FoIia Psychiatrica et Neurologica Japonica, Vol. 33, No.2, 1979
Computed Tomographic Findings in Subacute Sclerosing Panencephalitis Ryosuke Murata, M.D., Kunio Fujii, M.D.* and Ryozo Yoshida, M.D.** Department of Pediatrics, Osaka City University, Osaka *Department of Pediatrics, Osaka Rosai Hospital, Sakai **Department of Pediatrics, Osaka Medical College, Takatsuki
INTRODUCTION
Even since subacute sclerosing panencephalitis (SSPE) was found to be a slow viral infection caused by the measles virus, extensive studies on the disease have been made from the pathological, immunological and virological points of view. Especially, intense interest has developed on the onsetmechanism of the disease to suggest some hypothesis. On the contrary, the disease displays a typical characteristic time-course of symptoms, so that, clinically, it has been divided into three stages by Freeman3 and four stages by J a b b ~ u r .In ~ the typical clinical course of this disease, intellectual deterioration, myoclonic seizures and characteristic findings in the electroencephalogram (EEG) are found at the early stage.6 Then, neurological impairment of pyramidal/extrapyramidal tracts follow, and finally, a severe loss of cerebral function leads to death. Establishment of the diagnosis has been carried out by observing the clinical timecourse of symptoms, characteristic EEG findings and elevated antibody titer of the measles virus in both serum and spinal fluid. Computed tomography (CT) has emerged as a safe, noninvasive method for evaluating patients with central nervous system diseases. ____
Received for publication Feb. 5 , 1979.
This communication presents our experiences in which the CI' may help in establishing the SSPE diagnosis and prognosis. The scanner used is the Hitachi CT-H 250. CASE REPORT
A 3-year-1 0-month-old girl
She was required to undergo medical examination following a complaint that she often fell down while walking. Growth after birth was normal. She began to walk without help at the age of one year and four months. She suffered from measles 10 months after birth, but the prognosis was favorable. The onset of myoclonic seizures occurred during the first 10 days of April 1977, and the seizures increased to 50 times a day so that finally she had difficulty in walking. Generalized two to four high voltage delta waves were repeated at intervals of 5-7 sec on the EEG pattern at the first medical examination. By administrating anticonvulsants, the symptoms of tumbling down lessened, but slowness of action/speaking became rather conspicuous. During the first 10 days of June, tremor of the right hand was often observed when grasping something and then, the hand gradually ceased to move. On 17th June, due to the right hemi-
154
R. Murata
ct ai.
Fig. 1: There are bilateral lowdensity areas without contrast enhancement or mass effect.
Fig. 2: Severe cerebral atrophy
plegia and walking difficulty, she was hospitalized. After entering the hospital, disturbance of consciousness became much worse, and difficulty in oral feeding, involuntary movement of the limbs as well as myoclonic seizures of the whole body were observed. During the middle of July, the symptoms became much worse and reached the Freeman's third stage. A trial of simultaneous administration of steroids, anticonvulsants and L-DOPA brought a decrease in the involuntary movement as well as the sudden flexion movements of the extremities, head and trunk without any advance in the symptoms. A plain CT scan on entering the hospital (Fig. 1 ) revealed a low density area in the left temporal, parietal, occipital and right occipital lobes. The second week following the plain CT, a contrast enhanced CT scan revealed a wide lesion of low density in the same area mentioned above, but displacement of the midline as well as enlargement of the ventricles were not observed. The antibody titer of complement fixation (CF) to measles virus in serum was 2,048 times as high as that of normal, and in the cerebrospinal
fluid 32 times as high as that of normal. Moreover, in the protein fraction of cerebrospinal fluid, the y-globulin level increased extremely to 34.3%. We established the diagnosis as SSPE from the clinical findings and the CF mentioned above. For three and a half months after entering the hospital, the symptoms remained unchanged. In the CT scan findings in March, 1978 ( 1 1 months after the onset), an enlargement of the ventricles, as shown in Fig. 2, was observed with marked atrophic lesions. The EEG pattern was hypsarrhythmia. DISCUSSION
Few reports on CT findings of SSPE have been presented. Following the report by Onoda et al." on three cases, the characteristic CT findings corresponding precisely to Freeman's III-A/III-B stages have been the wide enlargement of the ventricles with the mediated grade enlargement of the ventricle, and the obvious atrophic picture of irregular border, of variable in size and spread in the cerebral white matter of the frontal, parietal, temporal and occipital lobes. O n
CT Findings in Subacute Sclerosing Panencephalitis the contrary, in the case of an acute course leading to death, only a slight low density area had been found in the CT scan made four months after the onset. With respect to our case, the low density areas were found in the left parietal, temporal and occipital lobes by the plain/contrast enhanced CT scan corresponding to Freeman’s 11-A/II-B stages, but not the mass effects. The marked uneven distribution of the low density area on each side of lobe, clinically corresponded to the neuropathological findings such as right hemiplegia and right side Babinski reflex. In the CT scan at giving remission to the 11-B stage after the progression to Freeman’s stage 111, the same atrophic picture was observed with the mediated enlargement of the third ventricle and lateral ventricle as well as expansion of the cerebral sulci and longitudinal cerebral fissure as mentioned in the report by Onoda el al. Because brain lesions such as necrosis, edema, cystic formation, accumulation of lipids, demyelination and so on have often brought about areas of decreased radiodensity in the lobes observed by CT, CT may be very useful for establishing the diagnoses of demyelinating7 and inflammatory diseases.l The onset of SSPE is found in the posterior cerebral regions, especially the parieto-occipital and posterotemporal areas with further progress in the cerebellum, brain stem and upper cervical cord. Microscopical examination reveals inflammatory changes in the gray and white matter with perivascular lymphocytes and plasma cells, as well as astrocytic proliferation in relation to areas of neuronal and fiber damage. Inclusions are seen with both the nucleus and cytoplasma of neurons and glial cells. Demyelinatioii is particularly evident in the more chronic cases. With further progression of the stages, a much wider portion of the cerebral white matter was destroyed, decreasing the volume of brain due to changes in the tissue. Consequently, an atrophic picture of the cerebrum was thought to be produced.
155
The slow viral infection also leads to the serious underlying disease-to the onset of progressive multifocal leukoencephalopathy (PML). In PML, many demyelination foci of some variance in size are formed to fuse into a much wider area. The low density area in the lobes is also a characteristic of PML observed by CT,?designating similar CT patterns to that of the SSPE without the midline displacement. The CT picture of SSPE may resemble those seen in brain tumor, brain abscess, encephalitis and demyelinating diseases. On the other hand, there are many atrophic cerebral diseases, so that a definite diagnosis of SSPE should not be performed only by observing the clinical/CT findings. Consequently, several examinations in expectation of finding many diseases are naturally required. In this SSPE case that we experienced, we observed that areas of decreased density were found in the lobes of which parietal/ occipital took a leading part at Freeman’s stage 11, and that the atrophic picture was found by CT scan at Freeman’s stage 111. Moreover, failure of the lesion to become enhanced with the contrast material on the CT scan may aid diagnosis of SSPE. Experienced findings are believed to be available for establishing the diagnosis of SSPE as well as for judgment of its prognosis. SUMMARY We used a computed tomography to look for evidence of cerebral changes in a three-year-10-month-old girl with SSPE. The area of decreased density on CT corresponded to inflammatory and demyelinative lesions in the brain. CT scans seem to be a useful tool for more accurate assessment in the diagnosis of SSPE. REFERENCES 1 Claveria, L. E., Boulay, G. H. and Moseley, I. F. : Intracranial infection: investigation by computerized axial tomography, Neuro-
radiology, 12: 59-71, 1976.
156
k. Murata
2 Conomy, J. P., Weinstein, M. A., Agamanolis, D. and Holt, W.S.: Computed tomography in progressive multifocal leukoencephalopathy, Am J Roentgenol, 127: 663665, 1976. 3 Freeman, J. M.: The clinical spectrum and early diagnosis of Dawson’s encephalitis, J Pediat, 75: 590-603, 1969. 4 Jabbour, J. T., Garcia, J., Lemmi, H., Ragland, J., Duenas, D. A. and Sever, J. L.: Subacute sclerosing panencephalitis: a multidisciplinary study of eight cases, JAMA, 207: 2248-2254, 1969.
et af.
5 Markand, 0. N. and Panszi, J. G.: The
electroencephalogram in subacute sclerosing panencephalitis, Arch Neurol, 32: 719726, 1975. 6 Onoda, M., Takahashi, A., Shiozawa, Z. and Sobue, I.: A clinical evaluation of computed tomography in subacute sclerosing panencephalitis, Neurol Med, 8: 84-87, 1978 (in Japanese). 7 Robertson, W. C., Gomez, M. R., Reese, D. F. and Okazaki, H.: Computerized tomography in demyelinating disease of the young, Neurology, 27: 838-842, 1977.
Computed Tomographic Findings in Subacute Sclerosing Panencephalitis Ryosuke Murata, M.D., Kunio Fujii, M.D.* and Ryozo Yoshida, M.D.** Department of Pediatrics, Osaka City University, Osaka *Department of Pediatrics, Osaka Rosai Hospital, Sakai **Department of Pediatrics, Osaka Medical College, Takatsuki
INTRODUCTION
Even since subacute sclerosing panencephalitis (SSPE) was found to be a slow viral infection caused by the measles virus, extensive studies on the disease have been made from the pathological, immunological and virological points of view. Especially, intense interest has developed on the onsetmechanism of the disease to suggest some hypothesis. On the contrary, the disease displays a typical characteristic time-course of symptoms, so that, clinically, it has been divided into three stages by Freeman3 and four stages by J a b b ~ u r .In ~ the typical clinical course of this disease, intellectual deterioration, myoclonic seizures and characteristic findings in the electroencephalogram (EEG) are found at the early stage.6 Then, neurological impairment of pyramidal/extrapyramidal tracts follow, and finally, a severe loss of cerebral function leads to death. Establishment of the diagnosis has been carried out by observing the clinical timecourse of symptoms, characteristic EEG findings and elevated antibody titer of the measles virus in both serum and spinal fluid. Computed tomography (CT) has emerged as a safe, noninvasive method for evaluating patients with central nervous system diseases. ____
Received for publication Feb. 5 , 1979.
This communication presents our experiences in which the CI' may help in establishing the SSPE diagnosis and prognosis. The scanner used is the Hitachi CT-H 250. CASE REPORT
A 3-year-1 0-month-old girl
She was required to undergo medical examination following a complaint that she often fell down while walking. Growth after birth was normal. She began to walk without help at the age of one year and four months. She suffered from measles 10 months after birth, but the prognosis was favorable. The onset of myoclonic seizures occurred during the first 10 days of April 1977, and the seizures increased to 50 times a day so that finally she had difficulty in walking. Generalized two to four high voltage delta waves were repeated at intervals of 5-7 sec on the EEG pattern at the first medical examination. By administrating anticonvulsants, the symptoms of tumbling down lessened, but slowness of action/speaking became rather conspicuous. During the first 10 days of June, tremor of the right hand was often observed when grasping something and then, the hand gradually ceased to move. On 17th June, due to the right hemi-
154
R. Murata
ct ai.
Fig. 1: There are bilateral lowdensity areas without contrast enhancement or mass effect.
Fig. 2: Severe cerebral atrophy
plegia and walking difficulty, she was hospitalized. After entering the hospital, disturbance of consciousness became much worse, and difficulty in oral feeding, involuntary movement of the limbs as well as myoclonic seizures of the whole body were observed. During the middle of July, the symptoms became much worse and reached the Freeman's third stage. A trial of simultaneous administration of steroids, anticonvulsants and L-DOPA brought a decrease in the involuntary movement as well as the sudden flexion movements of the extremities, head and trunk without any advance in the symptoms. A plain CT scan on entering the hospital (Fig. 1 ) revealed a low density area in the left temporal, parietal, occipital and right occipital lobes. The second week following the plain CT, a contrast enhanced CT scan revealed a wide lesion of low density in the same area mentioned above, but displacement of the midline as well as enlargement of the ventricles were not observed. The antibody titer of complement fixation (CF) to measles virus in serum was 2,048 times as high as that of normal, and in the cerebrospinal
fluid 32 times as high as that of normal. Moreover, in the protein fraction of cerebrospinal fluid, the y-globulin level increased extremely to 34.3%. We established the diagnosis as SSPE from the clinical findings and the CF mentioned above. For three and a half months after entering the hospital, the symptoms remained unchanged. In the CT scan findings in March, 1978 ( 1 1 months after the onset), an enlargement of the ventricles, as shown in Fig. 2, was observed with marked atrophic lesions. The EEG pattern was hypsarrhythmia. DISCUSSION
Few reports on CT findings of SSPE have been presented. Following the report by Onoda et al." on three cases, the characteristic CT findings corresponding precisely to Freeman's III-A/III-B stages have been the wide enlargement of the ventricles with the mediated grade enlargement of the ventricle, and the obvious atrophic picture of irregular border, of variable in size and spread in the cerebral white matter of the frontal, parietal, temporal and occipital lobes. O n
CT Findings in Subacute Sclerosing Panencephalitis the contrary, in the case of an acute course leading to death, only a slight low density area had been found in the CT scan made four months after the onset. With respect to our case, the low density areas were found in the left parietal, temporal and occipital lobes by the plain/contrast enhanced CT scan corresponding to Freeman’s 11-A/II-B stages, but not the mass effects. The marked uneven distribution of the low density area on each side of lobe, clinically corresponded to the neuropathological findings such as right hemiplegia and right side Babinski reflex. In the CT scan at giving remission to the 11-B stage after the progression to Freeman’s stage 111, the same atrophic picture was observed with the mediated enlargement of the third ventricle and lateral ventricle as well as expansion of the cerebral sulci and longitudinal cerebral fissure as mentioned in the report by Onoda el al. Because brain lesions such as necrosis, edema, cystic formation, accumulation of lipids, demyelination and so on have often brought about areas of decreased radiodensity in the lobes observed by CT, CT may be very useful for establishing the diagnoses of demyelinating7 and inflammatory diseases.l The onset of SSPE is found in the posterior cerebral regions, especially the parieto-occipital and posterotemporal areas with further progress in the cerebellum, brain stem and upper cervical cord. Microscopical examination reveals inflammatory changes in the gray and white matter with perivascular lymphocytes and plasma cells, as well as astrocytic proliferation in relation to areas of neuronal and fiber damage. Inclusions are seen with both the nucleus and cytoplasma of neurons and glial cells. Demyelinatioii is particularly evident in the more chronic cases. With further progression of the stages, a much wider portion of the cerebral white matter was destroyed, decreasing the volume of brain due to changes in the tissue. Consequently, an atrophic picture of the cerebrum was thought to be produced.
155
The slow viral infection also leads to the serious underlying disease-to the onset of progressive multifocal leukoencephalopathy (PML). In PML, many demyelination foci of some variance in size are formed to fuse into a much wider area. The low density area in the lobes is also a characteristic of PML observed by CT,?designating similar CT patterns to that of the SSPE without the midline displacement. The CT picture of SSPE may resemble those seen in brain tumor, brain abscess, encephalitis and demyelinating diseases. On the other hand, there are many atrophic cerebral diseases, so that a definite diagnosis of SSPE should not be performed only by observing the clinical/CT findings. Consequently, several examinations in expectation of finding many diseases are naturally required. In this SSPE case that we experienced, we observed that areas of decreased density were found in the lobes of which parietal/ occipital took a leading part at Freeman’s stage 11, and that the atrophic picture was found by CT scan at Freeman’s stage 111. Moreover, failure of the lesion to become enhanced with the contrast material on the CT scan may aid diagnosis of SSPE. Experienced findings are believed to be available for establishing the diagnosis of SSPE as well as for judgment of its prognosis. SUMMARY We used a computed tomography to look for evidence of cerebral changes in a three-year-10-month-old girl with SSPE. The area of decreased density on CT corresponded to inflammatory and demyelinative lesions in the brain. CT scans seem to be a useful tool for more accurate assessment in the diagnosis of SSPE. REFERENCES 1 Claveria, L. E., Boulay, G. H. and Moseley, I. F. : Intracranial infection: investigation by computerized axial tomography, Neuro-
radiology, 12: 59-71, 1976.
156
k. Murata
2 Conomy, J. P., Weinstein, M. A., Agamanolis, D. and Holt, W.S.: Computed tomography in progressive multifocal leukoencephalopathy, Am J Roentgenol, 127: 663665, 1976. 3 Freeman, J. M.: The clinical spectrum and early diagnosis of Dawson’s encephalitis, J Pediat, 75: 590-603, 1969. 4 Jabbour, J. T., Garcia, J., Lemmi, H., Ragland, J., Duenas, D. A. and Sever, J. L.: Subacute sclerosing panencephalitis: a multidisciplinary study of eight cases, JAMA, 207: 2248-2254, 1969.
et af.
5 Markand, 0. N. and Panszi, J. G.: The
electroencephalogram in subacute sclerosing panencephalitis, Arch Neurol, 32: 719726, 1975. 6 Onoda, M., Takahashi, A., Shiozawa, Z. and Sobue, I.: A clinical evaluation of computed tomography in subacute sclerosing panencephalitis, Neurol Med, 8: 84-87, 1978 (in Japanese). 7 Robertson, W. C., Gomez, M. R., Reese, D. F. and Okazaki, H.: Computerized tomography in demyelinating disease of the young, Neurology, 27: 838-842, 1977.
