Neurology India
menu-bar5 Open access journal indexed with Index Medicus
  Users online: 1273  
 Home | Login 
About Editorial board Articlesmenu-bullet NSI Publicationsmenu-bullet Search Instructions Online Submission Subscribe Videos Etcetera Contact
  Navigate Here 
  » Next article
  » Previous article 
  » Table of Contents
 Resource Links
  »  Similar in PUBMED
 »  Search Pubmed for
 »  Search in Google Scholar for
 »Related articles
  »  Article in PDF (113 KB)
  »  Citation Manager
  »  Access Statistics
  »  Reader Comments
  »  Email Alert *
  »  Add to My List *
* Registration required (free)  

  In this Article
 »  Abstract
 »  Introduction
 »  Case report
 »  Discussion
 »  References

 Article Access Statistics
    PDF Downloaded122    
    Comments [Add]    

Recommend this journal

Year : 2001  |  Volume : 49  |  Issue : 3  |  Page : 308-10

Dural arteriovenous malformation : a rare cause of epilepsy in childhood.

Department of Paediatrics, Faculty of Medicine, Yuzuncu Yil University, Van, Turkey.

Correspondence Address:
Department of Paediatrics, Faculty of Medicine, Yuzuncu Yil University, Van, Turkey.
[email protected]

  »  Abstract

A 3 year and 6 month old girl with epilepsy associated with dural arteriovenous malformation (DAVM), diagnosed on the MRI, is presented to emphasise the importance of DAVM in the aetiology of childhood epilepsy.

How to cite this article:
Caksen H, Unal O, Tombul T, Cesur Y, Abuhandan M. Dural arteriovenous malformation : a rare cause of epilepsy in childhood. Neurol India 2001;49:308

How to cite this URL:
Caksen H, Unal O, Tombul T, Cesur Y, Abuhandan M. Dural arteriovenous malformation : a rare cause of epilepsy in childhood. Neurol India [serial online] 2001 [cited 2021 Oct 22];49:308. Available from:

   »   Introduction Top

Arteriovenous malformation (AVM) develops as a consequence of the failure of normal capillary bed development between arteries and veins during embriogenesis.[1] They are typically located in the cerebral hemispheres, but may be situated in the cerebellum, brain stem, or spinal cord. Dural arteriovenous malformations (DAVMs) or AV fistulae are vascular malformations that have different angioarchitecture, clinical presentation, natural history, imaging features, and therapy, as compared to parenchymal AVMs of brain.[2] DAVMs comprise about 10% to 15% of all intracranial vascular malformations. In this article, a case of epilepsy associated with DAVM is presented to emphasize its importance in the aetiology of childhood epilepsy.

   »   Case report Top

A 3 year and 6 month old girl was admitted with history of right partial seizures becoming generalised. The first convulsion occurred at the age of 11 months and she had eight convulsive attacks till admission. She was managed as a case of idiopathic epilepsy in another hospital and treated with phenobarbital for two years and carbamazepine for 7 months. However, her seizures continued. Physical examination revealed weight of 15 Kg (50th percentile) and height of 93 cm (10th percentile). General physical and neurological examination was normal.
Investigations disclosed haemoglobin of 12.1 g/dl and leukocyte count of 5,400/mm3. The urine analysis, renal and liver function tests were normal. Electroencephalogram showed paroxismally focal spike activity and disorganisation on the left cerebral hemisphere during awaking and asleep [Figure - 1]. Magnetic resonance imaging (MRI) showed superficial vascular enlargement in the left frontal dural area as regions of signal void on T1WI and T2WI [Figure - 2]. Cortical sulcal dilatation was noted in the frontotemporal lobes. In addition, vascular enlargement and signal void were seen in the quadrigeminal cistern. MR angiography demonstrated malformation in the quadrigeminal cistern and deep cortical venous drainage in the left frontobasal region [Figure - 3]. DAVM in the frontal region and quadrige-minal cistern and atrophy in frontotemporal area were diagnosed. Her seizures markedly decreased with an increase of carbamazepine to 30 mg/Kg/day. She was referred to the department of neurosurgery.

   »   Discussion Top

AVMs may be located in the different area of brain.[1],[2] In a series of 24 children, AVM was located in noncritical cortical areas in 7 cases, in critical cortical areas in 6 cases, in the midline structures or in the basal ganglia in 8 cases, in the posterior fossa in 2 cases, and was dural in only 1 case.[3] In our case, the AVM was located in the dural area which is a rare localisation.
Both adults and children with DAVM can present with a wide clinical spectrum of signs and symptoms, depending on the location, size and venous drainage of the arteriovenous shunt.[4] Children are sometimes born with congenital DAVM; these congenital fistulas tend to have larger connections and increased flow compared to their adult (acquired) counterparts. The cause of congenital dural malformations remains unknown. Meyers et al reported that paediatric dural malformations often present in the neonatal period. They can present much like a vein of Galen malformation, with macrocephaly, heart murmur, cardiac failure, distended scalp veins, increased intracranial pressure, and haemorrhage.[6] We do believe that the DAVM is a congenital lesion because our patient's first symptom of convulsion occurred at the age of 11 month. However, our patient did not have macrocephaly, heart murmur, cardiac failure or increased intracranial pressure during the new born period or later because her DAVM was very small.
MRI is extremely useful in the evaluation of the patients with DAVM, particularly those with the potential for an aggressive course, because of pial venous drainage [Table I]. Since the clinical presentation of these patients is often nonspecific and vague, the often subtle clues to the diagnosis on MRI can play an extremely important role in the management of these patients.[7] Minford and Forsythe[8] reviewed the computed tomography (CT) findings in 82 children with partial seizures of unknown aetiology. CT was normal in 64 children (78%) and abnormal in 18 children (22%). Four children had potentially correctable lesions (two tumours and two arteriovenous malformations). Therefore, they suggested that CT was indicated in every child with partial seizures.[8] Our patient was treated as idiopathic epilepsy in another hospital, but CT or MRI was not planned. Therefore, we would also like to stress that a contrast CT scan or MRI should be planned in children with partial seizures.
Treatments of DAVM include transarterial embolisation, transvenous embolisation and surgical exicision. In the large congenital dural malformations, the goal is to reduce shunting adequately to treat the heart failure. Intravascular therapy is the procedure of choice to reduce the flow, with subsequent combined intravascular/surgical therapy, when the child is older and more stable.[5],[9] In our patient, we used carbamazepine in order to control the seizure, and then she was referred to the department of neurosurgery. She could not be followed later.


  »   References Top

1.Haslam RHA : The nervous system. In : Textbook of pediatrics (16th ed); Behrman RE, Kliegman RM, Jenson HB, (eds). WB Saunders Co. Philadelphia, 2000; 1793-1866.   Back to cited text no. 1    
2.Vinuela F, Fox AJ, Pelz DM et al :Unusual clinical manifestations of dural arteriovenous malformations. J Neurosurg 1986; 64 : 554-558.   Back to cited text no. 2    
3.Mazza C, Pasqualin A, Scienza R et al : Intracranial arteriovenous malformations in the pediatric age: experience with 24 cases. Childs Brain 1983; 10 : 369-380.   Back to cited text no. 3    
4.Lasjaunias P, Chui M, Ter Brugge K et al : Neurological manifestations of intracranial dural arteriovenous malformations. J Neurosurg 1986; 64 : 724-730.   Back to cited text no. 4    
5.Meyers FM, Halbach VV, Barkovich AJ : Anomalies of cerebral vasculaure : Diagnostic and endovascular considerations. In : Pediatric Neuroimaging (3rd ed); Barkovich AJ. Lippincott Willams and Wilkins. Philadelphia. 2000; 771-814.   Back to cited text no. 5    
6.Albright AL, Latchaw RE, Price RA : Posterior dural arteriovenous malformations in infancy. Neurosurgery 1983; 13 : 129-135.   Back to cited text no. 6    
7.Chaudhary , Sachdev VP, Cho SH et al : Dural arteriovenous malformations of the major venous sinuses: an acquired lesion. AJNR1982; 3 : 13-19.   Back to cited text no. 7    
8.Minford AM, Forsythe WI : Computed tomography findings in partial seizures. Arch Dis Child 1992; 67 : 693-696.   Back to cited text no. 8    
9.Barnwell SL, Halbach VV, Higashida RT et al : Complex dural arteriovenous fistulas results of a new combined neurosurgical and intraventional neuroradiology treatment in 16 patients. J Neurosurg 1989; 71 : 352-358.   Back to cited text no. 9    


Print this article  Email this article
Previous article Next article
Online since 20th March '04
Published by Wolters Kluwer - Medknow