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What's new in neurology

Last literature review version 17.3: September 2009  |  This topic last updated: October 14, 2009   (More)

The following represent additions to UpToDate since the last version that were considered by the authors and editors to be of particular interest. The new material described below represents a small subset of the updating that has been performed, since approximately 40 percent of the topic reviews are updated during each four-month cycle.

CEREBROVASCULAR DISEASE

Cerebral venous thrombosis — A report from the prospective International Study on Cerebral Vein and Dural Sinus Thrombosis (ISCVT) cohort shows that women with cerebral venous thrombosis have a better long-term prognosis than men. This difference is driven by the more favorable outcome for the subgroup of women with gender specific risk factors (mainly oral contraceptives, pregnancy or puerperium) for cerebral venous thrombosis [1]. (See "Treatment and prognosis of cerebral venous thrombosis", section on 'Long-term outcome'.)

Intracerebral hemorrhage — A large retrospective study adds to the findings of previous studies that have associated the presence of small foci or larger areas of contrast extravasation within the hematoma on CT angiography source images ("CTA spot sign") with risk of hematoma expansion [2]. (See "Spontaneous intracerebral hemorrhage: Prognosis and treatment", section on 'Hematoma growth'.)

DEMENTIA

Vascular risk factors — An observational study suggests that aggressive treatment of vascular risk factors is associated with a slower rate of cognitive decline in patients with AD [3]. (See "Treatment of dementia", section on 'Risk factor control'.)

While prior observational studies have associated less tight diabetes control with increased risk of cognitive decline and/or dementia, a recent study associated a history of severe hypoglycemic episodes with dementia risk among individuals with type II diabetes [4]. This suggests that some caution is appropriate in pursuing tight glycemic control in older adults. (See "Risk factors for dementia", section on 'Diabetes mellitus'.)

DEMYELINATING DISEASE

Active relapsing-remitting multiple sclerosis — The role of glucocorticoids combined with beta interferons for the treatment of relapsing remitting multiple sclerosis (RRMS) remains uncertain. In the NORMIMS trial of 130 adults with RRMS and recent relapse on subcutaneous interferon beta-1a, adjunctive treatment with oral methylprednisolone led to a significant reduction in mean yearly relapse rate compared with placebo (0.22 versus 0.59) [5]. However, small patient numbers and a high drop out rate preclude definitive conclusions. (See "Treatment of relapsing-remitting multiple sclerosis in adults", section on 'Glucocorticoids in combination therapy'.)

Multiple sclerosis and natalizumab — Since the reintroduction of natalizumab to the market in June 2006, more than 10 new cases of progressive multifocal leukoencephalopathy (PML) have been confirmed through June 2009 in patients with multiple sclerosis treated with natalizumab monotherapy [6-9]. The incidence of PML among patients on natalizumab for 18 months or longer approximates 1 in 1000. (See "Natalizumab for relapsing-remitting multiple sclerosis in adults", section on 'Risk of progressive multifocal leukoencephalopathy'.)

Vanishing white matter disease — The adult form of vanishing white matter (VWM) disease is generally less severe than childhood and juvenile forms, but data are limited. In the largest adult series of 16 patients with causative EIF2B mutations followed for a mean of 11 years, the initial manifestations in most were gait disturbances with spastic paraparesis and/or cerebellar ataxia [10]. However, one patient remained asymptomatic. Brain MRI findings included cerebral atrophy in all 16 patients, cystic leukoencephalopathy in 13, and cerebellar atrophy in 12. Stress-induced deterioration at onset or during the course of the disease was common. Among 14 survivors, disease evolution was progressive in all but three. (See "Vanishing white matter disease", section on 'Clinical features'.)

EPILEPSY

Vigabatrin approved — Vigabatrin was approved by the FDA in 2009 for the treatment of infantile spasms as well as for adjunctive treatment of adults with refractory partial seizures [11,12]. A boxed warning alerts clinicians to safety concerns regarding permanent vision loss with this medication. Physicians must be registered with the SHARE program in order to prescribed vigabatrin. (See "Pharmacology of antiepileptic drugs", section on 'Vigabatrin' and "Management and prognosis of infantile spasms", section on 'Vigabatrin'.)

HEADACHE

Migraine and MRI lesions — In the longitudinal AGES-Reykjavik Study, subjects (mainly women) who had migraine with aura at the initial evaluation (n = 361) had an increased risk of infarct-like lesions on brain MRI 25 years later compared with those not reporting headache once or more per month at baseline (adjusted odds ratio 1.4) [13]. Earlier studies also suggested an association between migraine with aura and silent infarct-like lesions on MRI. However, the etiology, nature, and clinical significance of the MRI lesions reported in these studies is unclear [14]. (See "Headache, migraine, and stroke", section on 'Subclinical brain lesions'.)

NEUROMUSCULAR DISEASE

Carpal tunnel syndrome — A randomized controlled trial with 116 patients demonstrated that surgical decompression for carpal tunnel syndrome is more effective than nonsurgical treatment consisting of multimodal interventions [15]. However, the benefit of surgery was small and of modest clinical significance. (See "Treatment of carpal tunnel syndrome", section on 'Surgery'.)

PEDIATRIC NEUROLOGY

Neonatal encephalopathy — Hypothermia is the only effective neuroprotective therapy currently available for treatment of neonatal hypoxic-ischemic encephalopathy, and is safe and easy to administer. However, therapeutic hypothermia has only limited efficacy. The TOBY trial of 325 near-term infants with hypoxic-ischemic encephalopathy found that whole body cooling started within six hours of birth and continued for 72 hours did not significantly reduce the composite outcome of death or severe disability at 18 months (RR 0.86, 95% CI 0.68-1.07) [16]. However, a statistically significant benefit for whole body cooling was observed for rate of survival without a neurologic abnormality (44 versus 28 percent) and risk of cerebral palsy among survivors (28 versus 41 percent). (See "Clinical features, diagnosis, and treatment of neonatal encephalopathy", section on 'Therapeutic hypothermia'.)


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REFERENCES

  1. Coutinho, JM, Ferro, JM, Canhao, P, et al. Cerebral venous and sinus thrombosis in women. Stroke 2009; 40:2356.
  2. Delgado Almandoz, JE, Yoo, AJ, Stone, MJ, et al. Systematic characterization of the computed tomography angiography spot sign in primary intracerebral hemorrhage identifies patients at highest risk for hematoma expansion: the spot sign score. Stroke 2009; 40:2994.
  3. Deschaintre, Y, Richard, F, Leys, D, Pasquier, F. Treatment of vascular risk factors is associated with slower decline in Alzheimer disease. Neurology 2009; 73:674.
  4. Whitmer, RA, Karter, AJ, Yaffe, K, et al. Hypoglycemic episodes and risk of dementia in older patients with type 2 diabetes mellitus. JAMA 2009; 301:1565.
  5. Sorensen, PS, Mellgren, SI, Svenningsson, A, et al. NORdic trial of oral Methylprednisolone as add-on therapy to Interferon beta-1a for treatment of relapsing-remitting Multiple Sclerosis (NORMIMS study): a randomised, placebo-controlled trial. Lancet Neurol 2009; 8:519.
  6. Tysabri update. Available at http://investor.biogenidec.com/phoenix.zhtml?c=148682&p=irol-TPME. (Accessed on September 22, 2009).
  7. Wenning, W, Haghikia, A, Laubenberger, J, et al. Treatment of progressive multifocal leukoencephalopathy associated with natalizumab. N Engl J Med 2009; 361:1075.
  8. Linda, H, von Heijne, A, Major, EO, et al. Progressive multifocal leukoencephalopathy after natalizumab monotherapy. N Engl J Med 2009; 361:1081.
  9. Major, EO. Reemergence of PML in natalizumab-treated patients--new cases, same concerns. N Engl J Med 2009; 361:1041.
  10. Labauge, P, Horzinski, L, Ayrignac, X, et al. Natural history of adult-onset eIF2B-related disorders: a multi-centric survey of 16 cases. Brain 2009; 132:2161.
  11. http://www.fda.gov/NewsEvents/Newsroom/PressAnnouncements/ucm179855.htm. (Accessed on August 30, 2009).
  12. Wohlrab, G, Leiba, H, Kastle, R, et al. Vigabatrin therapy in infantile spasms: solving one problem and inducing another?. Epilepsia 2009; 50:2006.
  13. Scher, AI, Gudmundsson, LS, Sigurdsson, S, et al. Migraine headache in middle age and late-life brain infarcts. JAMA 2009; 301:2563.
  14. Kurth, T, Tzourio, C. Migraine and cerebral infarct-like lesions on MRI: an observation, not a disease. JAMA 2009; 301:2594.
  15. Jarvik, JG, Comstock, BA, Kliot, M, et al. Surgery versus non-surgical therapy for carpal tunnel syndrome: a randomised parallel-group trial. Lancet 2009; 374:1074.
  16. Azzopardi, DV, Strohm, B, Edwards, AD, et al. Moderate hypothermia to treat perinatal asphyxial encephalopathy. N Engl J Med 2009; 361:1349.
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UpToDate performs a continuous review of over 430 journals and other resources. Updates are added as important new information is published. The literature review for version 17.3 is current through September 2009; this topic was last changed on October 14, 2009. The next version of UpToDate (18.1) will be released in March 2010.

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