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PERSONAL PRACTICE
tive in eradicating the virus. So, M-protein the meninges and in the brain parenchyma.
is necessary for correct assembly of pro- Inclusion bodies are seen within both
geny virus at the surface of infected cells, nucleus and cytoplasm of neurons and glial
mutations in this protein lead to an anti- cells. Cowdry type-A inclusion bodies con-
genically distinct form, that can no longer sisting of homogenous eosinophilic materi-
bind to viral nucleocapsid that initiate al, are diffusely seen in neurons and oligo-
virus maturation. The absence of function- dendroglia in patients with rapidly pro-
ally intact, budding virus particle, results gressive fatal disease. Another, Cowdry
in intracellular accumulation of incomplete type-B inclusion bodies are small and mul-
measles virus in brain cells(7-12). tiple, and are almost always present in
brainstem. These later type of inclusions
In addition, modulation of measles anti-
are less often seen. Late in the course of the
gen on the surface of infected cells by
disease it may be difficult to find typical
antimeasles antibody has been described,
and this modulation might make the cell areas of inflammation, and the main
vulnerable to attack by the immune system histopathological changes are marked with
parenchymal necrosis and gliosis(1,14,15).
and could alter expression of virus within
the cell. It is also possible that antigenic Measles viral antigens can be demon-
challenge of a second infection could alter strated by labeled antibody technique with-
the dormant state of SSPE virus and result in the inclusions as well in cells without in-
in disease expression. Sero-epidemiological clusions. Data from several studies suggest
data suggest that an exposure to another that the endothelial cells in the brain are
virus, such as Epstein-Barr virus or influen- frequently infected during acute fatal mea-
za type I virus may transform the measles sles. This site of infection may provide a
virus into a defective virus(13,14). portal of entry for virus in persons who
subsequently develop SSPE or measles in-
Pathology clusion body encephalitis(16,17).
Brain biopsy performed in the early Clinical Features and Classification
stages of SSPE shows mild inflammation of
the meninges and brain parenchyma in- The clinical features and time course of
volving cortical and subcortical grey matter SSPE are highly variable. The initial symp-
as well as white matter, with cuffs of plas- toms are subtle and include intellectual de-
ma cells and lymphocytes around blood terioration and behavioral changes without
vessels. In the subsequent stage, gross neurological signs or findings. As disease
examination of brain may reveal mild progresses non-specific manifestations give
to moderate atrophy of cerebral cortex. way to marked disturbance in motor func-
Microscopic examination shows wide- tion and the development of periodic
spread degeneration of neurons and dis- myoclonic jerks. Myoclonic jerks initially
organization in the cortex. Parieto-occipital involve the head and subsequently trunk
region of cerebral hemisphere is most and limbs. Muscular contraction is fol-
severely affected; subsequently, it spreads lowed by 1 to 2 seconds of relaxation
to the anterior portions of cerebral hemi- associated with a decrease in muscle action
sphere, the subcortical structrures, brain- potential or complete electrical silence. The
stem and spinal cord. Focal or diffuse myoclonic jerks do not interfere with con-
perivascular infiltrates of lymphocytes, sciousness. They are exaggerated by excite-
plasma cells and phagocytes are present in ment, and may disappear during sleep.
338
INDIAN PEDIATRICS VOLUME 35-APRIL 1998
Initially they are infrequent and might be logical signs, partial seizures, or papill-
regarded as stumbling or clumsiness. At edema, these findings can lead to an erro-
this stage seizures may occur. Macular neous diagnosis of an intracranial space-
chorioretinitis is seen in up to 50% of cases. occupying,lesion.
Retinopathy and optic atrophy may devel-
op in addition to cerebellar ataxia and Cerebrospinal Fluid (CSF)
dystonia. Few patients may present with The CSF pattern may be diagnostic.
cortical blindness. The late stage of the dis- The fluid is usually cellular with a normal
ease is marked by stupor and coma, auto- or mildly elevated protein level and a
nomic failure with loss of thermoregulation markedly elevated gamma-globulin level
leading to temperature fluctuations and (comprising at least 20% of total CSF
disturbed sweating. In late stages, protein)(21,22).
myoclonic jerks diminish in intensity and
eventually disappear. In majority of cases Electroencephalography (EEG)
death occurs between 1 and 3 years after
onset of symptoms. Ten per cent of the pa- Early in the course of disease, the EEG
tients have a fulminant course, dying with- may be normal or show only moderate,
in months, and 10 per cent survive for 4-10 non-specific slowing. The typical EEG pat-
years with extended periods of stabiliza- tern is usually seen in myoclonic phase and
tion. Spontaneous improvement may also is diagnostic. EEG picture is characterized
occur during any of the stage of the disease by periodic complexes consisting of bi-
and may last for a variable period of time laterally symmetrical, synchronous, high-
before eventual relapse occurs(5,18,19). voltage (200-500mv) bursts of polyphasic
stereotyped delta waves. They remain
Staging of SSPE is done according to identical in any given lead. These periodic
modified Jabbour classification(20), as fol- complexes repeat at fairly regular 4 to 10
lows: Stage-I: mental and behavioral second intervals and have a 1 :1 relation-
changes, forgetfulness, irritability and leth- ship with myocolonic jerks (Fig. 1). Earlier
argy; Stage-II: mycolonic jerks, dyskinesia, each complex lasts between 0.5 and 2 sec-
choreoathetosis, ataxia; Stage-Ill: decer- onds, may recur as infrequently as every 5
brate rigidity and decorticate rigidity; and minutes. These complexes may be present
Stage-IV: severe loss of all cortical function, only during sleep, and may be elicited
flexion posturing of limbs and mutism. by afferent stimuli. Later in the course
of illness, the EEG becomes increasingly
Diagnosis disorganized and shows high-amplitude,
Despite characteristic signs and symp- random dysrhytmic slowing, in terminal
toms early diagnosis of SSPE is not always stages the amplitude may fall(23-25).
easy, early detection of myoclonus is im- In addition to type-I EEG abnormalities
portant to make reasonable diagnosis. Sub- just described, few other types of EEG ab-
tle behavioral changes are easily missed normalities have also been recognized
by the relatives and these patients are often which have some effect on prognosis of the
treated by a psychiatrist at this stage. In disease. Type-II abnormalities are charac-
some cases myoclonus is not present in the terized by periodic giant delta waves inter-
early stages, only atonia may be present mixed with rapid spikes as fast activity.
and can be overlooked. Occasionally, the EEG background is usually slow. Type-Ill
patient can demonstrate lateralizing neuro- EEG pattern in patients of SSPE is charac-
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INDIAN PEDIATRICS VOLUME 35-APRIL 1998
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INDIAN PEDIATRICS VOLUME 35-APRIL 1998
7. Hall WW, Choppin PW. Evidence for the 16. Vani KR, Yasha TC, Rao TV, Das S, Ravi
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