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What's new in neurology
All topics are updated as new evidence becomes available and our peer review process is complete.
Literature review current through: Apr 2012. | This topic last updated: Apr 2, 2012.

The following represent additions to UpToDate since the last version of What’s New that were considered by the authors and editors to be of particular interest.

CEREBROVASCULAR DISEASE

Glucose control in acute stroke — Hyperglycemia is common in patients with acute stroke and is associated with poor functional outcomes. This has led to studies evaluating whether tight control of glucose with intravenous insulin in the acute phase of ischemic stroke might be beneficial. However, the available evidence suggests that it is not. A 2011 systematic review identified seven controlled trials involving nearly 1300 adults with acute ischemic stroke who were randomly assigned to either intensively monitored insulin infusion therapy or to usual care [1]. There was no difference between the treatment and control groups for the combined outcome of death or disability and dependence, and no difference between groups for the outcome of final neurologic deficit. In addition, the intervention group had a higher rate of symptomatic hypoglycemia. (See "Initial assessment and management of acute stroke", section on 'Hyperglycemia'.)

DEMYELINATING DISEASE

Fingolimod for multiple sclerosis — Eleven deaths in patients with multiple sclerosis (MS) have been linked to the use of fingolimod internationally as of late February 2012, including four patients who had cardiac events and seven with unexplained death [2]. Although these deaths may be coincidental and unrelated to fingolimod, we suggest not starting fingolimod for patients with MS until the cause of death in these cases has been clarified. (See "Treatment of relapsing-remitting multiple sclerosis in adults", section on 'Clinical use of fingolimod'.)

Treating cognitive deficits in patients with multiple sclerosis — Nonpharmacologic approaches to the treatment of cognitive deficits in patients with multiple sclerosis (MS) primarily consist of support and improvement of coping strategies, particularly because the benefits of medication and rehabilitation are limited. Cognitive rehabilitation techniques are another approach, but data related to clinical application of these methods are limited. A recent systematic review and meta-analysis identified 14 studies of cognitive training or cognitive-behavioral interventions in a total of 770 patients with MS [3]. Cognitive training combined with other neuropsychologic rehabilitation methods did not lead to statistically significant improvement for most outcomes, but did have statistically significant benefit for three subcategories of cognitive performance – memory span, working memory, and immediate visual memory. Despite mostly negative results from the meta-analyses of the pooled data, some evidence of benefit was noted in 12 of the 14 included studies when analyzed individually. Further research into the application of cognitive rehabilitation techniques is clearly needed. (See "Comorbid problems associated with multiple sclerosis in adults", section on 'Nonpharmacologic approaches'.)

EPILEPSY

Midazolam for prehospital treatment of status epilepticus — Intramuscular midazolam was shown to be at least as effective as intravenous lorazepam in terminating status epilepticus in a clinical trial of 893 adults and children treated in the prehospital setting [4]. On arrival to hospital, seizure remission was more likely in patients treated with IM midazolam compared with IV lorazepam (73 versus 63 percent). Intravenous lorazepam treatment was associated with a shorter median time from active treatment to cessation of convulsions while IM midazolam was associated with a shorter median time to administration of active treatment. These results suggest that midazolam may be most appropriate for preshospital, rather than in-hospital, treatment of status epilepticus. (See "Status epilepticus in adults", section on 'Midazolam' and "Management of status epilepticus in children", section on 'Midazolam'.)

Clobazam now available to treat Lennox-Gastaut syndrome — Clobazam became available in the United States in late 2011. In two randomized trials of children with Lennox-Gastaut syndrome, adjunctive therapy with clobazam (0.5 to 1 mg/kg per day) was associated with a significant reduction in seizure frequency compared to patients taking placebo or low dose clobazam (0.25 mg/kg per day) [5,6] (See "Epilepsy syndromes in children", section on 'Lennox-Gastaut syndrome'.)

Prevention of SUDEP in patients with refractory epilepsy — A meta-analysis of randomized controlled trials in patients with refractory epilepsy found that adjunctive antiepileptic drug therapy was associated with a reduced risk of sudden unexpected death in epilepsy (SUDEP) (OR=0.17), suggesting that ongoing efforts to reduce seizure frequency in patients with uncontrolled seizures is appropriate [7]. (See "Sudden unexpected death in epilepsy", section on 'Prevention and counseling'.)

HEADACHE

Diagnosis of low cerebrospinal fluid pressure headache — The diagnosis of spontaneous low cerebrospinal fluid (CSF) pressure headache, also known as spontaneous intracranial hypotension, requires evidence of low CSF pressure or evidence of a CSF leak. The latter is generally detected on neuroimaging studies, mainly CT myelography or radioisotope cisternography, that require subarachnoid contrast injection. A novel and noninvasive approach, subtraction MRI, employs rapid postprocessing image analysis of standard T2 and T1 MRI sequences in order to delineate the dural sac of the spinal cord and distinguish between fluid and fat. In a report of 17 patients with spontaneous intracranial hypotension, subtraction MRI of the spinal cord identified the epidural CSF fluid collection in all patients [8]. Additional studies are needed to determine the sensitivity and specificity of this method. (See "Pathophysiology, clinical features, and diagnosis of spontaneous low cerebrospinal fluid pressure headache", section on 'MRI of brain and spine'.)

NEUROMUSCULAR DISEASE

Meralgia paresthetica — Meralgia paresthetica is a clinical syndrome of pain and dysesthesia in the anterolateral thigh associated with entrapment or compression of the lateral femoral cutaneous nerve. Although the condition is not uncommon, few studies have evaluated the etiology and epidemiology of meralgia paresthetica. However, a recent population-based report found that the most frequent conditions associated with meralgia are obesity, diabetes mellitus, and older age [9]. Compared with the general population, subjects with diabetes have a seven-fold increase in the occurrence of meralgia paresthetica. (See "Meralgia paresthetica (lateral femoral cutaneous nerve entrapment)", section on 'Etiology and epidemiology'.)

PEDIATRIC NEUROLOGY

Congenital myopathies — A novel condition, known as early onset myopathy, areflexia, respiratory distress, and dysphagia (EMARDD), is a rare autosomal recessive congenital myopathy characterized by diaphragmatic paralysis that presents at birth or in early infancy [10,11]. A recent report identified mutations in the MEGF10 gene as the cause [10]. (See "Congenital myopathies", section on 'Early onset myopathy, areflexia, respiratory distress, and dysphagia'.)

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REFERENCES

  1. Bellolio MF, Gilmore RM, Stead LG. Insulin for glycaemic control in acute ischaemic stroke. Cochrane Database Syst Rev 2011; :CD005346.
  2. The Globe and Mail. Health Canada launches investigation of oral MS drug. www.theglobeandmail.com/life/health/new-health/health-news/health-canada-launches-investigation-of-oral-ms-drug/article2351665/ (Accessed on February 28, 2012).
  3. Rosti-Otajärvi EM, Hämäläinen PI. Neuropsychological rehabilitation for multiple sclerosis. Cochrane Database Syst Rev 2011; :CD009131.
  4. Silbergleit R, Durkalski V, Lowenstein D, et al. Intramuscular versus intravenous therapy for prehospital status epilepticus. N Engl J Med 2012; 366:591.
  5. Conry JA, Ng YT, Paolicchi JM, et al. Clobazam in the treatment of Lennox-Gastaut syndrome. Epilepsia 2009; 50:1158.
  6. Ng YT, Conry JA, Drummond R, et al. Randomized, phase III study results of clobazam in Lennox-Gastaut syndrome. Neurology 2011; 77:1473.
  7. Ryvlin P, Cucherat M, Rheims S. Risk of sudden unexpected death in epilepsy in patients given adjunctive antiepileptic treatment for refractory seizures: a meta-analysis of placebo-controlled randomised trials. Lancet Neurol 2011; 10:961.
  8. Bonetto N, Manara R, Citton V, Cagnin A. Spinal subtraction MRI for diagnosis of epidural leakage in SIH. Neurology 2011; 77:1873.
  9. Parisi TJ, Mandrekar J, Dyck PJ, Klein CJ. Meralgia paresthetica: relation to obesity, advanced age, and diabetes mellitus. Neurology 2011; 77:1538.
  10. Logan CV, Lucke B, Pottinger C, et al. Mutations in MEGF10, a regulator of satellite cell myogenesis, cause early onset myopathy, areflexia, respiratory distress and dysphagia (EMARDD). Nat Genet 2011; 43:1189.
  11. Hartley L, Kinali M, Knight R, et al. A congenital myopathy with diaphragmatic weakness not linked to the SMARD1 locus. Neuromuscul Disord 2007; 17:174.
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