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Rasmussen's encephalitis presenting as focal cortical dysplasia.

O'Rourke DJ, Bergin A, Rotenberg A, Peters J, Gorman M, Poduri A, Cryan J, Lidov H, Madsen J, Harini C - Epilepsy Behav Case Rep (2014)

Bottom Line: Seizures are usually the hallmark of presentation, but antiepileptic drug treatment fails in most patients and is ineffective against epilepsia partialis continua, which often requires surgical intervention.Co-occurrence of focal cortical dysplasia has only rarely been described and may have implications regarding pathophysiology and management.We describe a rare case of dual pathology of Rasmussen's encephalitis presenting as a focal cortical dysplasia (FCD) and discuss the literature on this topic.

View Article: PubMed Central - PubMed

Affiliation: Dept. of Neurology, Boston Children's Hospital, USA.

ABSTRACT
Rasmussen's encephalitis is a rare syndrome characterized by intractable seizures, often associated with epilepsia partialis continua and symptoms of progressive hemispheric dysfunction. Seizures are usually the hallmark of presentation, but antiepileptic drug treatment fails in most patients and is ineffective against epilepsia partialis continua, which often requires surgical intervention. Co-occurrence of focal cortical dysplasia has only rarely been described and may have implications regarding pathophysiology and management. We describe a rare case of dual pathology of Rasmussen's encephalitis presenting as a focal cortical dysplasia (FCD) and discuss the literature on this topic.

No MeSH data available.


Related in: MedlinePlus

Coronal T1 image showing blurring of the gray–white matter interface at the left precentral gyrus suspicious for focal cortical dysplasia.
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f0005: Coronal T1 image showing blurring of the gray–white matter interface at the left precentral gyrus suspicious for focal cortical dysplasia.

Mentions: Follow-up MRI 2 months postseizure onset demonstrated blurring of the gray–white junction in the left precentral gyrus and was concerning for a cortical dysplasia (Fig. 1). However, differential diagnosis still included a Rasmussen's encephalitis or a nonspecific autoimmune process. Given the persistent pharmacologically refractory focal seizures, the patient underwent a surgical evaluation. Electroencephalogram showed multiregional left hemispheric spikes and slowing. Positron emission tomography (FDG-PET) showed an area of hypometabolism in the left precentral gyrus, but there was no focal increased radiotracer uptake with ictal single-photon emission computed tomography. Magnetoencephalography showed a spike dipole cluster at the left precentral gyrus.


Rasmussen's encephalitis presenting as focal cortical dysplasia.

O'Rourke DJ, Bergin A, Rotenberg A, Peters J, Gorman M, Poduri A, Cryan J, Lidov H, Madsen J, Harini C - Epilepsy Behav Case Rep (2014)

Coronal T1 image showing blurring of the gray–white matter interface at the left precentral gyrus suspicious for focal cortical dysplasia.
© Copyright Policy - CC BY-NC-ND
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4307873&req=5

f0005: Coronal T1 image showing blurring of the gray–white matter interface at the left precentral gyrus suspicious for focal cortical dysplasia.
Mentions: Follow-up MRI 2 months postseizure onset demonstrated blurring of the gray–white junction in the left precentral gyrus and was concerning for a cortical dysplasia (Fig. 1). However, differential diagnosis still included a Rasmussen's encephalitis or a nonspecific autoimmune process. Given the persistent pharmacologically refractory focal seizures, the patient underwent a surgical evaluation. Electroencephalogram showed multiregional left hemispheric spikes and slowing. Positron emission tomography (FDG-PET) showed an area of hypometabolism in the left precentral gyrus, but there was no focal increased radiotracer uptake with ictal single-photon emission computed tomography. Magnetoencephalography showed a spike dipole cluster at the left precentral gyrus.

Bottom Line: Seizures are usually the hallmark of presentation, but antiepileptic drug treatment fails in most patients and is ineffective against epilepsia partialis continua, which often requires surgical intervention.Co-occurrence of focal cortical dysplasia has only rarely been described and may have implications regarding pathophysiology and management.We describe a rare case of dual pathology of Rasmussen's encephalitis presenting as a focal cortical dysplasia (FCD) and discuss the literature on this topic.

View Article: PubMed Central - PubMed

Affiliation: Dept. of Neurology, Boston Children's Hospital, USA.

ABSTRACT
Rasmussen's encephalitis is a rare syndrome characterized by intractable seizures, often associated with epilepsia partialis continua and symptoms of progressive hemispheric dysfunction. Seizures are usually the hallmark of presentation, but antiepileptic drug treatment fails in most patients and is ineffective against epilepsia partialis continua, which often requires surgical intervention. Co-occurrence of focal cortical dysplasia has only rarely been described and may have implications regarding pathophysiology and management. We describe a rare case of dual pathology of Rasmussen's encephalitis presenting as a focal cortical dysplasia (FCD) and discuss the literature on this topic.

No MeSH data available.


Related in: MedlinePlus