Indian J Nucl Med. 2013 AprJun; 28(2): 93–95. PMCID: PMC3800318
doi: 10.4103/09723919.118257
Hemimegalencephaly: A rare cause of hemihypoperfusion on 99m
technetiumethyl cysteinate dimer brain perfusion singlephoton
emission computed tomography
Nishikant A Damle, Abhinav Singhal, Anirban Mukherjee, Manas Kumar Sahoo, Madhavi Tripathi, and Chandrasekhar
Bal
Department of Nuclear Medicine, All India Institute of Medical Sciences, New Delhi, India
Address for correspondence: Dr. Nishikant A Damle, Department of Nuclear Medicine, All India Institute of Medical Sciences, New Delhi
110 029, India. Email: nkantdamle@gmail.com
Copyright : © Indian Journal of Nuclear Medicine
This is an openaccess article distributed under the terms of the Creative Commons AttributionNoncommercialShare Alike 3.0 Unported,
which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Abstract
Hemimegalencephaly is a rare congenital neuronal migration disorder that can presents with the equally
rare finding of hemihypoperfusion on brain perfusion singlephoton emission computed tomography
(SPECT). It is an extremely rare cause of intractable epilepsy. Technetium99m ethyl cysteinate dimer
(ECD) brain perfusion SPECT is useful in excluding other foci of hypoperfusion in the contralateral since
hemispherectomy has been suggested to be the treatment of choice. Furthermore, hemimegalencephaly
may present with hyper as well as hypoperfusion on ECD SPECT. We present the case of an 11yearold
male child with intractable seizures who showed hemihypoperfusion in the hemimegalecephalic
hemisphere.
Keywords: 99m technetiumethyl cysteinate dimer brain singlephoton emission computed tomography,
hemihypoperfusion, hemimegalencephaly
INTRODUCTION
Hemimegalencephaly is a rare congenital neuronal migration disorder characterized by unilateral
hypertrophy of a cerebral hemisphere and ipsilateral ventriculomegaly.[1,2] It can be an isolated
abnormality, or it can be associated with neurocutaneous syndromes.[1] Clinical manifestations include
intractable motor seizures, impaired psychomotor development, hemiparesis, and hemianopsia.[1] Patients
having hemimegalencephaly usually have a very poor prognosis. These patients require early surgical
interventions to prevent progression to intractable epilepsy.[3] Hemispherectomy is the treatment of
choice.[1] The definition of the dysfunctional areas of dysplastic cortex may affect surgical management of
these anomalies.[4] Evaluation of regional cerebral blood flow using technetium99m ethyl cysteinate
dimer (Tc99m ECD) singlephoton emission computed tomography (SPECT) provides useful information
in the patient of hemimegalencephaly[5,6] because it clearly delineates the border of the dysplastic cortex
by showing altered blood flow to the dysfunctional areas.[4]
CASE REPORT
An 11yearold male child was referred to a tertiary care hospital with history of gradually increasing
seizures frequency since infancy. The child had delayed milestones and right hemiparesis. Seizures were
mainly right sided, complex partial in nature and refractory to medical treatment over the past decade. The
child was born out of nonconsanguineous marriage, at term with a normal vaginal delivery. There was no
family history of neurologic diseases. Noncontrast computed tomography (CT) of head showed diffuse
enlargement of left cerebral hemisphere with midline shift towards right while the right cerebral
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hemisphere was normal [Figure 1a]. T2W magnetic resonance imaging (MRI) images showed diffuse
gyral thickening involving the left cerebral cortex causing midline shift and scalloping of inner table of left
calvarium [Figure 1b]. White matter in both cerebral hemispheres show normal myelination. Linear
hyperintensities involving the subcortical U fibers of left frontoparietal region were seen on the fluid
attenuation inversion recovery image [Figure 1c].
Postgadolinium T1W image in axial plane showed no abnormal enhancement [Figure 1d]. Tc99m ECD
brain perfusion SPECT revealed diffusely decreased tracer uptake in the left cerebral hemisphere, with
normal uptake in the right hemisphere suggestive of left cerebral hemihypoperfusion [Figure 2ac].
DISCUSSION
Tc99m ECD is a perfusion agent similar to Tc99m hexamethylpropyleneamine oxime (HMPAO).
Regional differences in perfusion within the affected hemisphere with relative interictal hypoperfusion and
ictal hyperperfusion of the seizure focus in a neonate with hemimegalencephaly has been reported on Tc
99m HMPAO brain SPECT imaging.[7] Ictal SPECT using Tc99m HMPAO is also useful in
demonstrating the site of epileptogenesis and its spread.[2] Sometimes it has been found that brain SPECT
shows increased radiotracer uptake in the pathological hemisphere in the early part of life, which gets
reversed in the later stage with decreased radiotracer uptake in the pathological hemisphere. These serial
changes of radiotracer uptake on SPECT probably reflect either changes in epileptic activity or
maturational changes in cerebral perfusion in hemimegalencephaly.[8] Brain SPECT helps in excluding
seizure foci or other perfusion abnormalities in the contralateral side before surgery.[1]
Considering recent recommendations for hemispherectomy in these patients, Tc99m ECD/HMPAO
SPECT scan should be used to complement electroencephalogram (EEG) as a method to define the extent
of abnormality, which may be more relevant to longterm prognosis than EEG alone.[9] Also, while
interpreting a brain SPECT, hemimegalencephaly must be considered as a differential in
hemihypoperfusion, for which CT/MRI correlation should be sought before reporting. The other
differentials for hemispheric hypoperfusion are Rasmussen's encephalitis,[10] Sturge Weber syndrome,[11]
Aspergers syndrome,[12] exanthema subitum[13] and gliomatosis cerebri,[14] which can even be mistaken
for hemimegalencephaly.
Footnotes
Source of Support: Nil
Conflict of Interest: None declared
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Figures and Tables
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Figure 1
Noncontrast computed tomography of head (a) Axial shows diffuse enlargement of left cerebral hemisphere (*) with
midline shift towards right while the right hemisphere is normal. Noncontrast magnetic resonance imaging T2W (b)
Images (axial) show diffuse gyral thickening (thin arrow) in the left cerebrum causing midline shift and scalloping of
inner table (arrow head) of left calvarium. White matter in both cerebral hemispheres show normal myelination. Linear
hyperintensities involving the subcortical U fibers of left frontoparietal region seen on fluid attenuation inversion
recovery image (c) Postgadolinium T1W image in axial plane (d) shows no abnormal enhancement
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Figure 2
99m technetiumethyl cysteinate dimer brain perfusion singlephoton emission computed tomography transaxial, coronal
and sagittal images reveal diffusely decreased tracer uptake in the left cerebral hemisphere (arrow), with normal uptake in
the right hemisphere. Findings are suggestive of left cerebral hemihypoperfusion
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