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LETTERS TO EDITOR
Year : 2018  |  Volume : 66  |  Issue : 3  |  Page : 836-838

Magnetic resonance imaging and magnetic resonance spectroscopy in varicella zoster necrotizing encephalitis


1 Department of Radiology, Sri Ramachandra University, Chennai, Tamil Nadu, India
2 Department of Paediatrics, Sri Ramachandra University, Chennai, Tamil Nadu, India

Date of Web Publication15-May-2018

Correspondence Address:
Dr. Remya Baburaj
Department of Radiology, Sri Ramachandra University, Porur, Chennai - 600 116, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.232343

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How to cite this article:
Baburaj R, Rangasami R, Rajakumar P S. Magnetic resonance imaging and magnetic resonance spectroscopy in varicella zoster necrotizing encephalitis. Neurol India 2018;66:836-8

How to cite this URL:
Baburaj R, Rangasami R, Rajakumar P S. Magnetic resonance imaging and magnetic resonance spectroscopy in varicella zoster necrotizing encephalitis. Neurol India [serial online] 2018 [cited 2018 Nov 18];66:836-8. Available from: http://www.neurologyindia.com/text.asp?2018/66/3/836/232343




Sir,

Acute necrotizing encephalopathy of childhood (ANEC) is a rare and distinct form of encephalopathy associated with respiratory infections, progressive deterioration of consciousness, and seizures. The disease was exclusively noted in east Asian countries with the first case being described in Japan by Mizuguchi et al., in 1995.[1] The common causative organisms are Influenza A virus, herpes simplex virus, and mycoplasma. ANEC caused by varicella zoster virus is extremely rare. We report the magnetic resonance imaging (MRI) and magnetic resonance spectroscopy (MRS) findings in a case of ANEC caused by varicella zoster virus.

A 21-month old child was admitted to the Pediatric Intensive Care Unit (ICU) with presenting symptoms of fever and multiple episodes of seizures for 2 days. There was no history of previous trauma, drug intake, or hospital admission. On examination, the child was febrile (temperature: 101°F), had tachycardia (131 beats per min), had a respiratory rate of 35/min, blood pressure of 80/40mmHg, and Glasgow Coma Scale of 8/15. Laboratory findings revealed a total leukocyte count of 9000/cu mm with 60% polymorphs and C-reactive protein level of 2.4 mg/dl. Fever panel was found to be inconclusive. On the day of admission, the patient was referred for magnetic resonance imaigng (MRI) of the brain. The study revealed multiple nonenhancing symmetrical T1 hypointense/T2 hyperintense lesions in bilateral thalami, cerebellar hemispheres, periventricular regions, corona radiata, and centrum semiovale [Figure 1]a, [Figure 1]b, [Figure 1]c and [Figure 1]e, [Figure 1]f, [Figure 1]g. Some of these lesions showed hemorrhage [Figure 1]d. These lesions showed restricted diffusion on diffusion-weighted images [Figure 1]h. Magnetic resonance spectroscopy with intermediate TE (144 ms) showed increased choline (cho) and decreased N-acetyl aspartate (NAA), with cho/NAA ratio of 1.1. Associated lipid lactate peak was present suggesting necrosis [Figure 2]. Based on the above features, a radiological diagnosis of acute necrotizing encephalitis was made. Lumbar puncture was done and cerebrospinal fluid analysis was positive for varicella zoster virus. The child was empirically administered a course of acyclovir and was treated symptomatically. The child was discharged after 1 month with residual spasticity and cognitive impairment with advice for physiotherapy and follow-up. The follow-up MRI done after 1 year showed gliosis in bilateral thalami, cerebellar hemispheres, periventricular regions, corona radiata, and centrum semiovale [Figure 3]a, [Figure 3]b, [Figure 3]c. Even though the child improved, there was residual spasticity and impaired cognition, at a 1 year follow-up visit.
Figure 1: (a-c): T2-weighted images show hyperintense lesions in the pons, bilateral cerebellar hemispheres, thalami, periventricular and centrum semiovale regions (white arrows).(d) Gradient axial image shows blooming within the thalamic lesions suggesting the presence of hemorrhages (arrow head). (e-g) Post contrast T1 images show no enhancement within these lesions (open arrow).(h) Diffusion-weighted image showing restricted diffusion in these lesions (black arrows)

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Figure 2: Magnetic resonance spectroscopy (TE: 144 ms) of the lesion shows elevated choline, decreased N-acetyl aspartate with Cho/N-acetyl aspartate ratio of 1.1. Associated lipid lactate peak is seen

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Figure 3: MR images obtained after 1 year. (a-c) T2-weighted images show gliotic lesions in the pons, bilateral cerebellar hemispheres, thalami, periventricular and centrum semiovale regions (white arrows)

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ANE, first proposed by Mizuguchi et al., in 1995, is a rare but distinctive type of acute encephalopathy with global distribution.[2] ANEC usually develops due to a viral infection caused by influenza A and influenza B, parainfluenza, varicella, and enterovirus. It usually presents as symmetrical lesions involving the thalami, basal ganglia, cerebellar and cerebral white matter, and tegmentum that are demonstrated by computed tomography (CT) or MRI.[1] It is characterized by a rapidly progressive disease with children developing convulsions, altered consciousness, and vomiting, a course that can be lethal. From a pathologic perspective, the lesions show edema, petechial hemorrhage, and necrosis.[3] The various infective organisms and the outcomes described so far are summarized in [Table 1].
Table 1: The various infective organisms causing ANEC and the outcomes so far described

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The differential diagnoses are acute disseminated encephalomyelitis (ADEM), acute hemorrhagic encephalitis, and hypoxic ischemic encephalopathy (HIE). Acute hemorrhagic encephalitis may be difficult to differentiate from ANE, though the former may show inflammatory cells on pathology and the latter may not show inflammatory cells. HIE can be excluded by clinical history of circulatory or hypoxic episode. ADEM is a postinfective benign disease that shows asymmetrical lesions without restricted diffusion on MRI.

The outcome of ANEC has been reported to be generally poor; approximately 65% of the affected patients died or were left with severe neurologic sequelae.[8] The affected patients are managed with supportive and symptomatic treatment that includes a combination of corticosteroids, mannitol, and anticonvulsants. Administration of acyclovir is controversial. MRI and MRS offer a noninvasive means of diagnosis of the disease and its complications. MRS plays an important role in the detection of lipid lactate peak indicative of necrosis.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
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 Psychiatry1995;58:555-61.  Back to cited text no. 1
[PUBMED]    
2.
Wu X, Wu W, Pan W, Wu L, Liu K, Zhang HL. Acute necrotizing encephalopathy: An underrecognized clinicoradiologic disorder. Mediators Inflamm 2015;2015:792578.  Back to cited text no. 2
[PUBMED]    
3.
Mizuguchi M. Acute necrotizing encephalopathy of childhood: A novel form of acute encephalopathy prevalent in Japan and Taiwan. Brain Dev 1997;19:81-92.  Back to cited text no. 3
[PUBMED]    
4.
Wang HS, Huang SC. Acute necrotizing encephalopathy of childhood. Chang Gung Med J 2001;24:1-10.  Back to cited text no. 4
[PUBMED]    
5.
Goo HW, Choi CG, Yoon CH, Ko TS. Acute necrotizing encephalopathy: Diffusion MR imaging and localized proton MR spectroscopic findings in two infants. Korean J Radiol 2003;4:61-5.  Back to cited text no. 5
[PUBMED]    
6.
Wong AM, Simon EM, Zimmerman RA, Wang HS, Toh CH, Ng SH. Acute necrotizing encephalopathy of childhood: Correlation of MR findings and clinical outcome. Am J Neuroradiol 2006;27:1919-23.  Back to cited text no. 6
[PUBMED]    
7.
Yadav S, Das CJ, Kumar V, Lodha R. Acute necrotizing encephalopathy. Indian J Pediatr 2010;77:307-9.  Back to cited text no. 7
[PUBMED]    
8.
Salehiomran MR, Nooreddini H, Baghdadi F. Acute necrotizing encephalopathy of childhood; A case report. Iran J Child Neurol 2013;7:51-4.  Back to cited text no. 8
[PUBMED]    


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