This Month in Archives of Neurology

Sugg and colleagues in the Argatroban tPA Stroke Study (TARTS) provide the first human data for using both argatroban and intravenous recombinant tissue plasminogen activator (rtPA) therapies in patients with acute stroke. Of 15 patients enrolled in this ongoing, prospective, open-label, dose escalation, safety, and activity study of argatroban and rtPA in patients with ischemic stroke, they report that recanalization was complete in 6 patients and partial in another 4 patients and reocclusion occurred in 3 patients within 2 hours of rtPA bolus administration. Using argatroban combined with intravenous rtPA for producing fast and complete recanalization is a promising strategy.

Lehmann et al provide a timely and elegant review of the biological mechanisms and clinical efficacy of using plasma exchange to treat patients who have immunological disease of the peripheral nervous system.

Hoffman-Snyder and colleagues determined in this 24-month retrospective study of 100 consecutive patients that the prevalence of undiagnosed abnormal fasting glucose metabolism was found to be nearly 2-fold higher in patients with chronic idiopathic axonal polyneuropathy than in similar age-matched groups from the general population. Their study adds to the emerging evidence that abnormal glucose metabolism may be a risk factor for chronic idiopathic axonal polyneuropathy. John T. Kissel, MD, provides editorial perspective.

Sanossian et al evaluated the effects of in-hospital initiation of statins on 3-month treatment adherence and achievement of national guideline cholesterol goals. They report that statin initiation during hospitalization for an ischemic cerebrovascular event is associated with high adherence to treatment, decreased levels of low-density lipoprotein cholesterol, and higher rates of achievement of national cholesterol guidelines.

Morris and colleagues examined whether high dietary intake of copper is associated with increased cognitive decline among persons who also consume a diet high in saturated and trans fats. In a detailed and comprehensive study, they report that high dietary intake of copper in association with a diet high in saturated and trans fats may be associated with accelerated cognitive decline.

Houeto et al analyzed the presence of behavioral disorders and social maladjustments in patients with Parkinson disease who were successfully treated by continuous bilateral subthalamic stimulation. Of note, they report that provided patients with Parkinson disease are rigorously selected for neurosurgery, subthalamic stimulation improves mood, anxiety, and quality of life. It neither results in severe permanent psychiatric disorders nor modifies patients' personalities, and it does not inhibit social adaptation.

Jain et al conclude that 1 in 3 tremor patients are misdiagnosed as having essential tremor. The most frequent false diagnoses are Parkinson disease and dystonia.

Burneo and colleagues in a large and comprehensive study conclude that although sex plays a role in temporal lobe epilepsy surgery outcome, race and socioeconomic status do not.

The natural history and pathophysiologic nature of ischemic spinal cord infarction are still incomplete. After reviewing the data for 27 patients with acute spinal cord infarction, Novy and colleagues report that there are 2 main types of spinal cord ischemia, radicular artery territory infarction and extensive spinal cord hypoperfusion. They conclude that each type has characteristic clinical, imaging, pathophysiological, and prognostic features.

Oskoui and colleagues describe the clinical and molecular genetic features of patients with mitochondrial DNA depletion syndrome and mutations in the TK2 gene. They report that the clinical spectrum of TK2 mutations is not limited to severe infantile myopathy with motor regression and early death but includes spinal muscular atrophy type 3–like presentation, rigid spine syndrome, and subacute myopathy without motor regression and with longer survival.

The clinical spectrum of DGUOK-related mitochondrial DNA (mtDNA) depletion syndrome is described by Freisinger et al. They studied 12 cases with infantile hepatoencephalopathies and mtDNA depletion syndrome and found pathogenic DGUOK mutations in 6 cases, indicating that this gene defect is a frequent but not exclusive cause of the hepatic form of mtDNA depletion syndrome.

Cellini et al evaluated the presence of fragile X premutation in male patients with sporadic ataxia. The CGG repeat size of the FMR1 gene was evaluated by fluorescent polymerase chain reaction. The FMR1 premutation alleles with a repeat number above 55 were detected in 3 probands out of 142 male subjects referred for sporadic ataxia. Genetic analysis of the FMR1 gene provides a reliable diagnostic tool for the definitive diagnosis of late-onset ataxias.

Contemporary patients with amyotrophic lateral sclerosis (ALS), as reported by Czaplinski and colleagues, had significantly prolonged survival and slower disease progression compared with patients from a historical group. They conclude that the improved outcome appeared independent of specific ALS outcome-modifying therapies. It is proposed from their data that the course of ALS has changed over time and is becoming less aggressive in recent years.

The cerebral N-acetylaspartyl (NAA) and myo-inositol (Ins) ratio as an index of cerebral involvement with amyotrophic lateral sclerosis (ALS) using magnetic resonance spectroscopy is reported by Kalra et al. They report that in subjects with ALS, the greatest abnormality was a 22% decrease in the NAA/Ins ratio. They conclude that the NAA/Ins ratio provides a meaningful biomarker in ALS.

Heatwole et al provide a spectrum of the frequency of abnormal laboratory values in patients with myotonic muscular dystrophy that forms a basis for early screening and monitoring and provide insight into the spectrum of tissues involved in this disease.

Seppi and colleagues studied patients with progressive supranuclear palsy (PSP) by applying iodine I 123–labeled 2β-carbomethoxy-3β-(4-iodophenyl)tropane ([¹²³I]β-CIT) single-photon emission computed tomography (SPECT) to localize the cerebral dopamine transporter in the early stages of PSP. They found that SPECT images of patients with PSP show a widespread decline of monoaminergic transporter availability, including the striatum and brain stem, which discriminates patients with PSP from patients with Parkinson disease but not from patients with multiple system atrophy. Thus, quantification of midbrain dopamine transporter signal may enhance the utility of SPECT imaging in the differential diagnosis of patients with parkinsonism.

Yao et al found that compared with controls, autistic children had significantly higher urinary levels of isoprostane F_2α-VI; 2,3-dinor-thromboxane B₂; and 6-keto-prostaglandin F_1α. Lipid peroxidation levels directly correlated with both vascular biomarker ratios. These findings support the view that platelet and vascular endothelium activation could also contribute to the development and clinical manifestations of autism.

Ramos and colleagues evaluated the association between promoter region polymorphisms in the tumor necrosis factor α and interleukin 10 genes and risk of late-onset Alzheimer disease. They report that tumor necrosis factor α promoter region variation, or possibly polymorphisms in nearby genes, could affect cerebral inflammatory response and be influencing risk of late-onset Alzheimer disease.

Chung et al conducted a systematic analysis of clinical features in association with gene mutations in Korean patients with early-onset Parkinson disease. They find that earlier onset of levodopa-induced dyskinesia and off dystonia were characteristic features of early-onset Parkinson disease.

Sepulcre and colleagues report that atrophy is most obvious in deep grey matter, including bilateral thalamic atrophy, in clinically early primary progressive multiple sclerosis. This may reflect increased sensitivity of these regions to neurodegeneration.