Sequential MRI follow-up
Naveen Sankhyan, MD
Suvasini Sharma, MD
Mahesh Kamate, MD
A 42-month-old boy presented with frequent left
partial motor seizures of 3 months duration. The
symptoms began after an acute episode of fever, en-
cephalopathy, vomiting, and left partial seizures last-
ing 1 week. CSF was unremarkable and negative for
HSV PCR. Evaluation for procoagulant states was
negative. He recovered with left hemiparesis. MRI
during the acute illness (figure 1) and at 3 months
(figure 2) suggested a diagnosis of hemiconvulsion-
hemiplegia-epilepsy (HHE) syndrome.
HHE syndrome is characterized by prolonged
unilateral convulsions with fever in children under 4
years of age, who subsequently develop hemiplegia,
partial epilepsy, and extensive atrophy of the in-
volved hemisphere.1,2The pathogenesis is believed to
be an interplay among genetic predisposition; viral
infection (e.g., influenza, HHV 6) or toxin (theoph-
ylline) exposure; excitotoxicity due to prolonged ictal
activity; and contributory systemic factors such as cy-
tokine excess, hypoxia, ischemia, and fever.2
1.Toldo I, Calderone M, Boniver C, Dravet C, Guerrini R,
Laverda AM. Hemiconvulsion-hemiplegia-epilepsy syn-
drome: early magnetic resonance imaging findings and
neuroradiological follow up. Brain Dev 2007;29:109–111.
2. Mizuguchi M, Yamanouchi H, Ichiyama T, Shiomi M.
Acute encephalopathy associated with influenza and other
viral infections. Acta Neurol Scand 2007;115 (suppl 186):
From the Division of Pediatric Neurology, Department of Pediatrics (N.S., S. Sharma, M.K.), and Department of Radiodiagnosis (S. Subramanian),
All India Institute of Medical Sciences, New Delhi, India.
Disclosure: The authors report no disclosures.
Address correspondence and
reprint requests to Dr. Naveen
Sankhyan, Senior Resident,
Division of Pediatric Neurology,
Department of Pediatrics, All
India Institute of Medical
Sciences, New Delhi, 110029
Figure 1 T1 axial (A), T2 axial (B), and coronal (C) (FLAIR) sections of brain show thickening of cortical gray
matter with increased signal intensity on T2W and FLAIR images with effacement of sulcal
spaces and midline shift suggestive of unilateral encephalitis
Figure 2 T1 axial (A), T2 axial (B), and coronal (C) (T2) sections of brain showing right hemispherical atrophy
with gliotic changes and ventricular dilation, as well as right subdural hematoma
Mitchell S.V. Elkind,
Copyright © 2008 by AAN Enterprises, Inc.
Naveen Sankhyan, Suvasini Sharma, Mahesh Kamate, et al.
: Hemiconvulsion-hemiplegia-epilepsy syndrome: Sequential
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