Atormac
Neurology India
menu-bar5 Open access journal indexed with Index Medicus
  Users online: 1387  
 Home | Login 
About Editorial board Articlesmenu-bullet NSI Publicationsmenu-bullet Search Instructions Online Submission Subscribe Videos Etcetera Contact
  Navigate Here 
 Search
 
  » Next article
  » Previous article 
  » Table of Contents
  
 Resource Links
  »  Similar in PUBMED
 »  Search Pubmed for
 »  Search in Google Scholar for
 »Related articles
  »  [PDF Not available] *
  »  Citation Manager
  »  Access Statistics
  »  Reader Comments
  »  Email Alert *
  »  Add to My List *
* Registration required (free)  


  In this Article
 »  Abstract
 »  Introduction
 »  Case report
 »  Discussion
 »  References

 Article Access Statistics
    Viewed8696    
    Printed179    
    Emailed12    
    PDF Downloaded0    
    Comments [Add]    
    Cited by others 7    

Recommend this journal

   
Year : 1999  |  Volume : 47  |  Issue : 4  |  Page : 304-7

Subacute sclerosing panencephalitis : CT and MR imaging in a rapidly progressive case.


Departments of Neurosciences, Radiodiagnosis and Pathology, Christian Medical College and Hospital, Vellore, Tamilnadu, 632004, India.

Correspondence Address:
Departments of Neurosciences, Radiodiagnosis and Pathology, Christian Medical College and Hospital, Vellore, Tamilnadu, 632004, India.

  »  Abstract

We report the findings on CT and MR imaging in a patient with rapidly progressive subacute sclerosing panencephalitis (SSPE), which correlated with the clinical progression of the disease. In view of the rapid neurological deterioration and CSF pleocytosis, a brain biopsy was done and this confirmed the diagnosis.

How to cite this article:
Alexander M, Singh S, Gnanamuthu C, Chandi S, Korah I P. Subacute sclerosing panencephalitis : CT and MR imaging in a rapidly progressive case. Neurol India 1999;47:304


How to cite this URL:
Alexander M, Singh S, Gnanamuthu C, Chandi S, Korah I P. Subacute sclerosing panencephalitis : CT and MR imaging in a rapidly progressive case. Neurol India [serial online] 1999 [cited 2019 Aug 18];47:304. Available from: http://www.neurologyindia.com/text.asp?1999/47/4/304/1584




   »   Introduction Top


SSPE is a slowly progressive disorder that is typically seen in children and adolescents with an invariably fatal outcome. It is a unique slow viral disease in which the measles virus has been identified as the pathogen. The usual age of onset is between 5-12 years.[1],[2],[3],[4] The natural history of SSPE is characterized by a highly variable temporal course ranging from an almost acute onset with rapid progression and death within a few weeks, to a protracted course after several years with phases of stabilization and even improvement.[3],[4],[5] The diagnosis is usually established by the clinical manifestation, presence of abnormal complexes on EEG, laboratory findings including high levels of CSF gamma globulin, and elevated titer of measles antibody in the CSF and serum.[1],[2],[3],[4],[5],[6],[7] Generally, CT and MR imaging characteristics do not correlate with progression of the disease.[6],[7],[8] Our patient showed a rapid radiological evolution that correlated with the progression of the disease.


   »   Case report Top


A 17-year-old male presented with a rapidly progressive neurological syndrome characterized by spasticity, very infrequent myoclonic jerks and dystonic posturing of the right upper limb of 1 month's duration and three episodes of generalized seizures. There was no history of measles in childhood, nor was he immunized against the disease. He was stuporous with a Glasgow Coma Scale of 10/15 (E4 V1 M5). Measles antibody titer revealed a Serum: CSF ratio of 40:1, which was suggestive of SSPE. The other antibody titers were unremarkable. CT imaging of the brain after 1 week into the illness was unremarkable [Figure. 1], however MR imaging revealed ill-defined slightly high signal intensity areas on T2-weighted images in the periventricular white matter and centrum semiovale bilaterally, predominantly involving the parietal lobes [Figure. 2]. On proton density weighted images, there was slight effacement of the gray-white matter definition. On T1-weighted images however, these lesions were not identified. EEG revealed periodic complexes and CSF showed lymphocytic pleocytosis (40 cells) with normal sugar and protein levels. While in the hospital he developed severe dystonia of all four limbs, opisthotonus, seizures, and dysautonomia. A CT repeated after 1 month revealed patches of ill-defined low density lesions with isolated enhancement in the cortical and subcortical white matter of the frontal and parietal lobes bilaterally [Figure. 1B]. A few lesions were also noted in the frontal periventricular white matter and centrum semiovale. MR imaging performed at this time demonstrated diffuse large high signal intensity lesions of varied sizes in the cerebrum bilaterally, involving the cortex, subcortical, deep and periventricular white matter, basal ganglia and thalami bilaterally, on T2-weighted and proton density weighted images [Figure. 3A].
On T1-weighted images, these lesions were of low signal intensity with loss of gray-white definition and obliteration of the sulci [Figure. 3B]. Since there was rapid clinical deterioration and CSF examination showed pleocytosis, which is very unusual in SSPE, a brain biopsy was advised. Microscopic examination of the biopsied brain tissue showed moderate perivascular infiltrates of lymphocytes as well as a few neutrophils and plasma cells in the cerebral cortex and white matter associated with neuronal depletion, widespread spongiosis, and gliosis as well as rarefaction of the white matter in areas. There were intranuclear homogenous eosinophilic inclusions in several glial cells chiefly astrocytes. The pia-arachnoid was also inflamed [Figure. 4]. He was treated with intrathecal interferon, 1 million units on alternate days for 15 days, and also with isoprinosine, carbamazepine, sodium valproate, clonazepam, and pacitane.

   »   Discussion Top


SSPE is a fatal encephalitis of children and adolescents, associated with an episode of measles several years before the onset of encephalitis.[1],[2],[3],[4] Our patient had no definite past history of measles. Clinically, the classical disease takes 1 to 3 years to progress from stage I (mental and behavioral abnormalities) to stage IV (mutism, occasional myoclonus and loss of cortical function).[2],[4],[7] However, our patient rapidly deteriorated within 1 month to stage III of Risk and Haddad.[5] Correlation between radiological changes and clinical findings have been poor with normal MRI in bedridden patients or improvement despite clinical progression have been found.[7] Murata et al had reported serial MRI in a case that showed radiological improvement parallel to partial clinical improvement.[8] Brismar et al reported partial resolution of lesions inspite of clinical progression.[7] Though changes seen on CT and MRI are non-specific, MRI can be used to assess the extent of brain involvement in early stages of encephalitis as it is more sensitive. Depending on white matter changes and atrophy, Brismar et al classified radiological changes into 6 stages.[7] Neuropathological findings suggest that the disease initially affects the occipital cortex and then progresses to involve the frontal cortex and finally the subcortical white matter, brain stem, and spinal cord.[7]

As in this patient, early changes are detectable as ill-defined high signal intensity areas on T2-weighted images, more commonly in the occipital subcortical white matter than in the frontal region. In most of the cases the gray matter is spared even in advanced clinical and MRI stages.[7] In the later stage of disease high signal intensity lesions are better defined in both gray and white matter with loss of gray-white matter differentiation.[2] Cerebellar, pontine, thalamic and spinal cord involvement have been reported.[5],[7] On T1-weighted images, lesions are seen as low signal intensity.

In the early stage of the disease, the CT may be normal,[4],[7],[8] or may show slight cerebral oedema. In the later stages, however bilateral symmetrical or asymmetrical low-density enhancing or non-enhancing lesions in the cerebrum involving the gray and white matter[6],[7],[8] may be seen. Cortical atrophy is found in the advanced stages of disease.[2],[4],[6] Mass effect is not a feature in SSPE. Typically the lesions do not enhance,[2] however, our patient showed patchy enhancement on CT. Brismar et al have reported a case with rapid clinical progression which showed multiple areas of enhancement.[7] Contrast enhancement have also been reported in cases of relapse.[7] The findings in our patient concurred with this pattern.

Although measles antibody titer in serum and CSF was suggestive of SSPE in this patient, the rapid neurological deterioration and CSF pleocytosis warranted a brain biopsy to rule out other possibilities such as encephalitides of other etiologies. This case emphasizes the role of MR imaging in delineating early brain changes and their extent radiologically in SSPE, though the extent of the changes may or may not correlate clinically.

 

  »   References Top

1.Miller DH, Robb SA, Ormerod IEC et al : Magnetic resonance imaging of inflammatory and demyelinating white-matter diseases of childhood. Dev Med Child Neurol Suppl 1990; 32 : 97-107.   Back to cited text no. 1    
2.Woodward KG, Weinberg PE, Lipton HL : Basal ganglia involvement in subacute sclerosing panencephalitis: CT and MR demonstration. J Comput Assist Tomogr 1988; 12 : 489-491.   Back to cited text no. 2    
3.Prabhakar S, Alexander M : Subacute sclerosing panencephalitis. Tropical Neurology; WB Saunders Co. Ltd. 1996; 77-93.   Back to cited text no. 3    
4.Tsuchiya K, Yamauchi T, Furui S et al : MR Imaging vs CT in subacute sclerosing panencephalitis. AJNR 1988; 9 : 943-946.   Back to cited text no. 4    
5.Risk WS, Haddad FS : The variable natural history of subacute sclerosing panencephalitis. A study of 118 cases from the middle East. Arch Neurol 1979; 36 : 610-614.  Back to cited text no. 5    
6.Weiner JB, Pires M, Kermode A : Resolving MRI abnormalities with progression of subacute sclerosing panencephalitis. Neuroradiology 1991; 33 : 178-180.   Back to cited text no. 6    
7.Brismar J, Gascon GG, Von Steyern KV et al : Subacute sclerosing panencephalitis: Evaluation with CT and MR. AJNR 1996; 17 : 761-722.   Back to cited text no. 7    
8.Tuncay R, Akman-Demir G, Gokyigit A et al : MRI in subacute sclerosing panencephalitis. Neuroradiology 1996; 38 : 636-640.   Back to cited text no. 8    
9.Murata R, Mutsuoka O, Nakajima S et al : Serial magnetic resonance imaging in subacute sclerosing panencephalitis. Jpn J Psychiatri Neurol 1987; 41 : 277-282.   Back to cited text no. 9    

 

Top
Print this article  Email this article
Previous article Next article
Online since 20th March '04
Published by Wolters Kluwer - Medknow