Double trouble: Bilateral cerebral involvement in Sturge-Weber syndrome

Sturge-Weber syndrome, also known as encephalotrigeminal angiomatosis or meningofacial angiomatosis, is characterised in its classical form by a congenital, usually unilateral, ‘portwine stain’ (capillary naevus) on the face, convulsions, typical intracranial calcification and some degree of mental retardation and hemiparesis. The clinical correlation of intractable seizures with the presence of bilateral intracranial disease has management and prognostic implications, thus making the presence of bilateral disease an important factor to all those involved in the management of the child with Sturge-Weber syndrome.

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Bilateral Sturge Weber Syndrome- a rare case report

Nepalese Journal of Ophthalmology ◽

10.3126/nepjoph.v5i1.7841 ◽

2013 ◽

Vol 5 (1) ◽

pp. 129-132 ◽

Cited By ~ 1

Author(s):

P Singh ◽

S Singh

Keyword(s):

Case Report ◽

Rare Case ◽

Unusual Case ◽

The Other ◽

Advanced Stage ◽

Weber Syndrome ◽

Rare Case Report ◽

The Face ◽

Bilateral Condition ◽

Sturge Weber Syndrome

Background: Sturge-Weber syndrome is a rare congenital neuro- oculo- cutaneous disorder. Objective: To report a very rare unusual case of bilateral manifestation of Sturge Weber syndrome. Case: We report an unusual case of a 17-year-old female with advanced stage of bilateral glaucoma associated with facial nevus extending to the other half of the face as well and bilateral intracranial calcification. Conclusion: Sturge -Weber syndrome can manifest as a bilateral condition. Nepal J Ophthalmol 2013; 5(9):129-132 DOI: http://dx.doi.org/10.3126/nepjoph.v5i1.7841

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Encephalotrigeminal Angiomatosis: A Review with Emphasis on Head and Neck Manifestations

Neurographics ◽

10.3174/ng.2000061 ◽

2021 ◽

Vol 11 (3) ◽

pp. 166-174

Author(s):

B.R. Larsen ◽

M.S. Rosztoczy ◽

D.C. Van Tassel ◽

C.R. Larson ◽

S.A. Avedikian ◽

...

Keyword(s):

Head And Neck ◽

Soft Tissues ◽

Clinical Findings ◽

Imaging Features ◽

Facial Soft Tissue ◽

Vascular Abnormalities ◽

Weber Syndrome ◽

The Face ◽

Sturge Weber Syndrome ◽

Tissue Abnormalities

Encephalotrigeminal angiomatosis, also known as Sturge-Weber syndrome or Sturge-Weber-Dimitri disease, is a sporadic congenital neurocutaneous syndrome characterized by angiomas of the face, eyes, and meninges. The typical clinical findings include seizures, a port-wine facial nevus, hemiparesis, and developmental delay. The classic intracranial imaging features of Sturge-Weber syndrome include cerebral hemispheric atrophy, hypervascularity and/or calcification of the cortex, and ipsilateral choroid plexus enlargement. Extracranial findings primarily include the vascular abnormalities of the ocular and facial soft tissues as well as hypertrophy of the calvarial and maxillofacial osseous structures. Additionally, we report involvement of the extraocular muscles, salivary glands, and lacrimal glands, findings that have not been well described in the literature. While a developmental venous pathology underlying Sturge-Weber syndrome has been proposed resulting in many of the findings, the mechanism behind the facial soft-tissue abnormalities and the clinical sequelae associated with these glandular abnormalities is still uncertain.Learning Objective: To recognize typical clinical and various imaging manifestations of Sturge-Weber syndrome, including head and neck and intracranial imaging findings

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STURGE WEBER SYNDROME- A RARE CASE REPORT ORIGINAL

10.36106/ijar/9502925 ◽

2021 ◽

pp. 4-5

Author(s):

Om Prakash Singh ◽

Vikas Kumar ◽

Pushp Kant Tiwari

Keyword(s):

Rare Case ◽

Signs And Symptoms ◽

Port Wine ◽

Occipital Area ◽

Weber Syndrome ◽

Rare Case Report ◽

The Face ◽

Sturge Weber Syndrome ◽

The Brain ◽

Embryonic Vessels

Sturge-Weber Syndrome (SWS) is one of the encephalotrigeminal angiomatosis and one of the important segmental vascular neurocutaneous disorders .The occurrence is not very uncommon and the prevalence is 1:20000 to 1:50000.(1) SWS occurs due to the presence of residual embryonic vessels . The various signs and symptoms include capillary malformation in the face a port wine birthmark and similar malformation in the brain involving leptomeniges as well as blood vessels of the eye causing glaucoma. The patient presents with seizures , hemiparesis and stroke like symptoms, headaches and developmental delay.(2) The imaging nding in SWS children is the calcication in the parietal and occipital area of the brain. The EEG ndings in SWS are the attenuation and the excess of slow activities.We are presenting here a rare case of , a fourteen year old male child who presented to our emergency department with status epilepticus. The aim of presenting this case is to share the classical presentation and the challenges involved in the management

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Use of Intralesional Bleomycin for Oral Hemangioma of Sturge-Weber Syndrome

International Journal of Head and Neck Surgery ◽

10.5005/jp-journals-10001-1211 ◽

2015 ◽

Vol 6 (1) ◽

pp. 17-19

Author(s):

Devayani Shinde ◽

Yogesh G Dabholkar ◽

Akanksha A Saberwal ◽

Haritosh Kamalakar Velankar ◽

Adip K Shetty

Keyword(s):

Vascular Malformations ◽

Accurate Diagnosis ◽

Port Wine Stain ◽

Port Wine ◽

Weber Syndrome ◽

Neurocutaneous Disorder ◽

The Face ◽

Sturge Weber Syndrome ◽

Venous Angiomas ◽

Head Neck

ABSTRACT Sturge-Weber syndrome (SWS) or encephalotrigeminal angiomatosis is a rare neurocutaneous disorder characterized with vascular malformations and capillary venous angiomas involving the face, choroid of the eye and leptomeninges with port wine stain, seizures activity and mental retardation. In this paper we report a case of a patient affected by Sturge-Weber syndrome presenting with tongue hemangioma and emphasize the importance of an accurate diagnosis and management in the clinical practice. How to cite this article Shinde D, Dabholkar YG, Saberwal AA, Velankar HK, Shetty AK. Use of Intralesional Bleomycin for Oral Hemangioma of Sturge-Weber Syndrome. Int J Head Neck Surg 2015;6(1):17-19.

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A case of adult-onset Sturge-Weber syndrome type III without intracranial calcification, presenting with transient homonymous hemianopia

Rinsho Shinkeigaku ◽

10.5692/clinicalneurol.cn-001473 ◽

2021 ◽

Vol 61 (2) ◽

pp. 132-135

Author(s):

Yuto Hayashi ◽

Yuri Sugiura ◽

Rie Nakatani ◽

Katsuya Araki ◽

Masayuki Moriya ◽

...

Keyword(s):

Intracranial Calcification ◽

Homonymous Hemianopia ◽

Syndrome Type ◽

Adult Onset ◽

Type Iii ◽

Weber Syndrome ◽

Sturge Weber Syndrome

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Sturge-Weber syndrome: Clinico-neuroradiological features and treatment profile

Nepal Journal of Neuroscience ◽

10.3126/njn.v18i3.34969 ◽

2021 ◽

Vol 18 (3) ◽

pp. 49-54

Author(s):

Anirban Chatterjee ◽

Malay Kumar Sinha

Keyword(s):

Genetic Disorder ◽

Early Age ◽

Neurodevelopmental Delay ◽

Gradual Loss ◽

Weber Syndrome ◽

The Face ◽

Sturge Weber Syndrome ◽

Lower Intelligence ◽

Nevus Flammeus

Introduction: Sturge Weber syndrome (SWS) is a rare, nonhereditary genetic disorder. SWS belongs to a diverse group of Neurocutaneous disorders. A somatic mutation in the early development of cells in SWS patients causes the formation of a congenital capillary vascular malformation. SWS is characterized by nevus flammeus on the face (also called Port-Wine Birthmark), brain (leptomeningeal angioma), and eyes (glaucoma). Methods and Materials: We present three patients diagnosed with Sturge Weber syndrome who attended a teaching hospital between 2013 to 2016. The clinical, neuroradiological features and treatment of SWS patients were reviewed retrospectively from medical records. Results: Two males and females with SWS were reviewed. The facial nevus flammeus was unilateral in one patient and bilateral in two patients. The extensive nevus flammeus including the face, trunk, and upper limb was found in one patient. Epilepsy, neurodevelopmental delay, and intellectual disability were the most common presentation in the series. Episodic hemiparesis was manifested in one patient. Levetiracetam and oxcarbazepine, either single or combined, were used in every SWS patient (n=3). Six months seizure-free was obtained in a patient with SWS with combined anticonvulsant therapy. Conclusion: Drug-refractory and early-age epilepsy is associated with lower intelligence level that consequently affects poor social skill and quality of life in the patients with SWS. Sudden or gradual loss of vision is also a threat for SWS patients. Diagnosis at birth or during early age, multidisciplinary intervention, and follow-up is a must to improve absolute outcomes in the patients with SWS.

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Sturge-Weber syndrome presenting with intractable seizures

Journal of Institute of Medicine ◽

10.3126/jiom.v32i1.4002 ◽

2010 ◽

Vol 32 (1) ◽

Author(s):

YB Roka ◽

PR Puri ◽

WS Ahmad ◽

BK Bista

Keyword(s):

Intractable Seizures ◽

Weber Syndrome ◽

Sturge Weber Syndrome

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Sturge-Weber Syndrome. Study of 55 Patients

Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques ◽

10.1017/s0317167100008878 ◽

2008 ◽

Vol 35 (3) ◽

pp. 301-307 ◽

Cited By ~ 81

Author(s):

Ignacio Pascual-Castroviejo ◽

Samuel-Ignacio Pascual-Pascual ◽

Ramón Velazquez-Fragua ◽

Juán Viaño

Keyword(s):

Mental Retardation ◽

Brain Lesion ◽

Poor Response ◽

Cerebral Lesions ◽

Upper Eyelid ◽

Gainful Employment ◽

Aesthetic Appearance ◽

Weber Syndrome ◽

Sturge Weber Syndrome ◽

Nevus Flammeus

Purpose:To review the clinical and neuroimaging features of a large series of patients with Sturge-Weber syndrome (SWS) seen over a 40-year period.Methods:Fifty-five patients with SWS (30 males and 25 females), were studied between 1965 and 2004. Results of neurological and ophthalmological examinations, electroencephalographic, and neuroimaging studies were reviewed. All patients were seen by one of the authors (I. P-C).Results:Epilepsy, hemiparesis, mental retardation and ocular problems were the most frequent and severe features of patients with Sturge-Weber syndrome in this series. The facial nevus flammeus was unilateral in 35 (63.5%) patients, bilateral in 17 (31%) and absent in 3 (5.5%) of the patients with leptomeningeal angiomas. Seven (41%) of the 17 patients with bilateral nevus flammeus had unilateral leptomeningeal angiomas. Seizures occurred in 47 patients (85.5%). Complete seizure control was obtained in 20 patients (42.5%), but in 2 of these 20 patients seizures were controlled only after lobectomy. All patients with unilateral or bilateral upper eyelid nevus flammeus had ipsilateral, unilateral or bilateral choroid-retinal angiomas. Only 20 (36%) of the 55 patients had low-normal or borderline intelligence (IQs<70). No relationship was observed between the size of the facial nevus flammeus and the severity of the brain lesion.Conclusions:Epilepsy, hemiparesis, mental retardation and ocular problems were the most frequent and severe features of patients with Sturge-Weber syndrome in this series. Cerebral lesions followed a progressive course during early childhood, but not later. Early surgical treatment controlled the seizures but other neurological problems such as hemiparesis and intellectual deficits showed a less satisfactory response. Early onset of seizures and poor response to medical treatment, bilateral cerebral involvement and unilateral severe lesions were indicative of a poor prognosis. Limited intelligence and social skills, poor aesthetic appearance and seizures complicated the integration of SWS patients. These features must be addressed in order for the patients improve social interactions, obtain gainful employment and achieve a better quality of life.

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