Limits...
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

Leptomeningeal and cortical lymphocytes.
© Copyright Policy - CC BY-NC-ND
Related In: Results  -  Collection

License
getmorefigures.php?uid=PMC4307873&req=5

f0010: Leptomeningeal and cortical lymphocytes.

Mentions: Biopsy subsequently showed a mild to moderate T lymphocyte predominant inflammatory infiltrate in the cortex and white matter with microglial nodules, which was consistent with Rasmussen's encephalitis, but the overall pathological picture was mild (Fig. 2, Fig. 3). In addition, rare dysplastic neurons were seen, suggesting a possible cortical dysplasia (Fig. 3d). Balloon cells were not identified. There was reactive gliosis, without vasculitis or viral inclusions.


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)

Leptomeningeal and cortical lymphocytes.
© Copyright Policy - CC BY-NC-ND
Related In: Results  -  Collection

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

f0010: Leptomeningeal and cortical lymphocytes.
Mentions: Biopsy subsequently showed a mild to moderate T lymphocyte predominant inflammatory infiltrate in the cortex and white matter with microglial nodules, which was consistent with Rasmussen's encephalitis, but the overall pathological picture was mild (Fig. 2, Fig. 3). In addition, rare dysplastic neurons were seen, suggesting a possible cortical dysplasia (Fig. 3d). Balloon cells were not identified. There was reactive gliosis, without vasculitis or viral inclusions.

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