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.

P168 Epileptic seizures and control of epilepsy in children with tuberous sclerosis and Sturge Weber syndrome

2009 ◽  
Vol 13 ◽  
pp. S72
Author(s):  
L. Cvitanovic-Sojat ◽  
M. Malenica ◽  
R. Gjergja-Juraski ◽  
M. Pavlovic ◽  
A. Bocan
Keyword(s):  
Tuberous Sclerosis ◽  
Epileptic Seizures ◽  
Weber Syndrome ◽  
Sturge Weber Syndrome ◽  
And Control

Surgical outcomes in children with bottom-of-sulcus dysplasia and drug-resistant epilepsy: a retrospective cohort study

2021 ◽  
pp. 1-11
Author(s):  
Puneet Jain ◽  
Ayako Ochi ◽  
Carter McInnis ◽  
Hiroshi Otsubo ◽  
O. Carter Snead ◽  
...  
Keyword(s):  
Surgical Treatment ◽  
Medical Records ◽  
Focal Cortical Dysplasia ◽  
Seizure Freedom ◽  
Language Mapping ◽  
Resective Surgery ◽  
Histological Data ◽  
Drug Resistant Epilepsy ◽  
Video Eeg

OBJECTIVE Bottom-of-sulcus dysplasia (BOSD) is challenging to identify radiologically. The aim of this study was to explore seizure outcomes after resective surgery or MR-guided laser interstitial thermal therapy (MRgLITT) in children with BOSD. METHODS Children with radiologically defined BOSD who underwent resective surgery or MRgLITT, with at least 1 year of follow-up were included. Clinical, radiological, neurophysiological, and histological data were extracted from medical records. Invasive video EEG (IVEEG) was used to evaluate the ictal onset zone or motor/language mapping, wherever appropriate. Histology of MRI-visible BOSD, including the overlying and adjacent cortex, was also evaluated. RESULTS Forty-one children with BOSD underwent surgical treatment. The lesion was initially overlooked on MRI in 20 patients (48.8%). Of 34 patients who underwent IVEEG and who had available ictal data, the ictal onset zone extended beyond the MRI-visible BOSD in 23 patients (67.6%). Surgical treatment included lesionectomy (24 patients), extended lesionectomy (12 patients), lobectomy (1 patient), and ablation of BOSD (4 patients). The pathology in 37 patients who underwent resection showed focal cortical dysplasia type IIB and type IIA in 21 (53.8%) and 16 patients (41%), respectively. Seizure freedom was achieved in 32 patients (78.1%) after a mean follow-up of 4.3 years. CONCLUSIONS Seizure outcomes after resective surgery or MRgLITT in children with BOSD were generally favorable. The authors found that the neurophysiological abnormality and pathology often extended beyond the MRI-visible BOSD.

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.

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.

scholarly journals Sturge-Weber syndrome coexisting with polydactyly: a case report

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Hongxi Wang ◽  
Nana Dong ◽  
Li Tan ◽  
Chukai Huang
Keyword(s):  
Intraocular Pressure ◽  
Case Report ◽  
Port Wine ◽  
Case Presentation ◽  
Port Wine Stains ◽  
Weber Syndrome ◽  
Left Neck ◽  
Sturge Weber Syndrome ◽  
Nevus Flammeus ◽  
Underlying Pathogenesis

Abstract Background Sturge-Weber syndrome (SWS) is a sporadic congenital disorder, characterized by unilateral facial nevus flammeus associated with ipsilateral glaucoma, choroidal angioma and leptomeningeal hemangiomas. SWS can comorbid with other disorders in some patients, however, there has been no prior described case of SWS and polydactyly occurring in the same patient. Case presentation A 15-year-old girl with diagnosis of SWS presented to our hospital. She had bilateral glaucoma and extensive port-wine stains distributing in bilateral faces, left neck and left upper limb. Meanwhile, the patient was noted to demonstrate the superfluous digit attaching on the left thumb and was diagnosed as polydactyly. Trabeculectomy, with intraoperative application of mitomycin C and postoperative subconjunctival injections of 5-fluorouracil, was successful in controlling the intraocular pressure in both eyes. Conclusions We report a case with bilateral SWS coexisting with unilateral polydactyly, which, to our knowledge, has not been recognized previously and adds further evidence to the existing literature. In view of the rare concurrence of SWS and polydactyly, the etiology is unclear and further investigation is required to explore the underlying pathogenesis.

scholarly journals Epilepsy in Sturge-Weber syndrome: a literature review and description of a clinical case

2020 ◽  
Vol 12 (1) ◽  
pp. 67-73
Author(s):  
N. I. Shova ◽  
V. A. Mikhailov ◽  
S. A. Korovina ◽  
D. V. Alekseeva
Keyword(s):  
Clinical Observation ◽  
Epileptic Seizures ◽  
Pediatric Practice ◽  
Antiepileptic Therapy ◽  
Weber Syndrome ◽  
Comprehensive Examination ◽  
Long Time ◽  
Sturge Weber Syndrome ◽  
Ischemic Attack ◽  
The Moment

The Struge-Weber syndrome is the third most common neurodermal disease after neurofibromatosis and tuberous sclerosis. This disease is not inherited, but occurs exclusively sporadically, both in men and in women, and in all races and ethnic groups. In 90% of cases, this syndrome is diagnosed in pediatric practice. Adult cases of this syndrome are sporadic, since they remain unrecognized due to the polymorphism of this disease. Comorbidity between epilepsy and the Sturge-Weber syndrome was noted.Aim. To conduct a literature analysis and describe the clinical observation of epilepsy with the Sturge-Weber syndrome.Materials and methods. Patient M., 50 years old has been observed for a long time due to complaints on episodes of numbness according to the hematotype with transient paresis. The examination (MRI) revealed changes characteristic for Sturge-Weber syndrome. The presence of specific paroxysmal changes according to the results of electroencephalography helped to verify the genesis of paroxysmal conditions.Results. The patient underwent a comprehensive examination. As a result, the diagnosis of epilepsy was verified. The prescription of adequate antiepileptic therapy allowed for stabilization and jugulation of epileptic seizures.Conclusion. In this clinical observation, we focus on the differential diagnosis between transient ischemic attack and epilepsy. Unfortunately, at the moment, adults with a previously undetectable syndrome undergo multiple hospitalizations and do not receive antiepileptic therapy, since the prior disease (epilepsy) is not verified.

Surgical treatment of epilepsy in Sturge–Weber syndrome in children

2007 ◽  
Vol 106 (1) ◽  
pp. 20-28 ◽  
Author(s):  
Marie Bourgeois ◽  
Darach William Crimmins ◽  
Ricardo Santos De Oliveira ◽  
Alexis Arzimanoglou ◽  
Matthew Garnett ◽  
...  
Keyword(s):  
Surgical Treatment ◽  
Treatment Of Epilepsy ◽  
Weber Syndrome ◽  
Sturge Weber Syndrome

Sturge-Weber Syndrome: A Case Study

2006 ◽  
Vol 25 (2) ◽  
pp. 89-98 ◽  
Author(s):  
Linda Welty
Keyword(s):  
Mental Retardation ◽  
Clinical Presentation ◽  
Occipital Region ◽  
Port Wine ◽  
Case Presentation ◽  
Port Wine Stains ◽  
Weber Syndrome ◽  
Sturge Weber Syndrome ◽  
Congenital Syndrome

Sturge-Weber syndrome (SWS) is a rare, sporadic, progressive, congenital syndrome. In its complete trisymptomatic form, SWS is physically characterized by port-wine stains over the trigeminal area, leptomeningeal angiomas usually over the parieto-occipital region, and eye abnormalities. Clinical manifestation for infants with SWS depends on the affected organs, but can include seizures, mental retardation, and glaucoma. This article begins with a case presentation of an infant with SWS and then presents the etiology, embryology, pathophysiology, clinical presentation, management, and prognosis of SWS.

scholarly journals Surgical Treatment of Sturge Weber Syndrome

1989 ◽  
Vol 29 (1) ◽  
pp. 60-64 ◽  
Author(s):  
Masanori ITO ◽  
Kiyoshi SATO ◽  
Chikashi MARUKI ◽  
Taizo NITTA ◽  
Akira OHNUKI ◽  
...  
Keyword(s):  
Surgical Treatment ◽  
Weber Syndrome ◽  
Sturge Weber Syndrome

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.

Sign in / Sign up

Export Citation Format

Share Document