Childs Nerv Syst DOI 10.1007/s00381-015-2757-8

CASE REPORT

Rasmussen encephalitis with dual pathology in a patient without seizures: case report and literature review Vijay M. Ravindra 1,2 & Marcus D. Mazur 1,2 & Carrie A. Mohila 3 & Matthew T. Sweney 4,5 & Aimee Hersh 6,7 & Robert J. Bollo 1,2

Received: 25 February 2015 / Accepted: 21 May 2015 # Springer-Verlag Berlin Heidelberg 2015

Abstract Purpose Rasmussen encephalitis without seizures is rare. We report a case of Rasmussen encephalitis and cortical dysplasia without epilepsy as well as describe the imaging, pathology, and clinical course and review the literature to investigate whether this may represent a rare subset of Rasmussen encephalitis. Case report We report the case of a 12-year-old girl with a history of cognitive decline and right arm weakness. Magnetic resonance imaging demonstrated diffuse left hemispheric cortical and subcortical atrophy suggestive of Rasmussen encephalitis. The patient had no clinical history of seizures, and electroencephalography did not demonstrate epileptiform abnormalities. Craniotomy for open brain biopsy was

performed, and histopathologic evaluation identified Rasmussen encephalitis with cortical dysplasia (dual pathology). Conclusions To the best of our knowledge, this is the third case of Rasmussen encephalitis diagnosed by both imaging and histopathology that had no clinical or electroencephalographic evidence of seizures and is the only case of Rasmussen encephalitis with cortical dysplasia without epilepsy. Keywords Rasmussen encephalitis . Electroencephalography . T-cell dominant . Cortical dysplasia

Introduction * Robert J. Bollo [email protected] 1

Department of Neurosurgery, University of Utah School of Medicine, Salt Lake City, UT, USA

2

Division of Pediatric Neurosurgery, Primary Children’s Hospital, 100 North Mario Capecchi Drive, Suite 1475, Salt Lake City, UT 84113, USA

3

Department of Pathology, Texas Children’s Hospital, Baylor College of Medicine, Houston, TX, USA

4

Department of Neurology, University of Utah School of Medicine, Salt Lake City, UT, USA

5

Division of Pediatric Neurology, Primary Children’s Hospital, Salt Lake City, UT, USA

6

Department of Rheumatology/Immunology, University of Utah School of Medicine, Salt Lake City, UT, USA

7

Division of Pediatric Rheumatology/Immunology, Primary Children’s Hospital, Salt Lake City, UT, USA

Rasmussen encephalitis (RE) is a chronic inflammatory disease of unknown origin typically isolated to one cerebral hemisphere that leads to unilateral hemispheric atrophy [28–31, 34]. Clinically, RE is characterized by epilepsy and progressive neurological deficits. The most common clinical presentation involves focal-onset seizures, including epilepsia partialis continua (EPC), which is observed in 50 % of cases [22, 24, 32, 36]. EPC is defined as regular or irregular clonic muscular twitches affecting a portion of the body that occur for a minimum of 1 h and recur at intervals of no more than 10 s [36]. Patients with RE typically develop drug-resistant epilepsy and progressive deterioration in hemispheric function. This description has been incorporated into the international classification of epilepsies and epileptic syndromes [1]. While epilepsy is typically a central clinical feature of RE, it is not required for diagnosis [6]. We report the

Childs Nerv Syst

case of a 12-year-old girl with a history of cognitive and motor decline without seizures who had a confirmed histopathological diagnosis consistent with Rasmussen encephalitis and cortical dysplasia [6]. This is the third case of RE diagnosed by both imaging and histopathology without clinical or electroencephalographic (EEG) evidence of seizures. Further, this is the first report of a patient with RE and cortical dysplasia without evidence of seizures [7]. We describe the imaging, pathology, and clinical course and review the literature to investigate whether this may represent a rare subset of RE.

Case report Presentation and diagnosis A 12-year-old, right-handed girl presented with headaches, worsening handwriting, decline in school performance, and social withdrawal over a 3-month period. Detailed neurological examination revealed little spontaneous speech but no dysarthria, subtle left hemiglossal atrophy, pronator drift of the right upper extremity, and mild weakness of the hand intrinsic muscles, grip, and wrist extension on the right. There was no sensory loss, and tone in the upper and lower extremities was normal. Magnetic resonance imaging (MRI) demonstrated significant atrophy of the left cerebral hemisphere, including patchy T2 hyperintensity in the frontal lobe, and a normal structural appearance of the right hemisphere, cerebellum, and brainstem (Fig. 1). Time-of-flight magnetic resonance angiography findings were normal. The results of serological testing, including complete blood count and immunoglobulin G (IgG) level, were also normal. Cerebrospinal fluid (CSF) obtained via lumbar puncture was also unrevealing (CSF total protein 17 mg/dL, glucose 54 mg/dL, red and white blood cell counts both

Rasmussen encephalitis with dual pathology in a patient without seizures: case report and literature review.

Rasmussen encephalitis without seizures is rare. We report a case of Rasmussen encephalitis and cortical dysplasia without epilepsy as well as describ...
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