Palop and MuckeArticle review recent experimental data demonstrating that high levels of β-amyloid peptide (Aβ) in the brain can cause epileptiform activity and cognitive deficits in transgenic mouse models. They conclude that Aβ may contribute to cognitive decline in Alzheimer disease by eliciting similar aberrant neuronal activity in humans, and they discuss the potential clinical and therapeutic implications of this hypothesis.
DichterArticle asks 2 fundamental questions in his quest to understand the physiological mechanisms underlying seizure generation: how do seizures develop in the brain, and how does a normal brain become an epileptic brain after injury? These questions form the discussion of this most elegant and insightful review.
Ma and colleaguesArticle report the highly significant finding that sorLA/LR11, a transmembrane neuronal protein that reduces amyloid precursor protein trafficking to secretases, was significantly reduced in the cerebrospinal fluid of patients with Alzheimer disease. This finding may have great potential as a diagnostic biomarker for patients with LR11 deficits that promote β-amyloid peptide production or as an index of therapeutic responses in late-onset Alzheimer disease. Editorial perspective is provided by Richard Mayeux, MD, MSc, and Peter St. George-Hyslop, MD.Article
Niehusmann et alArticle describe a new form of encephalitis associated with ovarian teratoma and with seizures and psychiatric symptoms. They have identified 19 female patients aged 15 to 45 years with unexplained new-onset epilepsy. Five of the 19 patients had antibodies against N-methyl-D-aspartate (NMDA) receptors. Only 1 patient had a neoplasm; thus the disorder is not always paraneoplastic.
Clinical courses of the 5 patients positive for N-methyl-D-aspartate receptor (NMDAR) antibodies. CSF indicates cerebrospinal fluid; IVIG, intravenous immunoglobulin.
Isaias and colleaguesArticle find that pallidal deep brain stimulation is a safe and effective treatment for primary generalized dystonia, with improvement sustained for up to 8 years in one patient.
Oh et alArticle show that reduction of regulatory T cells (Treg) did not negatively affect maintenance of central nervous system tolerance during anti-CD25 antibody treatment. Sustained reduction in the frequency of CD4+CD25+ Treg was observed during treatment.
Bielekova and colleaguesArticle report that daclizumab monotherapy is an effective treatment in most patients with persistent disease activity during interferon beta therapy. Either a combination of daclizumab with interferon beta or higher doses of daclizumab may be necessary to achieve optimal therapeutic responses in all patients.
Lozeron et alArticle show that the influence of anti–TNF-α continuation on the long-term course of neuropathy is highly variable, suggesting that anti–TNF-α withdrawal is not always necessary for neuropathic control.
Delmaire and colleaguesArticle id demonstrate the presence of diffusion abnormalities in fiber tracts connecting primary sensorimotor areas with subcortical structures in writer's cramp. These abnormalities support the role of the cortico-subcortical pathways in the pathophysiology of writer's cramp.
Brugman et alArticle provide evidence that in most patients with a sporadic adult-onset upper motor neuron syndrome, differentiation of sporadic presentations of hereditary spastic paraparesis from primary lateral sclerosis based on clinical characteristics is unreliable and therefore depends on genetic testing.
Fang and colleaguesArticle report that the incidence of amyotropic lateral sclerosis has been increasing during the last 1½ decades in Sweden. The specific basis for this increase remains unknown.
This Month in Archives of Neurology. Arch Neurol. 2009;66(4):431–432. doi:10.1001/archneurol.2009.51