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Neurology FREE

Robert J. Joynt, MD, PhD
JAMA. 1994;271(21):1686-1687. doi:10.1001/jama.1994.03510450058032
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Advances in molecular genetics marked the major advances last year in neurology; however, major developments also occurred in other areas, particularly in new types of therapy. Late last year, two excellent reviews summarizing advances in the genetics of neurologic diseases were published.1,2 The unstable trinucleotide repeat continues to turn up as the genetic defect in several inherited neurologic disorders, including Huntington's disease, spinobulbar muscular atrophy, myotonic dystrophy, and spinocerebellar ataxia type 1.1,2 The number of repeats is related to severity or latency in the different disorders.

Late-onset familial Alzheimer's disease (FAD) has been associated with a defect on chromosome 19.3 The defect is linked with apolipoprotein E, which transports cholesterol into the brain. Apolipoprotein E binds with amyloid and is found with amyloid and with the neurofibrillary tangles in the brain of those with Alzheimer's disease. The E4 allele of apolipoprotein E is found in 45% of

REFERENCES

Rosenberg RN.  An introduction to the molecular genetics of neurological disease: recent advances. Arch Neurol . 1993;;50:1123-1128.
Martin JB.  Molecular genetics in neurology. Ann Neurol . 1993;;34:757-773.
Strittmatter WJ, Saunders AM, Schmechel D, et al.  Apolipoprotein E: high-avidity binding to βamyloid and increased frequency of type 4 allele in late-onset familial Alzheimer disease. Proc Nati Acad Sci U S A . 1993;;90:1977-1981.
Román GC, Tatemichi TK, Erkinjuntti T, et al.  Vascular dementia: diagnostic criteria for research studies: report of the NINDS-AIREN International Workshop. Neurology . 1993;;43:250-260.
Langhorne P, Williams BO, Gilchrist W, Howie K.  Do stroke units save lives? Lancet . 1993;;342:395-398.
Kalia KK, Yonas H.  An aggressive approach to massive middle cerebral artery infarction. Arch Neurol . 1993;;50:1293-1297.
Sisti MB, Kader A, Stein BM.  Microsurgery for 67 intracranial arteriovenous malformations less than 3 cm in diameter. J Neurosurg . 1993;;79:653-660.
Steiner L, Lindquist C, Karlsson B, Steiner M.  Microsurgery and radiosurgery in brain arteriovenous malformations. J Neurosurg . 1993;;79:647-652.
Egre HJ, Crowley JJ, Townsend JJ, et al.  A randomized trial of radiotherapy versus radiotherapy plus CCNU for incompletely resected low-grade gliomas: a Southwest Oncology Group study. J Neurosurg . 1993;;78:909-914.
Siva A, Radhakrishnan K, Kurland LT, O'Brien PC, Swanson JW, Rodriguez M.  Trauma and multiple sclerosis: a population-based cohort study from Olmsted County, Minnesota. Neurology . 1993;;43:1878-1882.
Sibley WA.  Physical trauma and multiple sclerosis. Neurology . 1993;;43:1871-1874.
Schaad UB, Lips U, Gnehm HE, et al.  Dexamethasone therapy for bacterial meningitis in children. Lancet . 1993;;342:457-461.
Galgiani JN, Catanzaro A, Cloud GA, et al.  Fluconazole therapy for coccidioidal meningitis. Ann Intern Med . 1993;;119:28-35.
Aubourg P, Adamsbaum C, Lavallard-Rousseau M-C, et al.  A two-year trial of oleic and erucic acids ('Lorenzo's oil') as treatment for adrenomyeloneuropathy. N Engl J Med . 1993;;329:745-752.
Myasthenia Gravis Clinical Study Group.  A randomized clinical trial comparing prednisone and azathioprine in myasthenia gravis: results of the second interim analysis. J Neurol Neurosurg Psychiatry . 1993;;56:1157-1163.
Posner M.  Seeing the mind. Science . 1993;;262:673-674.

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Country-Specific Mortality and Growth Failure in Infancy and Yound Children and Association With Material Stature

Use interactive graphics and maps to view and sort country-specific infant and early dhildhood mortality and growth failure data and their association with maternal

Rosenberg RN.  An introduction to the molecular genetics of neurological disease: recent advances. Arch Neurol . 1993;;50:1123-1128.
Martin JB.  Molecular genetics in neurology. Ann Neurol . 1993;;34:757-773.
Strittmatter WJ, Saunders AM, Schmechel D, et al.  Apolipoprotein E: high-avidity binding to βamyloid and increased frequency of type 4 allele in late-onset familial Alzheimer disease. Proc Nati Acad Sci U S A . 1993;;90:1977-1981.
Román GC, Tatemichi TK, Erkinjuntti T, et al.  Vascular dementia: diagnostic criteria for research studies: report of the NINDS-AIREN International Workshop. Neurology . 1993;;43:250-260.
Langhorne P, Williams BO, Gilchrist W, Howie K.  Do stroke units save lives? Lancet . 1993;;342:395-398.
Kalia KK, Yonas H.  An aggressive approach to massive middle cerebral artery infarction. Arch Neurol . 1993;;50:1293-1297.
Sisti MB, Kader A, Stein BM.  Microsurgery for 67 intracranial arteriovenous malformations less than 3 cm in diameter. J Neurosurg . 1993;;79:653-660.
Steiner L, Lindquist C, Karlsson B, Steiner M.  Microsurgery and radiosurgery in brain arteriovenous malformations. J Neurosurg . 1993;;79:647-652.
Egre HJ, Crowley JJ, Townsend JJ, et al.  A randomized trial of radiotherapy versus radiotherapy plus CCNU for incompletely resected low-grade gliomas: a Southwest Oncology Group study. J Neurosurg . 1993;;78:909-914.
Siva A, Radhakrishnan K, Kurland LT, O'Brien PC, Swanson JW, Rodriguez M.  Trauma and multiple sclerosis: a population-based cohort study from Olmsted County, Minnesota. Neurology . 1993;;43:1878-1882.
Sibley WA.  Physical trauma and multiple sclerosis. Neurology . 1993;;43:1871-1874.
Schaad UB, Lips U, Gnehm HE, et al.  Dexamethasone therapy for bacterial meningitis in children. Lancet . 1993;;342:457-461.
Galgiani JN, Catanzaro A, Cloud GA, et al.  Fluconazole therapy for coccidioidal meningitis. Ann Intern Med . 1993;;119:28-35.
Aubourg P, Adamsbaum C, Lavallard-Rousseau M-C, et al.  A two-year trial of oleic and erucic acids ('Lorenzo's oil') as treatment for adrenomyeloneuropathy. N Engl J Med . 1993;;329:745-752.
Myasthenia Gravis Clinical Study Group.  A randomized clinical trial comparing prednisone and azathioprine in myasthenia gravis: results of the second interim analysis. J Neurol Neurosurg Psychiatry . 1993;;56:1157-1163.
Posner M.  Seeing the mind. Science . 1993;;262:673-674.
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