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Table of Contents    
Year : 2015  |  Volume : 63  |  Issue : 6  |  Page : 979-981

Sturge-Weber syndrome with bilateral cerebral calcifications but without a facial nevus

1 Department of Neurology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India
2 Department of Internal Medicine, Moti Lal Nehru Medical College, Allahabad, Uttar Pradesh, India

Date of Web Publication20-Nov-2015

Correspondence Address:
Vimal Kumar Paliwal
Department of Neurology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.170085

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How to cite this article:
Kumar S, Anand S, Ambesh P, Paliwal VK. Sturge-Weber syndrome with bilateral cerebral calcifications but without a facial nevus. Neurol India 2015;63:979-81

How to cite this URL:
Kumar S, Anand S, Ambesh P, Paliwal VK. Sturge-Weber syndrome with bilateral cerebral calcifications but without a facial nevus. Neurol India [serial online] 2015 [cited 2022 Aug 19];63:979-81. Available from: https://www.neurologyindia.com/text.asp?2015/63/6/979/170085


 Sturge- Weber syndrome More Details More Details (SWS) is a neurocutaneous syndrome characterized by capillary-venous angiomas involving the face, ipsilateral leptomeninges, and the eye. It is a rare sporadic-congenital syndrome (1/50,000 births) and is generally classified into three types. Type 1 manifests with classical facial nevus, leptomeningeal angioma, and glaucoma; Type 2 SWS presents with facial nevus and glaucoma; whereas, Type 3, the rarest of the three (incidence 5–15%),[1] presents only with isolated leptomeningeal angiomas. Bilateral calcifications can be seen in upto 20% patients with SWS but bilateral affliction without a facial nevus is extremely rare.[2] In the absence of a facial nevus, the disease poses a diagnostic challenge, and therefore, we report this case.

An 8-year-old girl presented with generalized tonic-clonic seizures of 4 year duration associated with behavioral abnormality for 2 years. Her illness started with seizures (frequency 5/year) and soon progressed to poor scholastic performance. Her speech was reduced to single words. She stopped informing about her natural calls. There were no other seizure types, exanthematous illness, head trauma or febrile encephalopathy. She was born full term and had attained her early milestones normally. On examination, she was irritable and hyperkinetic with poor attention span and without any neurocutaneous markers. Her neurological examination was normal including the presence of a normal head size and normal vision/hearing. Her hematological investigations including calcium studies were normal. Cranial computed tomography scan revealed bilateral parieto-occipital atrophy, gliosis, and gyriform calcification. Magnetic resonance imaging of the head revealed leptomeningeal enhancement over the parieto-occipital sulci with underlying periventricular white matter hyperintensities [Figure 1]. Electroencephalography revealed an attenuation of alpha rhythm over the parietooccipital leads [Figure 1].
Figure  1: (a) Cranial computed tomography scan showing parieto-occipital gyriform calcification;   (b) postgadolinium T1-weighted magnetic resonance imaging showing leptomeningeal enhancement  (arrows); (c and d) T2 fluid attenuated inversion recovery image showing periventricular and parieto-occipital white matter hyperintensity and cerebral atrophy; (e) electroencephalography, occipital leads  (box) showing attenuation of alpha rhythm  (12  Hz, frontal/central leads)

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Differential diagnosis in a child presenting with these manifestations may include inborn errors of metabolism, mitochondrial disorders, childhood epileptic disorders such as Landau-Kleffner syndrome, progressive myoclonic epilepsies, and encephalopathies like subacute sclerosing panencephalitis. However, the radiological assessment of the lesions causing intracranial calcifications in childhood may significantly shorten the list. The causes include congenital TORCH infections, calcified neurocysticercosis, Fahr's disease, tuberous sclerosis, tumors, celiac disease, purulent meningitis, and ossifying meningoencephalitis. TORCH infections, meningitis, and meningoencephalitis produce static encephalopathy. Calcified neurocysticercosis/brain tumors produce nongyriform calcification. Calcifications in Fahr's syndrome involve the globus pallidus, the caudate-putamen complex and the cerebellum (straito-pallidal-dentate region). Our patient lacked subependymal calcifications/tubers of tuberous sclerosis. Celiac disease may cause bilateral occipital calcifications but a preceding history suggestive of malabsorption syndrome was lacking in our patient. Moreover, cognitive dysfunction is uncommon in celiac disease. Additionally, calcification in celiac disease occupies both cortical/subcortical locations and lacks enhancement as well as cortical atrophy/gliosis, quite unlike the findings in our patient.[3]

Electroencephalographic abnormalities in SWS include ipsilateral slowing, attenuation of the background rhythm, and spike-wave discharges.[4] Alpha-rhythm, predominantly seen in occipital leads, was attenuated in our patient possibly due to the abnormal underlying cortex. We conclude that a careful clinico-radiological correlation may be helpful in diagnosing SWS especially when there is lack of a facial nevus.

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 » References Top

Roach ES. Neurocutaneous syndromes. Pediatr Clin North Am 1992;39:591-620.  Back to cited text no. 1
Alkonyi B, Chugani HT, Karia S, Behen ME, Juhász C. Clinical outcomes in bilateral Sturge-Weber syndrome. Pediatr Neurol 2011;44:443-9.  Back to cited text no. 2
Magaudda A, Dalla Bernardina B, De Marco P, Sfaello Z, Longo M, Colamaria V, et al. Bilateral occipital calcification, epilepsy and coeliac disease: Clinical and neuroimaging features of a new syndrome. J Neurol Neurosurg Psychiatry 1993;56:885-9.  Back to cited text no. 3
Kossoff EH, Bachur CD, Quain AM, Ewen JB, Comi AM. EEG evolution in Sturge-Weber syndrome. Epilepsy Res 2014;108:816-9.  Back to cited text no. 4


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