Diagnosis Of Subacute Sclerosing Panencephalitis Research Articles

Atypical Presentation of Subacute Sclerosing Panencephalitis in a Child

Background: Subacute sclerosing panencephalitis (SSPE) is a rare complication of measles, which emerges long after the initial infection. The diagnosis of SSPE can be challenging, especially when initial presentation is atypical. Clinical Description: A 6-year-old boy, apparently healthy previously, presented with a sudden onset of generalized myoclonic jerks with intact cognitive and scholastic skills. Past history revealed that the child had measles infection at 7 months of age. Clinical examination, including memory and intellect assessment, was normal except for the generalized myoclonus. Management and Outcome: Diagnosis of SSPE was confirmed with positive antimeasles antibodies and characteristic pseudoperiodic pattern on electroencephalogram. The child was started on standard treatment as per the existing guidelines. However, the child showed marked deterioration over the next 4 weeks with loss of cognition and intellect, causing hopelessness and grief in the family, leading to their self-withdrawal from further treatment. Conclusion: This case report describes the importance of considering SSPE as a potential diagnosis in patients with myoclonic jerks but intact cognitive and intellectual function. Furthermore, the case portrays the progressive deterioration and profound emotional impact on the family of child with this disease.

Neuroimaging Abnormalities in Patients with Subacute Sclerosing Panencephalitis : Prospective Follow-up Study.

This study aimed to assess the neuroimaging abnormalities and their progression in patients with Subacute sclerosing panencephalitis (SSPE) and identify clinical predictors of these imaging findings. This prospective observational study evaluated clinical and neuroimaging features in patients with SSPE. Patients were categorized using Dyken's criteria, Jabbour's staging system, and the definition of fulminant SSPE. They underwent comprehensive clinical assessments, cerebrospinal fluid examination, Electroencephalogram (EEG), and Magnetic Resonance Imaging (MRI) scans. Treatment involved intrathecal interferon‑α and antiepileptic medications. Functional disability was assessed using the modified Barthel index. Follow-ups were performed at 6 months, including reassessment of Modified Barthel Index (MBI) and Jabbour's staging and EEG and MRI scans. The mean age was 13.9 ± 6.7years, with males comprising 81.5% (44/54) of the cohort. Fulminant SSPE was noted in 33% (18/54) of cases. Disease duration before presentation varied significantly between fulminant and non-fulminant forms (p = 0.001). Neuroimaging abnormalities were more prevalent in JSIII stage patients, with diffuse cerebral atrophy being asignificant finding (p = 0.011). Basal ganglia involvement correlated with movement disorders. The 6‑month follow-up showed increased cerebral atrophy (p = 0.004). Increasing disease duration was an independent predictor of cerebral atrophy. An Intercomplex interval (ICI) of more than 10minutes correlated with normal neuroimaging, 10 patients died within the study period, 8 of whom had fulminant SSPE. Parieto-occipital White matter hyperintensity (WMH) is the most prevalent and sensitive neuroimaging finding for the diagnosis of SSPE. Despite interferon treatment, cerebral atrophy progressed in both aggressive and fulminant SSPE. Increasing disease duration is an independent predictor of cerebral atrophy.

Low glycemic index therapy in children with sub-acute sclerosing panencephalitis (SSPE): an experience from a measles-endemic country.

Sub-acute sclerosing panencephalitis (SSPE) is a chronic, progressive neurodegenerative disorder, commonly seen in measles-endemic countries leading to progressive neuronal loss and death. Currently, there is no proven cure for this devastating disease. We started a low glycemic index therapy (LGIT) in children with SSPE using the same principle as per its role in intractable epilepsy. Low glycemic index diet was started in children with a confirmed diagnosis of SSPE based on Dyken's criteria. All children were then classified into four stages according to disease progression. The response to diet was evaluated by improvement in their myoclonic jerks, motor activities, and changes in their stage of the disease. A total of 12 children were enrolled. The mean age was 6.65 years (range 3.3-10 years), with a male-to-female ratio of 2:1. Five children were at stage IV, five were at stage III, and two were at stage II at the start of the diet. Nine (75%) children showed improvement in their stage of illness. Of three children who were at stage IV at the initiation of the diet, one improved to stage II and two to stage III. Four children at stage III reverted to stage II. Two children initiated at stage II went into total remission. Seven (58.3%) children showed a >50% reduction in myoclonic jerks with three (25%) having a 100% reduction. Three (25%) children died due to pneumonia. LGIT may play an effective role in the management of SSPE and gives hope to families having children with this potentially life-threatening disease.

Bilateral retinitis as the initial presentation of subacute sclerosing panencephilitis

Subacute sclerosing panencephalitis (SSPE) is a ravaging disease of the central nervous system caused by persistent mutant measles virus. The diagnosis of SSPE is based on characteristic clinical and EEG findings and demonstration of elevated antibody titres against measles in cerebrospinal fluid. SSPE may have atypical clinical features at the onset. Herein, we report an atypical case of SSPE in an adolescent with bilateral retinitis as the initial presenting feature. The disease progressed with an appearance of cognitive worsening, myoclonic jerks, periodic high amplitude generalized complexes on EEG, and elevated titers of measles antibodies in cerebrospinal fluid leading to the final diagnosis of SSPE. The case warrants a high degree of suspicion on ophthalmologist’s part to clinch the definitive diagnosis of not so common deadly disease.

Subakutni sklerozirajući panencefalitis - promene fenotipa tokom poslednje decenije

Introduction: Subacute sclerosing panencephalitis (SSPE) is a rare, progressive, neurodegenerative disease with poor outcome. Anti-measles vaccination contributed to a decreasing number of SSPE patients, but not to its eradication. The aim of our study is to evaluate the course of the disease in our SSPE patients with a focus on vaccinated children. The main goal is considering possibilities for improving prevention of the disease. Methods: A retrospective study included the patients with SSPE treated in the period from December 2010 to December 2020 at the Pediatric Clinic of the Institute. The inclusion criteria were the patients with the diagnosis of SSPE based on clinical presentation, neuroimaging, electroencephalography and positive IgG anti-measles antibodies, both in serum and CSF. Results: Five children with fulminant course of SSPE were included. All these patients were suffering from measles at an early age. Three of them had been vaccinated against measles and two had not. All of them had previously been healthy, immune-competent children, with normal general development. The course was extremely fulminant with lethal outcome within three months since the initial symptoms in four cases. Progressive motor and cognitive decline, behavior changes, movement disorders, myoclonic jerks and seizures were dominant in clinical presentation. Conclusion: Despite vaccination, SSPE has not been eradicated. An increasing number of vaccinated immune-competent children with fulminant form of SSPE and history of measles infection at an early age were treated at our Clinic. As a measure for improving prevention, we suggest considering weaning of vaccine-derived immunity, and re-vaccination of girls at reproductive period.

A CASE REPORT OF PAEDIATRIC SUBACUTE SCLEROSING PANENCEPHALITIS

Subacute sclerosing panencephalitis (SSPE) is a devastating disease of the central nervous system (CNS) caused by persistent measles virus infection. The diagnosis of SSPE is based on characteristic clinical and EEG findings and demonstration of elevated antibody titres against measles in cerebrospinal fluid. Subacute sclerosing panencephalitis can have atypical clinical features at the onset.We report a 10 year old boy presented with convulsions and visual disturbances. The disease progressed with behavioural and cognitive disturbances and periodic high amplitude generalised complexes on EEG, and elevated titers of measles antibodies in cerebrospinal fluid leading to the final diagnosis of subacute sclerosing panencephalitis.

Subacute Sclerosing Panencephalitis (SSPE) in Toddlers and Young Children: A Case Series

SSPE is a neurodegenerative disorder caused by persistent defective or mutant measles virus. The disease has a gradual progressive course leading to death within 1-3 yrs or even early. SSPE is a disease of childhood and early adolescence. The classic age at presentation is 8-11 years and usually occurs after a latent period of average 6 years. Here we report 3 cases of SSPE in toddlers. Diagnosis was made on the basis of clinical presentation, EEG pattern and elevated CSF anti measles antibody titer as described in Dykan criteria for diagnosis of SSPE. Clinical presentation was very early with a relatively shorter latency and fatal progression. Two patients had past history of measles and all patients were immunized against measles. One patient died within 4 months of disease expression and 2 patients went into vegetative state within 3 months of disease onset.
 Bangladesh J Child Health 2020; VOL 44 (2) :114-117

Adult Onset Subacute Sclerosing Panencephalitis: A case report

Subacute sclerosing panencephalitis (SSPE) is a chronic encephalitis of childhood and young adolescence due to persistent measles virus infection of the central nervous system (CNS). In majority of cases, onset occurs between 5-10 years of age. SSPE generally occurs 5-10 years after measles virus infection1. The diagnosis of SSPE is based on characteristic clinical and electroencephalogram (EEG) findings, increase measles antibody titer in cerebrospinal fluid (CSF) and serum. As onset of SSPE in adults is rare and may have atypical feature it requires high index of suspicion for early and accurate diagnosis. Herein, we report a case of SSPE in a male of 26 years with recurrent episodes of myoclonic jerks. Journal of National Institute of Neurosciences Bangladesh, 2016;2(1): 40-42

Subacute sclerosing panencephalitis: clinical and demographic characteristics.

To determine the clinical and demographic characteristics of children diagnosed with Subacute sclerosing panencephalitis (SSPE). Case series. The Aga Khan University Hospital, Karachi, from January 2000 to June 2012. A retrospective analysis was done, regarding medical charts of 43 children under the age of 16 years with a discharge diagnosis of SSPE. Demographic and clinical characteristics were recorded. RESULTS were expressed as percentages. Most of the 43 patients were male (72%). The average age at presentation was 8.7 years with average duration of symptoms being 100.6 days. History of measles was present in 17 patients (39.5%). All children had seizures at presentation and 65% had cognitive impairment. Most patients required poly therapy for control of seizures. Sodium valproate was the most commonly used anti-epileptic agent; Isoprinosine was tried in 22 (51%) patients. CSF for antimeasles antibodies was positive in approximately 86% of the 40 (93%) children. EEG showed burst suppression pattern in 36 (83.7%) cases. Forty-two patients (97.6%) were discharged home in a vegetative state. SSPE is progressive neurodegenerative disorder. It can be prevented by timely immunization against measles. Measles antibody in the CSF is diagnostic for SSPE and is helpful in early diagnosis. Most patients experience a gradual but progressive decline in motor and cognitive functions.

Spectrum of MRI brain findings in subacute sclerosing panencephalitis

Background and objectives: Subacute sclerosing panencephalitis (SSPE) is a progressive catastrophic neurodegenerative disease because of persistent measles viral infection in the brain. This study was designed to determine the spectrum of magnetic resonance imaging (MRI) findings in subacute sclerosing panencephalitis. Design: Case series. Methods: We described the brain MRI findings in 20 pediatric patients with confirmed SSPE with their clinical and electroencephalogram (EEG) correlates. This study was conducted at Aga Khan University Hospital, Karachi, Pakistan between January 2006 and June 2016. Diagnosis of SSPE was on the basis of the clinical signs and symptoms, the characteristic EEG patterns (burst suppression in the early stage and a diffuse, random, slow arrhythmia pattern in the late stage), and high titers of measles antibody in the cerebro-spinal fluid. Results: The mean age at presentation was 7.4 ± 3.3 years. MRI abnormalities included diffuse white matter changes (n = 8), subcortical T2 hyperintesities in both grey and white matter in 1 patient and the brainstem changes in 2 patients. MRI was normal in 8/20 patients. Magnetic resonance spectroscopy (MRS) was performed in 4 patients out of whom 1 patient showed reduced N-acetyl aspartate (NAA) peak with elevated choline peak and inverted doublet lactate peak, 1 showed only reduced NAA, 1 showed isolated choline peak and 1 patient had a normal MRS. Conclusion: MRI brain to date is supportive in understanding the pathology of SSPE. MRI can be normal in patients with SSPE if done early on at the start of the disease.

Atypical magnetic resonance imaging features in subacute sclerosing panencephalitis.

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.

Atypical Presentation of Sub Acutesclerosing Pan Encephalitis With Short Onset Latency

The most severe sequel of measles virus infection is sub acutesclerosing pan encephalitis (SSPE), a fatal disease of the central nervous system that generally develops 7–10 years after infection. A 7 ½ year old boy presented with gradual difficulty in walking and speech along with jerky movements of right upper limb which progressed to become generalized and progressive loss of cognition was also observed within days of onset of symptoms. Diagnosis of SSPE was made on the basis of burst suppression pattern on EEG and anti measles antibody in CSF. A differential diagnosis of SSPE should be considered in all forms of acute encephalopathy for early diagnosis and treatment.J Nepal Paediatr Soc 2015;35(1):62-63

Nonconvulsive status epilepticus and decreased frontal glucose metabolism in a case with SSPE

We report a 9-year-old boy with atypical presentation of subacute sclerosing panencephalitis (SSPE). He was admitted due to staring spells and cognitive deterioration. Electroencephalography (EEG) demonstrated bilaterally symmetrical, synchronous, frontal sharp-and-slow wave discharges in 80% of the record. Diazepam or midazolam administration during EEG recording attenuated the sharp-and-wave discharges, but revealed no periodic complexes. Magnetic resonance imaging delineated bilateral frontal T2-weighted hyperintense foci. Fluorodeoxyglucose-positron emission tomography imaging indicated bilaterally decreased glucose metabolism in frontal cortex. The other laboratory tests including measles antibody in cerebrospinal fluid and serum suggested no specific etiology. Interestingly, a month later, the previously detected sharp-and-slow wave discharges disappeared, and periodic slow-wave paroxysms appeared on EEG recording. Measles antibody titers showed seroconversion and confirmed the diagnosis of SSPE. The present case suggests that clinicians be cautious while excluding SSPE.

Subacute sclerosing panencephalitis presenting with hypersexual behavior.

Sir, Subacute sclerosing panencephalitis (SSPE) is a rare progressive demyelinating disease of the central nervous system associated with a chronic infection of brain tissue with measles virus. The disease is commonly seen in children and young adults. It is characterized by progressive intellectual deterioration, focal and/or generalized seizures, myoclonus, ataxia, and visual disturbances.[1] The presentation varies according to the age of the patient, and the diagnosis is often delayed in adults due to the atypical presentation. The hypersexual disorder is classically defined in persons aged above 18 years and is characterized by the recurrent and intense sexual urge irrespective of the situation persisting beyond 6 months.[2] The disease is usually associated with significant psychiatric ailments and also with central nervous disorders such as stroke, brain injury, and medications. SSPE presenting with hypersexual behavior is uncommon and is rarely reported in the literature. We retrospectively reviewed the data pertaining to four patients of SSPE treated at our hospital between 2012 and 2014. The diagnosis of SSPE is based on the Dyken's criteria.[3] SSPE is diagnosed, if the patient meets any three of the following five criteria: (a) Progressive intellectual deterioration with signs of myoclonus; (b) characteristic electroencephalographic (EEG) pattern; (c) elevated cerebrospinal fluid (CSF) globulin levels; (d) elevated CSF measles antibody titers; and (e) brain biopsy suggestive of measles. The demographic profile and investigation findings of all the patients are summarized in the Table 1.Table 1: Clinical details of all the four patientsBriefly, all our patients are young adults of both sexes and hail from poor socioeconomic strata of the society. All the patients presented with classical behavioral abnormalities, scholastic regression, and a couple of them presented with the hypersexual syndrome. All the patients showed a characteristic EEG pattern of periodic lateralized epileptiform discharge sequences neuroimaging revealed nonspecific white matter hyperintensities in few patients. The paired serum and CSF samples for measles ELISA IgG were positive in all the patients confirming the diagnosis of SSPE. The patients were treated with intrathecal interferon, Isoprinosine, and antipsychotic drugs including risperidone. Sodium valproate was used to control the myoclonus and seizure activity. One patient had a persistent worsening illness with recurrent seizures and succumbed to his illness at the end of 1-year of observation. Behavioral abnormalities improved in the remaining three patients, and they are under regular follow-up. In India, SSPE is still a common neurodegenerative disorder despite an increase in the measles vaccination. The diagnosis and treatment are usually delayed due to the varying presentation of the disease. Our data revealed that three patients were receiving psychiatric treatment for more than a year prior to the proper diagnosis. Poor scholastic performance and cognitive regression are important clues to the underlying diagnosis of SSPE and EEG is often diagnostic in these cases.[4] The clinical presentation of adult onset differs from childhood onset as, personality changes and ophthalmic manifestations are common presenting features and survival with treatment have been better in certain series.[5] Our data had patients from the second and third decades of life, and none of the patients had ophthalmic manifestations. Hypersexual behavior leads to social stigma and is often underreported by the patients. The same has been linked with a variety of neuropsychiatric disorders including obsessive-compulsive disorder, bipolar disorder, drugs such as dopaminergic agonists and other disorders including stroke and brain injury. The prevalence of SSPE is common among lower socioeconomic strata, large family size, and rural areas due to overcrowding. The measles virus is transmitted by respiratory secretions via aerosol exposure and also through direct contact with larger droplets.[1] A variety of metabolic disorders leads to dementia in young including Wilson's disease, hypothyroidism, B12 deficiency, etc. Our patients were investigated for these conditions and the negative screen resulted in the psychiatric diagnosis. It is essential for all the practitioners to look for myoclonus and also investigate for the rare causes of dementia such as SSPE and prion diseases. The therapy of SSPE involves the use of isoprinosine and an immunomodulator like interferon. The response to therapy depends on the clinical stage of SSPE at which the treatment was started. SSPE staging is done as per the modified Jabbour's classification and patients with stage 1 disease respond remarkably to the therapy whereas, stage 2 disease progresses invariably resulting in fatal outcomes in 2–5 years. To conclude, SSPE is a devastating disorder of public health importance in the developing countries. A high index of suspicion for SSPE is required during the evaluation of young adults with myoclonus, behavioral changes, and scholastic regression. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

Subacute sclerosing panencephalitis in a child with recurrent febrile seizures.

Subacute sclerosing panencephalitis (SSPE) is a devastating disease of the central nervous system (CNS) caused by persistent mutant measles virus infection. The diagnosis of SSPE is based on characteristic clinical and EEG findings and demonstration of elevated antibody titres against measles in cerebrospinal fluid. Subacute sclerosing panencephalitis can have atypical clinical features at the onset. Herein, we report an unusual case of subacute sclerosing panencephalitis in a child with recurrent febrile seizures. The disease progressed with an appearance of myoclonic jerks, periodic high amplitude generalized complexes on EEG, and elevated titers of measles antibodies in cerebrospinal fluid leading to the final diagnosis of subacute sclerosing panencephalitis.

Fulminant subacute sclerosing panencephalitis presenting with acute ataxia and hemiparesis in a 15-year-old boy.

BackgroundSubacute sclerosing panencephalitis (SSPE) is a delayed and fatal manifestation of measles infection. Fulminant SSPE is a rare presentation in which the disease progresses to death over a period of 6 months. The clinical features are atypical and can be misleading.Case ReportWe report herein a teenage boy who presented with acute-onset gait ataxia followed by right hemiparesis that evolved over 1 month, with left-hemispheric, delta-range slowing on the electroencephalogram (EEG). Magnetic resonance imaging disclosed multiple white-matter hyperintensities, suggesting a diagnosis of acute disseminated encephalomyelitis. He received intravenous steroids, and within 4 days of hospital admission he developed unilateral slow myoclonic jerks. Repeat EEG revealed Rademecker complexes, pathognomonic of SSPE, and an elevated titer of IgG antimeasles antibodies was detected in his cerebrospinal fluid. The disease progressed rapidly and the patient succumbed within 15 days of hospitalization. The diagnosis of SSPE was confirmed by autopsy.ConclusionsThis case illustrates the difficulty of recognizing fulminant SSPE when it manifests with asymmetric clinical and EEG abnormalities.

Atypical subacute sclerosing panencephalitis with short onset latency

An 11-month-old boy presented with focal seizures, myoclonic jerks and altered sensorium of one month duration, with a history of measles at eight months of age. A diagnosis of Subacute sclerosing panencephalitis (SSPE) was made on the basis of typical EEG changes and presence of anti-measles antibody in cerebrospinal fluid. A differential diagnosis of SSPE should be considered in all forms of acute encephalopathy in infants for early diagnosis and management.

Atypical clinical presentation of subacute sclerosing panencephalitis (SSPE)

SSPE (Subacute sclerosing panencephalitis) is a chronic and slowly progressive inflammatory disease of the central nervous system caused by a persistent measles virus usually affecting the childhood and adolescent age group. We present the case of a 19 years old adolescent, the product of a normal full-term pregnancy, with a primary measles infection at the age of 7 months. Neurological examination at admission discloses unilateral myoclonic jerks of upper left limb and choreoathetosis of the left hand fingers. The particularity of our case is the unilaterality of the myoclonic jerks at the onset and relatively old the age of debut. The findings of periodic complexes on EEG, elevated gamma globulin and oligoclonal bands in the CSF and elevated measles antibody titers in the serum and CSF were sufficient to make diagnosis of SSPE.

Atypical subacute sclerosing panencephalitis with short onset latency

An 11-month-old boy presented with focal seizures, myoclonic jerks and altered sensorium of one month duration, with a history of measles at eight months of age. A diagnosis of Subacute sclerosing panencephalitis (SSPE) was made on the basis of typical EEG changes and presence of anti-measles antibody in cerebrospinal fluid. A differential diagnosis of SSPE should be considered in all forms of acute encephalopathy in infants for early diagnosis and management.

831 An Analysis of the Progressive Clinical Deterioration in the Cases with Subacute Sclerosing Panencephalitis

Subacute sclerosing panencephalitis (SSPE) is devastating progressive neurodegenerative disorder of the central nervous system by an aberrant measles virus. SSPE is still important health problem in countries where measles immunization...