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Abstract

Objectives: Subacute sclerosing panencephalitis (SSPE) is rare chronic, progressive encephalitis that affects primarily children and young adults, caused by a persistent infection with measles virus. No cure for SSPE exists, but the condition can be managed by medication if treatment is started at an early stage. Methods and results: Heterogeneity of imaging findings in SSPE is not very uncommon. But pial and gyral enhancements are very rarely noticed. Significant asymmetric onset as well as pial-gyral enhancements is not reported. Herein we present a case of 16 years adolescent of SSPE having remarkable asymmetric pial-gyral enhancements, which were misinterpreted as tubercular infection. Conclusion: Early diagnosis and treatment is encouraging in SSPE, although it is not curable with current therapy. Clinico-radiological and electrophysiological correlation is very important in diagnosis of SSPE, more gravely in patients having atypical image findings as in our index case.

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... Das et al reported adolescents of SSPE having remarkable asymmetric pialgyral enhancements, which were misinterpreted as tubercular infection. 20 Hasirci reported late development of occipital hyperintensities after 3 months of the onset of visual symptoms, along with multilocular narrowing of medium and small vessels on diffusion subtraction angiography while our two patients with visual loss had occipital hyperintense signals right at presentation. 21 Vilas et al, reported diffuse demyelinating leukoencephalopathy among atypical presentations of SSPE while we had 4.3% patients of each focal white matter and occipital hyperintensities. ...
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Objective: To determine the variations in clinical presentation, neuroimaging and electroencephalography patterns of subacute sclerosing panencephalitis. Study Design: Cross-sectional study. Place and Duration of Study: Children's Hospital & Institute of Child Health, Lahore, Pakistan, from Jul to Dec 2020. Methodology: We recruited children presented with clinical features suggestive of subacute sclerosing panencephalitis, along with positive anti-measles antibodies on cerebrospinal fluid. Association between variables was determined to formulate an early diagnosis of subacute sclerosing panencephalitis. Results: Out of 47 children, 29 were males with a mean age of 6.54 ± 2.9 years. Only 23% were fully immunized against measles, 36.2% were unvaccinated and 40.4% received partial immunization. The mean age of measles infection was 1.49 ± 1.2 years; the mean interval between measles and onset of SSPE was 4.13 ± 3 years. Atypical clinical presentation was seen in 38.3% with intractable epilepsy (8.5%), focal deficit (8.5%) and extrapyramidal symptoms (8.5%) being commonest followed by coma (6.4%), visual loss (4.3%) and psychosis (2.1%). Neuroimaging was suggestive of cortical hyperintensities in 46.8% and was normal in 46.8%. Electroencephalography showed burst suppression in 55.3% and atypical findings in 19.1%. Younger age (1-1.5 years) of measles and unimmunized status were associated with early onset of SSPE with a p-value of 0.001 and 0.05 respectively. Non-immunized status was associated with atypical presentation of SSPE (p-value <0.05). Conclusion: The younger age of measles infection and failure to receive complete immunization led to early onset of Subacute sclerosing panencephalitis with an atypical presentation.
... Earliest described changes are T2 white-matter hyperintensities. Asymmetric involvement being common [26]. Basal ganglia changes and cortical gray matter changes were seen in a third and fourth of cases in a study, respectively [27]. ...
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A 7-year-old child who suffered from symptomatic focal epilepsy as a sequel to perinatal hypoxia used to have frequent seizures. This time she developed prolonged status epilepticus lasting for over 5 hours. She received a treatment in the form of intravenous midazolam and reinitiation of sodium valproate and clobazam that were discontinued previously. Seizures were controlled over a couple of hours, but she remained unresponsive. Later, she developed acute onset dystonia (day 3 post-status epilepticus) and also myoclonic jerks. She presented to us after 3 weeks of onset of these complaints and we considered hypoxic encephalopathy resulting from prolonged status epilepticus or acute encephalitis or non-convulsive status epilepticus. However, acute onset dystonia and periodicity of myoclonic jerks were pointers against it, and on evaluation, she was diagnosed with atypical fulminant subacute sclerosing panencephalitis (SSPE). Knowing the atypical presentations of SSPE is important in planning management and prognostication.
... Stages 3 and 4 involve progressive unresponsiveness leading to coma, autonomic dysregulation, and death [42]. MRI findings are variable and include decreased gray matter volume [43], diffuse symmetric white matter changes, subcortical and juxtacortical T2 hyperintensities [44], pial-gyral enhancement [45], or hyperintensities in the basal ganglia [46]. Involvement of optic nerves, brainstem, and spinal cord could resemble NMOSD [47,48]. ...
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Purpose of Review Pediatric central nervous system demyelinating diseases include multiple sclerosis (MS), neuromyelitis optica spectrum disorder (NMOSD), and acute disseminated encephalomyelitis (ADEM). As diagnostic criteria become more inclusive, the risk of misdiagnosis of atypical demyelinating diseases of rheumatologic, infectious, and autoimmune etiology increases. Recent Findings We review mimics of multiple sclerosis, neuromyelitis optica spectrum disorder, and acute disseminated encephalomyelitis, including rheumatologic diseases: systemic lupus erythematosus and neuro-Behçet disease; infectious diseases: human immunodeficiency virus, progressive multifocal leukoencephalopathy, and subacute sclerosis panencephalitis; and autoimmune diseases including X-linked Charcot-Marie-Tooth disease, chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) and autoimmune glial fibrillary acidic protein (GFAP) encephalopathy. Summary Atypical demyelinating disease may mimic classic neuroinflammatory diseases of the central nervous system. Imaging may meet criteria for a diagnosis of multiple sclerosis, or patients may present with optic neuritis and transverse myelitis consistent with neuromyelitis optica spectrum or myelin oligodendrocyte glycoprotein (MOG) antibody disorders. Through careful history-taking and review of atypical MRI findings, we may avoid misdiagnosis and mistreatment.
... They can range from hyperintensities in the parietal and occipital region, brain stem lesions, and marked atrophy, causing prominence of sulci. Diagnosis is usually done on the basis of MRI findings, electroencephalogram (EEG) findings, and the detection of anti-measles IgG antibodies in the cerebrospinal fluid (CSF) or serum [4]. ...
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Subacute Sclerosing Panencephalitis (SSPE) is a debilitating disorder associated with the measles infection in childhood. It is a very rare manifestation in children. It usually presents with measles before the age of two. We report a similar case of SSPE in a 14-year-old girl who developed this life-threatening condition in spite of receiving the measles vaccination. Despite the vaccination, the patient had suffered from measles before the age of two. This highlights the dilemma of ineffective vaccinations in developing countries. We also describe the radiologic features of SSPE in this patient, with marked atrophy seen in the occipital region following hyperintensities noticed at a relatively earlier stage.
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To evaluate the progression of CT and MR changes of the brain in subacute sclerosing panencephalitis (SSPE) as a basis for assessing the effects of different types of therapy. Fifty-two patients with SSPE were examined, 44 with MR imaging and 42 with CT of the brain on one or more occasions. A total of 92 MR and 67 CT studies were performed. Correlation between the clinical status and the MR findings in admission was poor. Of 20 patients with clinically advanced disease, only 8 had marked MR abnormalities; 6 had normal or almost normal findings on MR examinations. Two of 4 patients with clinically mild disease had advanced MR changes. The progression of the MR findings appeared to follow a constant pattern. The earliest pathologic finding was focal, high-T2-intensity white matter changes; later atrophic changes followed. The atrophy lagged behind the white matter changes and was thus mild when white matter changes were moderate or severe. In the most advanced stage, when the patient was in a neurovegetative state, an almost total loss of white matter had usually taken place. At this stage, the corpus callosum was also thin. Basal ganglia changes, usually involving the putamina, were seen in one third of patients and cortical gray matter changes were seen in one fourth of patients examined with MR imaging. In 2 of 20 patients, MR changes regressed in parallel with clinical improvement following therapy, but in 5 patients clinical improvement was accompanied by progression of MR changes. The progress of MR abnormalities seen in patients with SSPE seems to follow a constant pattern, but the severity of MR changes does not always correlate well with the clinical findings. Caution must therefore be used when evaluating the effects of therapy.
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Subacute sclerosing panencephalitis (SSPE) is a progressive neurological disorder of childhood and early adolescence. It is caused by persistent defective measles virus. Brain biopsies or postmortem histopathological examination show evidence of astrogliosis, neuronal loss, degeneration of dendrites, demyelination, neurofibrillary tangles, and infiltration of inflammatory cells. Patients usually have behavioral changes, myoclonus, dementia, visual disturbances, and pyramidal and extrapyramidal signs. The disease has a gradual progressive course leading to death within 1-3 years. The diagnosis is based upon characteristic clinical manifestations, the presence of characteristic periodic EEG discharges, and demonstration of raised antibody titre against measles in the plasma and cerebrospinal fluid. Treatment for SSPE is still undetermined. A combination of oral isoprinosine (Inosiplex) and intraventricular interferon alfa appears to be the best effective treatment. Patients responding to treatment need to receive it life long. Effective immunisation against measles is the only solution presently available to the problem of this dreaded disease.
Article
The clinical features and outcome of disease in 14 cases of subacute sclerosing panencephalitis (SSPE) diagnosed at the King Khalid University Hospital, Riyadh during an 8-year period are similar to those described elsewhere. Therapy was associated with arrest of deterioration for 2.5 years in one patient, and with survival after diagnosis for 2-7 years in four others. Many of the cases had initial misdiagnoses because of the frequently bizarre modes of presentation. It is thought that many more cases of SSPE occur in Saudi Arabia and also in many other tropical countries than are currently recognized. The establishment of national SSPE registries is advocated to improve early identification and management of cases.
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Serial MRI was performed on a 15 year old girl with Subacute Sclerosing Pan-encephalitis (SSPE]. After a period of remission she entered a phase of progressive deterioration. A repeat MRI showed significant resolution of the previous abnormalities. Her pathology and MRI scans are discussed.
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Twenty-four computed tomographic scans of 12 patients with confirmed subacute sclerosing panencephalitis were studied using standardized techniques of radiological assessment. Abnormalities encountered were of four types--(1) lateral ventricular dilatation, (2) cerebral cortical atrophy and sylvian fissure widening, (3) low parenchymal attenuation, and (4) brainstem atrophy and cerebellar atrophy--and of varying degrees. The abnormalities correlated best with the stage and duration of disease, but not necessarily well with the patient's mental state. The fewest radiological abnormalities were encountered in the acute or early stages, whereas more signs of parenchymal disturbances in the form of low attenuations emerged during intermediate periods. Chronic periods were accompanied by atrophic changes in the form of cortical atrophy, ventricular dilatation, and brainstem cerebellar atrophy.
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Thirty-four MRI studies of 26 patients with subacute sclerosing panencephalitis are reported. Lesions of high signal intensity on T 2-weighted images are the most common finding; they frequently involve the periventricular or subcortical white matter. Lesions tend to start in the cortex-subcortical white matter and progress with periventricular white matter involvement and diffuse cerebral atrophy. Pial and parenchymal contrast enhancement, local mass effect of parenchymal lesions, and involvement of the splenic portion of the corpus callosum are not infrequent. Basal ganglia and brainstem lesions were rare in this series. Although cortical and subcortical lesions have some correlation with clinical findings, the extent and location of the periventricular white matter lesions and cerebral atrophy did not reflect the neurologic status in many patients. NEUROLOGY 1996;47: 1278-1283
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Subacute sclerosing panencephalitis (SSPE) is a progressive, slow virus infection of the brain, caused by the measles virus, attacking children and young adults. We investigated 15 patients with SSPE by MRI, with 5 normal and 10 pathological results. In the early period, lesions were in the grey matter and subcortical white matter. They were asymmetrical and had a predilection for the posterior parts of the hemispheres. Later, high-signal changes in deep white matter and severe cerebral atrophy were observed. Parenchymal lesions significantly correlated with the duration of disease. A significant relationship between MRI findings and clinical stage was observed in the 1st year of the disease.
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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.