scholarly journals STURGE-WEBER SYNDROME: A CASE REPORT

2018 ◽  
Vol 11 (3) ◽  
pp. 3
Author(s):  
Janani R ◽  
Kiruthika S ◽  
Arunachalam Ramachandran ◽  
Kumaresan A
Keyword(s):  
Developmental Delay ◽  
Clinical Manifestations ◽  
Cerebral Atrophy ◽  
Physiotherapy Treatment ◽  
Physical Diagnosis ◽  
Medical College ◽  
Developmental Age ◽  
Weber Syndrome ◽  
Sturge Weber Syndrome ◽  
The Right

  To report a clinical case of Sturge-Weber syndrome (SWS) 1 year 8-month-old male child reported to Saveetha Medical College, Physiotherapy Department presented with complaints of difficulty in sitting, standing, and walking. Computed tomography brain revealed unilateral calcification and cerebral atrophy on the right side. Physical diagnosis revealed the child had left hemiparesis with developmental delay. The child had a developmental delay with a motor developmental age of 2 months. As clinical manifestations of SWS are wide, it leads to multidisciplinary approaches. Physiotherapist’s knowledge is important for early diagnosis and to provide an adequate physiotherapy treatment.

scholarly journals Surgical treatment of drug resistant epilepsy in Sturge-Weber syndrome: review of the literature and clinical case presentation

2019 ◽  
Vol 11 (1) ◽  
pp. 53-62
Author(s):  
G. S. Ibatova ◽  
S. K. Akshulakov ◽  
S. M. Malyshev ◽  
R. G. Khachatryan ◽  
T. M. Alekseeva ◽  
...  
Keyword(s):  
Surgical Treatment ◽  
Medical Records ◽  
Clinical Manifestations ◽  
Epileptic Seizures ◽  
Positive Outcomes ◽  
Class Iii ◽  
Case Presentation ◽  
Weber Syndrome ◽  
Sturge Weber Syndrome ◽  
Drug Resistant Epilepsy

The paper addresses the relatively rare inherited neurodermal disorder – Sturge-Weber syndrome that can manifest in epileptic seizures. We describe updated concepts, epidemiology, etiology, pathogenesis, clinical manifestations, and surgical treatment of the disease. We examined medical records of 21 patients (aged from 1 to 11 years) with Sturge-Weber syndrome treated over the period of 1996-2016. After surgical treatment of 10 patients (five cases with hemispheretomy and five – with multifocal resection), positive outcomes (Engel class I, II) were found in 70% of cases, and negative (Engel class III, IV) – in 20% of cases. Оne child suddenly died during epileptic seizures. In non-operated children (age from 2 to 5 years) under our observation, an improvement was noted in six cases, no changes – in three cases, and a further progression of the disease – in three cases. In this article, we analyze two of these cases in detail.

scholarly journals Isolated leptomeningeal angiomatosis in the sixth decade of life, an adulthood variant of Sturge Weber Syndrome (Type III): role of advanced Magnetic Resonance Imaging and Digital Subtraction Angiography in diagnosis

BMC Neurology ◽  
2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Vetrivel Muralidharan ◽  
Gaetano Failla ◽  
Mario Travali ◽  
Tiziana Liliana Cavallaro ◽  
Marco Angelo Politi
Keyword(s):  
Clinical Presentation ◽  
Subcortical White Matter ◽  
Digital Subtraction ◽  
Resonance Imaging ◽  
Radiological Findings ◽  
Hyperintense Signal ◽  
Right Cerebral Hemisphere ◽  
Weber Syndrome ◽  
Sturge Weber Syndrome ◽  
The Right

Abstract Background Sturge-Weber syndrome (SWS) is primarily diagnosed in pediatric population, but clinical presentation in late adulthood is rarely reported. Evolution of radiological findings in the adulthood variant of SWS with isolated leptomeningeal angiomatosis has never been reported to our knowledge. Case presentation We report here a case of an isolated temporo-parieto-occipital leptomeningeal angiomatosis on the right cerebral hemisphere in a sixty-two-year-old male who presented with generalized seizure, GCS score 14/15 (E4 V4 M6) with equal and reacting pupils, psychomotor slowing, left hemineglect and grade 4 power in the left upper and lower limbs. Over a period of 48 h his neurological status deteriorated, but recovered spontaneously over a week on titration with anticonvulsants. He had a prior history of treatment for focal leptomeningitis, three years ago. Cerebrospinal fluid (CSF) analysis showed glucose of 75 mg/dL, proteins of 65 mg/dL and culture grew no organisms. On follow-up, he had intermittent episodes of focal seizure for two years. Initial, computed tomography of brain showed hyperdense lesion in the parieto-occipital convexity subarachnoid space on the right cerebral hemisphere mimicking subarachnoid hemorrhage and computed tomography angiography showed no significant abnormality. Magnetic resonance imaging (MRI) of brain showed intense pial enhancement in the right temporo-parieto-occipital region with a subtle T2W hyperintense signal in the underlying subcortical white matter without edema or infarct or mass effect. Digital subtraction cerebral angiography (DSA) showed hypertrophy of the cerebral arteries, arteriolo-capillary bed and venules in the right temporo-parieto-occipital territory associated with early arterio-capillary and venous opacification. Serial MRI done after six months, one and two years showed increase in the T2W hyperintense signal in the subcortical white matter and cortical atrophy with no changes in the pial enhancement. MR perfusion imaging showed reduced cerebral blood flow (CBF) and cerebral blood volume (CBV) in the right parieto-temporo-occipital cortical and subcortical regions and increased perfusion in the leptomeninges with reduction of the NAA / Cr ratios in spectroscopy. Conclusion Conglomeration of various radiological findings in MRI, Perfusion, MRS and DSA with the clinical presentation can aid in establishing the diagnosis of this rare presentation of SWS-type 3 variant in late adulthood.

Sturge Weber syndrome

2019 ◽  
Vol 16 (2) ◽  
pp. 46-49
Author(s):  
Yam Bahadur Roka
Keyword(s):  
Cerebral Atrophy ◽  
Partial Seizures ◽  
Weber Syndrome ◽  
Daily Dose ◽  
History Of ◽  
Scalp Wound ◽  
Skin Nodules ◽  
Sturge Weber Syndrome ◽  
Neurocutaneous Syndrome

Sturge-weber syndrome is a type of neurocutaneous syndrome/ neurooculo cutaneous/ phakomatoses that is characterized by facial capillary Port Wine stain, leptomeningealangioma and glaucoma with a incidence of 1 per 20-50,000 live births. A case is reported that presented to the outpatient clinic with discharging wound over the left side of his head for a week. On examination he had a large left facial PW stain involving all the divisions of the Trigeminal nerve. Further examination revealed he had history of partial seizures since last 10 years with incomplete and irregular treatment. He also had right sided hemiatrophy of the limbs along with medial gaze deviation of the left eyeball. Skull skiagram was done outside which revealed calcifications in his left hemisphere and CT was then advised for his headache and seizure that revealed Left hemispheric Tram track calcifications along with cerebral atrophy and loss of cortical volume. EEG was unremarkable. He was managed with antibiotics for the scalp wound and discharged with Leveteracetam 1000 mg daily dose and asked to attend eye hospital for the reduced vision. With the PW stain, skin nodules, eye findings and the radiological features he was diagnosed as SWS and counseled for regular follow up, use of anti-epileptic medications, probable side effects and surgery for eye abnormalities.

An unusual case of acoria in Sturge–Weber syndrome

2018 ◽  
Vol 30 (1) ◽  
pp. NP5-NP6
Author(s):  
Miriam Rahhal-Ortuño ◽  
Manuel Díaz-Llopis ◽  
Luis Alonso-Muñoz ◽  
MS Rahhal
Keyword(s):  
Unusual Case ◽  
Clinical Manifestations ◽  
Weber Syndrome ◽  
Sturge Weber Syndrome ◽  
Nevus Flammeus

We present an unusual case of Sturge–Weber syndrome whose main clinical manifestations were nevus flammeus, seizures, glaucoma, and acoria. To our knowledge, the combination of Sturge–Weber syndrome and acoria has not been previously reported.

On Angiomas of Retina, Brain, and Skin

PEDIATRICS ◽  
1981 ◽  
Vol 67 (5) ◽  
pp. 753-754
Author(s):  
Louis I. Sobel ◽  
Jeffrey G. Odel ◽  
Alan H. Friedman
Keyword(s):  
Growth Hormone ◽  
Vascular Malformations ◽  
Hormone Deficiency ◽  
Upper Eyelid ◽  
Cerebral Angiogram ◽  
Suprasellar Region ◽  
Weber Syndrome ◽  
Sturge Weber Syndrome ◽  
Clinical Description ◽  
The Right

Recently Russell et al1 (Pediatrics 66: 306, 1980) described a child with a hemangioma of the right side of the forehead and right upper eyelid, an arteriovenous malformation of the basal ganglia and suprasellar region, and an ipsilateral angiomatous ocular lesion, who had an isolated growth hormone deficiency. They concluded that the vascular malformations were consistent with a variant of the Sturge-Weber syndrome. It is our opinion that their fundus and cerebral angiogram illustrations and the clinical description of the cutaneous vascular nevi represent a case of Wyburn-Mason syndnome.2-4

Sturge-Weber syndrome with late onset hemiplegic migraine-like attacks and progressive unilateral cerebral atrophy

Cephalalgia ◽  
2013 ◽  
Vol 34 (1) ◽  
pp. 73-77 ◽  
Author(s):  
Vincent Planche ◽  
Olivier Chassin ◽  
Louise Leduc ◽  
Wendy Regnier ◽  
Antony Kelly ◽  
...  
Keyword(s):  
Mental Retardation ◽  
Late Onset ◽  
Cerebral Atrophy ◽  
Venous Drainage ◽  
Developmental Venous Anomaly ◽  
Hemiplegic Migraine ◽  
Preventive Medication ◽  
Weber Syndrome ◽  
Sturge Weber Syndrome ◽  
Fdg Pet Ct

Background Sturge-Weber syndrome (SWS) is an uncommon etiology of hemiplegic migraine-like (HM-like) attacks, associated with epilepsy and mental retardation. Case We report the case of a 40-year-old woman with SWS who has been suffering from HM-like episodes since she was 24, with no history of seizure or mental retardation. Susceptibility weighted imaging (SWI)-MRI and CT scans have shown bilateral calcifications of the choroidal plexuses, a developmental venous anomaly with dilated transmedullary veins and a left parieto-occipital leptomeningeal angioma. 18F-Fluorodeoxyglucose (FDG)-PET/CT revealed a diffuse left-hemisphere hypometabolism. The comparison between the MRI performed at the age of 24 and the one performed at the age of 40 highlighted a progressive unilateral fronto-temporo-parietal atrophy. Surprisingly, even now, cognitive functions of this patient are relatively preserved. Lamotrigine permitted an improvement of HM-like attacks. Discussion Explanations for this minimally symptomatic form of SWS may be the absence of seizure, the importance of her deep venous drainage, the absence of cortical calcification and white matter impairment in the affected hemisphere, and, paradoxically, the severely asymmetric cortical metabolism. Furthermore, this case reinforces the hypothesis that alteration of cerebral hemodynamics could precipitate the cortical spreading depression giving rise to migraine with aura. Conclusion We propose to consider SWS as a cause of apparently isolated hemiplegic migraine and lamotrigine as a preventive medication in HM-like attacks.

scholarly journals Sturge-Weber Syndrome

KYAMC Journal ◽  
2017 ◽  
Vol 4 (1) ◽  
pp. 366-368
Author(s):  
Md Alamgir Hossain ◽  
AKM Bazlul Karim ◽  
Abu Md Shakurul Alam
Keyword(s):  
Right Hemisphere ◽  
Resonance Imaging ◽  
Mentally Disabled ◽  
Weber Syndrome ◽  
The Face ◽  
Sturge Weber Syndrome ◽  
Slow Activity ◽  
The Right ◽  
Venous Angiomas ◽  
Professional Counselling

Introduction: Sturge-Weber syndrome sometimes referred to as encephalotrigeminal angiomatosis, vascular malformation with capillary venous angiomas involving face, choroid of eye, leptomeninges, is a rare congenital neurological and skin disorder.Case presentation: This is case report of 7 years old mentally disabled boy, with long-standing seizures, with a port-wine nevi on the right side of the face along the distribution of trigeminal nerve. Interictal encephalogram showed bilateral slow activity, pronounced in the right hemisphere, with epileptogenic activity in the right fronto-parietal region. Computerized tomography and magnetic resonance imaging showed intracranial calcifications, abnormally large veins in deep medullary and subependymal periventricular region, atrophy of the right hemisphere of brain and ipsilateral thickening of skull.Conclusion: Professional counselling and support in addition to drug treatment an provide help to patients and their family to overcome their problems and improve the treatment outcome.KYAMC Journal Vol. 4, No.-1, July 2013, Page 366-368

scholarly journals Management of childhood glaucoma in Sturge Weber Syndrome: A challenge

2016 ◽  
Vol 7 (2) ◽  
pp. 194-197
Author(s):  
Poonam Lavaju ◽  
Pawan Mahat
Keyword(s):  
Anterior Chamber ◽  
Visual Outcome ◽  
Progressive Increase ◽  
It Management ◽  
Increased Risk ◽  
Weber Syndrome ◽  
Sturge Weber Syndrome ◽  
Shallow Anterior Chamber ◽  
The Right ◽  
Choroidal Effusion

Background: Sturge-Weber syndrome (SWS) is a rare congenital neuro- oculo- cutaneous disorder. Glaucoma is one of the ocular abnormalities associated with it. Management of glaucoma in SWS is challenging. Objective: To report surgical and visual outcome of trabeculectomy in bilateral glaucoma in Sturge-Weber syndrome and the challenges encountered in its management. Case description: A six-year-old girl presented with gradually progressive increase in size of both the eyes associated with watering, photophobia and diminution of vision since birth. On examination she was diagnosed to have bilateral SWS with glaucoma, refractive to antiglaucoma medications. She underwent trabeculectomy with intraoperative use of 5- FU. The post operative period was complicated with choroidal effusion in both the eyes with shallow anterior chamber. In the second post operative day, the right eye anterior chamber re- formation was done with ringer lactate solution. There was spontaneous resolution of choroidal effusion with normalization of intraocular pressure with no adverse effect on visual acuity within one week. Conclusion: Treatment of glaucoma associated with Sturge-Weber syndrome is challenging. When planning surgical intervention an increased risk of choroidal effusion associated with it, should be kept in mind. 

scholarly journals CRYO-SURGICAL MANAGEMENT OF ANGIOMATOUS GINGIVAL OVERGROWTH ASSOCIATED WITH STURGE WEBER SYNDROME (SWS) - A CASE REPORT.

2020 ◽  
pp. 27-28
Author(s):  
A Chaitanya ◽  
NVS. Sruthima. G ◽  
Gautami S Penmetsa ◽  
Ramesh KSV
Keyword(s):  
Treatment Option ◽  
Type I ◽  
Effective Treatment Option ◽  
Recurrence Rates ◽  
Excessive Bleeding ◽  
Gingival Overgrowth ◽  
Patient Reported ◽  
Weber Syndrome ◽  
Sturge Weber Syndrome ◽  
The Right

A 22 year old male patient reported with extra oral unilateral port wine stain on the right side of the face with intraoral diffused angiomatous gingival overgrowth in the maxillary posterior region and palate on the right side. It is identifiedon Roach scale as type I Sturge Weber Syndrome (SWS).Due to the typical involvement of multiple feeder vessels in SWS, tendency towards excessive bleeding intrasurgically and higher recurrence rates reported, cryotherapy is opted as a novel treatment option. The treatment outcome achieved suggests that cryotherapy can be an effective treatment option in treating intraoral hemangiomas.

scholarly journals Sturge Weber Syndrome – Roach’s Type II Variant

2020 ◽  
Vol 18 (1) ◽  
pp. 76-79
Author(s):  
Muna Bista ◽  
Sudha Agrawal ◽  
Sweta Taparia
Keyword(s):  
Wide Spectrum ◽  
Clinical Manifestations ◽  
Corneal Edema ◽  
Congenital Glaucoma ◽  
Type Ii ◽  
Ophthalmic Manifestations ◽  
Wide Range ◽  
Weber Syndrome ◽  
History Of ◽  
Sturge Weber Syndrome

Sturge-Weber syndrome (SWS) is a neurocutaneous sporadic disorder caused by mutation in GNAQ gene responsible for persistence of vascular plexus around cephalic portion of neural tube. It has a wide spectrum of cutaneous, neurologic and ophthalmic manifestations, which may or may not be associated with one another. Roach scale has classified it into three types. Here, we present a case of Roach’s Type II variant of SWS with Port-wine stain (PWS) and ocular abnormalities without Central Nervous System (CNS) involvement. A 24 months old female presented with hemangioma involving the left side of face since birth. She had history of corneal edema and buphthalmos at two days of life. There was no history of seizure or developmental delay and Magnetic Resonance Imaging (MRI) of the head ruled out cranial hemangioma. Roach’s Type II is a rare variant of SWS and should be suspected in any case having PWS along the course of trigeminal nerve with congenital glaucoma because the neurologic involvement in a given case may vary from an absence to overt clinical manifestations with or without radiological changes. Due to its wide range of manifestations, a multidisciplinary approach is required for proper management of these patients.

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