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History
McCrory et al. (p. 1888) provide a historic account of cerebellar seizure concepts as originally described by Jackson in 1871 and decerebrate rigidity developed under the influence of Sherrington at the turn of the century.
Sleep
Nogués et al. (p. 1777) studied 30 patients with syringomyelia using pulmonary function studies and polysomnography, and determined that severe abnormalities in respiratory rhythm generation during sleep occur in patients with syringobulbia, particularly when the combination of dysphagia and dysphonia is present. These disturbances are not due to muscle weakness or correlated with the size of the cavity. Malow’s accompanying editorial (p. 1732) accents the occurrence of disordered sleep in neurologic disease.
Parchi et al. (p. 1757) established a sporadic variant of prion disease with the fatal familial insomnia phenotype. They propose that the condition be called sporadic fatal insomnia.
Frucht et al. (p. 1908) describe patients treated with the newer dopamine agonists, pramipexole and ropinirole, who experienced motor vehicle accidents after falling asleep while driving. Five of the eight individuals had no warning before falling asleep; hence, the authors characterize the events as "sleep attacks" resembling narcolepsy. The authors propose that these dopamine agonists may have triggered sleep attacks by downregulating dopaminergic input to the reticular activating system.
Multiple sclerosis
Gayo et al. (p. 1764) measured baseline levels of mRNA in freshly isolated cells from patients before interferon beta (IFNß) therapy and at 3, 6, and 12 months to determine whether the treatment had an initial and sustained effect on the in vivo synthesis and secretion of tumor necrosis factor-
and IFN
. The authors conclude that IFNß treatment decreases the spontaneous expression of these proinflammatory cytokines. In their accompanying editorial, Moses and Sriram (p. 1729) review the immunomodulatory role of IFNß. They note that despite increasing and important knowledge about the effects of IFNß, translating this information into the clinic for patient management has not yet been accomplished.
Movement disorders
Elbaz et al. (p. 1876) used case-control methodology to investigate the occurrence of PD in families. They found that familial factors (genetic, environmental, or both) clearly play a role in pathogenesis of PD.
Neuroimaging and stroke
Three articles in this issue, accompanied by an editorial, explore new MRI techniques for evaluating acute ischemic stroke. Ay et al. (p. 1784) studied 782 consecutive patients scanned when stroke-like neurologic deficits were present and diffusion-weighted imaging (DWI) was normal. They conclude that, although a normal DWI in such patients should stimulate a search for nonischemic causes, more than half of the patients have an ischemic cause as the best clinical diagnosis. In their study of 12 patients, Marks et al. (p. 1792) characterized the effects of rt-tPA therapy and early reperfusion on DWI and perfusion-weighted imaging changes observed following acute ischemic injury. They noted early reperfusion more frequently when rt-tPA was used. Fisher and Albers (p. 1750) provide a thorough review of these MRI techniques. In their editorial, Prichard and Grossman (p. 1733) note that recent literature supports the use of MRI as the principal imaging modality for initial evaluation of acute stroke syndrome. They suggest that perfusion-weighted imaging can be combined with DWI to guide acute stroke treatment.
Neuromuscular
Orrell et al. (p. 1822) review a large experience in DNA testing of patients with facioscapulohumeral dystrophy. Definitive molecular diagnosis is now possible in virtually 100% of cases.
Epilepsy
Sachdeo et al. (p. 1882) performed a double-blind, randomized trial of topiramate in Lennox–Gastaut syndrome. Topiramate adjunctive therapy was effective in reducing the number of drop attacks and major motor seizures and in improving seizure severity.
Cerebral aneurysms
Johnston et al. (p. 1799) performed a cohort study of patients treated for unruptured cerebral aneurysms at 60 university hospitals from 1994 through 1996 to compare surgical treatment with endovascular coil embolization. The embolization technique resulted in fewer adverse outcomes. The companion paper by Johnston et al. (p. 1806) is a cost–utility analysis comparing surgical clipping and endovascular coil embolization with no treatment for unruptured cerebral aneurysms. The treatment of small (<10 mm), asymptomatic, unruptured aneurysms in patients without a history of subarachnoid hemorrhage was neither effective nor cost-effective. Treatment appears cost-effective for aneurysms that are 
10 mm or symptomatic, or when there is a history of subarachnoid hemorrhage.
Neurologists on the Internet
Rivner (p. 1891) reviews experience with a free Web site designed for neurologists to use for discussion of clinical matters. Beresford and Brooke (p. 1730) discuss some of the practical issues, particularly legal ones, surrounding neurologists’ use of the Internet.
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