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.     

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.     

Atypical presentations of SSPE: a clinical study in four cases

Subacute sclerosing panencephalitis (SSPE) is a progressive, fatal disease of the central nervous system caused by a persistent measles virus. It is clinically characterized by insidious onset of intellectual deterioration and behavioral changes followed by myoclonias and eventually complete neurologic deterioration. The diagnosis is based on characteristic clinical features, periodic electroencephalography (EEG) complexes of high slow waves and increased antibody titer against measles in cerebrospinal fluid. Here, we report four SSPE cases, two of whom manifested with hemiparesis; in the third and fourth cases, cerebellar ataxia and acute encephalopathy with focal seizures were the presenting symptoms at the onset of disease, respectively. The typical periodic EEG complexes in our patients led to the diagnosis of SSPE. Our findings show that SSPE should be considered in the differential diagnosis of hemiparesis, cerebellar ataxia and acute encephalopathy, and highlight the diagnostic significance of EEG in unidentified cases.     

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.     

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.     

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.     

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     

Brainstem involvement in subacute sclerosing panencephalitis

Subacute sclerosing panencephalitis (SSPE), which usually develops 2-10 years after measles infection, is a progressive neurologic disorder with an insidious onset. The neurologic dysfunctions associated with SSPE include generalized myoclonic jerks and seizure activity, and progression of the disease usually results in coma and death within one to two years after onset. Most of the cerebral lesions in SSPE are observed in the periventricular and subcortical white matter. Brainstem involvement in SSPE is very rare. In this paper, we report two cases with brainstem involvement in SSPE that was accompanied by other intracranial lesions with magnetic resonance imaging (MRI). These two patients died in a short time. Thus, brainstem involvement should be considered in patients with SSPE.     

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.     

Subacute sclerosing panencephalitis with remission in a Bosnian refugee child

Subacute sclerosing panencephalitis (SSPE) virtually disappeared from the US after mass measles immunization programs dating from the 1960s. However, SSPE has reappeared in internationally adopted children and in refugee children emigrating from developing or war torn countries lacking effective measles immunization programs. SSPE usually occurs 6 to 8 years after a bout of measles in early childhood; death typically follows within 1 to 3 years. What is often not reported in textbooks is that spontaneous remission occurs in a small subset of children with documented SSPE.     

Subakut sklerosierende Panenzephalitis nach Masernexposition im Säuglingsalter

Background

Subacute sclerosing panencephalitis (SSPE) is a lethal complication of measles virus infection. A risk factor for developing SSPE is measles exposure preceding the first vaccination. We report on two cases of SSPE with different clinical courses.

Case presentation

Both patients acquired an inapparent measles infection during their first year of life. They were vaccinated according to the current national recommendations. On reaching school age, early symptoms of SSPE were apparent and severe encephalopathy developed. Necrotizing chorioretinitis was observed in one of the patients. Only one patient was able to receive immunomodulatory treatment which induced transient clinical improvement.

Conclusion

SSPE should always be considered as a differential diagnosis in progressive encephalopathy in childhood. The disease can only be prevented by consistent measles vaccination in all children to minimize the risk of infection.     

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     

Subacute sclerosing panencephalitis (SSPE) in an adolescent Ethiopian girl

Some 50 years have elapsed since Dawson first described a 16-year-old girl with an inclusion encephalitis. Since then, numerous publications on subacute sclerosing panencephalitis (SSPE) have appeared in the world literature. However, most of these reports are from the developed world. There have been few reports from Africa. One of them was from Kenya, where a retrospective analysis of EEGs of patients with epilepsy over a 5-year period identified 53 probable cases of subacute sclerosing panencephalitis (1). The other one was from South Africa where an incidence of 1.2 per million per year was reported on the basis of 15 cases collected from two hospitals in the Cape Province (2). No case, as yet, has been reported from Ethiopia. This paper reports SSPE presenting in an adolescent Ethiopian girl who had measles at the age of 18 months.     

Neurological deterioration following head injury: The eyes had it

A 17-year-old male presented with confusion following a mild head injury. Repeated CT scans of the head were normal. There was a 3 year history of decreased vision, associated with a focal pigmentary retinopathy. On assessment he demonstrated visual agnosia and early dementia. An MRI scan showed symmetrical demyelination of the white matter, particularly of the occipital lobes. The diagnosis of subacute sclerosing panencephalitis (SSPE) was confirmed by the typical EEG findings and the presence of measles antibodies in the CSF. The head injury was the precipitating factor which led to a diagnosis of SSPE. This disease should be considered in young patients who have persisting cognitive dysfunction out of keeping with the severity of the initial trauma. A focal pigmentary retinopathy, especially with macular involvement, should also raise the possibility of SSPE, despite the absence of neurological symptoms initially. We report the longest interval to date between the visual symptoms and onset of neurological signs of 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 influence of inosiplex treatment on the neurological disability of patients with subacute sclerosing panencephalitis

Subacute sclerosing panencephalitis is a central nervous system degenerative disease that rapidly progresses to death in most untreated cases. In this study we compare the level of neurological disability longitudinally in a group of SSPE patients receiving inosiplex (Isoprinosine) treatment (12) to a historical control group of untreated patients (15). The mean ND did not differ between the groups from onset of SSPE through 21 months. From two years through four and one-half years the inosiplex group had significantly lower ND compared to the nontreatment group. The subjects were then divided into four subgroups: Group 1, rapidly progressing SSPE, not treated; Group 2, slowly progressing SSPE, not treated; Group 3, rapidly progressing SSPE, inosiplex treated; and Group 4 slowly progressing SSPE, inosiplex treated. The rapidly developing groups did not differ in ND at any time. The slowly developing treated group had significantly lower ND than the slowly developing untreated group from two and one-half years to four and one-half years after onset of SSPE. These findings suggest that inosiplex is effective in the slowly developing or chronic form of SSPE.     

Diagnostic and pathogenetic aspects of subacute sclerosing panencephalitis

Subacute sclerosing panencephalitis, usually a rapidly progressive and fatal disease, is a slow virus infection, where measles virus persists in cells of the CNS and in lymphocytes. Four patients, 3 boys and 1 girl, are described, who presented a characteristic disease course, beginning at the age of 12 to 14 years after they had contracted measles infection during infancy or early childhood. The diagnostic criteria including clinical and laboratory CSF findings are summarized, and epidemiologic features related to measles and measles immunization are briefly discussed. In two patients specific measles virus protein antibodies in serum and CSF were analyzed. The results confirm postulated mechanisms for viral persistence in the CNS and suggest in addition a possible role of the viral protein H in the pathogenesis of SSPE.     

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.     

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).