Spontaneous obliteration of MRI-silent cerebral angiomatosis revealed by CT angiography in a patient with Sturge–Weber syndrome

Aguglia, Umberto; Latella, Maria Adele; Cafarelli, Franco; Piane, Emilio Le; Gangemi, Antonio; Labate, Angelo; Gambardella, Antonio; Quattrone, Aldo

doi:10.1016/j.jns.2007.07.024

Cited by 5 publications

(6 citation statements)

References 15 publications

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“…To the best of our knowledge, six cases of SWS with intracranial hemorrhage have been reported to date (Anderson and Duncan 1974 ; Pozzati et al 1983 ; Dolkart and Bhat 1995 ; Aguglia et al 2008 ; Lopez et al 2013 ; Nakajima et al 2014 ; Table 1 ). The median age of the patients was 15.1 years (range 1–62 years old).…”

Section: Discussionmentioning

confidence: 99%

“…The hormonal or hemodynamic changes associated with pregnancy were suspected to be responsible for the bleeding. Aguglia et al described a 37-year-old woman with intracranial hemorrhage in the left temporal lobe that was derived from the spontaneous thrombosis of left temporal angiomatous malformation (Aguglia et al 2008 ). Lopez et al reported a 20-month-old boy with right subdural hematoma that appeared to be associated with head trauma of an occipital lesion (Lopez et al 2013 ).…”

Section: Discussionmentioning

confidence: 99%

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Sturge–Weber syndrome with intracerebral hemorrhage: a case report

et al. 2016

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Introduction Sturge–Weber syndrome (SWS) is a rare congenital disease that affects the brain, skin, and eyes, and is a sporadically occurring neurocutaneous syndrome that affects intracerebral veins, which is associated with venous thrombosis. However, intracranial hemorrhage in patients with SWS is rare. We herein report a rare case of SWS with intracerebral hemorrhage derived from sinus thrombosis.Case descriptionA 62-year-old man suddenly fell into a coma and was admitted to our hospital. His neurological status was assessed as GCS 6 (E1V1M4) with right-sided hemiparesis. At birth, he had a right-sided facial port-wine stain typical of SWS that involved the ophthalmic division of the trigeminal nerve. Laboratory findings showed that he was dehydrated, and his serum D-dimer concentration was increased. Computed tomography revealed left thalamic hemorrhage with acute hydrocephalus and cortical calcification in the right occipital lobe. Magnetic resonance imaging displayed a vascular malformation of the right cerebral hemisphere consistent with SWS. Magnetic resonance venography showed steno-occlusion of the superior sagittal sinus, straight sinus, and left internal cerebral vein (ICV). Emergency ventricular drainage was performed. Seven days after surgery, his consciousness improved to GCS 14 (E4V4M6). Rehydration therapy was performed to prevent sinus thrombosis.Discussion and EvaluationHis postoperative course was uneventful. Sudden congestion of the left ICV may have caused left thalamic hemorrhage.ConclusionsSWS with major sinus occlusion needs to be diagnosed with utmost caution in order to allow for preoperative neurological and radiological assessments.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Sturge–Weber syndrome with intracerebral hemorrhage: a case report

et al. 2016

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“…3 To our knowledge, all patients with intracranial hemorrhage associated with SWS in previous reports 9 had some type of vascular le- sion, including angioma, 7 arteriovenous malformation, 1,11 or aneurysm. 13 Therefore, spontaneous intracerebral hemorrhage in a patient with SWS is extremely rare.…”

Section: Causes Of Intracerebral Hemorrhagementioning

confidence: 93%

Sturge-Weber syndrome with spontaneous intracerebral hemorrhage in childhood

Nakajima¹,

Sugano²,

Iimura³

et al. 2014

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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.

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“…Since then, other cases of hemorrhage in Sturge-Weber syndrome have been published: a ruptured aneurysm, an intraventricular hemorrhage due to choroid plexus angioma, a subdural hemorrhage after minimal trauma, a thalamic hemorrhage due to internal cerebral vein thrombosis, and 3 additional reports of parenchymal hemorrhage. [10][11][12][13][14][15][16] These patients presented with variable symptoms, including seizure exacerbation, altered mental status, headaches and nuchal rigidity, and hemiparesis. Interestingly, only one of the patients was taking aspirin at the time of hemorrhage.…”

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confidence: 99%

Yield of Emergent Neuroimaging in Patients With Sturge-Weber Syndrome Presenting With Acute Neurologic Symptoms

et al. 2018

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Background/Aims: Seizures, strokelike episodes, and headaches are common complications in patients with Sturge-Weber syndrome. Based on our experience, we hypothesized that patients with Sturge-Weber syndrome have frequent urgent neuroimaging studies when presenting acutely to the emergency department. In this study, we aimed to determine the incidence of acute imaging studies in this patient population and to evaluate the prevalence of findings such as acute intracranial hemorrhagic or ischemic strokes. Methods: To determine the frequency and yield of brain imaging, we conducted a retrospective chart analysis in patients with Sturge-Weber syndrome who presented to Boston Children's Hospital with acute neurologic symptoms between 1996 and 2016. Results: We reviewed 136 encounters of patients with Sturge-Weber syndrome. In 73 of 136 encounters (53.7%), patients underwent a total of 89 imaging studies, consisting of 47 head computed tomographies (CTs) and 42 brain magnetic resonance images (MRIs). Twenty-two percent of patients imaged underwent both CT and MRI scanning of the brain. Patients with strokelike episodes or headaches were more likely to be imaged compared to patients presenting with seizures (89.7% and 100% vs 34.4%, respectively). None of the neuroimaging studies showed acute hemorrhagic or ischemic strokes. Conclusions: Acute neurologic manifestations of Sturge-Weber syndrome frequently lead to urgent neuroimaging. In our cohort, there was no imaging evidence of acute hemorrhagic or ischemic strokes. In addition, emergent imaging in patients presenting with breakthrough seizures did not result in meaningful changes in management.

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Spontaneous obliteration of MRI-silent cerebral angiomatosis revealed by CT angiography in a patient with Sturge–Weber syndrome

Cited by 5 publications

References 15 publications

Sturge–Weber syndrome with intracerebral hemorrhage: a case report

Sturge–Weber syndrome with intracerebral hemorrhage: a case report

Sturge-Weber syndrome with spontaneous intracerebral hemorrhage in childhood

Yield of Emergent Neuroimaging in Patients With Sturge-Weber Syndrome Presenting With Acute Neurologic Symptoms

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