scholarly journals A case report of a pregnant woman with Sturge-Weber syndrome

2020 ◽  
Vol 48 (4) ◽  
pp. 030006052091370
Author(s):  
Ying Luo ◽  
Yang Yang ◽  
Xiu Chen
Keyword(s):  
Magnetic Resonance Imaging ◽  
Pregnant Woman ◽  
Cerebral Hemisphere ◽  
Surgical Intervention ◽  
Port Wine ◽  
Resonance Imaging ◽  
Intracranial Vessel ◽  
Weber Syndrome ◽  
Sturge Weber Syndrome ◽  
The Right

A 23-year-old pregnant woman presented with headache and paroxysmal spasm of the right limb. She was born with a port-wine birthmark around the left eye frame. Magnetic resonance imaging revealed evidence of atrophy, calcification and vascular malformation in the left cerebral hemisphere. She was diagnosed with Sturge-Weber syndrome. She gave birth to a child without a port-wine birthmark through a caesarean section and her headache eased without surgical intervention of the intracranial vessel hyperplasia. This case suggests that pregnant women with SWS can deliver safely.

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.

scholarly journals Spectrum of Magnetic Resonance Imaging Findings in Sturge Weber Syndrome: A Rare Neurocutaneous Syndrome

2021 ◽  
pp. 20-26
Author(s):  
Varun Singh ◽  
Bhushita Lakhkar ◽  
Rajasbala P. Dhande ◽  
Shreya Tapadia ◽  
Bhavik Unadkat
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Clinical History ◽  
Final Diagnosis ◽  
Neurological Deficits ◽  
Resonance Imaging ◽  
Mri Findings ◽  
Weber Syndrome ◽  
Sturge Weber Syndrome ◽  
Neurocutaneous Syndrome

Sturge Weber syndrome is a type of neurocutaneous disorder also known as meningofacial angiomatosis. It is generally seen in paediatric age group and more  commonly effects the  males which present with delayed milestones, seizure disorder, loss of vision and other neurological deficits. Clinical suspicion of Sturge weber syndrome should be made on the  presence of port wine stain(facial nevus) in a young children. MRI (magnetic resonance imaging) plays a key role in detecting the various spectrum of cortical and vascular defects associated with the syndrome. Clinicians can come to a final diagnosis of Sturge weber syndrome, when the clinical history is supplemented with MRI findings. We in our case report will be discussing the various spectrum of MR findings in this rare neurocutaneous syndrome.

scholarly journals Early magnetic resonance imaging to detect presymptomatic leptomeningeal angioma in children with suspected Sturge–Weber syndrome

2019 ◽  
Vol 62 (2) ◽  
pp. 227-233 ◽  
Author(s):  
Claire Bar ◽  
Jean‐Michel Pedespan ◽  
Olivia Boccara ◽  
Nicolas Garcelon ◽  
Raphael Levy ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Resonance Imaging ◽  
Weber Syndrome ◽  
Sturge Weber Syndrome

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

Sturge-Weber syndrome with spontaneous intracerebral hemorrhage in childhood

2014 ◽  
Vol 13 (1) ◽  
pp. 90-93 ◽  
Author(s):  
Madoka Nakajima ◽  
Hidenori Sugano ◽  
Yasushi Iimura ◽  
Takuma Higo ◽  
Hajime Nakanishi ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Intracerebral Hemorrhage ◽  
Venous System ◽  
Deep Coma ◽  
Resonance Imaging ◽  
Deep Venous System ◽  
Thalamic Hemorrhage ◽  
Weber Syndrome ◽  
Sturge Weber Syndrome

A girl aged 2 years 10 months suddenly went into a deep coma and demonstrated left hemiplegia. At birth, she had exhibited a left-sided facial port-wine stain typical of Sturge-Weber syndrome (SWS) and involving the V1 and V2 distributions of the trigeminal nerve. Computed tomography showed a right thalamic hemorrhage with acute hydrocephalus. Magnetic resonance imaging with Gd enhancement 8 months before the hemorrhage had shown a patent superior sagittal sinus (SSS) and deep venous system. Magnetic resonance imaging and MR angiography studies 2 months before the hemorrhage had revealed obstruction of the SSS and right internal cerebral vein (ICV). Given that a digital subtraction angiography study obtained after the hemorrhage did not show the SSS or right ICV, the authors assumed that impaired drainage was present in the deep venous system at that stage. The authors speculated that the patient's venous drainage pattern underwent compensatory changes because of the occluded SSS and deep venous collectors, shifting outflow through other cortical venous channels to nonoccluded dural sinuses. Sudden congestion (nearly total to total obstruction) of the ICV may have caused the thalamic hemorrhage in this case, which is the first reported instance of pediatric SWS with intracerebral hemorrhage and no other vascular lesion. Findings suggested that the appearance of major venous sinus occlusion in a child with SWS could be a warning sign of hemorrhage.

Sturge-Weber Syndrome: Brain Magnetic Resonance Imaging and Neuropathology Findings

2016 ◽  
Vol 58 ◽  
pp. 25-30 ◽  
Author(s):  
Anna L. Pinto ◽  
Liam Chen ◽  
Rachel Friedman ◽  
Patricia E. Grant ◽  
Annapurna Poduri ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Brain Magnetic Resonance Imaging ◽  
Resonance Imaging ◽  
Weber Syndrome ◽  
Sturge Weber Syndrome

Sturge-Weber Syndrome

2000 ◽  
Vol 3 (3) ◽  
pp. 301-305 ◽  
Author(s):  
Venita Jay
Keyword(s):  
Nerve Tissue ◽  
Port Wine ◽  
Difficult Question ◽  
Probable Relationship ◽  
Weber Syndrome ◽  
Mucous Membranes ◽  
Sturge Weber Syndrome ◽  
The Right ◽  
The Brain

I have no intention of going into the difficult question of the pathology of port-wine mark. The point to which I wish to call particular attention is the probable relationship between the mark and the fits …. From the nature of the fits, and from their mode of onset, I think there can be no doubt that they are due to some cause external to the nerve-tissue … and this external cause is in all probability to be found in the presence of a “port-wine mark” on the surface of the right side of the brain, just as we have found it in the skin, mucous membranes, and retina of that side. William Allen Sturge, 1879

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