Focal cortical dysplasia with calcification: a case report - Springer Link

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Dec 22, 2007 - Abstract. Case report Focal cortical dysplasia (FCD) with calcifica- tion is rare. We presented a 13-year-old epileptic patient with. FCD and ...
Childs Nerv Syst (2008) 24:619–622 DOI 10.1007/s00381-007-0566-4

CASE REPORT

Focal cortical dysplasia with calcification: a case report Kazuhiro Samura & Takato Morioka & Fumiaki Yoshida & Kimiaki Hashiguchi & Yasushi Miyagi & Masahiro Mizoguchi & Tadahisa Shono & Shinji Nagata & Satoshi O. Suzuki & Tomio Sasaki

Received: 24 August 2007 / Published online: 22 December 2007 # Springer-Verlag 2007

Abstract Case report Focal cortical dysplasia (FCD) with calcification is rare. We presented a 13-year-old epileptic patient with FCD and calcification in the left frontal lobe. At age 24, the FCD lesion and the surrounding epileptogenic cortex and underlying subcortex were removed after chronic subdural electrode recording. Histological examination showed that the calcified lesion was not independent of the FCD lesion but located in the subcortical area of the FCD lesion. A neoplastic nature was ruled out for the lesion. Discussion The pathophysiological mechanism involved in the coexistence of FCD and calcification is discussed.

accepted that FCD can cause intractable epilepsy. Seizures occur at an early age and are often resistant to medication; however, surgical resection has been found to be beneficial in such patients [9]. Although extensive histopathological and immunohistochemical examinations of FCD have been reported [8, 11, 14], calcification in FCD is rare [18, 19]. We reported a case of FCD with calcification demonstrated on a computed tomographic (CT) scan at age 13 years and histologically verified at age 24.

Keywords Focal cortical dysplasia . Calcification . Epilepsy surgery

A 13-year-old, right-handed boy had been having seizures since the age of 3 years. His seizure began when he felt sick, followed by cloudy consciousness and hand motor automatism, and often secondary generalized tonic–clonic seizures. A CT scan demonstrated a small calcification in the subcortical white matter of the left frontal lobe (Fig. 1a). Although his seizures were almost controlled with various antiepileptic drugs, including carbamazepine, zonisamide, and clobazam, they became intractable at the age of 18. At the age of 24, presurgical evaluation was performed. A CT scan showed a small calcification in the subcortical white matter of the left frontal lobe, identical to the CT finding at age 13 (Fig. 1b). Magnetic resonance imaging (MRI) was performed with a 1.5-tesla unit. Axial fluid-attenuated inversion-recovery (FLAIR) image showed focal cortical thickening, blurring of the gray–white matter junction, and subcortical hyperintensity in the left frontal lobe (Fig. 1c,d). It was estimated that the calcified lesion was located at the lower part of the area with subcortical hyperintensity. The lesion was not enhanced with gadolinium-diethylenetriamine pentaacetic acid. 18F-labeled fluorodeoxyglucose positron emission tomography and Tc-99m ethyl-cysteinate dimer

Introduction Focal cortical dysplasia (FCD) [18], a form of neuronal migration disorder, is a malformative lesion of the neocortex that occurs during brain development. It is widely K. Samura : T. Morioka (*) : F. Yoshida : K. Hashiguchi : Y. Miyagi : M. Mizoguchi : T. Shono : S. Nagata : T. Sasaki Department of Neurosurgery, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka 812-8582, Japan e-mail: [email protected] S. O. Suzuki Department of Neuropathology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan Y. Miyagi Division of Digital Patient, Digital Medicine Initiative, Kyushu University, Fukuoka, Japan

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Fig. 1 CT scans at age 13 (a) and 24 (b) years demonstrate a small calcification in the subcortical white matter of the left frontal lobe. (c, d) An axial FLAIR MRI shows focal cortical thickening, blurring of the gray–white matter junction, and subcortical hyperintensity in the left frontal lobe. It is estimated that the calcified lesion is located at the lower part of the subcortical hyperintensity area

Fig. 2 (a) A surface anatomy scan image. The white box indicates the extent of the craniotomy. (b) An operative photograph, shown in an orientation that matches with (a), demonstrates the position and number of the subdural and grid electrodes. Electrode numbers 36–39 are located on the interhemispheric surface of the frontal lobe. The arrows indicate the pachygyric cortex. (c) ECoG reveals that ictal discharges begin at the electrode on the interhemispheric surface just medial to the pachygyric cortex (arrow; electrode number 36) and spread to the lateral and posterior frontal lobe (electrode numbers 23, 27, and 31)

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single photon emission-computed tomography showed hypometabolism and hypoperfusion at the left anterior frontal lobe, respectively. Interictal electroencephalography (EEG) showed paroxysmal discharges in the left frontal region. On ictal EEG, rhythmic slow waves with the maximal amplitude on Fp1 and F3 appeared. Through left fronto–temporo–parietal craniotomy, chronic subdural grid and strip electrodes were placed on the lateral and interhemispheric aspects of the left frontal lobe (Fig. 2a,b). The pachygyric cortex was observed at the most medial site of the craniotomy (Fig. 2b; arrows); it was confirmed as firm by palpation. Ictal electrocorticography (ECoG) revealed that ictal discharges began at the electrode on the interhemispheric surface just medial to the pachygyric cortex (electrode number 36 in Fig. 2b, arrow in Fig. 2c) and spread to the lateral and posterior frontal lobe (electrode number 23, 27, and 31 in Fig. 2b,c). Cortical and subcortical resection of the pachygyric cortex and the surrounding ictal onset zone was performed. Resection was extended to the underlying periventricular zone. Surgical specimens exhibited disruption of cortical lamination and dysmorphic neurons and balloon cells in the cortex and subcortical white matter (Palmini type IIB; Fig. 3a). A focal calcified lesion was observed in the subcortical white matter (Fig. 3a); however, the boundary between FCD and the calcified lesion was blurred (Fig. 3b).

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Fig. 3 (a) Lower magnification of the surgical specimen shows the topographical relationship between FCD and calcification (Ca). H & E, ×2. There is disruption of cortical lamination and dysmorphic neurons and balloon cells in the cortex and subcortical white matter (Palmini type IIB). A focal calcified lesion is observed in the subcortical white matter. Arrows indicate the cortical surface. (b) Higher magnification of the black box in (a) shows that the boundary between FCD and Ca is blurred. ×10

Immunohistochemical examination including MIB-1 ruled out a neoplastic nature for the lesion. During the first 3 years postoperative, the patient's seizures were controlled by monotherapy of carbamazepine.

Discussion In the present case, histological examination clearly showed that the calcified lesion was not independent from the FCD lesion but located in the subcortical area of the FCD lesion, which was epileptogenic. Calcification had been demonstrated on a CT scan at age 13, and this finding remained unchanged for 9 years.

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Focal cortical dysplasia with calcification is rare [18, 19]; to our knowledge, only two cases have been reported. Maehara et al. [6] reported a case of an 8-year-old epileptic patient with FCD with extensive intracerebral ossification. Pathological examination revealed a number of dysplastic neurons in the whole lesion, which consisted of an epileptogenic cortex and a nonepileptogenic hamartomatous lesion with adipose tissue, vascular tissue, calcification, and ossification, suggesting that FCD has multipotentiality for cellular differentiation including various hamartomatous tissues. You et al. [21] reported a case of an epilepsy surgery in a patient with Lennox-Gastaut syndrome and FCD with mottled calcification in the right frontal lobe. Although lesionectomy of the FCD had a seizure-free result, the precise topographical relationship between the epileptogenic area, FCD, and calcification was not reported. Cortical dysplasia is known to sometimes be associated with glioneuronal tumors, such as ganglioglioma and dysembryoplastic neuroepithelial tumors [5, 10, 15]; these are sometimes accompanied with calcification [12]. In our case, immunohistochemical examination including MIB-1 ruled out a neoplastic nature for the lesion. The pathological findings in FCD bear some resemblance to those of a cortical tuber: a disordered cortex with large neurons and bizarre glial cells (balloon cells) [3]. Bronen et al. [1] suggested that FCD with balloon cells and formes fruste of tuberous sclerosis (cortical tuber without other stigma of tuberous sclerosis) might indeed represent the same entity or that FCD with balloon cells might simply be a solitary form of tuberous sclerosis. Calcification is frequently shown in cortical tubers [2, 7]. However, Yagishita and Arai [20] noted that forme fruste of tuberous sclerosis is a separate entity from FCD. Pathologically, abnormal neurons are numerous in FCD but sparse in cortical tubers [18]. Cytoarchitectural abnormalities are much more extensive in cortical tubers than in FCD, and a characteristic feature of a cortical tuber is subpial clusters of giant astrocytes and sheaves of astrocytic processes [3]. Pathological findings of the present case were indeed those of FCD rather than of a cortical tuber. Another possible mechanism for the coexistence of FCD and calcification is by neuronal injury; since calcification is often observed as sequelae of an injury. Previous studies have indicated that the pathogenesis of the various histopathological patterns of FCD is multifactorial [14]. Among them, interestingly, in utero injuries at different stages of brain development [13, 16] and even perinatal or postnatal insults [4, 17] may contribute to their etiology. This notion can explain and unify the coexistence of FCD and calcification; however, it is unlikely, because if so, FCD with calcification should not be rare. Although the precise pathophysiology in this case of the coexistence of FCD and calcification remains

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unknown, the case demonstrates that calcification can be associated with FCD.

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