Enhanced magnetic resonance imaging of leptomeningeal angiomatosis

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Enhanced Magnetic Resonance Imaging of Leptomeningeal Angiomatosis Seiichi Sugama, MD**, Hiroshi Yoshimura, MD*, Karou Ashimine, MD*, Yoshikatsu Eto, MD, PhD*, and Kihei Maekawa, MD* We present two patients with unilateral occipital gyriform calcification and seizures. Gyriform or serpentine calcification as revealed by computed tomography (CT) scan is rare and is a characteristic finding of Sturge-Weber syndrome (SWS) and celiac disease (CD). These patients had neither the facial nevus flammeus or neurological deficits characteristic of SWS, nor the gastrointestinal symptoms characteristic of CD. CD is often accompanied by cerebral occipital calcification indistinguishable from that of SWS. We demonstrate the presence of cerebral leptomeningeal angiomatosis (LA) by Gadolinium-DTPA-enhanced magnetic resonance imaging (MRI) but could not detect LA by either CT scanning or angiography. It has been reported that contrast-enhanced MRI is useful to detect LA in SWS. However, we found no reports of enhanced MRI in patients with SWS without facial angioma. If future studies can demonstrate the absence of cortical enhancement by contrast-enhanced MRI in CD with cerebral calcifications, enhanced MRI would become an important tool for differentiating CD from SWS. © 1997 by Elsevier Science Inc. All rights reserved. Sugama S, Yoshimura H, Ashimine K, Eto Y, Maekawa K. Enhanced magnetic resonance imaging of leptomeningeal angiomatosis. Pediatr Neurol 1997;17:262-265.

From the *Departments of Pediatrics; *Institute of DNA Medicine; Tokyo Jikei University School of Medicine; Tokyo; and *Department of Pediatrics; Okinawa Chubu Hospital; Okinawa; Japan.

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Introduction Many conditions involve intracranial calcification, such as encephalitis, congenital infection, purulent meningitis, neonatal hemorrhage, disturbance of calcium metabolism, tuberous sclerosis, arteriovenous malformation, and chemotherapy. However, gyriform or serpentine calcification as revealed by cerebral computed tomography (CT) scan is rare and is a characteristic finding of both Sturge-Weber syndrome (SWS) [1-3] and celiac disease (CD) [4-8], as well as following chemotherapy and radiotherapy treatment for childhood leukemia [9]. We present two patients with unilateral occipital gyriform calcification and seizures, neither of whom had facial nevus flammeus, a characteristic manifestation of SWS, nor the gastrointestinal symptoms characteristic of CD. We demonstrated the presence of cerebral leptomeningeal angiomatosis (LA) by Gadolinium-DTPA (Gd-DTPA)-enhanced magnetic resonance imaging (MRI) but could not detect LA by either CT scanning or angiography. It has been reported that contrast-enhanced MRI is useful to detect cerebral LA in SWS [10-12]. However, to our knowledge, there have been no reports of enhanced MRI in patients with SWS without facial angioma. Contrast-enhanced MRI may be an important tool for differentiating CD with cerebral calcifications from SWS without cutaneous manifestations.

Patients and Methods Patient 1. A 9-year-oldboy was admittedto our hospitalfollowingthe first episode of afebrile right-sided status epilepticusfollowing upward globe deviationand vomiting.Birth and developmenthad been uneventful. There was no familyhistory of neurologicor gastrointestinaldisease. Physical examination revealed no cutaneous manifestations,including those of the face. Ophthalmologicexaminationwas normal. His neuromental developmentand neurologicexaminationrevealed no abnormalities. Electroencephalogram demonstrated left-occipital high voltage slow waves. He was treated with carbamazepine,and no further seizures have been reported. Patient 2. A 4-year-old girl was referred to us following the first episode of repeated afebrile seizures featuring ocular deviation toward the left side, loss of awareness, and loss of tone of extremities. Previously, she had had two episodes of febrile seizures. Pregnancyand parturition were normal; psychomotor development was uneventful. Examination revealed no cutaneous manifestations,including those of the face, and normalophthalmologicfindings.Her intellectualand motor development were normal. Electroencephalogramwas normal. Following the administrationof carbamazepinetherapy, no further seizures have been reported.

Communicationsshould be addressed to: Dr. Sugama;Depamnentof Pedialrics;TokyoJikei UniversitySchoolof Medicine;2-25-18Nishi-shinbashi;Minato-ku;Tokyo 105,Japan. Received January 28, 1997; accepted May 2, 1997.

© 1997 by Elsevier Science Inc. All rights reserved. PII S0887-8994(97)00093-3 • 0887-8994/97/$17.00

Figure 1. Patient 1. (A) Enhanced cerebral CT scan, reveals gyriform calcification in the left occipital region with no modification after contrast medium injection (arrows). (B, C) Enhanced cerebral (coronal sections) postcontrast T~-weighted magnetic resonance images (TR: 400 ms; TE: 15 ms) reveal focal atrophic ~hange and a thin stripe of enhancement covering the left occipital lobe and the posterior part of the left temporal lobe' (arrows). Neither patient had evidence of transient malabsorption. Results of routine laboratory tests were normal, including that of folic acid levels. Cerebral CT and MRI. In Palient 1, a CT scan revealed gyriform calcification in the left occipital region with no modification after contrast medium injection (Fig IA). MRI was performed with a 0.5-T scanner and the Tl-weighted image, obtained without Gd-DTPA, demonstrated atrophic changes in the ipsilateral occipital region in both patients. Images obtained after injection of Gd-DTPA depicted a thin stripe of enhancement covering the left occipital lobe and the posterior section of the left temporal lobe in Patient 1 (Fig. 1B, C). In Patient 2, a CT scan revealed unilateral light calcification in the left parieto-occipital cortico-subcortical region (Fig. 2A). Enhanced CT scan demonstrated

increased band-like density adjacent to the calcifications and gyral enhancement in the left parieto-occipital region (Fig. 2B). Gd-DTPAenhanced MRI of Patient 2 depicted extensive gyriform enhancement in the parieto-occipital area (Fig. 2C), and the coronal slice also revealed impressive enhancement of LA and slight staining of subcortical white matter. A congested basal vein of Rosenthal draining the angioma from the left occipital lateral ventricle was noted. No enhancement or enlargement of the choroid plexus, which is a characteristic finding of SWS, was observed in either patient. Angiography. In Patient 1, angiography revealed no distinct abnormality except for a slight dilatation of Labbe's vein, In Patient 2, both hyperemia and tortuosity of subependymal and medullary veins, and paucity of superficial cortical veins was seen.

Figure 2. Patient 2. (A ) Cerebral CT scan reveals light calcification in the left parieto-occipital cortico-subcortical region (arrows). (B) CT scan reveals increased band-like density adjacent to the calcifications and gyral enhancement in left parieto-occipital region (arrows). (C) Enhanced cerebral (axial section) postcontrast T~-weighted magnetic resonance image (TR: 400 ms; TE: 15 ms) reveals extensive leptomeningeal enhancement and focal atrophic change in posterior left cerebral hemisphere (arrows).

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Discussion SWS, a phakomatosis characterized by a "port-wine" vascular nevus flammeus in the area of trigeminal nerve distribution, is a congenital disorder of the vasculature of the brain and of the eye. Other clinical manifestations of SWS are seizures, hemiparesis, hemianopsia, glaucoma, and mental retardation. Typically, a patient with CD has gastrointestinal findings, such as recurrent diarrhea and malabsorption, and anemia; however, it has been reported that many CD patients have no gastrointestinal problems [5]. Laboratory tests reveal high concentrations of antibodies to gluten, low serum folic acid and characteristic pathological findings of biopsied intestinal mucosa [5]. Occipital calcifications in both of our patients were similar to those found in SWS [1,2] and CD [4-8], but neither patient had other typical features, specifically a facial vascular nevus in the territory of the trigeminal nerve, glaucoma, and neurological deficit as seen in SWS, or the malabsorption symptoms and serum folic acid deficiency typical of CD. Some investigators [6,7] have reported that patients with CD accompanied by cerebral calcification can be differentiated from SWS with cerebral calcification on the basis of the following neuroradiological characteristics typical of CD: 1) absence of choroid plexus enlargement by CT and MRI; 2) absence of abnormal deep cerebral veins detected by enhanced CT; 3) absence of neurological deficits; 4) cerebral calcification in the cortico-subcortical locus in CD, as opposed to the mainly cortical locus in SWS; 5) absence of lobar or hemispheric atrophy, which is almost always present in SWS; and, 6) presence of bilateral rather than unilateral calcification in most CD cases. Patient 1 had features 1-3 but not 4-6, and Patient 2 had features 1,3, 4 but not 2, 5, and 6. Therefore, we could not confirm the diagnosis by the above findings. Although angiography did not demonstrate direct evidence of LA, Gd-DTPAenhanced MRI T~-weighted images clearly exhibited regional distribution of angiomatosis in the meninges of the left occipital lobe in both patients. The hallmark of SWS is LA in the area of the trigeminal nerve as well as ipsilateral brain and meninges; however, not all SWS patients have facial lesions [13,14]. Roach [14] classified LA of the brain without facial angiomatosis as type 3 SWS, although this classification remains controversial. Cerebral LA is the fundamental lesion in SWS, and the minimum clinical and diagnostic criteria for SWS requires the presence of intracranial LA [13]. CD is often accompanied by cerebral occipital calcifications indistinguishable from those of SWS. As in SWS, LA is ordinarily confined to the pia matter and these calcifications occur in a pericapillary distribution, usually in the fourth layer of the atrophied cerebral cortex. Alterations in circulatory dynamics in the abnormal vessels are thought to induce anoxic lesions of the endothelium [3]. In CD, calcifications appear at the

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interface between the white and gray matter spreading to the cortical surface, and few or no foci of pial angiomatosis have been identified [7,8]. Therefore, the detection of cerebral LA by MRI is more likely to lead to a diagnosis of SWS than to CD. The precise etiology of SWS is unknown, but incomplete involution of the embryonal vasculature has been proposed [15]. The enhanced MRI findings in our two patients were the same as those reported for patients with SWS; the origin of their angiomatosis may be similar to that of SWS. Direct demonstration of LA is difficult or impossible either by CT [1] or angiography [16,17]. Visualization of LA by Gd-DTPA-enhanced MRI has been described recently in patients with SWS [3,10,11,17]. Lipski et al. [10] reported cortical enhancement actually representing the pial angioma itself as confirmed by surgical specimens. To our knowledge, there have been no reports of Gd-DTPA-enhanced MRI studies in SWS without cutaneous manifestations. It is important to distinguish these two diseases: SWS is a fundamentally progressive disease, while CD can be treated with a gluten-free diet. If the absence of cortical enhancement by Gd-DTPA MRI is confirmed in CD with cerebral calcifications in future studies, Gd-DTPA-enhanced MRI may become an important tool for differentiating CD and SWS. We propose that enhanced MRI is essential in patients with cerebral calcifications and facial cutaneous angioma but normal findings by CT scan. The authors thank Drs. Jun-ichiro Okamoto and Kiyoshi Ohmine for their assistance in the interpretation of cerebral angiography and MRI.

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