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Year : 2020  |  Volume : 15  |  Issue : 1  |  Page : 54-56

A case of deep cerebral venous thrombosis presenting like acute necrotizing encephalopathy

Department of Paediatrics, Pt. Bhagwat Dayal Sharma Post Graduate Institute of Medical Sciences, Rohtak, Haryana, India

Date of Submission18-Sep-2019
Date of Acceptance30-Oct-2019
Date of Web Publication19-Mar-2020

Correspondence Address:
Dr. Sachin Dangi
2776/1 Lal Bahadur Shastri Nagar, Rohtak, Haryana.
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jpn.JPN_117_19

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Acute necrotizing encephalopathy (ANEC) is a rapidly progressive neurologic disorder that occurs in children after common viral infections such as influenza A and herpes simplex virus. It is observed more commonly in Asian countries. Magnetic resonance imaging findings in ANEC include symmetrical brain lesions, preferentially affecting the thalamus bilaterally. However, similar neuroimaging findings are also observed in deep cerebral vein thrombosis, which can lead to misdiagnosis. We report a case of 2½-year-old child who presented like acute necrotizing encephalopathy but on further investigations was found to be having deep cerebral vein thrombosis.

Keywords: Acute necrotizing encephalopathy, deep cerebral vein thrombosis, magnetic resonance imaging, thalamus

How to cite this article:
Dangi S, Gwasikoti N. A case of deep cerebral venous thrombosis presenting like acute necrotizing encephalopathy. J Pediatr Neurosci 2020;15:54-6

How to cite this URL:
Dangi S, Gwasikoti N. A case of deep cerebral venous thrombosis presenting like acute necrotizing encephalopathy. J Pediatr Neurosci [serial online] 2020 [cited 2020 Oct 24];15:54-6. Available from: https://www.pediatricneurosciences.com/text.asp?2020/15/1/54/280998

   Introduction Top

Acute necrotizing encephalopathy (ANEC) is a rare, severe encephalopathy observed more commonly in Asian countries.[1] It is thought to be triggered by a viral infection (influenza and human herpes virus-6 (HHV-6)) in a genetically susceptible host.[2] Magnetic resonance imaging (MRI) findings are characterized by symmetrical brain lesions, preferentially affecting the thalamus bilaterally, which help to make a prompt diagnosis of acute necrotizing encephalopathy.[3] However, neuroimaging findings in such cases should be interpreted very cautiously because venous sinus thrombosis of deep cerebral veins can produce similar neuroimaging findings characterized by infarction and vasogenic edema of bilateral thalami.

   Case Presentation Top

We report a case of 2½-year-old girl who presented with complaints of irritability, which lasted for 2 days followed by altered sensorium for 1 day. Two weeks before this presentation, the patient was admitted for bronchopneumonia and was discharged after 7 days of intravenous (IV) antibiotics. Seven days after being discharged, she developed excessive irritability with decreased intake of oral feeds that lasted for 2 days, followed by altered sensorium. She was developmentally normal with no history of any other major illness in past. On admission, the patient’s Glascgow coma scale was E2V2M5 with blood pressure 116/68mm Hg, heart rate 120/min, and respiratory rate 26/min. Her cranial nerves were intact. She had no weakness, ataxia, sensory disturbance, or meningeal signs. Motor examination revealed increased tone of both upper limb and lower limb, power <3/5 with brisk deep tendon reflexes, and extensor plantar response. She developed seizures on admission, and was started on IV phenytoin, valproate, and levetiracetam.

MRI of brain revealed symmetrical large areas of restricted diffusion appearing hyperintense on T2-weighted (T2W)/fluid attenuation inversion recovery image and hypointense on T1-weighted (T1W) images involving bilateral thalami, suggestive of acute necrotizing encephalitis [Figure 1].
Figure 1: MRI of brain showing large areas of restricted diffusion appearing in T1W hypointense (A) and T2W hyperintense (B) images involving bilateral thalami, neuroimaging findings were consistent with acute necrotizing encephalitis. Patchy areas of blooming (C) on susceptibility weighted images are seen in the region of bilateral thalami

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On the basis of clinical presentation and neuroimaging findings, a tentative diagnosis of acute necrotizing encephalitis was made. The child was started on IV methylprednisolone pulse therapy (30mg/kg OD). However, she did not respond to the treatment, magnetic resonance venography was planned, which revealed deep cerebral venous thrombosis involving right transverse and sigmoid sinus, straight sinus, bilateral internal cerebral veins, and vein of Galen [Figure 2].
Figure 2: Magnetic resonance venography of brain showing absent flow-related enhancement in the right transverse and sigmoid sinuses, straight sinus, bilateral cerebral veins (A, B, C, and D, respectively)

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As the diagnosis of deep cerebral venous sinus thrombosis was made, methylprednisolone was stopped and low-molecular-weight heparin was started. The patient’s prothrombotic workup was sent. Her condition improved gradually, and oral feed was started. She was discharged after 1 month with a plan of repeat magnetic resonance venogram after 3 months on follow-up.

   Discussion Top

The real etiology and the pathogenesis of ANEC remain unclear. Usually, it develops secondary to viral infections, including influenza A, herpes simplex virus, HHV-6, and enterovirus.[4] Pathologically, there is edema, hemorrhage, and necrosis in thalamic region.

In most of the patients of ANEC, bilateral symmetrical thalamic involvement is observed. MRI of brain shows multiple areas of restricted diffusion involving bilateral thalami. Lesions are hypointense on T1W and hyperintense on T2W images. These findings can be extensive. Multiple areas of hemorrhagic spots, cavitations, and post-contrast enhancement are also observed.[3] Deep cerebral venous thrombosis also causes vasogenic edema, hemorrhage, and necrosis in the bilateral thalami, which results in similar neuroimaging findings.

   Conclusion Top

This case report highlights that in the presence of bilateral thalami lesions, deep cerebral venous thrombosis must be considered in addition to ANEC. Delay in the diagnosis of cerebral venous thrombosis and commencement of anticoagulant therapy can lead to unfavorable outcomes.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Mizuguchi M, Abe J, Mikkaichi K, Noma S, Yoshida K, Yamanaka T, et al. Acute necrotising encephalopathy of childhood: a new syndrome presenting with multifocal, symmetric brain lesions. J Neurol Neurosurg Psychiatry 1995;58:555-61.  Back to cited text no. 1
Sazgar M, Robinson JL, Chan AK, Sinclair DB. Influenza B acute necrotizing encephalopathy: a case report and literature review. Pediatr Neurol 2003;28:396-9.  Back to cited text no. 2
Kim JH, Kim IO, Lim MK, Park MS, Choi CG, Kim HW, et al. Acute necrotizing encephalopathy in Korean infants and children: imaging findings and diverse clinical outcome. Korean J Radiol 2004;5:171-7.  Back to cited text no. 3
Ito Y, Ichiyama T, Kimura H, Shibata M, Ishiwada N, Kuroki H, et al. Detection of influenza virus RNA by reverse transcription-PCR and proinflammatory cytokines in influenza-virus-associated encephalopathy. J Med Virol 1999;58: 420-5.  Back to cited text no. 4


  [Figure 1], [Figure 2]


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