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LETTER TO EDITOR
Year : 2010  |  Volume : 58  |  Issue : 4  |  Page : 671-672

Idiopathic anti-NMDA-receptor encephalitis in a young Indian girl


1 Department of Neurology, Lourdes Heart Institute and Neuro Center, Lourdes Hospital, Kochi, Kerala, India
2 Department of Nuclear Medicine, Amrita Institute of Medical Sciences, Kochi, Kerala, India

Date of Acceptance21-Jul-2010
Date of Web Publication24-Aug-2010

Correspondence Address:
Boby Varkey Maramattom
Department of Neurology, Lourdes Heart Institute and Neuro Center, Lourdes Hospital, Kochi, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.68692

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How to cite this article:
Maramattom BV, Philip C, Sundaram P S. Idiopathic anti-NMDA-receptor encephalitis in a young Indian girl. Neurol India 2010;58:671-2

How to cite this URL:
Maramattom BV, Philip C, Sundaram P S. Idiopathic anti-NMDA-receptor encephalitis in a young Indian girl. Neurol India [serial online] 2010 [cited 2019 Jun 26];58:671-2. Available from: http://www.neurologyindia.com/text.asp?2010/58/4/671/68692


Sir,

Anti-N-methyl-D-aspartate-receptor (NMDAR) encephalitis is a recently described paraneoplastic syndrome often associated with ovarian teratoma or idiopathic autoimmune encephalitis. It is associated with antibodies against NR1-NR2 hetromers of the NMDAR in serum and cerebrospinal fluid (CSF). [1] We describe the first case of idiopathic anti-NMDAR encephalitis from India.

A 13-year-old girl developed acute-onset behavioral abnormalities, gait disturbance and bruxism over a period of 1 month. An initial EEG and contrast magnetic resonance imaging (MRI) were normal. At admission, she was stuporous and mute. EEG showed frequent electrographic seizures of possible right-hemispheric onset. She was mechanically ventilated and required multiple anticonvulsants, including midazolam infusion for five days, before seizures could be controlled. CSF examination revealed 32 lymphocytes; protein, 52 mg/dL; and normal sugar. An extensive CSF encephalitic panel was negative. Twenty days after admission, she developed orofacial grimacing movements with oculogyric deviation and generalized chorea. Repeat contrast MRI of brain was normal. Subsequent multiple EEGs were normal. For another month, she continued to have episodic hypoventilation and cardiac dysautonomia. At this point of time, serum anti-NMDAR (N-methyl-D-aspartate-receptor) antibody testing was reported as positive (Prof. Angela Vincent, Weatherall Institute, Oxford). An 18F FDG PET-CT showed minimal-to-moderate FDG uptake in multiple lymph nodes, bone marrow and spleen, suggesting the possibility of a lymphoproliferative disorder, but no evidence of a teratoma [Figure 1]. There was hypometabolism in the left temporal and both occipital lobes [Figure 2]. Intense diffuse FDG uptake in both the lungs was attributed to nosocomial pneumonia. Multiple cervical lymph-node biopsies and bone marrow aspiration biopsies showed only reactive changes. These findings made us consider the diagnosis of idiopathic anti-NMDAR encephalitis. She was treated with two courses of IV methyl-prednisolone 1 g Χ 5 days, followed by IVIg 2 g/kg. Two weeks later, she became more responsive and was weaned off the ventilator. Five months later, she was ambulating independently and communicating with gestures.
Figure 1 :FDG PET-CT coronal images showing uptake in the lungs

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Figure 2 :FDG PET-CT axial images of brain showing hypometabolism in the left temporal and bilateral occipital lobes

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Anti-NMDAR antibodies bind to the NR2B or NR2A subunits of NMDAR. NR2B binds glutamate and is avidly expressed in the hippocampal and forebrain neurons of human beings. [2] These antibodies are thought to inhibit NMDARs in presynaptic GABAergic interneurons, resulting in reduced GABA release and disinhibition of postsynaptic glutamatergic transmission with excessive release of glutamate in the prefrontal/ subcortical structures. The pathogenic role of these antibodies is further strengthened by their disappearance during clinical improvement.

The typical clinical evolution of anti-NMDAR encephalitis includes five phases: phase I (prodromal phase)- 'viral-like' illness; phase II- acute psychosis and behavioral symptoms; phase III- intractable seizures, central hypoventilation and dysautonomia; phase IV- hyperkinetic phase with orofacial grimacing; and phase V- gradual recovery from the illness. MRI of the brain is either normal or shows nonspecific changes. [3] EEG shows only diffuse slowing. CSF shows features of inflammation with pleocytosis, increased protein and oligoclonal band.

ANMDARE was first described in a female in 1997 as ovarian teratoma-associated limbic encephalitis (OTLE). Besides, as a paraneoplastic syndrome, this disorder can be idiopathic in 30% to 40% of patients and has also been reported in males. [4] Rarely anti-NMDAR encephalitis has been associated with malignancies such as Hodgkin's lymphoma or testicular teratomas. [5],[6] The treatment is removal of the underlying neoplasm, combined with immunotherapy, plasma exchange, intravenous immunoglobulin, and corticosteroids. Idiopathic anti-NMDAR encephalitis may show a poor response to treatment.



 
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1.Kleinig TJ, Thompson PD, Matar W, Duggins A, Kimber TE, Morris JG, et al. The distinctive movement disorder of ovarian teratoma-associated encephalitis. Mov Disord 2008;23:1256-61.  Back to cited text no. 1  [PUBMED]  [FULLTEXT]  
2.Dalmau J, Tόzόn E, Wu HY, Masjuan J, Rossi JE, Voloschin A,et al. Paraneoplastic anti-N-methyl-D-aspartate receptor encephalitis associated with ovarian teratoma. Ann Neurol 2007;61:25-36.  Back to cited text no. 2      
3.Sansing LH, Tόzόn E, Ko MW, Baccon J, Lynch DR, Dalmau J. A patient with encephalitis associated with NMDA receptor antibodies. Nat Clin Pract Neurol 2007;3:291-6.  Back to cited text no. 3      
4.Dalmau J, Gleichman AJ, Hughes EG, Rossi JE, Peng X, Lai M,et al. Anti-NMDA-receptor encephalitis: Case series and analysis of the effects of antibodies. Lancet Neurol 2008;7:1091-8.  Back to cited text no. 4  [PUBMED]  [FULLTEXT]  
5.Zandi MS, Irani SR, Follows G, Moody AM, Molyneux P, Vincent A. Limbic encephalitis associated with antibodies to the NMDA receptor in Hodgkin lymphoma. Neurology 2009;73:2039-40.  Back to cited text no. 5  [PUBMED]  [FULLTEXT]  
6.Eker A, Saka E, Dalmau J, Kurne A, Bilen C, Ozen H, et al. Testicular teratoma and anti-N-methyl-D-aspartate receptor-associated encephalitis. J Neurol Neurosurg Psychiatry 2008;79:1082-3.  Back to cited text no. 6  [PUBMED]  [FULLTEXT]  


    Figures

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