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Nonconvulsive status epilepticus on electroencephalography: an atypical presentation of subacute sclerosing panencephalitis in two children.

Singhi P, Saini AG, Sahu JK - Case Rep Pediatr (2012)

Bottom Line: Subacute sclerosing panencephalitis is a neurodegenerative disease secondary to measles infection that usually has a typical presentation with progressive myoclonia, cognitive decline, and periodic slow-wave complexes on electroencephalography.Both had elevated measles antibodies in cerebrospinal fluid and blood.Pediatricians need to be aware of this atypical presentation of subacute sclerosing panencephalitis.

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatrics, Advanced Pediatric Center, Postgraduate Institute of Medical Education and Research, Chandigarh 160012, India.

ABSTRACT
Subacute sclerosing panencephalitis is a neurodegenerative disease secondary to measles infection that usually has a typical presentation with progressive myoclonia, cognitive decline, and periodic slow-wave complexes on electroencephalography. We report two pediatric cases who presented with periodic myoclonic jerks and cognitive decline. In both cases, the electroencephalogram showed continuous nonconvulsive status epilepticus activity. Both had elevated measles antibodies in cerebrospinal fluid and blood. Pediatricians need to be aware of this atypical presentation of subacute sclerosing panencephalitis.

No MeSH data available.


Related in: MedlinePlus

Magnetic resonance imaging of the brain (fluid attenuated inversion recovery sequence, coronal section) showing periventricular hyperintensities involving bilateral parieto-occipital white matter (Case  1).
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fig2: Magnetic resonance imaging of the brain (fluid attenuated inversion recovery sequence, coronal section) showing periventricular hyperintensities involving bilateral parieto-occipital white matter (Case  1).

Mentions: A clinical diagnosis of SSPE was considered in view of periodic myoclonus and progressive cognitive decline. Scalp electroencephalogram showed disorganized background with generalized spike-wave discharges 2–2.5/second occupying more than 80% of tracing, consistent with NCSE (Figure 1). Following intravenous diazepam (0.3 mg/kg) administration, transient normalization of background rhythm to 6–8/second along with intermittent, generalized high-voltage slow-wave discharges was seen, but typical periodic complexes were not seen. There was no change in the mental status of the child following diazepam. Magnetic resonance imaging of brain (fluid attenuated inversion recovery sequence) showed periventricular hyperintensities involving bilateral parieto-occipital white matter (Figure 2). Measles antibody titers by enzyme-immunoassay were raised in both cerebrospinal fluid and serum: 5.24 IU/mL (positive >1.1) and 5.11 IU/mL (positive >1.1), respectively. A diagnosis of SSPE was made with clinical stage IIIA according to Jabbour's criteria [1]. Child was started on valproic acid (30 mg/kg/day) and clonazepam (0.5 mg/kg/day). At four months follow-up, the child showed some improvement in sensorium and myoclonic jerks with electroencephalogram now showing the typical periodic complexes time locked with periodic myoclonus.


Nonconvulsive status epilepticus on electroencephalography: an atypical presentation of subacute sclerosing panencephalitis in two children.

Singhi P, Saini AG, Sahu JK - Case Rep Pediatr (2012)

Magnetic resonance imaging of the brain (fluid attenuated inversion recovery sequence, coronal section) showing periventricular hyperintensities involving bilateral parieto-occipital white matter (Case  1).
© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3472538&req=5

fig2: Magnetic resonance imaging of the brain (fluid attenuated inversion recovery sequence, coronal section) showing periventricular hyperintensities involving bilateral parieto-occipital white matter (Case  1).
Mentions: A clinical diagnosis of SSPE was considered in view of periodic myoclonus and progressive cognitive decline. Scalp electroencephalogram showed disorganized background with generalized spike-wave discharges 2–2.5/second occupying more than 80% of tracing, consistent with NCSE (Figure 1). Following intravenous diazepam (0.3 mg/kg) administration, transient normalization of background rhythm to 6–8/second along with intermittent, generalized high-voltage slow-wave discharges was seen, but typical periodic complexes were not seen. There was no change in the mental status of the child following diazepam. Magnetic resonance imaging of brain (fluid attenuated inversion recovery sequence) showed periventricular hyperintensities involving bilateral parieto-occipital white matter (Figure 2). Measles antibody titers by enzyme-immunoassay were raised in both cerebrospinal fluid and serum: 5.24 IU/mL (positive >1.1) and 5.11 IU/mL (positive >1.1), respectively. A diagnosis of SSPE was made with clinical stage IIIA according to Jabbour's criteria [1]. Child was started on valproic acid (30 mg/kg/day) and clonazepam (0.5 mg/kg/day). At four months follow-up, the child showed some improvement in sensorium and myoclonic jerks with electroencephalogram now showing the typical periodic complexes time locked with periodic myoclonus.

Bottom Line: Subacute sclerosing panencephalitis is a neurodegenerative disease secondary to measles infection that usually has a typical presentation with progressive myoclonia, cognitive decline, and periodic slow-wave complexes on electroencephalography.Both had elevated measles antibodies in cerebrospinal fluid and blood.Pediatricians need to be aware of this atypical presentation of subacute sclerosing panencephalitis.

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatrics, Advanced Pediatric Center, Postgraduate Institute of Medical Education and Research, Chandigarh 160012, India.

ABSTRACT
Subacute sclerosing panencephalitis is a neurodegenerative disease secondary to measles infection that usually has a typical presentation with progressive myoclonia, cognitive decline, and periodic slow-wave complexes on electroencephalography. We report two pediatric cases who presented with periodic myoclonic jerks and cognitive decline. In both cases, the electroencephalogram showed continuous nonconvulsive status epilepticus activity. Both had elevated measles antibodies in cerebrospinal fluid and blood. Pediatricians need to be aware of this atypical presentation of subacute sclerosing panencephalitis.

No MeSH data available.


Related in: MedlinePlus