Magnetic Resonance Imaging/magnetic Resonance Angiography Research Articles

Moyamoya Syndrome in Children With Sickle Cell Disease in Saudi Arabia: A Single-Center Experience.

Background Sickle cell disease (SCD) is relatively common in Saudi Arabia. Its neurologic complications such as stroke and Moyamoya syndrome (MMS) can be severe and devastating. Such complicationscan be minimized by modern investigative tools such as transcranial Doppler (TCD) ultrasound, which is unavailable in many high-risk places. Our aim is to investigate the prevalence and characteristics of these complications in children with SCD in our center where TCD is not available. Methods We conducted a retrospective record review of children with SCD admitted to the pediatric ward andvisited the pediatric hematology outpatient clinic of King Fahad Military Medical Complex, Dhahran, Saudi Arabia, from January 2010 to December 2021. The target population was children aged sixmonths to 14years with SCD and a history of stroke or transient ischemic attacks (TIAs). Their magnetic resonance imaging/magnetic resonance angiography (MRI/MRA) of the brain radiographic features were reviewed. A descriptive analysis was used to summarize the demographic characteristics and clinical features of patients with and without MMS. Results Twenty-six children (out of 385 with sickle cell anemia, originating mainly from the southwestern and eastern provinces of Saudi Arabia) experienced an overt stroke with an overall prevalence of 6.7%. All patients with stroke were originally from the Southwestern province. Their genotype was SS, and the median age at the onset of the first stroke was six years (IQR: 5.5). The main presenting symptoms were seizures (57.7%), motor weakness (42.3%), headache (15.3%), cranial nerve palsies (11.5%), cognitive deficit (7.6%), and dysphasia (3.8%). The majority of strokes were ischemic (92.3%). MMS was detected in 61.5% and was seen at the onset of the first stroke in all patients with this MRA abnormality. Seven children with moyamoya (43.8%) had recurrent strokes. Conclusion In this study, the prevalence of overt stroke is 9% in children with SCD originating from the southwestern region of Saudi Arabia (26/286), and 61.5% of them (16/26) had MMS. It is absent in the children of Eastern origin (99 children). In places lacking TCD facilities, further studies are required to determine if MRA brain screenings of children with SCD may detect MMS before the onset of stroke and help startprotective therapy.

Thoracic outlet syndrome: a review for the primary care provider.

Thoracic outlet syndrome (TOS) symptoms are prevalent and often confused with other diagnoses. A PubMed search was undertaken to present a comprehensive article addressing the presentation and treatment for TOS. This article summarizes what is currently published about TOS, its etiologies, common objective findings, and nonsurgical treatment options. The PubMed database was conducted for the range of May 2020 to September 2021 utilizing TOS-related Medical Subject Headings (MeSH) terms. A Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) systematic literature review was conducted to identify the most common etiologies, the most objective findings, and the most effective nonsurgical treatment options for TOS. The search identified 1,188 articles. The automated merge feature removed duplicate articles. The remaining 1,078 citations were manually reviewed, with articles published prior to 2010 removed (n=771). Of the remaining 307 articles, duplicate citations not removed by automated means were removed manually (n=3). The other exclusion criteria included: non-English language (n=21); no abstracts available (n=56); and case reports of TOS occurring from complications of fractures, medical or surgical procedures, novel surgical approaches, or abnormal anatomy (n=42). Articles over 5 years old pertaining to therapeutic intervention (mostly surgical) were removed (n=18). Articles pertaining specifically to osteopathic manipulative treatment (OMT) were sparse and all were utilized (n=6). A total of 167 articles remained. The authors added a total of 20 articles that fell outside of the search criteria, as they considered them to be historic in nature with regards to TOS (n=8), were related specifically to OMT (n=4), or were considered sentinel articles relating to specific therapeutic interventions (n=8). A total of 187 articles were utilized in the final preparation of this manuscript. A final search was conducted prior to submission for publication to check for updated articles. Symptoms of hemicranial and/or upper-extremity pain and paresthesias should lead a physician to evaluate for musculoskeletal etiologies that may be contributing to the compression of the brachial plexus. The best initial provocative test to screen for TOS is the upper limb tension test (ULTT) because a negative test suggests against brachial plexus compression. A positive ULTT should be followed up with an elevated arm stress test (EAST) to further support the diagnosis. If TOS is suspected, additional diagnostic testing such as ultrasound, electromyography (EMG), or magnetic resonance imaging/magnetic resonance angiography (MRI/MRA) might be utilized to further distinguish the vascular or neurological etiologies of the symptoms. Initial treatment for neurogenic TOS (nTOS) is often conservative. Data are limited, therefore there is no conclusive evidence that any one treatment method or combination is more effective. Surgery in nTOS is considered for refractory cases only. Anticoagulation and surgical decompression remain the treatment of choice for vascular versions of TOS. The most common form of TOS is neurogenic. The most common symptoms are pain and paresthesias of the head, neck, and upper extremities. Diagnosis of nTOS is clinical, and the best screening test is the ULTT. There is no conclusive evidence that any one treatment method is more effective for nTOS, given limitations in the published data. Surgical decompression remains the treatment of choice for vascular forms of TOS.

Acute Reversible Cerebral Vasoconstriction Syndrome With Low-Dose Dihydroergotamine Possibly Potentiated by Valproic Acid and Erenumab: A Case Report

Dihydroergotamine (DHE), in the setting of polypharmacy, may increase the possibility of reversible cerebral vasoconstriction syndrome (RCVS). A 64-year-old woman with chronic migraine and medication-overuse headache (on amitriptyline, duloxetine, erenumab) was electively admitted for 5 days of intravenous (IV) ketamine (up to 55 mg/h) to treat intractable migraine pain. On the seventh day, upon receiving her forth dose of IV DHE (0.25 mg) and the first of IV valproic acid (VPA) (500 mg) adjunctively, she developed acute bilateral decreased visual acuity, bitemporal visual field deficit, and unsteady gait. Brain magnetic resonance imaging/magnetic resonance angiography (MRI/MRA) showed confluent bilateral T2 hyperintensities with punctate restricted diffusion in the occipital lobes associated with multi-segmental narrowing involving anterior, middle, posterior cerebral, and basilar arteries consistent with RCVS. Verapamil was initiated, whereas DHE, neuroleptics, and serotonergic agents were discontinued. Though she continued to have constant, non-thunderclap migrainous headache, her other neurologic symptoms resolved in 24 h. Concomitant use of VPA and erenumab with DHE may result in RCVS. VPA likely displaces the protein-bound DHE causing a transient surge of free DHE level in the serum. Erenumab may have impaired the protective vasodilatory mechanism, augmenting DHE’s vasoconstrictive effect. This case report highlights the importance and awareness of such a drug-drug interaction with DHE. J Neurol Res. 2020;10(1):20-24 doi: https://doi.org/10.14740/jnr558

Acute Reversible Cerebral Vasoconstriction Syndrome With Low-Dose Dihydroergotamine Possibly Potentiated by Valproic Acid and Erenumab: A Case Report

Dihydroergotamine (DHE), in the setting of polypharmacy, may increase the possibility of reversible cerebral vasoconstriction syndrome (RCVS). A 64-year-old woman with chronic migraine and medication-overuse headache (on amitriptyline, duloxetine, erenumab) was electively admitted for 5 days of intravenous (IV) ketamine (up to 55 mg/h) to treat intractable migraine pain. On the seventh day, upon receiving her forth dose of IV DHE (0.25 mg) and the first of IV valproic acid (VPA) (500 mg) adjunctively, she developed acute bilateral decreased visual acuity, bitemporal visual field deficit, and unsteady gait. Brain magnetic resonance imaging/magnetic resonance angiography (MRI/MRA) showed confluent bilateral T2 hyperintensities with punctate restricted diffusion in the occipital lobes associated with multi-segmental narrowing involving anterior, middle, posterior cerebral, and basilar arteries consistent with RCVS. Verapamil was initiated, whereas DHE, neuroleptics, and serotonergic agents were discontinued. Though she continued to have constant, non-thunderclap migrainous headache, her other neurologic symptoms resolved in 24 h. Concomitant use of VPA and erenumab with DHE may result in RCVS. VPA likely displaces the protein-bound DHE causing a transient surge of free DHE level in the serum. Erenumab may have impaired the protective vasodilatory mechanism, augmenting DHE’s vasoconstrictive effect. This case report highlights the importance and awareness of such a drug-drug interaction with DHE. J Neurol Res. 2020;10(1):20-24 doi: https://doi.org/10.14740/jnr558

Red blood cell transfusion to treat or prevent complications in sickle cell disease: an overview of Cochrane reviews.

This is the protocol for a review and there is no abstract. The objectives are as follows: To summarize the evidence in Cochrane reviews of the effectiveness and safety of red cell transfusions versus no transfusion, or restrictive (to increase the total haemoglobin) versus liberal (to decrease the haemoglobin S level below a specified percentage) transfusion, for treatment or prevention of complications experienced by people with SCD.

Brain MRI/MRA Findings after Hydroxyurea Treatment in Children with Sickle Cell Anemia

Background: Up to 40% of children with sickle cell anemia (SCA) will have abnormalities on brain imaging due to their hematologic disorder, much of which is subclinical. Common abnormalities on brain magnetic resonance imaging/magnetic resonance angiography (MRI/MRA) include leukoencephalopathy from microvascular ischemic insult and vascular stenosis from endothelial damage. Silent cerebral ischemic insults are progressive; further neurologic abnormalities, including overt stroke, are more common among children with ischemic findings on MRI compared to children without them. Furthermore, poor performance on neuropsychological testing, lower IQ, and higher rates of grade retention are common in children with SCA and cerebral ischemic disease. The effect of hydroxyurea treatment on the development and progression of vascular stenosis and leukoencephalopathy is unclear. This study aimed to longitudinally evaluate the development of intracerebral abnormalities through serial MRI/MRA in children with SCA (HbSS and HbSβ0-thalassemia) who receive long-term therapy with hydroxyurea.Methods: Children with SCA and no prior history of overt stroke enrolled in the Hydroxyurea Study of Long-Term Effects (HUSTLE NCT00305175) underwent brain MRI/MRA, transcranial doppler (TCD) examinations, and laboratory evaluations immediately before hydroxyurea initiation and after 3 and 6 years of treatment to maximum tolerated dose. MRI/MRAs were reviewed for the presence or absence of vascular stenosis and leukoencephalopathy. Leukoencephalopathy was defined as white matter T2 hyperintensity. Proportions of abnormal MRI/MRA findings were compared between baseline and 3 years and baseline and 6 years using McNemar’s test and the Wilcoxon-Mann-Whitney exact test was used to explore associations with laboratory parameters.Results: Forty-six children with SCA, mean age 9.4 years (range 1-17.3), had an MRI/MRA at baseline and 3 years post-initiation of hydroxyurea. Ten children had an additional MRI/MRA after 6-years of hydroxyurea therapy. Frequencies of leukoencephalopathy and vascular stenosis are shown in the table. Prevalence of leukoencephalopathy before hydroxyurea therapy was higher than that reported in the literature for untreated children with SCA of similar age. There were no significant differences between baseline imaging findings and those at 3 and 6 years.Table:Prevalence of leukoencephalopathy and vascular stenosis at baseline, 3 and 6 yearsBaseline (n=47) Median age 9.6 years (range 1.0 to 17.3)3 years (n=46) Median age 13.1 years (range 4.4 to 21.1)6 years (n=10) Median age 14.5 years (range 8.3 to 18.3)Leukoencephalopathy27 (57.4%)28 (60.9%)*5 (50%)*Stenosis3 (6.4%)1 (2.2%)*0*all p>0.05Children with leukoencephalopathy at baseline and 3 years were older than those without (mean 10.7 vs. 7.7 years, p=0.01; 10.5 vs. 7.5 years, p=0.02 respectively). Lower HbF at baseline was associated with the presence of leukoencephalopathy at year 3 (median HbF 4.6% vs. 12.4%, p=0.008), but there was no association between HbF at 3-years and the presence of leukoencephalopathy at 3-years (median HbF 16.4% vs. 13.2%, p=0.55). When stratified by age, these findings were similar. TCD velocities and other hematologic parameters were not associated with MRI/MRA abnormalities. The small number of vascular stenosis cases precluded further analyses of this outcome.Conclusions: In this longitudinal study, children treated with hydroxyurea for 3-6 years did not demonstrate an increased frequency of vascular stenosis or leukoencephalopathy on brain MRI/MRA during treatment. Older age at hydroxyurea initiation and lower pre-hydroxyurea HbF percentage were associated with the presence of leukoencephalopathy at baseline and 3-years. These findings suggest that hydroxyurea may mitigate the expected progression of vascular stenosis and leukoencephalopathy in children with SCA, and that this therapy should be initiated early on, before the development of cerebrovascular disease, particularly among those with a low HbF percentage. DisclosuresOff Label Use: hydroxyurea for children with sickle cell disease.

Magnetic resonance imaging/angiography and transcranial Doppler velocities in sickle cell anemia: results from the SWiTCH trial

AbstractThe Stroke With Transfusions Changing to Hydroxyurea (SWiTCH) trial compared standard (transfusions/chelation) to alternative (hydroxyurea/phlebotomy) treatment to prevent recurrent stroke and manage iron overload in children chronically transfused over 7 years before enrollment. Standardized brain magnetic resonance imaging/magnetic resonance angiography (MRA) and transcranial Doppler (TCD) exams were performed at entry and exit, with a central blinded review. A novel MRA vasculopathy grading scale demonstrated frequent severe baseline left/right vessel stenosis (53%/41% ≥Grade 4); 31% had no vessel stenosis on either side. Baseline parenchymal injury was prevalent (85%/79% subcortical, 53%/37% cortical, 50%/35% subcortical and cortical). Most children had low or uninterpretable baseline middle cerebral artery TCD velocities, which were associated with worse stenoses (incidence risk ratio [IRR] = 5.1, P ≤ .0001 and IRR = 4.1, P < .0001) than normal velocities; only 2% to 12% had any conditional/abnormal velocity. Patients with adjudicated stroke (7) and transient ischemic attacks (19 in 11 standard/8 alternative arm subjects) had substantial parenchymal injury/vessel stenosis. At exit, 1 child (alternative arm) had a new silent infarct, and another had worse stenosis. SWiTCH neuroimaging data document severe parenchymal and vascular abnormalities in children with SCA and stroke and support concerns about chronic transfusions lacking effectiveness for preventing progressive cerebrovascular injury. The novel SWiTCH vasculopathy grading scale warrants validation testing and consideration for use in future clinical trials. This trial was registered at www.clinicaltrials.gov as #NCT00122980.

Hip Arthroscopy: Prevalence of Intra-articular Pathologic Findings After Traumatic Injury of the Hip

The purpose of this study was to document and compare the incidence of intra-articular hip pathologic findings identified using arthroscopy versus conventional imaging in patients with acute trauma to the hip. This was a blinded prospective case series study designed to review the incidence of intra-articular pathologic disorders in patients with post-traumatic injury between the ages of 18 and 65 years who were referred to a single surgeon. Injuries included hip dislocation, proximal femur fracture, pelvic ring fracture, acetabular fracture, penetrating injury (gunshot wound), and soft tissue injury. Preoperative radiographs, computed tomographic (CT) scans, or magnetic resonance imaging/magnetic resonance angiography (MRI/MRA) scans (or a combination of these) were obtained. Findings were documented and compared with intraoperative findings. A total of 29 post-traumatic hips were enrolled in this study. Hip arthroscopy identified 17 of 29 hips (59%) as having loose bodies, 11 of 29 (38%) hips as having an intra-articular step deformity, 14 of 29 (49%) hips as having an osteochondral lesion, and 27 of 29 (93%) hips as having a labral tear. Plain radiographs and CT scans yielded low sensitivity when compared with arthroscopy for the identification of loose bodies and step deformities. MRI/MRA comparison with arthroscopic findings suggest that MRI/MRA is an accurate toolfor identification of labral tears, because 91% of tears seen on arthroscopy were also identified by MRI/MRA. In 4hips,however, MRI/MRA failed to identify osteochondral lesions that were subsequently identified by arthroscopy. Traumatic injuries of the hip result in substantial intra-articular pathologic findings, including loose bodies, labral tears, step deformities, and osteochondral lesions. The arthroscope is a powerful tool in identifying these injuries. Plain radiographs and CT scans appear to underestimate the true incidence of loose bodies and step deformities within the joint when compared with hip arthroscopy after a traumatic injury of the hip. Level IV, diagnostic case series.

Diagnostic imaging and pacemaker implantation in a domestic goat with persistent left cranial vena cava

Difficulty was encountered with the insertion of a right atrial pacing lead via the left jugular vein during lead and pacemaker implantation in a clinically normal goat as part of an ongoing rapid atrial pacing – induced atrial fibrillation research project. Fluoroscopic visualization of an abnormal lead advancement path prompted angiographic assessment which revealed a persistent left cranial vena cava (PLCVC) and prominent coronary sinus communicating with the right atrium. Angiography facilitated successful advancement and securing of the pacing lead into the right side of the interatrial septum. Cardiac magnetic resonance imaging/magnetic resonance angiography (MRI/MRA) allowed further characterization of this rare venous anomaly. Even though PLCVC has been reported once in a goat, to the authors' knowledge this is the first report to include MRI/MRA characterization of PLCVC and prominent coronary sinus with successful cardiac pacemaker implantation using the PLCVC.

Non-invasive diagnostic assessment of extensive vertebral artery tortuosity with enlargement of the intervertebral foramen

Two cases with an incidental finding of a widened intervertebral foramen due to extensive tortuosity of the vertebral artery are presented. Computed tomography angiography and magnetic resonance imaging/magnetic resonance angiography (MRA) both proved to be useful as non-invasive techniques to provide the diagnosis. In-plane saturation is a disadvantage seen with the two-dimensional time-of-flight MRA technique.

Case study: Pharmacologic management of movement disorder after midbrain haemorrhage

Movement disorders following midbrain haemorrhage are infrequently encountered in rehabilitation, and are uncommonly corrected by pharmacologic means. This report describes a 20 year-old male with a prior history of cocaine abuse who presented with a 4 day history of dysarthria and blurred vision following methamphetamine abuse. Physical examination demonstrated hypertension, left facial hemispasm, bilateral upward gaze paresis and ataxic gait. Magnetic resonance imaging/magnetic resonance angiography (MRI/MRA) showed multifocal parenchymal haematomas in the mesencephalic tegmentum, subcortical left front region and right anterior thalamus consistent with cavernous angiomas. The patient was transferred to rehabilitation on hospital day 5. The following day, he developed choreoathetoid movements, dystonia, and aphasia, secondary to an extension of the midbrain haemorrhage. Cogentin was initiated with slight improvement in choreoathetoid movements. The patient began intensive multidisciplinary rehabilitation therapy but after 18 days of therapy, the patient remained totally dependent in activities of daily living (ADLs), transfers, mobility and was unable to communicate in any manner. A trial of Sinemet was initiated, with resultant steady improvement in functional ability over the next month. By discharge, the patient was independent in ADLs and ambulation. By 9 months post discharge follow-up, the patient was fully independent with normal cognition, and had self tapered all medications without ill effect. Dopamine agonist trials of appropriate duration appear indicated in cases of movement disorder (paucity or excess) following midbrain lesions.