EEG IN SSPE

Dr . K Anuroop
• Subacute sclerosing panencephalitis (SSPE)—also known
as Dawson disease—is a rare form of chronic progressive
brain inflammation caused by slow infection with certain
defective strains of hypermutated measles virus.
• The condition primarily affects children, teens, and young
adults. It has been estimated that about 1 in 10,000 people who
get measles will eventually develop SSPE
• However, a 2016 study estimated that the rate for babies who
contracted measles was as high as 1 in 609.
• No cure for SSPE exists, and the condition is almost always
fatal.
• SSPE should not be confused with
acute disseminated encephalomyelitis, which can also be
caused by the measles virus, but has a very different timing and
course
• SSPE is characterized by a history of primary measles infection,
followed by an asymptomatic period that lasts 7 years on
average but can range from 1 month to 27 years.
• After the asymptomatic period, progressive neurological
deterioration occurs, characterized by behavior change,
intellectual problems, myoclonic seizures, blindness, ataxia, and
eventually death.
• Symptoms progress through the following 4 stages:
• Stage 1: There may be personality changes, mood swings, or
depression. Fever and headache may also be present. This stage
may last up to 6 months.
• Stage 2: This stage may involve jerking, muscle spasms, seizures,
loss of vision, and dementia.
• Stage 3: Jerking movements are replaced by writhing (twisting)
movements and rigidity. At this stage complications may result in
death.
• Stage 4: The final stage, in which breathing, heart rate, and blood
pressure are affected, leading to coma and death.
• This neurodegenerative complication of measles virus infection in
childhood is worthy of mention, being one of very few disorders in
which the EEG shows relatively specific or pathognomonic changes.
• The characteristic EEG picture is of stereotyped high voltage periodic
complexes, usually generalised or bilateral .
• Morphology of the complexes is highly stereotyped within an
individual, but differs between patients.
• The periodicity varies from a few to many seconds, with a gradual
reduction of the interval between complexes, and eventual
disappearance of complexes as disease progresses.
• Background cerebral activity between complexes is normal initially,
with increasing slow activity and then attenuation in later stages.
• The complexes are usually associated with myoclonic jerks (the
myoclonus may be negative).
• Most SSPE presents in children and adolescents. Adult onset cases are
rare, but clinical and electrographic features can be atypical—
prominent early visual symptoms, lack of dementia, and a slow EEG
without stereotyped complexes.
• An awake electroencephalogram (international 10–20
system, average montage, sensitivity – 20 μV/mm and
sweep speed – 30 mm/s, low frequency filter – 1 Hz and
high frequency filter – 70 Hz) revealed periodic generalized
complexes consisting of bilaterally symmetrical, high-
voltage (>400 μV) bursts of polymorphic delta-waves with
sharps and background slowing and recurring every 4–8 s
• SSPE is an inflammatory disease occurring in children and
adolescents, believed to be caused by the measles virus and which is
characterized by abnormal movements, a progressive intellectual
deterioration, and a diagnostic electroencephalographic pattern.
• The characteristic electroencephalographic pattern consists of high-
voltage (300 to 1500 mv) repetitive polyphasic and sharp- and slow-
wave complexes ranging from 0.5 to 2 see in duration, usually
recurring every 4 to 15 see.
• On rare occasions, these complexes may occur at intervals ranging up
to 1 to 5 min. The periodic complexes may be present at any stage of
the disease, but they usually are seen during the intermediate stages.
• Although the form and appearance of the periodic complexes is fairly
constant and stereotyped in a single recording, the shape of the
complexes varies in different patients and can change from time to
time in the same patient at different stages of the disease process.
• The complexes are usually generalized and bisynchronous, but at
times they may be asymmetric, have a time lag from side to side or
front to back, or occur in a more lateralized or focal fashion,
particularly in the earlier stages of the disease.
• Initially, the complexes may occur at irregular intervals, but, once
established, the complexes recur at regular intervals, although the
repetition rate may vary during the course of the disease.
• Afferent stimuli do not usually affect the periodic complexes;
however, on rare occasions, the complexes can be evoked by external
stimuli.
• This occurs when the complexes are present in an inconstant manner,
either when they first make their appearance or toward the end of
the period of remission.
• Once the regular pattern of the complexes has been established,
however, the complexes are no longer influenced by external stimuli.
• Drugs usually have little effect on the periodic complexes, although
one report described the occurrence of periodic pattern after an
intravenous injection of diazepam.
• A prominent feature of SSPE is the stereotyped motor jerks or spasms
occurring with the periodic complexes.
• The movements are often described as myoclonic jerks; however, they
do not have the momentary lightning-quick nature of true myoclonus;
instead, the movements consist of an initial "shock-like abruptness,"
followed by a momentary arrest of the movement, and then a
gradually melting away to the position of rest.
• On less frequent occasions, the periodic complexes may be associated
with an inhibitory phenomenon such as an arrest of movement, loss
of tone, or drop attacks. The abnormal movements usually become
evident about the same time that the periodic complexes appear on
the EEG, however, on occasion, and particularly in the early stages of
the disease, the periodic complexes may be present without the
associated motor movements.
• On the other hand, the presence of the MOTOR jerks in the absence
of the periodic complexes is uncommon.
• The motor movements often disappear during sleep, despite a
persistence of the periodic complexes.
• Certain drugs, such as diazepam, may reduce or abolish the motor
movements without altering the electroencephalographic complexes.
• The resting EEG may be relatively normal when the complexes first
appear. As the disease evolves, however, the EEG shows various
changes, consisting of slowing and disorganization of the background,
an asymmetry of the background activity, or both.
• These changes are followed by an increase in the slow-wave
abnormalities, usually occurring in a diffuse manner but at times
having a focal or lateralized emphasis and coinciding with the area of
maximal neurologic involvement.
• Early in the course of the disease, the electroencephalogram (EEG)
may be normal or show only moderate, non-specific generalised
slowing. The typical EEG pattern is usually seen in myoclonic phase
and is virtually diagnostic. The EEG picture is characterised by
periodic complexes consisting of bilaterally symmetrical,
synchronous, high voltage (200–500 mv) bursts of polyphasic,
stereotyped delta waves. Waveforms remain identical in any given
lead.
• These periodic complexes repeat at fairly regular 4–10 second
intervals and have 1:1 relationship with myoclonic jerks .
• Frequently there is shortening of interval between periodic complexes
with progression of the disease.
• The periodic complexes of SSPE first appear during sleep, when they
are not accompanied by myoclonic spasms.
• Often these periodic complexes can be brought out when the patient is
awake, if diazepam is administered intravenously during the routine
electroencephalographic recording.
• Late in the course of disease, the EEG may become increasingly
disorganised and show high amplitudes and random dysrhythmic
slowing.
• In terminal stages the amplitude of waveforms may fall.
• In addition to type I periodic electroencephalographic complexes just
described, few other forms of periodic complexes have also been
recognised.
• These various types of periodic complexes have been shown to have
some association with the prognosis of the disease.
• Type II abnormalities are characterised by periodic giant delta waves
intermixed with rapid spikes as fast activity.
• In this pattern of periodic complexes, EEG background is usually
slow.
• The type III periodic complexes pattern is characterised by long
spike-wave discharges interrupted by giant delta waves.
• Yakub demonstrated that video-split EEG monitoring is a more
sensitive technique for early diagnosis and detection of atonia or
myoclonus, which are time related to EEG periodic complexes.
• He further observed that type III periodic complexes were associated
with the worst outcome, while patients with type II periodic
complexes had the best outcome.
• In this study outcome was determined by the rate of progression of
disease.
• . In the later stages of the disease, polymorphic delta activity or
intermittent frontal dominant monorhythmic slow-wave activity may
be present. On occasion, there may be a transient flattening Or
attenuation of activity after the periodic complexes.
• Various types of epileptiform discharges, spikes, sharp waves, or
spike-and-wave complexes occurring in a focal or generalized fashion
also may be present.
• Patients who have a remission or an improvement in the clinical state
show a corresponding improvement on the EEG.
• The typical stages of sleep become less recognizable as the disease
progresses, and identifiable sleep stages become limited to two main
types.
• These are a low-voltage fast pattern with or without spindle activity
and a high- voltage slow-wave pattern.
• In the later stages of the disease, sleep spindles, V waves, and K
complexes disappear and the electroencephalographic differentiation
of the various stages of sleep is no longer possible.
• The periodic complexes often persist during sleep, al- though their
shape and frequency may be modified. On rare occasions, periodic
complexes may be activated or occur predominantly during the sleep
recording.
• In the final stages of the disease, there is often a reduction in
amplitude and abundance of the electroencephalographic activity and
the recording may become almost isoelectric.
• In some instances, however, alpha activity may still be present shortly
before death.
• Although other entities may be associated with a periodic pattern, the
stereotyped electroencephalographic complexes occurring in a
regular and periodic fashion and having a constant relationship to
motor movements make this pattern one of the most characteristic
and specific of all electroencephalographic patterns.
• Close attention to the EEG and clinical features aid in the diagnosis of
SSPE and distinguish it from other types of encephalopathies or
disease entities.
• EEG in various stages of SSPE.
• A: The background activity is disorganized with few repetitive high
voltage delta bursts with fronto-central amplitude accentuation in the
early stages of the disease.
• B: Typical "metronomic" delta burst pattern with almost flat amplitude
during burst intervals recorded during the advanced stages of SSPE C:
A uniform pattern of monomorphic slow waves at a very advanced
stage of SSPE. stage II while diffuse fronto-parteital changes were
more common in stage II-III.
• At early stages of the disease, the EEG may be normal or show
moderate non-specific generalized slowing.
• As the diseases progresses, the periodic discharge pattern emerges, at
first only during sleep, before myoclonic jerks are evident.
• In early stages, administration of IV diazepam can bring out the typical
bursts.
• Somewhat later in the course and concomitantly with the appearance
of typical myoclonus, the characteristic periodic discharge pattern can
be seen also during wakefulness.
• This pattern consists of bilateral synchronous symmetrical bursts with
an amplitude of 200-500 mV. Each burst consists of polyphasic,
monomorphic delta waves.
• The bursts appear every 4-10 seconds with an identical morphology in
any recording lead and are rhythmic ("metronomic"). The bursts are
"time locked" with the myoclonic jerks.
• The interval between the bursts becomes shorter with disease
progression. In advanced stages this pattern is replaced by
disorganized slow delta rhythm.
• The voltage of the recording gets smaller and at stage IV it may
became almost iso- electric
• In a relatively recent study from India (Praveen-Kumar et al., 2007),
the EEG features of 58 patients with SSPE (stage I: 10.2%; II: 64%; III:
25.8%), were analyzed and related to the stage of disease and
neuroimaging findings.
• Bursts as defined above were found in 98% of the patients. Those
were periodic in 55%, "quasi-periodic" in 36% and non-periodic in 6%.
The periodic bursts were asymmetrical in 33%.
• . The burst morphology, frequency and inter-burst interval were not
influenced by hyperventilation, photic or sensory stimulation. Normal
background was recorded in all patients in stage I, and slow
background in all other stages.
• Spike and sharp wave epileptiform discharges were present in 72.4%
of the patients. Those were mostly generalized and only in 10 patients
epileptiform activity was lateralized or multifocal.
• The focal discharges were mostly fronto-parietal.
• Periodic Lateralizing Epileptic Discharges (PLED's), were present in 10
recordings. Asymmetry of background activity or bursts were
associated with lateralizing neurological deficits.
• However, there was no correlation between the EEG asymmetry and
CT or MRI findings
• Diagnostic Criteria SSPE can be diagnosed if the patient fulfills three of
the following five criteria:
• (a) typical clinical presentation;
• (b) typical electroencephalographic changes with stereotyped periodic
complexes;
• (c) typical histo-pathological finding in brain biopsy or autopsy;
• (d) elevated CSF globulin levels, greater than 20% of total CSF protein;
and
• (e) elevated CSF measles antibody titers of 1/4 or more by
hemagglutination inhibition method, or 1/20 or more by ELISA(25)
• . Many atypical findings like rhythmic delta or spike wave activity over
the frontal regions are reported. The interval in between the periodic
discharge further shortens with the progression of the disease .
• Triphasic waves (TWs) are distinct but nonspecific
electroencephalographic (EEG) pattern described as “blunted spike
and wave”.
• They are found in metabolic encephalopathy, toxic or hypoxic causes
• Triphasic waves are rarely reported in SSPE but definitely mention in a
few studies .
• Hypoxic or unknown metabolic pathology could be the reason for
these waves and usually seen in the end stage of the illness where
anoxic brain injury may set in
• Nonconvulsive Status Epilepticus on Electroencephalography: An
Atypical Presentation of Subacute Sclerosing Panencephalitis
• Scalp electroencephalogram showing disorganized background with
generalized spike-wave discharges 2–2.5/second occupying more than
80% of tracing, consistent with nonconvulsive status epilepticus
• Scalp electroencephalogram showing 1-2 Hz generalized synchronous
spike-wave discharges occupying more than 80% of tracing, consistent
with nonconvulsive status epilepticus
• subacute sclerosing panencephalitis presenting as depression
• The presence of normal EEG in the early stage makes clinicians even
more complacent about suspecting a diagnosis of SSPE.
• The typical EEG findings of SSPE appear in stage II of the disease. EEG in
a typical case in stage II is characterized by high amplitude 300–1500
microvolts repetitive polyphasic sharp and slow wave complexes of 0.5–
2 s duration, recurring every 4–15 s synchronously with myoclonic jerks .
• EEG in the end stages of the disease again becomes atypical, as the
background and sleep activities are disorganized, and there is a gradual
reduction in the amplitude till it finally becomes isoelectric.
• ATYPICAL PRESENTATION OF SSPE WITH ADEM
• Atypical form of SSPE occurs in about 10% of all patients. Unlike
classical SSPE, in atypical form there are no defined stages in clinical
presentation due to rapid course.
• Atypical features also include unusual age of onset, visual loss,
seizures and other focal symptoms as initial presentations, a lack of
SSPE-specific EEG pattern, and atypical fast progression of disease.
• As rare complication of measles, SSPE is difficult to diagnose & stamp.