Abstract:The incidence, diagnostic landscape, and workload impact of CNS inflammatory diseases other than multiple sclerosis (MS) (CIDOMS) in a tertiary setting is unknown. We describe a retrospective case series of 64 patients identified over a 2-year period (2009–2010) at the Wessex Neurological Centre in the United Kingdom, accounting for 4% of all patients seen at the center. As expected, neurosarcoidosis and neuromyelitis optica (NMO) were the most common diagnoses reached (14% each); other diagnoses singly accoun… Show more
“…The dynamic nature of cerebral perfusion via collateral blood vessels means that imaging of collateral blood flow is a snapshot in time and circumstances may change. Boulouis et al 1 examined collateral blood vessels using CTA performed after transfer. In some patients, initially poor collateral blood flow may have improved during transfer, but often this would be too late to reduce the extent of infarct growth.…”
Section: Opinion Editorialmentioning
confidence: 99%
“…The practical options for acute stroke imaging in primary stroke centers are some combination of noncontrast CT, CTA (single phase or multiphase), and CTP. Collateral assessment using single-phase CTA acquired at peak arterial phase, as applied in the study by Boulouis et al, 1 is less accurate than multiphase CTA or CTP because late-arriving collateral blood flow may be underestimated, potentially leading to unwarranted exclusion of patients from endovascular thrombectomy. The limited interrater reliability of ASPECTS and insensitivity to ischemic changes within the first 1 to 2 hours after stroke onset 12 are further complicating factors when determining the optimal primary stroke center imaging strategy.…”
Global measures of disability, such as the modified Rankin score, can be informative and are widely used in other neurologic contexts, such as stroke 18 and encephalitis, 19 but are useful only if measured using formalized definitions and preferably prospectively. A consensus NS-specific metric that measures change and severity within the affected neuroanatomy of a patient could provide a meaningful way forward.The analysis by Joubert and colleagues 16 valuably expands our knowledge about NS prognosis in the current era. Key findings are that neurologic relapses in patients with NS are common, relapses in other organ systems are remarkably common, and immunosuppression reduces relapse risk. Furthermore, certain immunosuppressants appear to be favorable, with particular note of the lack of an observed benefit with mycophenolic acid (mycophenolate), a finding consistent with another recent study in neurosarcoidosis. 20 Development of sarcoidosis-specific outcome measures and consensus approaches to reporting could help to catalyze efforts to advance better treatments and improve prognosis for patients with NS, an enigmatic disease that, despite deep historical roots, continues to perplex and beguile us.
“…The dynamic nature of cerebral perfusion via collateral blood vessels means that imaging of collateral blood flow is a snapshot in time and circumstances may change. Boulouis et al 1 examined collateral blood vessels using CTA performed after transfer. In some patients, initially poor collateral blood flow may have improved during transfer, but often this would be too late to reduce the extent of infarct growth.…”
Section: Opinion Editorialmentioning
confidence: 99%
“…The practical options for acute stroke imaging in primary stroke centers are some combination of noncontrast CT, CTA (single phase or multiphase), and CTP. Collateral assessment using single-phase CTA acquired at peak arterial phase, as applied in the study by Boulouis et al, 1 is less accurate than multiphase CTA or CTP because late-arriving collateral blood flow may be underestimated, potentially leading to unwarranted exclusion of patients from endovascular thrombectomy. The limited interrater reliability of ASPECTS and insensitivity to ischemic changes within the first 1 to 2 hours after stroke onset 12 are further complicating factors when determining the optimal primary stroke center imaging strategy.…”
Global measures of disability, such as the modified Rankin score, can be informative and are widely used in other neurologic contexts, such as stroke 18 and encephalitis, 19 but are useful only if measured using formalized definitions and preferably prospectively. A consensus NS-specific metric that measures change and severity within the affected neuroanatomy of a patient could provide a meaningful way forward.The analysis by Joubert and colleagues 16 valuably expands our knowledge about NS prognosis in the current era. Key findings are that neurologic relapses in patients with NS are common, relapses in other organ systems are remarkably common, and immunosuppression reduces relapse risk. Furthermore, certain immunosuppressants appear to be favorable, with particular note of the lack of an observed benefit with mycophenolic acid (mycophenolate), a finding consistent with another recent study in neurosarcoidosis. 20 Development of sarcoidosis-specific outcome measures and consensus approaches to reporting could help to catalyze efforts to advance better treatments and improve prognosis for patients with NS, an enigmatic disease that, despite deep historical roots, continues to perplex and beguile us.
“…between 20 and 50 years old) [1][2][3]. Multiple sclerosis (MS) is by far the most common of these disorders but it should be noted that diagnosis should only be applied in the presence of typical symptoms reminiscent of MS and in the absence of specific red flags (Table 1) suggesting the possibility of an alternative diagnosis [2,[4][5][6]. Indeed, in the presence of such red-flags, other non-MS inflammatory diseases must be carefully searched since their prognosis and treatments markedly differ [7][8][9].…”
“…This situation is not without consequence. Among neuroinflammatory disorders, diagnostic workups for NS account for a disproportionately large share of total inpatient costs, 1 and tremendous variability in clinical practice exists regarding application of various proposed diagnostic criteria. 2,3 Particularly in the absence of histopathologic findings, this can lead in some cases to premature or unwarranted diagnosis of NS.…”
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