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Volume 6, No 2 - Spring 2009

Volume 6, No 2 - Spring 2009

Table of Contents

The Neurofibromatoses. Part 1: NF1 Diagnosis and Treatment Review
The neurofibromatoses, including neurofibromatosis 1 (NF1), neurofibromatosis 2 (NF2), and schwannomatosis, comprise a group of genetically distinct disorders of the nervoussystem unified by the predisposition to nerve sheath tumors. NF1 is the most common neurogenetic disorder, with a birth incidence of 1 in 3000. NF1 is inherited in autosomal dominant fashion with full penetrance and variable expressivity. The hallmark lesion of NF1 is the neurofibroma, a benign tumor derived from the nerve sheath and composed of a mixture of proliferating Schwann cells, fibroblasts, mast cells, and pericytes. Other findings include gliomas, learning disability, vasculopathy, and bony abnormalities. Café au lait macules are typically the initial clinical manifestation of NF1 and tend to increase in size and number throughout childhood and puberty. Current treatment of patients with NF1 remains primarily surgical. Genetic counseling is essential for adult patients because molecular diagnostic testing can minimize the risk of transmission to children. [Rev Neurol Dis. 2009;6(2):E47-E53]
Intramedullary Spinal Cord Hemorrhage (Hematomyelia) Diagnosis and Treatment Review
Intramedullary spinal cord hemorrhage (hematomyelia) is an uncommon cause ofmyelopathy and can present in an acute, subacute, stepwise, or chronic fashion.Spinal vascular malformations such as intramedullary cavernomas and intraduralarteriovenous malformations are the most common cause of atraumatic intramedullaryspinal cord hemorrhage based on the existing literature. Additional considerationsinclude warfarin or heparin anticoagulation, hereditary or acquired bleeding disorders,primary spinal cord tumors, spinal cord metastases, Gowers’ intrasyringal hemorrhage,or a delayed complication of spinal radiation. Prompt diagnosis of hematomyeliafirst requires recognition of a myelopathy syndrome (transverse, central, anterior,posterior, or hemi-cord) often accompanied by sudden, severe back or neck pain andsometimes radicular pain. MRI with and without gadolinium is the preferred imagingmodality. There are no clinical trials to guide the management of acute intramedullaryspinal cord hemorrhage, and subsequent treatment is usually directed toward theunderlying cause.[Rev Neurol Dis. 2009;6(2):E54-E61]
Contraception for Women With Epilepsy Treatment Review
The choice of a contraceptive drug can be challenging for women with epilepsy due topossible interactions between antiepileptic drugs (AEDs) and hormonal contraception.Enzyme-inducing AEDs can cause hormonal contraception to fail and can increase therisk of teratogenicity. Higher doses of oral contraceptives can overcome pharmacologicfailure but may create additional risks. The effects of reproductive hormones on individualAEDs have recently been clarified, providing helpful guidelines for physiciansand patients. Studies show that lamotrigine has a significantly increased clearance(> 50%) when used with combined oral contraceptives, which results in an increasedseizure frequency in most patients. Useful alternatives to oral contraceptives includedepot injections and intrauterine devices. Subdermal implants may increase the riskof pregnancy in women with epilepsy on enzyme-inducing AEDs. Depot medroxyprogesteroneacetate is effective but can increase side effects. Intrauterine devices are analternative to pharmacologic approaches because they lack drug-drug interactionsand side effects.[Rev Neurol Dis. 2009;6(2):E62-E67]
Neuroimaging Advances Meeting Review
Neuroimaging AdvancesHighlights From the 32nd Annual Meeting of the American Society of Neuroimaging,January 22-25, 2009, Orlando, FL[Rev Neurol Dis. 2009;6(2):E68-E71]© 2009 MedReviews®, LLCKey
International Stroke Conference 2009 Meeting Review
Highlights From the International Stroke Conference 2009, February 18-20, 2009, San Diego, CA[Rev Neurol Dis. 2009;6(2):E72-E74]© 2009 MedReviews®, LLC
Multiple Brain Infarcts After Orbital Inflammation Case Review: Presentation
A 75-year-old woman developed trigeminal varicella-zoster virus infection complicatedby ophthalmoplegia, and visual loss followed by recurrent cerebral infarctions involvingsmall and large intracranial arteries. Medical therapy improved her ophthalmoplegia,but she developed a right hemiparesis and aphasia.[Rev Neurol Dis. 2009;6(2):E75-E76]
Migraine News and Views From the Literature