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Table of Contents    
LETTERS TO EDITOR
Year : 2018  |  Volume : 66  |  Issue : 2  |  Page : 536-538

Extreme delta brushes in a 14-year old girl with anti-NMDAR encephalitis


Epilepsy Services, Division of Neurology, The Hospital for Sick Children, Toronto, Ontario, Canada

Date of Web Publication15-Mar-2018

Correspondence Address:
Dr. Cristina Go
Epilepsy Services, Division of Neurology, The Hospital for Sick Children, Toronto, Ontario
Canada
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.227316

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How to cite this article:
Jain P, Whitney R, Go C. Extreme delta brushes in a 14-year old girl with anti-NMDAR encephalitis. Neurol India 2018;66:536-8

How to cite this URL:
Jain P, Whitney R, Go C. Extreme delta brushes in a 14-year old girl with anti-NMDAR encephalitis. Neurol India [serial online] 2018 [cited 2020 Jul 6];66:536-8. Available from: http://www.neurologyindia.com/text.asp?2018/66/2/536/227316




Sir,

A previously healthy, 14-year old girl presented with an explosive onset of seizures (generalized tonic-clonic and left focal) and new-onset behavioral change. She was treated with multiple antiepileptic drugs. Given the high seizure burden and altered behavior, a continuous electroencephalogram (EEG) monitoring was commenced [Figure 1], [Figure 2], [Figure 3].
Figure 1: EEG on day 1 of hospitalization: The record shows nearly continuous 1-2 Hz polymorphic delta activity over the right fronto-temporal head region (longitudinal bipolar montage with a timebase 30 mm/sec, sensitivity 7 μV/mm, high frequency filter at 70 Hz, low frequency filter at 1 Hz and sampling rate of 256 Hz)

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Figure 2: EEG on day 2 of hospitalization: The record shows 1-2 Hz delta activity at Fp2/F4/C4 with superimposed 25-30 Hz beta activity (black arrows) suggestive of extreme delta brushes (longitudinal bipolar montage with a timebase 30 mm/sec, sensitivity 7 μV/mm, high frequency filter at 70 Hz, low frequency filter at 1 Hz and sampling rate of 256 Hz)

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Figure 3: EEG on day 2 of hospitalization: The record shows sudden onset of 25 Hz beta activity at Fp2/F4 which evolves in frequency, location (right hemispheric spread and then left hemisphere involvement), amplitude and morphology (a-d). The seizure lasted for 45 seconds and there was no clinical correlate. Continuous EEG captured multiple similar electrographic seizures (longitudinal bipolar montage with a timebase 30 mm/sec, sensitivity 7 μV/mm, high frequency filter at 70 Hz, low frequency filter at 1 Hz and sampling rate of 256 Hz)

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Laboratory investigations for infections and metabolic derangements were unremarkable. Magnetic resonance imaging (MRI) of the brain revealed no abnormalities. Serum and cerebrospinal fluid (CSF) anti- N-methyl-D-aspartate (NMDA) antibodies were both positive. MRI of the pelvis and abdomen were normal. A diagnosis of anti-NMDA-receptor (NMDAR)-mediated encephalitis was made.

While in the hospital, she received treatment with intravenous immunoglobulins (IVIG), steroids, and rituximab. She showed a good clinical response to therapy and was discharged home after 2 months in hospital. An electroencephalogram (EEG) done before discharge revealed persistent delta slowing over the right frontal head region but no extreme delta brushes (EDB).

She continued to have relapses intermittently. These relapses were similarly characterized by altered behavior and clinical seizures with persistently positive CSF anti-NMDA antibodies. Serial oncological imaging was negative. The relapses were managed with steroids and additional cycles of rituximab.

Seizures are a common presentation in anti-NMDA encephalitis and have been estimated to affect up to 77% of the children.[1],[2] The associated EEG patterns described are usually nonspecific. One unique EEG pattern described is EDB. Ikeda et al.,[3] described this EEG pattern in a 19-year old woman with anti-NMDA receptor encephalitis with a high seizure burden. Schmitt et al.,[4] later described a similar pattern in 30% of the patients with anti-NMDA encephalitis. The pattern was characterized by rhythmic 1–3 Hz delta activity with superimposed 20–30 Hz beta activity.

The presence of EDB on EEG has been associated with a more protracted hospital course, abnormal neuroimaging, and a trend towards a worse outcome at discharge in individuals with anti-NMDA encephalitis.[4] Notably, the resolution of EDB on EEG has been associated with clinical improvement.

The mechanism of EDB is unknown. It has been postulated that in patients with anti-NMDA-receptor encephalitis, the levels of synaptic NMDA-receptor and NMDA-receptor mediated currents are reduced leading to altered rhythmic neuronal activity, which may produce the distinct EEG pattern.[4]

Although EDB has been described in the EEG of individuals with anti-NMDA encephalitis, its specificity is not known. Recently, EDB has been described among patients with other diagnoses, such as stroke, brain tumor, metabolic disturbances, and hypoxic encephalopathy.[5] Regardless, the presence of EDB on EEG, especially in the context of other clinical symptoms such as behavioral change, seizures, autonomic disturbances and movement disorders should alert the clinician about a possible diagnosis of anti-NMDAR encephalitis. Recognition of this unique EEG pattern may aid in the diagnosis and prevent delays in treatment.

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.
Florance NR, Davis RL, Lam C, Szperka C, Zhou L, Ahmad S, et al. Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis in children and adolescents. Ann Neurol 2009;66:11-8.  Back to cited text no. 1
    
2.
Dalmau J, Lancaster E, Martinez-Hernandez E, Rosenfeld MR, Balice-Gordon R. Clinical experience and laboratory investigations in patients with anti-NMDAR encephalitis. Lancet Neurol 2011;10:63-74.  Back to cited text no. 2
    
3.
Ikeda A, Matsui M, Hase Y, Hitomi T, Takahashi Y, Shibasaki H,et al. 'Burst and slow complexes' in nonconvulsive epileptic status. Epileptic Disord 2006;8:61-4.  Back to cited text no. 3
    
4.
Schmitt SE, Pargeon K, Frechette ES, Hirsch LJ, Dalmau J, Friedman D. Extreme delta brush: A unique EEG pattern in adults with anti-NMDA receptor encephalitis. Neurology 2012;79:1094-100.  Back to cited text no. 4
    
5.
Baykan B, Tuncer OG, Vanli-Yavuz EN, Kirac LB, Gundogdu G, Bebek N,et al. Delta brush pattern is not unique to NMDAR encephalitis: Evaluation of two independent long-term EEG cohorts. Clin EEG Neurosci 2017 DOI: 10.1177/15500594176931. journals.sagepub.com/doi/abs/10.1177/1550059417693168.  Back to cited text no. 5
    


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