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Table of Contents    
Year : 2012  |  Volume : 60  |  Issue : 5  |  Page : 521-522

Isolated bilateral basal ganglionic hyper intensities in early stage of Subacutesclerosing Panencephalitis: A case report

Department of Neurology, Nizam's Institute of Medical Sciences, Hyderabad, India

Date of Web Publication3-Nov-2012

Correspondence Address:
Surya Prabha Turaga
Department of Neurology, Nizam's Institute of Medical Sciences, Hyderabad
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.103205

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How to cite this article:
Turaga SP, Kaul S, Haritha Chowdary HA, Praveen P. Isolated bilateral basal ganglionic hyper intensities in early stage of Subacutesclerosing Panencephalitis: A case report. Neurol India 2012;60:521-2

How to cite this URL:
Turaga SP, Kaul S, Haritha Chowdary HA, Praveen P. Isolated bilateral basal ganglionic hyper intensities in early stage of Subacutesclerosing Panencephalitis: A case report. Neurol India [serial online] 2012 [cited 2022 May 28];60:521-2. Available from: https://www.neurologyindia.com/text.asp?2012/60/5/521/103205


Diagnosis of subacutesclerosing panencephalitis (SSPE) is based on past history of measles, typical clinical symptoms and signs, characteristic electroencephalographic (EEG) findings, and elevated serum and cerebrospinal fluid (CSF) anti-measles antibody titers. [1] Neuro-imaging findings can aid in the diagnosis in some situations. Classical magnetic resonance imaging (MRI) features in SSPE are white matter changes more posterior in location and cortical atrophy which lags behind the white matter changes. Basal ganglionic hyper intensities were described late in the disease course. [1] We report a case of SSPE in Gascon Stage 2A, [1] having isolated basalganglionic hyper intensities without cortical and subcortical changes at an early stage of disease which was not reported so often.

An 8-year-old girl born of consanguineous parentage, presented with history of sudden falls 8-10/day with sudden flexion of head and trunk without loss of consciousness of one and half month duration and poor scholastic performance and forgetfulness since 2 months. She had measles at the age of 1 year despite being vaccinated for measles. On examination she was alert but had memory and calculation deficits. She had generalized jerks involving entire body. She had no other deficits. Video EEG done showed semiperiodic burst suppression pattern [Figure 1]. CSF analysis showed no cells with normal sugar, protein, pyruvate and lactate. Anti-measles antibodies IgG were positive in CSF. MRI brain showed isolated lesions in the basal ganglion, hyper intense on T2-weighted images and hypo intense in T1-weighted images. FLAIR sequences suggested that the lesions were chronic rather than acute resolving lesions, without coexisting cortical and subcortical involvement [Figure 2]. Follow-up telephonic enquiry revealed that she had sudden death.
Figure 1: EEG showing semi- periodic complexes with R waves associated with myoclonic jerks

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Figure 2: MRI brain showing bilateral basal ganglionic hyperintensities on T2 sequence (a) without cortical and subcortical involvement and hypointense on FLAIR (b) and T1 sequences (c)

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In SSPE basal ganglionic hyper intensities along with cortical subcortical changes were seen in 35% of patients. [2],[3] An Indian study reported these changes in only 9% of patients. [4] As the disease progresses, there is increasing loss of white matter and cortical atrophy. In the final stage of SSPE, most of the white matter is lost, the ventricles and extra cerebral CSF spaces are severely widened and posterior fossa structures also show marked atrophy. Involvement of the brainstem and cortical neurons results in coma, dysautonomia and vegetative stage. [1],[2],[3],[4] Pathophysiologic basis of early involvement of basalganglia is unclear. But early death in our patient may be related to dysautonomia of last stages of SSPE. This knowledge may help in prognosticating and planning for certain tests like autonomic function tests in these patients. Another feature in our patient is that she had received regular vaccinations. Vaccination given early in life might not protect the baby from getting measles because it may be interfered with maternal antibodies.

  References Top

1.Brismar J, Gascon GG, von Steyern KV, Bohlega S. Subacutesclerosing panencephalitis: Evaluation with CT, MR. AJNR Am J Neuroradiol 1996;17:761-72.  Back to cited text no. 1
2.Akdal G, Baklan B, Cakmakçi H, Kovanlikaya A. MRI follow-up of basal ganglia involvement in subacute sclerosing panencephalitis. Pediatr Neurol 2001;24:393-5.  Back to cited text no. 2
3.Sawaishi Y, Yano T, Watanabe Y, Takada G. Migratory basal ganglia lesions in subacute sclerosing panencephalitis (SSPE): Clinical implications of axonal spread. J Neurol Sci 1999;168:137-40.  Back to cited text no. 3
4.Praveen Kumar S, Sinha S, Taly AB. The spectrum of MRI findings in subacute sclerosing panencephalitis with clinical and EEG correlates. J Pediatr Neurol 2011;9:177-85.  Back to cited text no. 4


  [Figure 1], [Figure 2]


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