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ISSN: Print -2349-0977, Online - 2349-4387


 
 Table of Contents  
CASE IN POINT - NEURORADIOLOGY
Year : 2016  |  Volume : 2  |  Issue : 4  |  Page : 209-210

Recognizing ulegyria—the lesser known form of hypoxic ischemic encephalopathy


1 Department of Radiology, Safdarjung Hospital, New Delhi, India
2 Department of Neurology, Safdarjung Hospital, New Delhi, India

Date of Web Publication22-Sep-2016

Correspondence Address:
Manish Kumar
Department of Radiology, Safdarjung Hospital, New Delhi
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2349-0977.191041

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  Abstract 

Derived from the Latin term ule, which means scarring, ulegyria is a manifestation of hypoxic ischemic encephalopathy. Recognizable on the magnetic resonance imaging sequences because of its characteristic features, the diagnosis is critical from the standpoint of clinical management. Seizures caused by ulegyria are refractory to pharmacotherapy and often do well with surgery.

Keywords: Hypoxic ischemic encephalopathy, MRI, seizures, ulegyria


How to cite this article:
Kumar M, Gupta R, Kaul B, Agarwal Y. Recognizing ulegyria—the lesser known form of hypoxic ischemic encephalopathy. Astrocyte 2016;2:209-10

How to cite this URL:
Kumar M, Gupta R, Kaul B, Agarwal Y. Recognizing ulegyria—the lesser known form of hypoxic ischemic encephalopathy. Astrocyte [serial online] 2016 [cited 2019 May 20];2:209-10. Available from: http://www.astrocyte.in/text.asp?2016/2/4/209/191041


  Introduction Top


Hypoxic ischemic encephalopathy due to perinatal asphyxia causes neurologic damage leading to a number of chronic disabilities, the most common ones being cerebral palsy, mental retardation, and epilepsy. The gestational age of an infant at the time of insult is the single most important determinant of the final neuropathological outcome. Ulegyria (scarred gyri) is an under-recognized neuropathological correlate of perinatal hypoxic ischemic encephalopathy, which typically affects full term infants. Its identification is important because it has characteristic magnetic resonance imaging (MRI) features, is often associated with pharmacoresistant seizures, and surgical outcomes are often favorable.

A 20-year-old male presented with a history of episodic involuntary body movements and seizures. Age of onset of seizures was 15 years and the patient had been on antiepileptics since then. There was no family history of epilepsy. History of perinatal distress was present in the form of prolonged obstructed labor and forceps delivery.

MRI brain was done which revealed bilaterally symmetrical shrunken and “mushroom-shaped” gyri in the parietal lobes with subcortical white matter hyperintensities and atrophy [Figure 1] and [Figure 2]. The abnormalities were typically located around the posterior arterial border zones.
Figure 1: Axial T1W image of the brain showing bilaterally symmetrical shrunken and “mushroom-shaped” gyri in the parietal lobes.

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Figure 2: Axial fluid-attenuated inversion recovery image at the same level showing white matter hyperintensities with atrophy underlying the symmetrical mushroom-shaped gyri. The lesions are located at the watershed zone between the middle and posterior cerebral arteries.

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  Discussion Top


Ulegyria refers to a specific pattern of hypoxemic-ischemic injury that affects full term infants.[1],[2] The characteristic gyral pattern consists of small convolutions with sparing at apex, and scarring and atrophy at the bottom of sulci with underlying white matter abnormalities. The lesions are usually bilateral, sometimes predominantly unilateral; the diagnosis is based on the following MRI criteria:[3]

  • Poorly demarcated lesion;
  • Atrophy and thinning of the cortex mainly involving the deep portion of the convolution and sparing the apex, resulting in mushroom shaped gyri; and
  • White matter signal abnormalities on T2-weighted and fluid-attenuated inversion recovery (FLAIR) sequences.


In a term infant, the crown of gyri are better perfused than the tissues at the bottom of sulci, and therefore, hypoxic injury causes atrophy and gliosis at depth of suci with spared apex, resulting in typical “mushroom shaped gyri.” The ulegyric lesions have been described to typically affect the occipital lobe [4] or the parasagittal watershed area.[5] This is because the insult occurs either in the region supplied by the posterior cerebral artery area or the arterial border zone between the middle cerebral and posterior cerebral arteries.

Ulegyria must be included as one of the etiologies of posterior cortex epilepsy. Its association with refractory seizures is well documented. Gill-Nagel et al.[4] reported eight cases of ulegyria presenting with posterior cortex epilepsy in a study cohort of 1020 epileptic patients.

Usui et al.[3] studied the amenability of ulegyric lesions to surgical resection and symptomatic seizure control. Out of 10 patients with posterior cortex epilepsy and ulegyria studied by them, 4 had unilateral and 6 had bilateral but unilateral predominant lesions. They reported significant seizure control following resection of posterior cortex including the MRI lesion. Bilaterality of lesions was not considered to be a contraindication to surgery.

Differential Diagnosis

Bilateral perislyvian polymicrogyria often presents with a clinical picture similar to bilateral perislyvian ulegyria.[6] However, the two can be differentiated on the basis of MRI findings. This is important to recognize because surgery is recommended for ulegyria but not for polymicrogyria.[6]

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflflicts of interest.

 
  References Top

1.
Barkovich AJ, Truwit CL. Brain damage from perinatal asphyxia: Correlation of MR findings with gestational age. AJNR Am J Neuroradiol 1990;11:1087-96.  Back to cited text no. 1
    
2.
Nikas I, Dermentzoglou V, Theofanopoulou M, Theodoropoulos V. Parasagittal lesions and ulegyria in hypoxic-ischemic encephalopathy: Neuroimaging findings and review of the pathogenesis. J Child Neurol 2008;23:51-8.  Back to cited text no. 2
    
3.
Usui T, Mihara T, Baba K, Matsuda K, Tottori T, Umeoka S et al. Posterior cortex epilepsy secondary to ulegyria: Is it a surgically remediable syndrome? Epilepsia 2008;49:1998-2007.  Back to cited text no. 3
    
4.
Gil-Nagel A, García Morales I, Jiménez Huete A, Alvarez Linera J, del Barrio A, Ruiz Ocaña C, et al. Occipital lobe epilepsy secondary to ulegyria. J Neurol 2005;252:1178-85.  Back to cited text no. 4
    
5.
Villani F, D'Incerti L, Granata T, Battaglia G, Vitali P, Chiapparini L, et al. Epileptic and imaging findings in perinatal hypoxic-ischemic encephalopathy with ulegyria. Epilepsy Res 2003;55:235-43.  Back to cited text no. 5
    
6.
Schilling LP, Kieling RR, Pascoal TA, Kim HI, Lee MC, Kim YH, et al. Bilateral perisylvian ulegyria: An under-recognized, surgically remediable epileptic syndrome. Epilepsia 2013;54:1360-7.  Back to cited text no. 6
    


    Figures

  [Figure 1], [Figure 2]


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