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Indian Pediatr 2018;55: 260 |
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Autoimmune Encephalitis Following Herpes Simplex Virus
Encephalitis in an Infant
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*Venkateswari Ramesh and Janani Sankar
Department of Pediatrics, CHILDS Trust Medical Research
Foundation, Kanchi Kamakoti CHILDS Trust Hospital,
Chennai, India.
Email: *[email protected]
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A 6-month-old developmentally normal boy presented to us with fever,
recurrent right focal seizures and altered sensorium for two days. He
was unconscious with poor respiratory effort requiring mechanical
ventilation. Cerebrospinal fluid (CSF) analysis showed high protein (96
mg/dL), normal glucose (60 mg/dL), and lymphocytic pleocytosis (90
lymphocytes and 250 RBC’s/HPF). Polymerase Chain Reaction for Herpes
Simplex Virus type 1 DNA (HSV-1 DNA PCR) in CSF was positive. Magnetic
Resonance Imaging (MRI) of brain showed multiple infarcts in bilateral
temporal, frontal regions and left insular cortex.
Electro-encephalography (EEG) showed focal slowing over left hemisphere.
Seizures were controlled with levetiracetam and fosphenytoin. The infant
was treated with parenteral acyclovir for 3 weeks. At discharge, he was
well without neurological deficit.
Ten days after discharge, he was re-admitted with
involuntary movements, loss of social smile, and not recognizing
parents. He was hypotonic with poor visual fixation. Perioral dyskinesia
and marked choreoathetotic movements was present. Repeat CSF analysis
showed high protein (83 mg/dL), and lymphocytic pleocytosis (96%
lymphocytes). Repeat CSF HSV PCR was negative. CSF NMDAR antibody was
positive. Infant was initially treated with Intravenous immunoglobulin
and Pulse methylprednisolone. As there was no response, he was
subsequently treated with 750 mg/m 2
of Rituximab followed by monthly 750 mg/m2
cyclophosphamide. There was good response; choreoathetoid
movements settled and encephalopathy improved.
Herpes Simplex Encephalitis triggers NMDAR antibodies
and potentially other brain autoimmunity [1]. These patients can present
with relapsing neurologic symptoms or choreoathetosis after Herpes
Simplex infection [2]. Prompt diagnosis and early immunotherapy has been
shown to improve clinical outcome in these children [3]. In any child
who presents with relapsing symptoms or choreoathetosis after HSV
encephalitis, the possibility of autoimmune encephalitis should be
considered.
Acknowledgement: Dr. Lakshmi Narayanan,
Consultant Pediatric Neurologist.
References
1. Armangue T, Leypoldt F, Málaga I, Raspall-Chaure
M, Marti I, Nichter C, et al. Herpes simplex virus encephalitis
is a trigger of brain autoimmunity. Ann Neurol. 2014;75:317-23.
2. Armangue T, Dalmau J, Autoimmune Encephalitis.
In: Kliegman RM, Stanton BF, St. Gema JW, Schor NF, Behrman RE,
editors: Nelson Textbook of Pediatrics, 20th edn. Philadelphia: WB
Saunders Co. p. 2905-10.
3. Armangue T, Moris G, Cantarín-Extremera V, Conde CE, Rostasy K,
Erro ME, et al.; Spanish Prospective Multicentric Study of
Autoimmunity in Herpes Simplex Encephalitis. Autoimmune post-herpes
simplex encephalitis of adults and teenagers. Neurology.
2015;85:1736-43.
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