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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

Scalp electroencephalogram showing 1-2 Hz generalized synchronous spike-wave discharges occupying more than 80% of tracing, consistent with nonconvulsive status epilepticus (Case  2).
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fig3: Scalp electroencephalogram showing 1-2 Hz generalized synchronous spike-wave discharges occupying more than 80% of tracing, consistent with nonconvulsive status epilepticus (Case  2).

Mentions: A previously well, six-year-old boy was admitted with frequent myoclonic jerks, progressive cognitive decline, and loss of ambulation for the last one year. He had measles infection at two years of age and was not vaccinated. On examination, he demonstrated periodic jerks as sudden flexion of the neck and arms over the trunk, associated with lapse in consciousness. He had dysphagia, slurred speech, and poor interaction with the examiner. There was generalized spasticity, hyperreflexia, and bilateral extensor plantar response. Fundus examination revealed bilateral optic atrophy. Scalp electroencephalogram showed 1-2 Hz generalized, synchronous, spike-wave discharges occupying more than 80% of tracing, consistent with NCSE (Figure 3). Following intravenous diazepam (0.3 mg/kg) administration, the spike-wave discharges disappeared transiently, but periodic complexes did not occur. Also, no change in mental state was noticed. Measles antibody titers by enzyme-immunoassay were raised in both cerebrospinal fluid and serum: 1 : 625 (positive >1 : 4) and 1 : 625 (positive >1 : 256), respectively. Magnetic resonance imaging of brain (fluid attenuated inversion recovery sequence) showed periventricular hyperintensities involving predominantly occipital brain. A diagnosis of SSPE was made with clinical stage IIIA [1]. Child was started on valproic acid (30 mg/kg/day) and clonazepam (0.03 mg/kg/day). At three month follow-up, he showed some improvement in sensorium and reduction of myoclonus, but NCSE persisted on electroencephalography.


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)

Scalp electroencephalogram showing 1-2 Hz generalized synchronous spike-wave discharges occupying more than 80% of tracing, consistent with nonconvulsive status epilepticus (Case  2).
© Copyright Policy
Related In: Results  -  Collection

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

fig3: Scalp electroencephalogram showing 1-2 Hz generalized synchronous spike-wave discharges occupying more than 80% of tracing, consistent with nonconvulsive status epilepticus (Case  2).
Mentions: A previously well, six-year-old boy was admitted with frequent myoclonic jerks, progressive cognitive decline, and loss of ambulation for the last one year. He had measles infection at two years of age and was not vaccinated. On examination, he demonstrated periodic jerks as sudden flexion of the neck and arms over the trunk, associated with lapse in consciousness. He had dysphagia, slurred speech, and poor interaction with the examiner. There was generalized spasticity, hyperreflexia, and bilateral extensor plantar response. Fundus examination revealed bilateral optic atrophy. Scalp electroencephalogram showed 1-2 Hz generalized, synchronous, spike-wave discharges occupying more than 80% of tracing, consistent with NCSE (Figure 3). Following intravenous diazepam (0.3 mg/kg) administration, the spike-wave discharges disappeared transiently, but periodic complexes did not occur. Also, no change in mental state was noticed. Measles antibody titers by enzyme-immunoassay were raised in both cerebrospinal fluid and serum: 1 : 625 (positive >1 : 4) and 1 : 625 (positive >1 : 256), respectively. Magnetic resonance imaging of brain (fluid attenuated inversion recovery sequence) showed periventricular hyperintensities involving predominantly occipital brain. A diagnosis of SSPE was made with clinical stage IIIA [1]. Child was started on valproic acid (30 mg/kg/day) and clonazepam (0.03 mg/kg/day). At three month follow-up, he showed some improvement in sensorium and reduction of myoclonus, but NCSE persisted on electroencephalography.

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