In pediatric patients, arterial ischemic strokes occur mostly in three main etiologic contexts: (1) congenital heart disease; (2) neonatal distress; (3) infections, focal or general inducing vasculitis; but many cases are considered idiopathic [4].

c

Fig. 2. Patient 2. (a) Unenhanced CT demonstrates hypodense area in the left basal ganglia. (b) Tl-weighted sagittal image demonstrates three small hypointense lesions, interpreted as infarct in the left nucleus lentiformis and corona radiata. (c) Bilateral slightly asymmetric punctate CBG.

T. Kaya et al./ European Journal of Radiology 20 (1995) 48-51

50

a

b

Fig. 3. Patient 3. (a) Unenhanced CT revealing a low-density lesion involving the left corona radiata and nucleus lentiformis. (b) Bilateral slightly asymmetric punctate CBG.

BGI are caused by occlusion of small penetrating blood vessels in the basal ganglia [7]. BGI in adults has a strong association with hypertension and atherosclerosis; but in children these lesions more often result from arteritis or embolism [4]. Dusser et al. demonstrated

a

that many of their cases with BGI were idiopathic [2]. Inagaki et al. reported in their series that nine cases had delayed hemiplegia after varicella infection and five cases were of idiopathic nature [8]. Histological studies of CBG have demonstrated that

b

Fig. 4. Patient 4. (a) Unenhanced CT demonstrates a hypodense area in the left basal ganglia. (b) Bilateral slightly asymmetric punctate CBG.

T. Kaya et al./ European Journal of Radiology 20 (1995) 48-51

the calcium is d e p o s i t e d in the m e d i a o f m e d i u m - s i z e d b l o o d vessels w i t h o u t c o m p r o m i s i n g the l u m i n a [3]. It has been r e p o r t e d that C B G is a recognised histological feature following ischemic episodes [3]. T h e other causes o f C B G include: birth asphyxia, i d i o p a t h i c hypercalcemia, diabetes, d i h y d r o p t e r i d i n e reductase deficiency, d e x a m e t h a s o n e therapy, c a r b o n m o n o x i d e , bismuth and lead intoxication [3], t r e a t a b l e p a r a t h y r o i d disease [9], m e t h o t r e x a t e t h e r a p y [10,11], a n g i o m a t o u s m a l f o r m a t i o n s with a n e u r y s m o f the vein o f G a l e n [12], congenital rubella, toxoplasmosis, cytomegalic inclusion disease, measles a n d c h i c k e n p o x [13], C o c k a y n e syndrome, Leigh's disease, m i t o c h o n d r i a l c y t o p a t h y [14,15], lipoid proteinosis, F a h r ' s disease [16], D o w n ' s s y n d r o m e [17] a n d A I D S [18]. Brain calcification due to hypoxic-ischemic injury is a well-known p h e n o m e n o n . A n s a r i et al. f o u n d that 99 cases o u t o f 486 consecutive autopsies h a d histologic evidence o f cerebral ischemic lesions. Their 39 o f 99 cases h a d different degrees o f calcification a n d four cases h a d p r o m i n e n t calcifications that were large enough to be detected b y r o u t i n e r a d i o l o g i c m e t h o d s [191. K a n a r e k a n d G i g e r o n [20] a n d B a b c o c k a n d Bell [21] reported cerebral calcification in p o s t a s p h y x i a l e n c e p h a l o p a t h y which was d e m o n s t r a t e d on C T a n d ult r a s o n o g r a p h y . M a c p h e r s o n et al. r e p o r t e d on two patients with basal ganglia calcifications following ischemic necrosis o f b r a i n tissue [22]. G r a n t et al. r e p o r t e d calcification in two patients with ischemia d e m o n s t r a t e d by C T [23]. K e n d a l l a n d C a v a n a g h analysed the C T images o f 18 000 children a n d f o u n d two cases o f diffuse basal ganglion calcification with b r a i n infarcts, d i a g n o s e d as a c a r d i o v a s c u l a r event a n d a C o k s a c k i e B infection [3]. This association was n o t compatible with o u r cases on the basis o f radiological a n d clinical findings. In the review o f literature, b o t h B G I a n d C B G can be seen in vascular m a l f o r m a t i o n s , infections, m e t a b o l i c disorders and perinatal complications. In o u r cases, there is no clinical o r l a b o r a t o r y m a n i f e s t a t i o n s o f these factors. W e conclude that the bilateral a n d the slight asymmetric p u n c t a t e basal ganglionic calcifications, d e m o n s t r a t e d in o u r patients, m a y be old a s y m p t o m a t i c calcified microinfarcts. But the n a t u r e o f the association between B G I a n d C B G is n o t well k n o w n a n d needs to be further investigated.

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