Regional cerebral metabolic rate for glucose in subacute sclerosing panencephalitis

Regional cerebral metabolic rates for glucose (rCMRglc) were measured in two cases of subacute sclerosing panencephalitis (SSPE) with different clinical courses. A marked decrease in rCMRglc was found in the cortical gray matter of a patient with rapidly developing SSPE (3.6–4.2 mg/100 g brain tissue per min). However, the rCMRglc was preserved in the caudate and lenticular nuclei of the patient (7.7 mg/100 g per min). The rCMRglc in a patient with slowly developing SSPE revealed patterns and values similar to those of the control. The rCMRglc correlated better with the neurological and psychological status of SSPE.Abbreviations rCMRglc regional cerebral metabolic rate - SSPE subacute sclerosing panencephalitis - PET position emission tomography - [F-18]FDG [F-18]2-fluorodeoxyglucose     

Subacute sclerosing panencephalitis after drug-induced immunosuppression.

A girl developed subacute sclerosing panencephalitis (SSPE). Eight years earlier she had had measles infection contracted shortly after cytotoxic treatment and radiotherapy for a spinal neuroblastoma. The case illustrates that typical SSPE, like immunosuppressive measles encephalopathy, can arise after drug-induced immunosuppression, and supports the view that these diseases probably represent opposite ends of a spectrum induced by measles virus infection in an individual with some form of immunological deficiency.     

Serological studies on subacute sclerosing panencephalitis

This study reports the clinical picture and measles virus antibody titres in 32 patients with cases of suspected subacute sclerosing panencephalitis (SSPE). The history of myoclonic jerks, mental regression, inability to walk and slurred speech were noted in these cases. The EEG showed generalised periodic complexes in twenty nine patients and only in three patients the EEG was not available. In all the above mentioned patients measles occurred at an early age (within a year).     

Patients with acute, fulminant form of SSPE

Subacute sclerosing panencephalitis (SSPE) usually begins insidiously and follows a subacute course with relentless but slow progression to death. In recent years, however, patients with acute or fulminant course were reported. In this article, we report on three patients (2 girls, 1 boy) with SSPE who developed an acute and fulminant course. Subacute sclerosing panencephalitis may be seen with more atypical symptoms and more acute and fulminant courses due to various undetermined reasons. Early diagnosis is very important for the effectiveness of treatment. Children presenting with acute or subacute neurologic symptoms should be examined for SSPE, especially if they have no risk factors for hereditary neurodegenerative/ neurometabolic diseases, and it is more important if those children were not vaccinated or were infected with measles.     

Two cases of subacute sclerosing panencephalitis associated with brainstem involvement

The most commonly involved areas in subacute sclerosing panencephalitis (SSPE) are periventricular and subcortical white matter. The basal ganglia, cerebellum, spinal cord and corpus callosum are less commonly involved. Brainstem involvement is rare and usually accompanied by other intracranial lesions. In this article, we report two cases of SSPE associated with brainstem involvement. The first case a 9-year-old girl had the typical symptom of SSPE. Magnetic resonance imaging (MRI) of the brain revealed a focal lesion 2 x 2.5 cm a diameter in the pons. The second case was a 6-year-old girl. On admission, MRI of the brain was normal. During 6th month of follow-up, T2-weighted MRI showed a hyperintense lesion in the pons and pedincule of cerebellum. On account of these cases we would like to stress that brainstem involvement may be seen in patients with SSPE; therefore, these patients should be monitored for this disorder.     

The Origin of Myoclonus and Periodic Synchronous Discharges in Subacute Sclerosing Panencephalitis

We report here a case of a patient with subacute sclerosing panencephalitis (SSPE) and we have analyzed periodic events using dipole tracing methods to clarify the origin of periodic synchronous discharges and myoclonus. Both source generators were located in the subcortical part of the cerebrum, an area adjacent to the thalamus. Although the pathophysiology of periodic events in SSPE has been controversial, dipole tracing methods may contribute to clarify the origin of periodic events in SSPE.     

18F-FDG PET and MRS of the early stages of subacute sclerosing panencephalitis in a child with a normal initial MRI

In subacute sclerosing panencephalitis (SSPE), conventional MRI findings have been reported. However, in the early clinical stages, imaging studies can appear normal. Moreover, with no history of infant measles infection, the diagnosis of SSPE can only be arrived at after extensive investigation that must eliminate a number of neurodegenerative diseases. We report here on ¹⁸ F-fluorodeoxyglucose positron emission tomography (¹⁸F-FDG PET) and magnetic resonance spectroscopy (MRS) findings in a 14-year-old girl with a normal initial MRI who had not contracted measles. Although ¹⁸ F-FDG PET and MRS are not specific or diagnostic for SSPE, these techniques can demonstrate substantial metabolic impairments when MRI findings show no obvious abnormalities, as is often the case in the early stages of this disease.     

Subacute sclerosing panencephalitis: a case report

Subacute sclerosing panencephalitis (SSPE) is a progressive neurological disorder of childhood and early adolescence caused by persistent defective measles virus. Clinical manifestations appear many years after the acute measles infection. The incidence of SSPE has substantially declined after the introduction of an effective vaccine. We report a case of a child with SSPE that began with atonia, dysarthria, and intellectual deterioration without the presence of any particular EEG anomalies. We have reported this girl who was affected by this severe affliction in the hope that, because of the rarity of SSPE, it would not go undiagnosed.     

Subacute sclerosing panencephalitis after intrauterine infection

Subacute sclerosing panencephalitis (SSPE), in the majority of cases, is caused by the wild measles virus, although there are some reports relating SSPE to vaccination. This paper presents an inborn that was infected during pregnancy by the measles virus and developed SSPE within the first year of life after a short incubation period. He progressed rapidly after a mild arrest with treatment. Subacute sclerosing panencephalitis is a fatal degenerative disease and, although it had largely disappeared because of nearly universal measles vaccination, it still remains a serious infection among children affected by human immunodeficiency virus (HIV). The lack of newer cases of SSPE occurring among normal children nowadays should not wane alertness by obstetricians and paediatricians, to recognize the risk with measles during pregnancy and the need for prevention and recognition of SSPE at an early stage. Although some references exist which report on SSPE cases related to vaccination, new work weakens the possible links between measles vaccine and SSPE. Conclusion: This report would like to stress the importance and success of reducing the SSPE problem with the aid of general measles vaccination with high coverage.     

Acute bilateral vision loss as the presenting feature of subacute sclerosing panencephalitis

A 12-yr-old boy with an atypical presentation of subacute sclerosing panencephalitis (SSPE) is described. Bilateral macular chorioretinitis preceded the neurological symptoms by 3 weeks. Both visual and neurological features had an acute onset. Clinicians need to be aware that macular chorioretinitis in a child may be the heralding feature of SSPE.     

Diagnostic dilemmas in fulminant subacute sclerosing panencephalitis (SSPE)

This report describes an eleven-year-old boy with atypical features of subacute sclerosing panencephalitis (SSPE), a rare complication of measles. He had only visual symptoms for 2 months followed by rapid neurological worsening to a vegetative state in 10 days. A diagnosis of SSPE was made based on the history of measles, characteristic ocular findings, compatible magnetic resonance imaging and electroencephalographic changes, and elevated ratio of cerebrospinal fluid to serum anti-measles antibody titers     

Antibodies to borna disease virus in subacute sclerosing panencephalitis

Mechanisms causing persistence and reactivation of measles virus in subacute sclerosing panencephalitis (SSPE) are unknown. Borna disease virus (BDV) frequently causes latent or persistent infection in the nervous system. We investigated a possible association of these viruses in SSPE. Although BDV seropositivity was similar in SSPE and control groups, SSPE patients with high antibodies to BDV had earlier and more rapid disease. The findings suggest that BDV might be involved in the course, but not in the etiopathogenesis, of SSPE.     

Non-leukemic disease of the central nervous system in children with acute lymphoblastic leukemia. III. Subacute sclerosing panencephalitis (author's transl)]

Diseases of the central nervous system (CNS) occurring during treatment of acute lymphoblastic leukemia (ALL) may be of leukemic or nonleukemic origin. Well known examples for CNS disease of nonleukemic origin are somnolence following prophylactic CNS irradiation, methotrexate-induced encephalopathy and acute infections caused by bacteria, viruses and toxoplasma gondii. Less known is the fact that also subacute CNS infections may occur in patients undergoing cytostatic therapy. Progressive multifocal leukoencephalopathy and subacute sclerosing panencephalitis (SSPE) are examples of this category of disease. Up to now 11 well documented cases of SSPE were reported occurring during treatment of ALL. Main clinical features were disorders of behaviour, consciousness and speach, seizures, paresis and inappropriate secretion of ADH. Several authors were able to demonstrate a deficiency of cellular immunity in patients with SSPE. In some cases this deficiency was consistent with reduced reactivity of T-lymphocytes against measles antigen only. The presence of inhibiting factors may be responsible for this phenomenon. Other authors found a normal or increased function of cellular immunity in SSPE; In hamsters occurrence of SSPE is induced by the simultaneous injection of hamster-adapted SSPE virus and antihamster lymphocyte serum. We, therefore, conclude that also in humans SSPE appearing during treatment of ALL is due to immunosuppression.     

Serodiagnosis of subacute sclerosing panencephalitis by enzyme linked immunosorbent assay

Thirty three young patients (23 males and 10 females), between 3–20 years of age, with a clinical suspicion of subacute, sclerosing panencephalitis (SSPE) were evaluated over a period of 4 years. A past history of measles was obtained in 42.4% of the cases. One child was immunised against measles. Measles specific antibodies (IgG) were demonstrated in the cerebrospinal fluid (CSF) from 30 (90.9%) of the 33 patients by ELISA. In the remaining three cases the antibodies were detected only in the serum. Serodiagnosis of SSPE by ELISA had a sensitivity of 100%, a specificity of 93.3% and a positive predictive value of 100%.     

A serial 18FDG-PET study of a patient with SSPE who had good prognosis by combination therapy with interferon alpha and ribavirin

We describe a 15-year-old girl with subacute sclerosing panencephalitis (SSPE) in stage II who was treated with isoprinosine, intraventricular interferon alpha (IFN-α), and ribavirin for 3 years. She is alive at three years from onset and studies at school with the assistance of a special educational teacher. To assess residual brain function, serial ¹⁸FDG-positron emission tomography (PET) was performed three times to measure cortical metabolism: at onset, a year later, and three years later. At onset, PET study revealed preserved glucose metabolism of the cerebral cortex. In serial PET study, glucose metabolism of the cerebral cortex was also preserved even after three years. Although SSPE is a progressive disease of the neuronal system, and typically leads to death in approximately 2–3 years, the neurological prognosis of our case was good. We consider that combination therapy in the very early stage without hypometabolism in the cerebral cortex may be effective for SSPE.     

Bilateral temporal cysts in a case of subacute sclerosing panencephalitis.

Magnetic resonance imaging in subacute sclerosing panencephalitis (SSPE) usually demonstrates changes in white matter signal intensity, cerebral atrophy, or, in early disease, normal findings. We observed bilateral cystic temporal lobe lesions in a patient with early stage SSPE. Other degenerative conditions associated with such lesions were ruled out based on a normal head circumference, the absence of white matter changes, and normal cerebrospinal fluid lactate level. This observation suggests the inclusion of SSPE in the differential diagnosis of cystic white matter lesions.     

The epidemiology of subacute sclerosing panencephalitis in England and Wales 1990-2002.

AIM: To assess the impact of measles/mumps/rubella (MMR) vaccine on the epidemiology of subacute sclerosing panencephalitis (SSPE) in England and Wales. METHODS: Cases of SSPE resident in England and Wales with onset between 1990 and 2002 were reviewed. RESULTS: A total of 47 cases were identified, 31 male and 16 female. There was an average annual decline of 14% in SSPE onset over the period, consistent with the decline in notified measles over the last 20 years. A history of measles was present in 35 (median age 1.3 years), the most recent recorded date being 1994; the interval from measles to onset of SSPE ranged from 2.7 to 23.4 years. Four children with a history of receipt of a measles containing vaccine were reported not to have had measles; two of these cases had a brain biopsy, and nucleotide sequence data confirmed wild measles infection. Brain biopsy specimens from a further three cases with a history of measles, of whom two had also had a history of vaccination, showed wild-type strain. CONCLUSION: The prevention of endemic circulation of measles virus in England and Wales through the high coverage achieved with MMR vaccine, together with the measles/rubella vaccination campaign of 1994, has resulted in the near elimination of SSPE. However, the recent decline in MMR vaccine coverage, with the associated increase in localised measles outbreaks and cases in young infants, is of concern. It underlines the importance of maintaining high vaccine coverage in order to protect indirectly those most vulnerable to SSPE, namely infants too young to be vaccinated.     

The epidemiology of subacute sclerosing panencephalitis in England and Wales 1990-2002

Aim: To assess the impact of measles/mumps/rubella (MMR) vaccine on the epidemiology of subacute sclerosing panencephalitis (SSPE) in England and Wales. Methods: Cases of SSPE resident in England and Wales with onset between 1990 and 2002 were reviewed. Results: A total of 47 cases were identified, 31 male and 16 female. There was an average annual decline of 14% in SSPE onset over the period, consistent with the decline in notified measles over the last 20 years. A history of measles was present in 35 (median age 1.3 years), the most recent recorded date being 1994; the interval from measles to onset of SSPE ranged from 2.7 to 23.4 years. Four children with a history of receipt of a measles containing vaccine were reported not to have had measles; two of these cases had a brain biopsy, and nucleotide sequence data confirmed wild measles infection. Brain biopsy specimens from a further three cases with a history of measles, of whom two had also had a history of vaccination, showed wild-type strain. Conclusion: The prevention of endemic circulation of measles virus in England and Wales through the high coverage achieved with MMR vaccine, together with the measles/rubella vaccination campaign of 1994, has resulted in the near elimination of SSPE. However, the recent decline in MMR vaccine coverage, with the associated increase in localised measles outbreaks and cases in young infants, is of concern. It underlines the importance of maintaining high vaccine coverage in order to protect indirectly those most vulnerable to SSPE, namely infants too young to be vaccinated.     

Prevalence of SSPE: a serological study.

The presence of measles antibodies in serum and cerebrospinal fluid (CSF) of 340 samples from children clinically suspected of subacute sclerosing panencephalitis (SSPE) were studied. One hundred and thirty eight (40%) of these children had SSPE based on the serological evidence. The mean age group of children affected was 8.2 years. The M:F ratio was 5:1. The titres of antibodies ranged from 1:2 to 1:32 in the CSF and from 1:16 to 1:512 in the serum.     

Subacute sclerosing panencephalitis: a still existing disease

Subacute sclerosing panencephalitis (SSPE) is a rare entity with an invariably fatal course that progressively affects the central nervous system. It is caused by persistent infection with the wild-type measles virus. While rare in industrial countries, it is not infrequent in developing countries, where there are still areas of endemic measles infection and immunization is not yet generalized. We describe an eight-year-old Spanish girl who presented rhythmic and symmetric myoclonus. She contracted measles at 13 months and required hospitalization. No cognitive deterioration was found. Neuroimaging and the initial electroencephalogram were normal. Oligoclonal bands and high titers of measles antibodies were found in serum and cerebrospinal fluid. She was treated with oral metisoprinol and intraventricular alpha-interferon (IFN-) and showed no further progression of her symptoms. The importance of including SSPE in the differential diagnosis of patients consulting for school failure, neurological deterioration or movement disorders is highlighted. Special attention should be paid to the immigrant population from countries where the incidence of SSPE is greater than in Spain.