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Images in Neurology

Isolated and Asymmetric Basal Ganglia Involvement in Early

Subacute Sclerosing Panencephalitis
Mahesh Kamate, Mayank Detroja1
Division of Pediatric Neurology, Department of Paediatrics, In‑Charge Child Development Clinic, KLE University’s J N Medical College,
1
Child Development Centre, KLE Prabhakar Kore Hospital, Belgaum, Karnataka, India

Neuroimaging does not have an obligatory role in the diagnosis

of subacute sclerosing panencephalitis (SSPE) but gives
important clues in the clinical assessment and differential
diagnosis. Magnetic resonance imaging (MRI) findings in SSPE
do not correspond to the clinical stages. In the early stages,
MRI findings are normal, or cortical/subcortical asymmetrical
hyperintense lesions may be observed in the posterior parts
of the brain. As the disease progresses, the lesions disappear
and new lesions occur symmetrically in the periventricular
white matter in association with mild cortical atrophy.[1,2]
The basal ganglia involvement is not common and is usually
affected later in the disease course after the cortex has already
shown signs of disease. In the cases reported earlier, the basal
ganglia involvement was bilateral and symmetric along with
white matter changes. We here report a case of SSPE who had
isolated and asymmetric basal ganglia involvement without Figure 1: Electroencephalography of the child showing characteristic
cortical or subcortical lesion. long‑interval generalized periodic epileptiform discharges in the
longitudinal montage characteristic of subacute sclerosing panencephalitis
A  12‑year‑old girl, born of nonconsanguineous parentage,
presented with a history of frequent falls with reduced
myoclonic jerks and classical EEG features, SSPE was
speech output and forgetfulness for the last 1 month, with
suspected and confirmed by raised cerebrospinal fluid
sudden extension of head without loss of consciousness
antimeasles antibody titers (1>:625).
for the last 15 days. There was no history of measles
in childhood and she was vaccinated for measles. On Neuroimaging in SSPE can be used to know the extent of the
examination, she was alert but had cognitive impairment. disease and for differential diagnosis. In SSPE, basal ganglia
She had generalized myoclonic jerks involving the neck lesions are not infrequent, but they tend to appear in patients
and trunk. The cranial nerve functions were normal, and with advanced clinical states, and/or in those with longer
deep tendon reflexes were exaggerated with intermittent disease duration.[2,3] Apart from the classical progression of MR
generalized dystonia. Electroencephalography (EEG) abnormalities in SSPE, many cases have been reported as early
showed generalized periodic complexes with asymmetrical involvement of thalamus, basal ganglia, brainstem, and corpus
background activity [Figure 1]. MRI was performed callosum with cortical or subcortical abnormalities. Praveen-
which showed isolated hyperintensities in left lentiform
nuclei on T2‑weighted/fluid‑attenuated inversion recovery Address for correspondence: Dr. Mahesh Kamate,
sequences [arrows in Figure 2]. The white matter was Division of Pediatric Neurology, Department of Paediatrics,
normal, and there was no cerebral atrophy. Although MRI KLE University’s J N Medical College, Belgaum ‑ 590 010, Karnataka, India.
brain findings were not classic of SSPE, in view of frequent E‑mail: drmaheshkamate@gmail.com

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DOI: How to cite this article: Kamate M, Detroja M. Isolated and asymmetric
10.4103/aian.AIAN_249_18 basal ganglia involvement in early subacute sclerosing panencephalitis. Ann
Indian Acad Neurol 2018;21:XX-XX.

© 2006 - 2018 Annals of Indian Academy of Neurology | Published by Wolters Kluwer - Medknow 1
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Kamate and Detroja: SSPE and basal ganglia

a b c

d e f
Figure 2: (a) T1‑weighted axial image of the brain at the level of basal ganglia showing left putaminal hypointensity (black arrows). (b and c) T2‑weighted
and fluid‑attenuated inversion recovery axial image of the brain at the level of basal ganglia showing left putaminal hyperintensity, respectively, with
normal white matter (black arrows). (d‑f) T2‑weighted axial image of the brain at the level of centrum semi‑ovale, temporal lobes, and upper midbrain,
respectively, showing normal white matter and absence of cerebral atrophy

Kumar  et al. reported basal ganglionic hyperintensities along
with cortical/subcortical changes which were seen in 6% of
patients.[4] However, in the cases reported, there is bilateral
involvement along with cerebral atrophy and white matter
changes. Unilateral and isolated basal ganglia involvement
as the initial MRI finding has not been reported earlier in the
literature.
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
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et al. MRI in subacute sclerosing panencephalitis. Neuroradiology
1996;38:636‑40.
3. BrismarJ, Gascon GG, Von Steyen KV, Bohlega S. Subacute sclerosing
panencephalitis: Evaluation with CT and MR. Am J Neuroradiol
1996;17:761‑772.
4. Praveen-Kumar S, Sinha S, Taly AB, Bharath RD, Bindu PS, Murthy SS,
et al The spectrum of MRI findings in subacute sclerosing panencephalitis
with clinical and EEG correlates. J Pediatr Neurol 2011;9:177-85.

2 Annals of Indian Academy of Neurology ¦ Volume XX ¦ Issue XX ¦ Month 2018