This Month in Archives of Neurology

Adams defines the clinical, imaging, and molecular pathologic findings for sickle cell–related strokes in children. He reviews the evidence for which he was a major contributor that transcranial Doppler is an effective method to show the developing features of cerebral vasculopathy requiring intervention. Through research that he and his colleagues began in 1986, they demonstrated that children with sickle cell disease who are developing high stroke risk can be detected months to years before the stroke using transcranial Doppler velocity. This major success story in neurology is graphically reviewed here.

Volkow and colleagues review the evidence that imaging studies have provided new insights on dopamine's role in drug abuse and addiction in the human brain. These studies have shown that the reinforcing effects of drugs of abuse in humans are contingent not just on dopamine increases per se in striatum (including nucleus accumbens) but on the rate of dopamine increases: the faster the increases, the more intense the reinforcing effects.

Hoppe and colleagues provide new data on the issue of patient seizure counts as a standard for individual treatment and clinical trials in epilepsy. In a comprehensive study using consecutive sampling of adult inpatients with focal epilepsies undergoing video electroencephalography monitoring, they found that patient seizure counts do not provide valid information. Editorial perspective is provided by Giridhar P. Kalamangalam, MD, DPhil; Jeremy D. Slater, MD; and James A. Ferrendelli, MD. ext-link xlink:href="ned70003"/

Grossman et al studied patients with frontotemporal dementia (FTD) to establish clinical, neuropsychological, and imaging features that discriminate between pathologically determined tau-positive FTD, tau-negative FTD, or frontal-variant Alzheimer disease. A discriminant function analysis grouped patients on the basis of clinical and neuropsychological features with 87.5% accuracy.

Giuliano et al studied tadalafil, a phosphodiesterase 5 (PDE5) inhibitor, to determine its efficacy and safety for use by men with erectile dysfunction (ED) secondary to traumatic spinal cord injury (SCI). The patients treated with tadalafil compared with placebo had significantly improved erectile function (Figure ), and tadalafil was well tolerated by men with ED secondary to traumatic SCI.

Hu and colleagues identified clinical features in behavioral variant frontotemporal dementia (bvFTD) that help to predict tau-positive pathology. They found that poor planning/judgment was associated with patients with bvFTD who had tau-positive pathology, and the constellation of impaired personal conduct and a paucity of dysexecutive symptoms identified tau-negative patients.

Chao et al report that patients with frontotemporal lobar degeneration who are in relatively early stages of the disease (ie, Clinical Dementia Rating scores of 1.0-1.2) have white matter atrophy that largely parallels the pattern of gray matter atrophy typically associated with these disorders.

Sproule and colleagues investigated the frequency of Wolff-Parkinson-White (WPW) syndrome among a cohort of patients with mitochondrial encephalopathy, lactic acidosis, and strokelike episodes (MELAS) and the A3246G mutation most commonly associated with MELAS. They report that the prevalence of WPW among subjects with MELAS and the A3246G mutation appears much higher than the normal population and may become manifest earlier than neurological symptoms. Patients with MELAS should be monitored for cardiac anomalies, including cardiomyopathy and WPW.

Jansen et al report on the clinical and molecular data of 3 families with enzyme-based diagnoses of mucopolysaccharidosis (MPS) type IIID or Sanfilippo syndrome type D. They found that major issues in the care of patients with MPS-IIID include behavioral problems, recurrent infections, and pain from orthopedic complications. To date, all mutations in the causal gene coding for N-acetylglucosamine-6-sulfatase (type D) predict premature termination of translation, and there is no obvious genotype-phenotype correlation.

Walter and colleagues used transcranial brain sonography (TCS) to exclude the diagnosis of idiopathic Parkinson disease in patients with sporadic parkinsonism. They show that distinct TCS features can exclude the diagnosis of Parkinson disease in patients with sporadic parkinsonism.

Louis et al found that mild parkinsonian signs are associated with a higher plasma homocysteine concentration in community-dwelling patients.