Atypical electroencephalographic pattern in a patient with subacute sclerosing panencephalitis.

Subacute sclerosing panencephalitis (SSPE) has become rare since the widespread use of the measles vaccine. In this patient with a 5 month history of seizures and progressive dementia due to SSPE, the atypical electroencephalographic pattern was characterized by generalized spike-and-wave discharges, maximal on the right, associated with clinical seizures. After diazepam (intravenously), more typical periodic complexes appeared.     

The electroencephalogram in subacute sclerosing panencephalitis.

Electroencephalogram studies of 31 patients with proved subacute sclerosing panencephalitis (SSPE) revealed periodic high-amplitude complexes in all except one. The periodic complexes consisted of two to four high-amplitude delta waves, were usually bisynchronous and symmetrical, and repeated once in five to seven seconds. When both the clinical myoclonic jerks and the periodic EEG complexes were present, a one to one relationship existed between the two phenomena. Besides periodic complexes, several atypical EEG findings were also noted that included frontal rhythmic delta activity in intervals between periodic complexes, electrodecremental periods following EEG complexes, paroxysm of bisynchronous spike wave activity, random spikes over frontal regions, and focal abnormalities, such as spike and slow wave foci. In spite of variability of EEG findings, there is usually no difficulty in making the diagnosis of SSPE if both the EEG and clinical findings are considered.     

Isoprinosine in subacute sclerosing panencephalitis.

An open therapeutic trial of isoprinosine was conducted in 15 patients with subacute sclerosing panencephalitis (SSPE). Long-term remissions occurred in 5 (33 percent), with documented improvement sustained for 2 or more years. Another patient was in remission 9 months after starting treatment, and three patients had transient remissions or stabilization. The disease was unaltered in five patients who had rapidly progressive SSPE when treatment started. These results compare with an average remission rate of about 5 percent in several series of untreated cases of SSPE or in cases treated with other antiviral agents. Patients in remission continued to have elevated cerebrospinal fluid (CSF) IgG and measles antibody titers, with one exception. Isoprinosine was tolerated for several years without side effects, except for mild hyperuricemia.     

Long survival in subacute sclerosing panencephalitis.

A case is reported of a boy who at the age of 14 years developed subacute sclerosing panencephalitis. He deteriorated over a period of 9 months, improved greatly and remained stable for 7 years before relapse. The final deterioration to death extended over 6 years. During the whole period he was examined regularly and the electroencephalogram recorded at 3 to 6 monthly intervals. The brain was examined histologically after death. Electroencephalographic and pathological features are described.     

Inosiplex in the treatment of subacute sclerosing panencephalitis.

Six patients with subacute sclerosing panencephalitis were treated with the antiviral agent inosiplex. The clinical condition of four children continued to deteriorate in spite of the treatment, the condition of one remained unchanged, and that of another improved minimally. The effect of inosiplex in these patients is not evident since this group demonstrated a pattern of progression similar to that seen in subacute sclerosing panencephalitis without specific treatment.     

Measles-vaccinated Israeli boy with subacute sclerosing panencephalitis

Subacute sclerosing panencephalitis is a rare neurologic disorder of childhood and adolescence. We describe a 16-year-old boy who manifested the disease despite proper vaccinations. He was hospitalized because of bedwetting, involuntary limb movements, abnormal speech, and balance disturbances. Immunoglobulin G antibodies against measles were strongly positive, with a high relative cerebrospinal fluid/serum ratio. Polymerase chain reaction for measles produced negative results. Electroencephalography registered slow activity with high voltage discharges every few seconds, and with triphasic complex morphology. Magnetic resonance imaging revealed diffuse white matter changes, mostly around the posterior regions and lateral ventricles. Treatment with valproic acid, levetiracetam, carbamazepine, and intravenous immunoglobulin G was ineffective. Inosiplex and interferon-β-1a were also administrated. The patient became comatose, with generalized myoclonic jerks, and died 1 year later. An autopsy was not performed. This patient illustrates that subacute sclerosing panencephalitis should be suspected among young vaccinated subjects.     

Intrathecal interferon in subacute sclerosing panencephalitis

Three patients at Stage II of subacute sclerosing panencephalitis (SSPE) were treated with semipurified alpha-interferon (IFN) using different combinations of intrathecal and intravenous routes: 1 x 10(6) IU of alpha-IFN were given every other day up to a total of 15 x 10(6) IU. Transient improvement of neurological symptoms and electroencephalogram were noted in all 3, while cognitive function slightly improved in 2 of them. Clinical benefits gradually disappeared 2 to 6 months after cessation of IFN. Intrathecal antibody production did not change substantially, but CSF Leu 3a/Leu 2a ratio appeared to increase. No significant side effects were observed, except for a mild meningeal inflammatory reaction after each intrathecal administration of IFN.     

Computed tomographic findings in subacute sclerosing panencephalitis.

We used a computed tomography to look for evidence of cerebral changes in a three-year-10-month-old girl with SSPE. The area of decreased density on CT corresponded to inflammatory and demyelinative lesions in the brain. CT scans seem to be a useful tool for more accurate assessment in the diagnosis of SSPE.     

Atypical EEG findings in subacute sclerosing panencephalitis.

OBJECTIVE: To evaluate atypical electroencephalographic features in subacute sclerosing panencephalitis (SSPE) and to detect its relation to clinical features. METHODS: Twenty-two patients aged 2-17 years (mean 9.4 years) with definite diagnosis of SSPE were studied. Their clinical data and EEG records were reviewed retrospectively. All EEG records were analysed for features of periodic complexes (PCs) in relation to age, age at onset, clinical stage and the rate of progression as well as duration of the disease. RESULTS: Classical periodic complexes of SSPE were found in EEGs of 13 patients (group I). Atypical patterns were observed in EEGs of nine patients (group II). Two new atypical findings were identified: prolonged discharges which include sharp waves and slow waves for 4-7 s followed by suppression for 1-4s; and periodic complexes which consist of four or five sharp waves in every 2 s. We observed atypical EEG patterns were more frequently in Stage III, acute form, and the disease duration was longer than in the typical group. CONCLUSIONS: Atypical EEG patterns in SSPE might be related to the progression of the disease, but this theory needs further longitudinal studies. SIGNIFICANCE: We suggest atypical EEG patterns might be observed more frequently in patients with severe neurologic disability, more rapidly progressive disease and longer duration of disease.     

Fulminant subacute sclerosing panencephalitis in association with pregnancy

Subacute sclerosing panencephalitis developed during pregnancy in a 27-year-old woman and immediately after delivery in an 18-year-old woman. In both, disease took an acute and fulminant course culminating in a vegetative state within several weeks. It is suggested that the relative older age of disease presentation and the unusually rapid neurologic deterioration were partially due to immunologic and hormonal alterations of pregnancy.     

Radionuclide imaging in subacute sclerosing panencephalitis

Three patients with subacute sclerosing panencephalitis (SSPE)--two with acute disease and one with an exacerbation--had abnormal radionuclide brain scans during periods of rapid neurologic deterioration. In two of the three patients radionuclide brain scan showed lesions of both cortex and deeper structures, indicating the panencephalic nature of the disease. There was no contrast enhancement on computerized tomography (CT) in the areas of radiopharmaceutical accumulation in the two patients studied. We feel that delayed radionuclide scanning is more sensitive in detecting acute SSPE than routine contrast-enhanced CT, because more time is allowed for tracer accumulation in lesions and for background activity to decrease.     

Intrathecal interferon in subacute sclerosing panencephalitis

Five patients with clinically advanced subacute sclerosing panencephalitis (SSPE) were given human leukocyte interferon (IFN) by the lumbar route, 1 million IU every other day for a total of 30 days. Intrathecal IFN produced a meningeal inflammatory reaction in all patients and was associated with transient hemiparesis in 1. It persisted in the cerebrospinal fluid at measurable levels for 48 hours after a single injection. Although improvement was temporally related to intrathecal IFN in 1 patient, it is not clear whether this was induced by IFN or a spontaneous remission. A randomized controlled trial would be necessary to evaluate IFN critically as a therapy for SSPE.     

Coincidental fluctuations of humoral immunity and clinical progression in a patient with subacute sclerosing panencephalitis

Summary Serum and cerebrospinal fluid were obtained from a 6-year-old male with subacute sclerosing panencephalitis (SSPE). Specimens were collected over a 9-month period beginning in the unusually acute phase and ending in a more quiescent phase of the disease. Immune complexes, auto-antibodies and viral antibodies were measured by radio-immunoassays. Fluctuations in these humoral immune parameters coincided with cessation of the acute phase of this disease. The results show that neurological changes in SSPE patients can be reflected in immune responses within both the peripheral circulation and the central nervous system.