Indian Journal of Nuclear Medicine
Home | About IJNM | Search | Current Issue | Past Issues | Instructions | Ahead of Print | Online submissionLogin 
Indian Journal of Nuclear Medicine
  Editorial Board | Subscribe | Advertise | Contact
Users Online: 699 Print this page  Email this page Small font size Default font size Increase font size


 
 Table of Contents     
INTERESTING IMAGE
Year : 2020  |  Volume : 35  |  Issue : 3  |  Page : 244-247  

18F-fluorodeoxyglucose positron emission tomography-computed tomography in initial diagnosis and treatment response evaluation of new onset refractory status epilepticus


1 Department of Nuclear Medicine and PET/CT, Mahajan Imaging Centre, Sir Ganga Ram Hospital, New Delhi, India
2 Department of Neurology, Sir Ganga Ram Hospital, New Delhi, India

Date of Submission20-Feb-2020
Date of Acceptance11-Mar-2020
Date of Web Publication01-Jul-2020

Correspondence Address:
Dr. Nikhil Seniaray
Department of Nuclear Medicine and PET/CT, Mahajan Imaging Centre, Sir Ganga Ram Hospital, Old Rajinder Nagar, New Delhi - 110 060
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijnm.IJNM_34_20

Rights and Permissions
   Abstract 


New onset refractory status epilepticus (NORSE), is a rare, neurological condition characterised by prolonged periods of refractory epileptic seizure with no readily identifiable cause in otherwise healthy individuals. Anatomical imaging like MRI and serology is usually unremarkable. In patients who have underlying etiology as auto-immune encephalitis without any evidence of auto-antibodies FDG PET may help in early diagnosis and treatment response as it tends to accumulate in the neuronal tissue whenever there is increased blood flow, metabolic demand or increased electrical activity which reverts back with clinical recovery.

Keywords: 18F-FDG PET, NORSE,MRI


How to cite this article:
Seniaray N, Verma R, Ranjan R, Belho E, Mahajan H. 18F-fluorodeoxyglucose positron emission tomography-computed tomography in initial diagnosis and treatment response evaluation of new onset refractory status epilepticus. Indian J Nucl Med 2020;35:244-7

How to cite this URL:
Seniaray N, Verma R, Ranjan R, Belho E, Mahajan H. 18F-fluorodeoxyglucose positron emission tomography-computed tomography in initial diagnosis and treatment response evaluation of new onset refractory status epilepticus. Indian J Nucl Med [serial online] 2020 [cited 2020 Aug 7];35:244-7. Available from: http://www.ijnm.in/text.asp?2020/35/3/244/288461



We present the case of a 14-year-old male patient who presented with fever of 5 days' duration followed with refractory status epilepticus (SE). His electroencephalography showed generalized epileptiform discharges. Contrast-enhanced magnetic resonance imaging (MRI) brain [Figure 1] and cerebrospinal fluid (CSF) examination were unremarkable except for mild pleocytosis. Blood tests including viral markers, HIV, scrub typhus, dengue, herpes, mycoplasma serology, onco-neuronal antibodies, and autoimmune profile were unremarkable. In view of clinical findings, it was suspected that the patient had occult autoimmune encephalopathy which was preceded by fever. The patient then underwent whole-body 18 F-fluorodeoxyglucose positron emission tomography-computed tomography (18 F-FDG PET-CT) brain scan to confirm the diagnosis and rule out other diagnostic possibilities. The whole-body 18 F-FDG PET-CT scan was unremarkable, however 18 F-FDG PET-CT scan of the brain revealed [Figure 2] multiple focal areas of hypermetabolism in bilateral fronto-parieto-temporal and cingulate cortices with globally reduced 18 F-FDG uptake in rest of the bilateral cerebral, cerebellar, and subcortical regions, without any obvious abnormality on the CT images that were likely to be of inflammatory etiology. Three-dimensional stereotactic surface projection (3D-SSP) analysis with Z score estimation of the 18 F-FDG PET brain images was done with Cortex ID software (Illinois, Chicago, USA) (GE Healthcare) using age-matched controls [Figure 3], which showed multiple areas of hypermetabolism (red-yellow color) and hypometabolism (blue color) in the brain parenchyma. Follow-up 18 F-FDG PET study [Figure 4] done at 6 months after the initial presentation and after a complete course of intravenous immunoglobulin, showed normalization of the cerebral glucose metabolism.
Figure 1: Axial magnetic resonance imaging scan showing T2-weighted, fluid-attenuated inversion recovery, diffusion-weighted imaging, and apparent diffusion coefficient images (a-d), which were unremarkable

Click here to view
Figure 2: Axial computed tomography (CT) (a-e), positron emission tomography (PET) (f-j), and fused positron emission tomography-computed tomography (k-o) images showing multiple focal areas of hypermetabolism in bilateral fronto-parieto-temporal and cingulate cortices with globally reduced globally reduced FDG uptake in rest of the bilateral cerebral, cerebellar, and subcortical regions, without any obvious abnormality on the computed tomography (CT) images that were likely to be of inflammatory etiology

Click here to view
Figure 3: 3D-SSP analysis with Z score estimation of the 18F-FDG PET brain images was done with Cortex ID software (GE Healthcare) using age-matched controls, which showed multiple areas of hypermetabolism (red-yellow color) and hypometabolism (blue color) in the brain parenchyma

Click here to view
Figure 4: Comparative 18F-FDG PET study showing initial baseline axial 18F-FDG PET brain images (a-h) done at the time of initial presentation of symptoms, and follow-up 18F-FDG PET brain images (i-p) done at 6 months after the initial presentation and after a complete course of intravenous immunoglobulin, showing normalization of the cerebral glucose metabolism

Click here to view


NORSE is a rare form of super-refractory SE where no etiological factor is identified in a patient with no prior history of epilepsy.[1] The common clinical features include super-refractory SE following a mild febrile illness with possible initial CSF pleocytosis and unremarkable anatomical imaging such as MRI as seen in our case.[1],[2],[3],[4] The outcome is frequently fatal with severe neurological sequelae as optimal management for this devastating condition remains unclear.[2],[3] Recently, few case series in literature have suggested an underlying etiology of autoimmune encephalitis without any evidence of autoantibodies and for such patients, immunotherapies could be a given treatment of choice, which could improve the patient outcome.[5],[6],[7] In our case, the patient made complete recovery following treatment with immunotherapy.18 F-FDG PET-CT not only helped in the diagnosis by documenting hypermetabolic areas in the brain suggesting cerebral inflammation, but also helped in monitoring response to immunotherapy and prognosis of the patient.

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.
Wilder-Smith EP, Lim EC, Teoh HL, Sharma VK, Tan JJ, Chan BP, et al. The NORSE (new-onset refractory status epilepticus) syndrome: Defining a disease entity. Ann Acad Med Singapore 2005;34:417-20.  Back to cited text no. 1
    
2.
Gall CR, Jumma O, Mohanraj R. Five cases of new onset refractory status epilepticus (NORSE) syndrome: Outcomes with early immunotherapy. Seizure 2013;22:217-20.  Back to cited text no. 2
    
3.
Van Lierde I, Van Paesschen W, Dupont P, Maes A, Sciot R. De novo cryptogenic refractory multifocal febrile status epilepticus in the young adult: A review of six cases. Acta Neurol Belg 2003;103:88-94.  Back to cited text no. 3
    
4.
Baxter P, Clarke A, Cross H, Harding B, Hicks E, Livingston J, et al. Idiopathic catastrophic epileptic encephalopathy presenting with acute onset intractable status. Seizure 2003;12:379-87.  Back to cited text no. 4
    
5.
Ishikura T, Okuno T, Araki K, Takahashi MP, Watabe K, Mochizuki H. A case of new-onset refractory status epilepticus (NORSE) with an autoimmune etiology. Rinsho Shinkeigaku 2015;55:909-13.  Back to cited text no. 5
    
6.
Gupta P, Patel S, Ranjan R, Agrawal CS. An interesting case of super-refractory status epilepticus. Neurol India 2015;63:628-9.  Back to cited text no. 6
[PUBMED]  [Full text]  
7.
van Baalen A, Häusler M, Boor R, Rohr A, Sperner J, Kurlemann G, et al. Febrile infection-related epilepsy syndrome (FIRES): A nonencephalitic encephalopathy in childhood. Epilepsia 2010;51:1323-8.  Back to cited text no. 7
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

Top
  
 
  Search
 
  
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

 
  In this article
    Abstract
    References
    Article Figures

 Article Access Statistics
    Viewed67    
    Printed2    
    Emailed0    
    PDF Downloaded20    
    Comments [Add]    

Recommend this journal