Activation of periodic complexes of subacute sclerosing panencephalitis by sleep

Abstract

Three patients with clinically proved subacute sclerosing panencephalitis (SSPE) had electroencephalograms i n which periodic stereotyped complexes characteristic of SSPE were apparent d u r i n g sleep b u t were not observed d u r i n g the initial awake recordings. The first patient had periodic complexes only d u r i n g sleep. The second and third patients had complexes that were inconstant and sporadic during the awake recordings b u t regular and periodic d u r i n g sleep. Although sleep c a n have a variable effect on t h e electroencephalographic pattern in SSPE, it may facilitate elicitation of typical periodic complexes In questionable cases of SSPE.Westmoreland BF, Gomez MR, Blume W T Activation of periodic complexes of subacute sclerosing panencephalitis by sleep Ann Neurol 1 [185][186][187] 1977 The electroencephalogram in subacute sclerosing panencephalitis (SSPE) shows a characteristic pattern of periodic stereotyped complexes that is usually diagnostic for SSPE. The patient, however, may n o t always present with t h e typical EEG pattern. T h i s r e p o r t describes 3 patients i n whom the characteristic periodic pattern of SSPE became apparent only w h e n a sleep recording was performed. Case ReportsThe first patient, a 13-year-old boy, was well until five months before being seen at our clinic, when he had the onset of progressive deterioration in intellect and behavior and episodes of falling to the ground without warning. Laboratory studies showed increased protein in the cerebrospinal fluid, with an elevated gamma globulin level and measles antibody titer. The initial EEG showed 6 to 7 Hz slowing with frequent trains of generalized and bilaterally synchronous 2 to 2.5 Hz spike-and-wave discharges. O n the following day a second tracing was obtained during which the patient was given 10 mg of diazepam intravenously; 15 minutes later he fell asleep, and generalized, periodic, and stereotyped slow waves at intervals of 2 to 3 seconds appeared ( Figure). This periodic pattern persisted throughout the sleep recording. After the patient woke up the periodic complexes disappeared, and the EEG again showed diffuse theta slowing. (This patient has been reported in more detail elsewhere [I]). In the second patient, a 13-year-old girl, progressive ataxia, deterioration in intellectual functioning, dysarthria, and athetoid movements of the extremities had developed four months before she was seen at our clinic. Laboratory studies showed elevated protein in the cerebrospinal fluid with an increased gamma globulin level and an elevated measles antibody titer. The awake EEG showed diffuse 4 to 6 Hz slowing with occasional sporadic, higher-amplitude, 2 to 3 Hz waveforms. During the recording the patient spontaneously wenr to sleep, at which timegeneralized, periodic, stereotyped slow-wave complexes, recurring every 1 0 seconds, became apparent. These were present throughout the sleep recording but disappeared after arousal from sleep. Four days later, periodic complexes were present during the awake...

Pearls, Perils, and Pitfalls in the Use of the Electroencephalogram

“…A sleep recording, therefore, is recommended in a suspected case of SSPE in which the awake tracing has failed to reveal periodic complexes. 48 Also, in the early stages there is asymmetry of the periodic complexes, which may be associated with asymmetry of the myoclonic jerks that occur contralateral to the periodic complexes. 47 Later in the disease, the periodic complexes are bilaterally symmetrical and synchronous.…”

Subacute sclerosing panencephalitis in a child with human immunodeficiency virus (HIV) infection on antiretroviral therapy

“…In contrast, our patient with WHO clinical stage IV HIV infection (past CMV retinitis) and SSPE has been stable in clinical Jabbour's stage II[ 8 ] for about seven months and so far seems to follow the natural typical course of SSPE, as described in most immunocompetent individuals. The classic periodic complexes as described by Cobb[ 9 ] (1966) occurs every 4-15 seconds, although there has been various reports (Westmoreland et al ., 1977)[ 10 ] where it occur every 1-5 minutes, depending on the stage and progression of disease. Our patients EEG showed periodic complexes, which were occurring every 18 seconds.…”