Flunarizine and Aspirin for Transient Hemiparesis in Sturge–Weber Syndrome

D’Arrigo, Stefano; Tessarollo, Valeria; Maselli, Erica; Pantaleoni, Chiara; Canafoglia, Laura

doi:10.1055/s-0039-1685528

Cited by 3 publications

(4 citation statements)

References 4 publications

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“…5,10 EDI SD-OCT can show choroidal thickness above 1000 m and B-Scan will show the diffuse choroidal hemangioma. 4,10…”

Section: Discussionmentioning

confidence: 99%

“…turge-weber syndrome (SWS) 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. 4,5 Somatic mutation in the GNAQ gene located on chromosome 9 affects the G proteins that act as modulators/translators in transmembrane signaling systems and is found in 80% of cases with SWS and the dysregulation of vascular MAPK and/or PI3K signaling…”

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confidence: 99%

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Sturge Weber syndrome

Roka

2019

Nep J Neurosci

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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.

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“…5,10 EDI SD-OCT can show choroidal thickness above 1000 m and B-Scan will show the diffuse choroidal hemangioma. 4,10…”

Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

Sturge Weber syndrome

Roka

2019

Nep J Neurosci

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“…Similar to headaches, hemiplegic migraine attacks can be triggered by factors such as head trauma (42,44). Many drugs have been used in the acute and prophylactic therapy of hemiplegic migraine, including antiepileptic drugs such as topiramate, valproate and lamotrigine, as well as flunarizine and ASA with variable efficacy (22,41). However, no studies have evaluated the retrospective or prospective observational response to such therapy in this condition.…”

Section: Hemiplegic Migrainementioning

confidence: 99%

Headache in Sturge–Weber syndrome: A systematic review

Ferretti,

Muscianese,

Fanfoni

et al. 2024

Cephalalgia

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Background Sturge–Weber syndrome (SWS) is a neurocutaneous disorder for which the neurological aspects, particularly headaches, remain poorly understood, despite significantly affecting morbidity. The present study aimed to elucidate the prevalence, characteristics and treatment strategies, as well as explore the pathogenesis of headaches, in SWS. Methods Using Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines, we systematically reviewed observational studies, case reports and series from eight databases (Cochrane Library, EBSCO, Embase, Medline, PubMed, Science Direct, Scopus and Web of Science), published from 1978 to 2023, to investigate the prevalence, characteristics, medication response and pathogenic theories of headaches in SWS. Results The review analyzed 48 studies, uncovering headache prevalence between 37% and 71%. Migraine-like headache affected up to 52% of individuals. Prophylactic and acute treatments included non-steroidal anti-inflammatory drugs, triptans and antiepileptic drugs, despite the lack of established guidelines. Life-threatening headaches in SWS are uncommon, typically accompanied by other neurological symptoms. The pathogenesis of headaches in SWS is considered to involve venous congestion and neuronal hyperexcitability linked to leptomeningeal angiomas. Conclusions Headaches occur more frequently in individuals with SWS than in the general population. Despite symptoms meeting migraine criteria, these headaches should be considered secondary to vascular conditions. Implementing acute and prophylactic treatment is advised to reduce the impact on patients’ lives.

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“…Moreover, no significant adverse effects have been detected [51]. Also a combination of antiaggregation and vasodilation (aspirin and flunarizine) has resulted effective, though in a single case so far [32]. Aspirin administration has been found also to have an impact in decreasing seizure outburst [135].…”

Section: Medical Therapymentioning

confidence: 99%

Sturge-Weber syndrome: an update on the relevant issues for neurosurgeons

et al. 2020

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Purpose Sturge-Weber syndrome (SWS) is a neurocutaneous facomatosis characterized by facial and leptomeningeal angioma, glaucoma, seizures, and neurological disability. Therefore, a challenging multidisciplinary interaction is required for its management. The goal of this paper is to review the main aspects of SWS and to present an illustrative pediatric series. Methods The pertinent literature has been analyzed, focused mainly on etiopathogenesis, pathology, clinical features, diagnostic tools, management, and outcome of the disease. Moreover, a series of 11 children operated on for refractory epilepsy between 2005 and 2015 (minimum follow-up 5 years, mean follow-up 9.6 years) is reported. The series consists of six boys and five girls with 6.5-month and 16.2-month mean age at seizure onset and at surgery, respectively. Seizures affected all children, followed by hemiparesis and psychomotor delay (81%), glaucoma (54%), and other neurological deficits (45%). Results All children underwent hemispherectomy (anatomical in three cases, functional in two cases, hemispherotomy in six cases); one patient needed a redo hemispherotomy. Mortality was nil; disseminated intravascular coagulation and interstitial pneumonia occurred in one patient each; three children had subdural fluid collection. Eight patients (72%) are in the ILAE Class 1 (completely seizure and aura free), two in Class 2 (only auras, no seizure), and one in Class 3 (1-3 seizure days per year). AEDs discontinuation was possible in 73% of cases. The most important news from the literature concerned the pathogenesis (role of the mutation of the GNAQ gene in the abnormal SWS vasculogenesis), the clinical findings (the features and pathogenesis of the stroke-like episodes are being understood), the diagnostic tools (quantitative MRI and EEG), and both the medical (migraine, seizures) and surgical management (epilepsy). The epileptic outcome of SWS patients is very good (80% are seizure-free), if compared with other hemispheric syndromes. The quality of life is affected by the neurological and cognitive deficits. Conclusions SWS still is an etiological and clinical challenge. However, the improvements over the time are consistent. In particular, the neurosurgical treatment of refractory epilepsy provides very good results as long as the indication to treatment is correct.

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Flunarizine and Aspirin for Transient Hemiparesis in Sturge–Weber Syndrome

Cited by 3 publications

References 4 publications

Sturge Weber syndrome

Sturge Weber syndrome

Headache in Sturge–Weber syndrome: A systematic review

Sturge-Weber syndrome: an update on the relevant issues for neurosurgeons

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