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LETTER TO EDITOR
Year : 2020  |  Volume : 68  |  Issue : 1  |  Page : 225-229

Severe Lingual and Orofacial Dyskinesias in Anti‑NMDA Receptor Encephalitis: A Case Report and Review of Indian Literature


Department of Neurology, King George's Medical University, Lucknow, Uttar Pradesh, India

Date of Web Publication28-Feb-2020

Correspondence Address:
Dr. Ravindra K Garg
Department of Neurology, King George's Medical University, Lucknow - 226 003, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.279693

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How to cite this article:
Rizvi I, Garg RK, Malhotra HS, Kumar N, Uniyal R. Severe Lingual and Orofacial Dyskinesias in Anti‑NMDA Receptor Encephalitis: A Case Report and Review of Indian Literature. Neurol India 2020;68:225-9

How to cite this URL:
Rizvi I, Garg RK, Malhotra HS, Kumar N, Uniyal R. Severe Lingual and Orofacial Dyskinesias in Anti‑NMDA Receptor Encephalitis: A Case Report and Review of Indian Literature. Neurol India [serial online] 2020 [cited 2020 Mar 31];68:225-9. Available from: http://www.neurologyindia.com/text.asp?2020/68/1/225/279693




Sir,

Anti-N-methyl-d-aspartate (NMDA) receptor encephalitis is an autoimmune disorder of the central nervous system in which auto-antibodies affect NMDA glutamate receptors of the brain. Initial clinical manifestations are dominated by a variety of neuropsychiatric symptoms. Anti-NMDA receptor encephalitis frequently affects young females and is often associated with ovarian teratomas. Orofacial–lingual dyskinesias are characteristically seen in a large number of patients with anti-NMDA receptor encephalitis. Immunotherapy and removal of ovarian malignancy are often life-saving.[1],[2] Here, we report a patient who presented with severe orolingual dyskinesia that responded well to immunotherapy.

A 22-year-old female presented with abnormal behavior, forgetfulness, seizures, and abnormal movements involving the tongue and face of 15 days duration. She acutely developed abnormal behavior and agitation. She became delusional and thought somebody was going to kill her; sometimes she also had auditory hallucinations. She stopped taking care of her personal hygiene and stopped cleaning and bathing herself. She also developed forgetfulness and could not remember where she had kept objects of day-to-day use and had to search for them. There was history of 5–6 episodes of seizures. On examination, she was conscious but disoriented and agitated. On higher mental function examination, her attention was impaired; recent memory was also impaired. There were persistent lingual and orofacial dyskinesias in the form of continuous tongue protrusion and orofacial movements [Video 1].



All laboratory investigations were normal. A contrast-enhanced MRI of the brain was normal. Electroencephalography was normal. Cerebrospinal fluid examination revealed normal protein, sugar, and cell count. Cerebrospinal fluid was also negative for virology, India ink, and gram staining. CSF IgG antibodies against the GluN1 subunit of the NMDA receptor were positive. A search for ovarian tumor was performed but ultrasonography of abdomen and pelvis, CT scan of abdomen, pelvis, and chest all were normal. She was treated with intravenous immunoglobulins (0.4 g/kg/day for 5 days) along with intravenous methyl prednisolone (1000 mg/day for 5 days). She had improvement in dyskinesias and behavior over the next 2 weeks [Video 2]. Maintenance immunotherapy, weekly intravenous immunoglobulins (0.4/kg weekly) administration for 12 weeks, along with tapering doses of was given. She was fully independent for all activities of daily living at 6 months of follow-up.



Orofacial dyskinesias, including repeated tongue protrusion, are characteristic movement disorder in anti-NMDA receptor encephalitis.[1],[2] A review of Indian cases revealed that, so far, a total of 81 cases had been reported across India. The majority of them belonged to the pediatric age group. An ovarian tumor was reported from five cases only. Fifty-two (64.2%) patients had some forms of orofacial–lingual dyskinesias. EEG was abnormal in 54 patients; a generalized background slowing was the most common EEG abnormality. The classic finding of extreme delta brush was also reported in some patients. MRI of the brain was abnormal in 19 (23.5%) patients only; hyperintensities on T2/FLAIR imaging was the most common imaging abnormality. A total of eight patients needed respiratory support; all such patients were successfully weaned off the ventilator. Almost all of the patients received some form of immunotherapy; 70 out of 81 patients had good outcome[3],[4],[5],[6],[7],[8],[9],[10],[11],[12],[13],[14],[15],[16],[17],[18],[19],[20],[21],[22],[23],[24],[25],[26],[27],[28] [Table 1].
Table 1: Summary of all Indian articles published in PubMed on anti-NMDA receptor encephalitis

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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.



 
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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. 2
    
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Reddy MS, Thippeswamy H, Ganjekar S, Nagappa M, Mahadevan A, Arvinda HR, et al. Anti-NMDA receptor encephalitis presenting as postpartum psychosis-a clinical description and review. Arch Womens Ment Health 2018;21:465-9.  Back to cited text no. 5
    
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Kumari K, Sahni N, Kumari V, Saini V. Anti-N-methyl-D-aspartate-receptor encephalitis in young females. Turk J Anaesthesiol Reanim 2017;45:377-9.  Back to cited text no. 6
    
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Basheer S, Nagappa M, Mahadevan A, Bindu PS, Taly AB, Girimaji SC. Neuropsychiatric manifestations of pediatric NMDA receptor autoimmune encephalitis: A case series from a tertiary care center in India. Prim Care Companion CNS Disord 2017;19(4).  Back to cited text no. 7
    
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Pastel H, Chakrabarty B, Saini L, Kumar A, Gulati S. A case of anti-N-methyl-D-aspartate (NMDA) receptor encephalitis possibly triggered by an episode of Japanese B encephalitis. Neurol India 2017;65:895-7.  Back to cited text no. 8
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Chatterjee SS, Ghosal MK, Mitra S. Psychosis and catatonia as presenting features of anti-N-methyl-D-aspartate (anti-NMDA) receptor encephalitis. Asian J Psychiatr 2017;27:112.  Back to cited text no. 9
    
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Patil VA, Kulkarni SD, Udwadia-Hegde A, Sayed RJ, Garg M. Anti-N-methyl-D-aspartate receptor encephalitis during relapse of herpes simplex encephalitis in a young boy: A brief review of literature. Neurol India 2017;65:393-7.  Back to cited text no. 10
[PUBMED]  [Full text]  
11.
Sharma P, Sagar R, Patra B, Saini L, Gulati S, Chakrabarty B. Psychotic symptoms in anti-N-methyl-d-aspartate (NMDA) receptor encephalitis: A case report and challenges. Asian J Psychiatr 2016;22:135-7.  Back to cited text no. 11
    
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Goenka A, Jain V. Anti-NMDA Receptor Encephalitis. Indian J Pediatr 2016;83:1032.  Back to cited text no. 12
    
13.
Mythri SV, Mathew V. Catatonic syndrome in anti-NMDA receptor encephalitis. Indian J Psychol Med 2016;38:152-4.  Back to cited text no. 13
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14.
Suthar R, Sankhyan N, Singhi P. Hyperkinetic movement disorder in a girl with anti-NMDA receptor encephalitis. Indian Pediatr 2016;53:81.  Back to cited text no. 14
    
15.
Suthar R, Saini AG, Sankhyan N, Sahu JK, Singhi P. Childhood anti-NMDA receptor encephalitis. Indian J Pediatr 2016;83:628-33.  Back to cited text no. 15
    
16.
Nagappa M, Bindu PS, Mahadevan A, Sinha S, Mathuranath PS, Taly AB. Clinical features, therapeutic response, and follow-up in pediatric anti-N-methyl-D-aspartate receptor encephalitis: Experience from a Tertiary Care University Hospital in India. Neuropediatrics 2016;47:24-32.  Back to cited text no. 16
    
17.
Shaikh MA, Dhansura T, Gandhi S, Shaikh T. Anaesthetic management of a patient with anti-NMDA receptor encephalitis. Indian J Anaesth 2015;59:248-50.  Back to cited text no. 17
[PUBMED]  [Full text]  
18.
Mangalwedhe SB, Pandurangi AA, Pandurangi AK, Dugani RI. Anti-N-methyl-D-aspartate receptor encephalitis presenting with psychiatric symptoms. J Neuropsychiatry Clin Neurosci 2015;27:e152-3.  Back to cited text no. 18
    
19.
Kaur S, Juneja M, Mishra D, Jain S. Anti-N-methyl-D-aspartate receptor encephalitis: A case report and review of the literature. J Pediatr Neurosci 2014;9:145-7.  Back to cited text no. 19
[PUBMED]  [Full text]  
20.
Kattepur AK, Patil D, Shankarappa A, Swamy S, Chandrashekar NS, Chandrashekar P, et al. Anti-NMDAR limbic encephalitis--a clinical curiosity. World J Surg Oncol 2014;12:256.  Back to cited text no. 20
    
21.
Shruthi TK, Shuba S, Rajakumar PS, Chitrambalam S. Anti-NMDA receptor encephalitis in an adolescent. Indian Pediatr 2014;51:405-6.  Back to cited text no. 21
    
22.
Sharma B, Handa R, Prakash S, Nagpal K, Gupta P. Anti-NMDA receptor encephalitis: A neurological disease in psychiatric disguise. Asian J Psychiatr 2014;7:92-4.  Back to cited text no. 22
    
23.
Chakrabarty B, Tripathi M, Gulati S, Yoganathan S, Pandit AK, Sinha A, et al. Pediatric anti-N-methyl-D-aspartate (NMDA) receptor encephalitis: Experience of a tertiary care teaching center from north India. J Child Neurol 2014;29:1453-9.  Back to cited text no. 23
    
24.
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27.
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