Crises épileptiques et épilepsie dans le cadre d’une panencéphalite sclérosante subaiguë (à propos de 30 cas)Epileptic seizures and epilepsy in subacute sclerosing panencephalitis (report of 30 cases).

Subacute Sclerosing Panencephalitis (SSPE) is becoming less frequent in Morocco since the generalization of measles vaccination in 1982. The aim of this study was first to analyze the semiological and elecrophysiological profiles of epilepsy in SSPE in both 'disease-revealing' seizures and sequellar ones, and second, to study the evolution of epilepsy and its possible prognostic value in SSPE. Among the neurological manifestations of SSPE, epilepsy is not as rare as frequently reported in the literature. In this longitudinal series concerning 70 cases of SSPE, 30 developed epilepsy. In two-thirds of our patients the epileptic seizures started in the first year of evolution; they revealed the SSPE in 23% of the cases and were sequellar in the rest. Seizures revealing the SSPE were widely dominated by partial seizures, secondarily generalized or not (86%), suggesting a focalized encephalitic process. Conversely, sequellar seizures were in most cases generalized tonic-clonic (43%), and therefore compatible with an already spread process. The EEG contributed both to the diagnosis of SSPE and to that of the epilepsy, showing epileptic abnormalities in ten patients. The outcome of epileptic seizures was very favorable under antiepileptic drugs, while that of SSPE remained severe and not modified by epilepsy. The authors underline the relative frequency of epilepsy in SSPE, the interest of the distinction between revealing and sequellar seizures, the good prognosis of epilepsy under adequate therapy, and the absence of prognostic value of epilepsy in SSPE.     

Subacute schlerosing panencephalitis in the middle east: Report of 99 cases

All patients entering with a differential diagnosis of subacute sclerosing panencephalitis (SSPE) at two major Lebanese medical centers since 1956 yielded 99 cases. Significant findings included: an epidemic pattern with 4 cases during 1956–57;95 cases during 1964–75; and, after 1966, 1.7 cases per year per million total population in Lebanon alone. The average age at which patients had measles shifted from 20 months in those who had onset of SSPE before 1971 to 35 months thereafter. Duration of illness was less than three months for 7% of the patients; four to 24 months for 69%; and more than 24 months for 24%. Four percent acieved significant long-term improvement.     

Acute SSPE.

Two patients had acute fulminanting encephalitis and the typical pathological changes of subacute slcerosing panencephalitis (SSPE). It is suggested that SSPE should be considered in the differential diagnosis of acute viral encephalitis.     

An acute variant of subacute sclerosing panencephalitis: an autopsy case report

An acute variant of subacute sclerosing panencephalitis (SSPE) was described in a 5-year-old boy who showed rapid progression of coma within 14 days of right hemiplegia. He had measles at 3 years of age. The diagnosis of SSPE was based on the following findings: high anti-measles antibody titer in the serum and in the spinal fluid, periodic complex of EEG, and typical pathological changes of the brain. Treatment with transfer factor failed to improve the worsening clinical course. It is suggested that SSPE should be considered in the differential diagnosis of acute fulminating encephalitides or intracranial vascular lesions.     

Cerebrospinal fluid measles antibody titer and γ globulins in subacute sclerosing panencephalitis

Summary Measles antibody determination on a series of CSF's confirm the physiopathological significance of measles in SSPE. The comparison of CSF protein electrophoretic patterns in agar gel and measles antibody titers contributes to the differential diagnosis of SSPE and other neurological disorders.

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Zusammenfassung Masern-Antikörperbestimmungen in einer Serie von Liquorproben bestätigen die physiopathologische Bedeutung der Masern bei der SSPE. Der Vergleich von Agar-Gel-Elektrophorese-Diagrammen der Liquorproteine mit Masern-Antikörper-Titer ist für die Differentialdiagnose der SSPE und anderer neurologischer Störungen aufschlußreich.

     

Indication of peripheral nerve hyperexcitability in adult-onset subacute sclerosing panencephalitis (SSPE)

Subacute sclerosing panencephalitis (SSPE) is a rare chronic, progressive encephalitis that affects primarily children and young adults, caused by a persistent infection of immune-resistant measles virus. Diagnostic hallmarks include widespread cortical dysfunction on EEG, myoclonus, white matter abnormalities on neuroradiological examination and the presence of IgG anti-measles antibodies in the cerebrospinal fluid. We present the first case of SSPE with signs of peripheral nerve hyperexcitability, observed as extra discharges following the compound motor action potential at motor nerve stimulation. In addition we demonstrate the importance of SSPE in the differential diagnosis of adult patients with psychiatric and neurological symptoms.     

Subacute sclerosing panencephalitis in two brothers

We report the occurrence of subacute sclerosing panencephalitis (SSPE) in two brothers two years after measles infection. The diagnosis was confirmed by compatible data from medical history, occurrence of autochthonic measles virus (MV) IgG production in the central nervous system (CNS), and pathognomonic EEG changes. Pathogenetically, SSPE is caused by a genome mutation of intracellularly persisting MV, causing viral nucleocapsides to accumulate in the brain cells. A specific predisposing immune defect is not known. The occurrence of two cases in one family is suggestive of a genetic predisposing factor.     

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.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.     

Subacute sclerosing panencephalitis and hypoparathyroidism

Subacute sclerosing panencephalitis (SSPE) occurred in 3 unrelated children affected with different genetic forms of hypoparathyroidism (HPT). Clinical or laboratory evidence of an immunological deficit before SSPE onset was ascertained in cases 1 and 3. At the onset of SSPE, all cases showed signs of increased central nervous system (CNS) excitability due to poorly regulated blood calcium levels, and cases 1 and 2 had papilledema. Epidemiological evaluation suggests that the association of SSPE and HPT is not accidental. In HPT, both a subtle immunological deficit and CNS involvement may be predisposing factors of SSPE.     

Fast-wave periodic complexes in a mentally retarded child who later developed subacute sclerosing panencephalitis: a modification of a classic EEG by preexisting brain damage?

The EEG of a 12-year-old girl with stage H subacute sclerosing panencephalitis (SSPE), who had also suffered from a non-progressive mental retardation of unknown aetiology since early childhood, revealed periodic generalised stereotyped fast wave bursts synchronous with myoclonic jerks. The background activity was nearly normal. The diagnosis of SSPE was established by raised serum and measles antibody titres, raised CSF IgO, and brain biopsy. This rare type of periodic complex has only once been described in the literature, again in a mentally retarded child who had developed SSPE. We suggest a mechanism of origin of this type of periodic complex drawn from observations in these two cases, and discuss its significance.     

Subacute sclerosing panencephalitis (SSPE) in women during pregnancy]

Four women with SSPE during pregnancy were observed. The influence of pregnancy on the course of illness was in all patients unfavourable: the disease exacerbated, what could be explained by physiological immunosuppression connected with gestation. Delivery (in three cases by sectio caesarea) had no beneficial influence on the fatal course of SSPE. In two cases intrauterine necrosis of foetus took place in the 8th and 20th week from the beginning of SSPE. In other two cases healthy children were born. One of them does not exhibit any neurological abnormalities during the first 5 years of life.     

Activation of periodic complexes of subacute sclerosing panencephalitis by sleep

Three patients with clinically proved subacute sclerosing panencephalitis (SSPE) had electroencephalograms in which periodic stereotyped complexes characteristic of SSPE were apparent during sleep but were not observed during the initial awake recordings. The first patient had periodic complexes only during sleep. The second and third patients had complexes that were inconstant and sporadic during the awake recordings but regular and periodic during sleep. Although sleep can have a variable effect on the electroencephalographic pattern in SSPE, it may facilitate elicitation of typical periodic complexes in questionable cases of SSPE.     

Subacute sclerosing panencephalitis

Subacute sclerosing panencephalitis (SSPE) is a subacute encephalopathy of childhood and young adolescence. Infrequently, SSPE can occur in adults and pregnant women. It is caused by an aberrant measles virus, known as the SSPE virus. SSPE virus differs from wild-type measles viruses in the form of several mutations affecting the viral genome. The matrix gene is most commonly affected by these mutations. The characteristic clinical manifestations of SSPE include behavioral changes, cognitive decline, myoclonic jerks, seizures, abnormalities in vision, bilateral pyramidal signs and coma. Ocular changes may occur in up to 50 % of patients. The most characteristic ophthalmological lesion is necrotizing retinitis. Cortical blindness can be the early feature of SSPE. The diagnosis of SSPE is often difficult in the early stages. In a typical case diagnosis is based on clinical, electroencephalographic, and cerebrospinal fluid findings. At present, there is no effective treatment to completely cure SSPE. Oral isoprinosine and intrathecal or intraventricular alpha-interferon may prolong survival to some extent. Immunization against measles is currently the most effective strategy against SSPE. Electronic Supplementary Material  The online version of this article DOI contains supplementary material, which is available to authorized users.     

Adult onset subacute sclerosing panencephalitis: clinical profile of 39 patients from a tertiary care centre

Clinical and laboratory characteristics of 39 patients with adult onset subacute sclerosing panencephalitis (SSPE) are described and compared to those of juvenile onset patients regarding preceding measles, age at onset, gender, interval between onset and diagnosis, clinical profile, and course during follow up. Diagnosis was based on clinical and electroencephalographic findings and raised anti-measles antibody titres in cerebrospinal fluid. Mean age at SSPE symptom onset was 20.9+/-4.9 years and mean interval from onset to diagnosis was 6.3+/-9.6 months. Referral diagnosis was accurate in only 12 patients. Presenting symptoms included myoclonus, behavioural changes, seizures, and cognitive, visual, and extrapyramidal disturbance. All patients received symptomatic therapy; 19 also received disease modifying agents. Five of seven pregnant women had successful deliveries. The follow-up period varied widely (maximum 60 months, median 9 months). The profile of adult onset SSPE did not differ from the rest of the cohort, except for a longer interval between measles infection and symptom onset (p<0.0001). SSPE in adults poses diagnostic challenges for clinicians. A high index of suspicion and appropriate investigations are necessary for early diagnosis and counselling.     

Prolonged remission in subacute sclerosing panencephalitis: 2 cases

Long survival in subacute sclerosing panencephalitis (SSPE), including total disappearance of clinical signs, is rare. Two cases are reported. They concern a girl and a boy who, at age 13 and 15, developed SSPE and are still in remission 6 and 5 years later. After a typical onset and course over periods of 12 and 18 months, clinical improvement was observed and periodic EEG complexes disappeared. However, the electrophoretic oligoclonal pattern of CSF proteins and the elevated measles titers persisted (in one case specific CSF IgM were still increased 6 years after the onset). MRI showed asymmetrical areas of high-intensity signal in both white and gray matter, predominant in the temporal, parietal and occipital regions. The age at which SSPE begins and the interval between measles and SSPE onset are not prognostic factors. On the other hand, in reported cases with lasting remission SSPE did not progress beyond Jabbour's stage II. The second typical feature of these long-term improvements is disappearance of EEG periodic complexes and emergence of a normal basic background activity. No other prognostic factor has been reported.     

Subacute Sclerosing Panencephalitis and Acquired Immunodeficiency Syndrome: Role of Electroencephalography and Magnetic Resonance Imaging

Subacute sclerosing panencephalitis (SSPE) had largely disappeared from the United States because of nearly universal measles vaccination, but it has reemerged in children infected with human immunodeficiency virus (HIV) . Two children with SSPE are described . The first was HIV positive and presented with seizures and encephalopathy at the age of 21 months. The second developed myoclonus and dementia at age 4 years; she was not infected with HIV, but her mother had acquired immunodeficiency syndrom. Magnetic resonance imaging findings were nonspecific and could have been compatible with HIV encephalopathy. Electroencephalography was characteristic of SSPE, showing high-voltage, periodic slow-wave complexes and background slowing . The diagnosis of SSPE was confirmed by brain biopsy or high measles antibody titers in the cerebrospinal fluid.     

Focal MRI findings in early SSPE

Many investigators have identified localized cortical involvement in subacute sclerosing panencephalitis (SSPE) by clinical and electrophysiologic criteria. Some investigators have reported such abnormalities in the posterior cerebrum early in the course of the disease, but without radiologic correlation. Recently, magnetic resonance imaging has been used to follow the progression of SSPE. The largest reported study of SSPE utilizing magnetic resonance imaging indicated that focal abnormalities were distinctly rare and actually mitigated against the diagnosis of SSPE. We report an SSPE patient with focal cerebral dysfunction and magnetic resonance imaging evidence of distinctly focal inflammatory disease early in the course of the illness.     

Subacute sclerosing panencephalitis in only one member of a monozygotic twin pair. Clinical, laboratory, electroencephalographic, and immunological study of both twins

Male monozygotic twins, one of whom developed subacute sclerosing panencephalitis (SSPE) are reported in detail. Both patients had measles at the age of 4 years and 6 months. The diagnosis of SSPE was based on clinical, electroencephalographic and laboratory findings. Titration of antibodies against measles was carried out in both cases. The healthy twin, who had been admitted with a clinical picture initially similar to that displayed by his brother, showed clinical manifestations mimicking those of the affected sib, but these vanished 4 days after his admission and were attributed to transient conversion hysteria.The genetic factors involved and the biological response of the antibodies against measles are discussed.     

Subacute sclerosing panencephalitis in Bulgaria (1978-2002)

The epidemiology of subacute sclerosing panencephalitis (SSPE) has changed substantially since the introduction of measles vaccine. We studied the incidence of SSPE in Bulgaria based on cases admitted to the Child Neurology Clinic, University Hospital of Neurology and Psychiatry, Sofia, for a 25-year period (1978-2002). The SSPE incidence prior to and during the period of routine measles immunization was analyzed. SSPE was diagnosed in 40 children (29 males and 11 females, mean age 8.5 years), 28 from 1978 to 1984 (average 4 patients/year), and 12 from 1995 to 2002 (average 1.7 patients/year). Thirty-eight cases (95%) were non-immunized and had early measles infection. Age at onset of SSPE ranged from 8 to 11 years (52.5%) with a mean latent period of 7 years following measles infection. The increase in SSPE incidence (1995-2002) following a 10-year disease-free period (1985-1994) appears to be related to early measles infection (mean age 11 months) during the measles epidemic of 1991-1992. During the period 1995-2002, children had earlier measles infection (average 11 months) and earlier onset of SSPE (mean age 8.4 years) than in the period 1978-1984 (mean age at measles infection 18 months, and of SSPE onset 11.2 years). The SSPE incidence in Bulgaria during the 25-year period from 1978 to 2002 confirms the importance of early measles infection as a risk factor for SSPE, and the role of routine measles immunization in SSPE prevention.     

Early signs of cognition disorders in children with subacute sclerosing panencephalitis in initial stage]

The purpose of the study was to find some characteristic features of cortical defects in the neuropsychological examination of children with SSPE. These findings could be very useful in early differential diagnosis. We analysed the results of the neuropsychological assessment of 13 children selected from a group of 100 patients. The most frequent defects were found in the visuo-spatial functions. The clinical analysis of the obtained results was studied in view to establish, whether there are the signs of the dysfunction of the right or both cerebral hemispheres.