Joel K. Curé

Stroke Prevention Trial in Sickle Cell Anemia

Stroke occurs in 7-8% of children with Sickle Cell Disease (Hb SS) and is a major cause of morbidity. Rates of recurrence have been reduced from 46-90% to less than 10% through chronic blood transfusions. Prevention of first stroke, however, would be preferable because even one stroke can cause irreversible brain injury. Transcranial Doppler (TCD) ultrasound can detect arterial blood flow rates associated with subsequent stroke risk. By combining TCD screening and a potentially effective treatment, first stroke may be prevented. The Stroke Prevention Trial in Sickle Cell Anemia (STOP) is the first stroke prevention trial in Hb SS and the first randomized, controlled use of transfusion in Hb SS. This multi-center trial is designed to test whether reducing sickle hemoglobin to 30% or less with periodic blood transfusions will reduce first-time stroke by at least 70% compared to standard care. Primary endpoints will be clinically evident symptoms of cerebral infarction with consistent findings on Magnetic Resonance Imaging and Angiography (MRI/MRA) or symptomatic intracranial hemorrhage. Secondary endpoints will be asymptomatic brain lesions detected by MRI in brain areas not involved in primary endpoints. The design calls for a 6-month start-up interval, 18 months of TCD screening and randomization, and observation for stroke from entry through month 54. Key features of the trial are standardized TCD and MRI/MRA protocols interpreted blindly, and blinded adjudication of endpoints. The sample size (60 per treatment group) is based on prospective data relating TCD velocity to risk of stroke. A time-averaged mean velocity of > or = 200 cm/sec is associated with a 46% risk of cerebral infarction over 39 months. The sample size is sufficient to detect 70% reduction in the primary endpoint at 90% power. This trial will determine if transfusion is effective in the primary prevention of stroke. Secondary aims may further the understanding of the effects of transfusion on the brain and guide future research into cerebrovascular disease in Hb SS.

Normal and variant anatomy of the dural venous sinuses

The normal and variant anatomy of the cerebral veins and dural venous sinuses is poorly understood by many radiologists. Beginning with a discussion of cerebral venous anatomy, this review illustrates clinically pertinent anatomy of the cerebral sinovenous system. Various methods of imaging cerebral veins and dural venous sinuses are described. Techniques and pitfalls of MR venography are emphasized.

DEVELOPMENTAL NEURAL ABNORMALITIES AND SEIZURES IN EPIDERMAL NEVUS SYNDROME

The epidermal nevus syndrome (ENS) is an unusual neurocutaneous disorder consisting of the combination of an epidermal nevus and a central nervous system (CNS), ophlhalmological, and/or skeletal abnormality. The study reports four new patients with ENS. Each had a confirmatory biopsy of the epidermal nevus, abnormal neurological examination findings, and documented CNS anatomical studies by imaging or autopsy. The paper also reviews the literature in English to determine neurological abnormalities found in skin‐biopsy‐proven cases of ENS. Hemi‐atrophy, hemimegalencephaly, migrational abnormalities and vascular anomalies were found to be the most frequent intracranial abnormalities associated with ENS. Seizures and/or disabling moderate to severe developmental delays were present in a majority of patients. Seizure onset during the neonatal period or early infancy was associated with major hemispheric malformations. Neuroectodermal‐derived ocular lesions were often bilateral. No consistent relation between laterality of the nevus and laterality of CNS abnormalities was found, supporting the gene mosaicism theory of pathogenesis.

Nonneoplastic intracranial cysts and cystic lesions

This article reviews the gamut of cystic lesions, unrelated to neoplastic disease, that are found in the brain, subarachnoid space, and ventricles. The utility of MRI in diagnosing these entities is shown.

Neonatal Hemangiomatosis Associated With Brachial Plexus Palsy

pathic. Our patient was treated with vecuronium for a brief period of time, and this diagnosis is highly unlikely. Thick-filament myopathy, characterized by acute diffuse areflexic weakness with increased creatine kinase levels, occurs mostly in asthmatics with respiratory failure requiring high-dose steroids.18-21 Recovery takes 10 days to 6 months. When compared with patients with prolonged neuromuscular blockade, patients with thick-filament myopathy tend to be younger and free of renal disease. Electrophysiologic and pathologic findings

Neurologic outcomes in children with post-pump choreoathetosis☆☆☆★

Eleven children who had post-pump choreoathetosis develop after cardiopulmonary bypass were evaluated in their perioperative course to determine factors that may correlate with their neurologic outcome. Results showed that preoperative cyanosis is associated with the development of a basal ganglia lesion. An acquired basal ganglia lesion and preoperative cyanosis are associated with persistence of post-pump choreoathetosis. The combination of cyanotic heart disease and a scan-identified basal ganglia lesion indicates a poor prognosis for the patient with persistent post-pump choreoathetosis. Also, the presence of total circulatory arrest is associated with a decrease in developmental quotient but not the persistence of post-pump choreoathetosis.

Congenital and acquired abnormalities of the dural venous sinuses.

The imaging features of a variety of dural venous sinus (DVS) abnormalities are reviewed. Congenital and heritable diseases affecting the DVS, tumor-related sinus compression, and traumatic injuries of the DVS are discussed. The causes, clinical manifestations, and imaging findings in cerebral sinovenous thrombosis are described, and pertinent imaging techniques and pitfalls are illustrated. Etiologic theories about the formation of dural arteriovenous malformations are discussed and their imaging features are demonstrated.

Neurotologic complications associated with deployment of airbags

ber of fatalities resulting from automobile accidents. Airbags, however, are associated with inherent risks. Various injuries resulting from the deployment of airbags have been reported.1 These include corneal abrasions, retinal detachment, facial fractures, lacerations and abrasions, extremity trauma, chest trauma, and even death. Otologic symptoms involving transient and permanent hearing loss, tinnitus, and dysequilibrium have also been described.2 This article describes an unusual, severe traumatic neurotologic complication of airbag deployment.