Cowden disease is a rare autosomal dominant disorder first described by Rachel Cowden in 1963 as one of the phosphatase and tensin homolog (PTEN) gene hamartoma tumor syndromes. Commonly affecting multiple systems, patients typically have an increased risk of breast and thyroid cancers as well as macrocephaly and benign hamartomatous growths. Due to the benign nature of many of the symptoms, true prevalence is likely higher than reported rates. Malformations associated with PTEN gene lead to dysregulation of cell proliferation yielding increased risk of neoplasm and frequently benign growths. Associated dysregulation of angiogenesis may result in arteriovenous malformations (AVMs). Intracranial AVMs may be asymptomatic. These AVMs present a risk of rupture and subsequent morbidity and mortality. Those with Cowden disease must be monitored for development of intracranial AVMs. Patients should be made aware that treatment for intracranial AVMs may be invasive and include serial embolization.
Objectives: The Distal Hyperintense Vessel (DHV) sign on FLAIR imaging is a radiographic marker of inadequate blood flow due to poor collateral flow distal to the stenotic artery. Previous studies suggest that the DHV sign was associated with early recurrent ischemic stroke in the anterior circulation secondary to intracranial atherosclerotic disease (ICAD). However, its significance in ischemic stroke in the posterior circulation is unknown. Here, we investigate the association of DHV sign in the basilar artery and outcomes at discharge in patients with posterior circulation stroke or TIA secondary to ICAD. Methods: We retrospectively reviewed patients with ischemic strokes or TIA attributed to ICAD of the basilar or vertebral arteries admitted to two comprehensive stroke centers affiliated with UTSW Medical Center from 2010 to 2022. Patients were included if they met VERiTAS criteria (≥50% vertebrobasilar stenosis). The DHV sign was defined as positive when the increased intensity in the basilar artery distal to stenosis was higher than the surrounding CSF signal. The DHV sign was evaluated by blinded vascular neurologists and neuro-radiologists. The primary outcome was mRS at discharge. The secondary outcome was the incidence of clinical deterioration during admission, defined as any worsened neurological exam with associated new infarct or infarct expansion in the posterior circulation. Results: A total of 135 patients were included in the study. A total of 33 (24%) patients had the presence of the DHV sign on admission MRI. Compared to patients without DHV sign, patients with DHV sign had higher NIHSS scores on admission (median 7 vs 2, p<0.001), higher risk of clinical deterioration during hospitalization (27% vs 2%, p<0.001), higher mRS scores at discharge (median 3.45 vs 1.8, p<0.001) and higher mortality rate (24% vs 0%, p<0.001). In multivariate analysis, the DHV sign was an independent predictor of higher mRS scores at discharge. Conclusions: In patients with ischemic stroke or TIA secondary to basilar or vertebral artery atherosclerotic stenosis, the presence of the DHV sign in the basilar artery is associated with more severe disease and poorer outcomes at discharge compared to patients without DHV sign.
April 28, 2017April 18, 2017Free AccessCopper Deficiency in patient with prior diagnosis of AIDP (P6.202)Parth Upadhyaya, OMS, IV, Farzan Ghodsianzadeh, DO, Rebecca Romero, MD, and Ratna Bhavaraju-Sanka, MDAuthors Info & AffiliationsApril 18, 2017 issue88 (16_supplement)https://doi.org/10.1212/WNL.88.16_supplement.P6.202 Letters to the Editor
Abstract Background : Tenecteplase is a tissue plasminogen activator with higher fibrin specificity compared with Alteplase. Accumulating data suggests that intravenous Tenecteplase 0.25mg/kg is non-inferior to Alteplase 0.9mg/kg for acute ischemic stroke. We describe our 10-months experience. Methods : At our MRI-based, urban comprehensive stroke center, we switched the intravenous thrombolytic agent for acute ischemic stroke to Tenecteplase 0.25mg/kg on March 23, 2021. Until January 31, 2022, 62 stroke patients were treated with Tenecteplase. We compared clinical and safety outcomes of Tenecteplase-treated patients with 94 Alteplase-treated patients. Results : During the study period, nine (15%) patients with unknown stroke onset were thrombolyzed with MRI screening. Nineteen (35%) patients underwent subsequent thrombectomy. When compared with Alteplase-treated patients, there was no difference with Tenecteplase-treated patients in 90-day functional outcome, death, symptomatic intracranial hemorrhage, or angioedema. Conclusions : The use of Tenecteplase for stroke thrombolysis was feasible with comparable safety and functional outcomes compared to Alteplase, even when Tenecteplase was administered based on MRI screening to stroke patients with unknown onset.
Cerebral microbleeds (cMBs) are common imaging findings in conditions related to cerebral amyloid angiopathy (CAA). Blood-brain barrier (BBB) leakage is considered pivotal in their pathogenesis. This study investigates the potential role of cerebral microenhancement (cME) as an imaging biomarker on 3D T1 black-blood MRI (BB-MRI) for BBB rupture, predicting the formation of cMBs in inflammatory CAA variants.
Abstract Purpose of Review Primary exercise headache has gone through many descriptors in the past but generally is a headache that is precipitated by strenuous exercise without significant intracranial pathology. Its presentation can remain vague, often confused with other primary and secondary headache disorders and thus undertreated. This review aims to discuss primary exercise headache in the context of epidemiology, presentation, pathophysiology, differential diagnosis, and treatment. Recent Findings Two large epidemiological studies in Iran and Japan have further characterized a predilection for female patients, comorbidity with migraine, and frequent bilateral nature of headache. Summary While large-scale epidemiological studies have aided in further characterization and determining varying prevalence, a lack of randomized clinical trials in the treatment of primary exercise headache remains. Indomethacin and beta-blocker use remain the mainstays of treatment based on case series with several case reports that urge caution when diagnosing said headache.
