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LETTER TO EDITOR
Year : 2012  |  Volume : 60  |  Issue : 6  |  Page : 656-657

Rapidly progressive SSPE masquerading as cerebral gliomatosis


1 Department of Neurological Sciences, Section of Neurology, Christian Medical College,Vellore, Tamil Nadu, India
2 Department of Radiology, Christian Medical College,Vellore, Tamil Nadu, India

Date of Web Publication29-Dec-2012

Correspondence Address:
Mathew Alexander
Department of Neurological Sciences, Section of Neurology, Christian Medical College,Vellore, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.105212

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How to cite this article:
Azad ZR, Patil AB, Sivadasan A, Mani S, Alexander M. Rapidly progressive SSPE masquerading as cerebral gliomatosis. Neurol India 2012;60:656-7

How to cite this URL:
Azad ZR, Patil AB, Sivadasan A, Mani S, Alexander M. Rapidly progressive SSPE masquerading as cerebral gliomatosis. Neurol India [serial online] 2012 [cited 2019 Dec 8];60:656-7. Available from: http://www.neurologyindia.com/text.asp?2012/60/6/656/105212


Sir,

Sub-acute sclerosing panencephalitis (SSPE) is a fatal disease of the central nervous system (CNS) related to persistent measles virus infection, usually seen in children and young adolescents. Typical neurological manifestations include progressive intellectual deterioration; behavioral changes; myoclonic jerks; and other type of seizures, pyramidal, and extra-pyramidal features. [1] This report presents a case of SSPE atypical features, occipital lobe seizures, and extensive unilateral hemispheric lesions on neuroimaging.

A 19-year-old boy presented with recurrent visual symptoms of 3 months' duration. The visual phenomena included flashes of light and distortion of images lasting 1-2 min and occurring two to three times a week. Magnetic resonance imaging (MRI) of the brain [Figure 1] done 3 months into the illness showed a T2-weighted hyper-intensive lesion involving the gray and white matter of the right parieto-occipital region, with swelling, sulcal effacement, and mild gyriform enhancement. There was extension into the splenium of the corpus callosum [Figure 1]a-f. MR spectroscopy showed a remarkable choline peak and a high Ch/Cr ratio. With these MRI findings a presumptive diagnosis of cerebral gliomatosis was considered and a stereotactic biopsy was planned. During this period he developed rapidly progressive gait and limb incoordination, generalized rigidity, and myoclonic jerks. In view of rapid progression with added neurologic features, SSPE was considered as one of the differentials. Cerebrospinal fluid (CSF) showed 6 cells/mm 3 (all lymphocytes), 30 mg% proteins, and 59 mg% glucose. Serum/CSF measles IgG titer ratio was significant (20; serum/CSF measles IgG titer ratio <64 is considered significant in our laboratory). Electroencephalogram (EEG) showed paroxysmal bursts of bilateral slow wave complexes every 3-5 s occurring synchronously with the myoclonic jerks [Figure 2].
Figure 1: T2 axial (a), Fluid-attenuated inversion-recovery (FLAIR) (b), and T2 coronal (c) images (arrow) showing hyper-intensity in the right parietooccipital lobe extending into the temporal lobe with sulcal effacement. Contiguous hyper-intensity crossing over into the splenium of the corpus callosum was also seen (b, arrowhead). There was no restriction on diffusion images (d,e). Post-gadolinium image (f) showed mild peripheral enhancement

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Figure 2: EEG showing periodic bursts of slow wave complexes occurring bilaterally at interval of 4 s and lasting for a period of around 1 s

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Classically SSPE has four characteristic stages of evolution. [2] Progression through these stages may be variable. [3] Our patient had occipital lobe seizures as the initial presentation 3 months before the onset of other characteristic clinical manifestation of SSPE. In SSPE, brain imaging usually shows bilateral parieto-occipital subcortical, periventricular white matter and deep gray nuclei involvement, with brainstem involvement in few cases. [4],[5],[6] MRI can be normal, show asymmetric changes in the early stages and may not correlate with the clinical stages in all cases with SSPE. [4],[5],[6] In our patient, MRI of brain showed a large right parieto-occipital T2-weighted hyper-intensity lesion crossing over to the other side across the splenium. Splenium has dense myelinated white matter tracts, relative to the adjacent hemispheric white matter. This acts as a barrier against the spread of interstitial edema and tumor cells, so only aggressive tumors (glioblastoma multiforme and lymphoma) and de-myelination typically cross or involve the corpus callosum. Our patient had splenium changes, which is very rare in SSPE and reported only with bilateral lesions. Our patient also had peripheral enhancement and parenchymal swelling with mass effect, which is also an uncommon radiological feature of SSPE. The subsequent rapid development of myoclonic jerks, extra-pyramidal, and cerebellar features helped in suspecting SSPE as a possibility and obviated the need for brain biopsy. SSPE can present with atypical clinical and neuroimaging features, especially with rapid onset, which could mimic a high-grade tumor. Our case highlights the importance of the clinical conundrum of SSPE and the lack of radiological correlation.





The authors acknowledge the contribution of Dr. Sanjit Aaron, Dr. Maya Thomas and Dr. Vivek Mathew during preparation of this manuscript.

 
 » References Top

1.Garg RK. Subacute sclerosing panencephalitis. J Neurol 2008;255:1861-71.  Back to cited text no. 1
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2.Jabbour JT, Garcia JH, Lemmi H, Ragland J, Duenas DA, Sever JL. Subacute sclerosing panencephalitis. JAMA 1969;207:2248-54.  Back to cited text no. 2
[PUBMED]    
3.Risk WS, Haddad FS. The variable natural history of sub acute sclerosing panencephalitis: A study of 118 cases from the Middle East. Arch Neurol 1979;36:610-4.  Back to cited text no. 3
[PUBMED]    
4.Tuncay R, Akman-Demir G, Gokygit A, Eraksoy M, Barlas M, Tolun R, et al. MRI in subacute sclerosing panencephalitis. Neuroradiology 1996;38:636-40.  Back to cited text no. 4
    
5.Brismar J, Gascon GG, von Steyern KV, Bohlega S. Subacute sclerosing panencephalitis: Evaluation with CT and MR. AJNR Am J Neuroradiol 1996;17:761-72.  Back to cited text no. 5
[PUBMED]    
6.Ozturk A, Gurses C, Baykan B, Gokyigit A, Eraksoy M. Subacute sclerosing panencephalitis: Clinical and magnetic resonance imaging evaluation of 36 patients. J Child Neurol 2002;17:25-9.  Back to cited text no. 6
    


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