Four out of five infants who experience a stroke around the time of birth develop neurologic disorders, according to a study in the August Annals of Neurology. Researchers observed 36 infants with perinatal arterial ischemic stroke for a period of 12 months and found that 58% developed cerebral palsy, 39% developed epilepsy, 25% experienced language delay, and 22% developed behavioral abnormalities. According to the researchers, large stroke size and injury to Broca’s area, internal capsule, Wernicke’s area, or basal ganglia were predictors of cerebral palsy. A delayed presentation of symptoms was associated with an increased risk of cerebral palsy.

Sulfasalazine, a drug currently used to treat inflammatory bowel disease and rheumatoid arthritis, can reduce tumor size by inhibiting the production of glutathione. Researchers reported in the August 3 Journal of Neuroscience that when administered by intraperitoneal injection, sulfasalazine “was able to reduce glutathione levels in tumor tissue and slow tumor growth in vivo in a commonly used intracranial xenograft animal model for human gliomas.” According to the researchers, sulfasalazine works by inhibiting the cystine uptake into glioma cells through the inhibition of system xc-.

Tricyclic antidepressants and opioids are as effective as gabapentin, tramadol, and pregabalin for the treatment of postherpetic neuralgia, according to results of a systematic review published in the July Public Library of Science Medicine. Topical therapies such as lidocaine and methylprednisolone were also efficacious. Though a spinal intrathecal administration of lidocaine and methylprednisolone demonstrated efficacy, further research about this approach is needed. Therapies that were not effective for the treatment of postherpetic neuralgia included certain N-methyl-D-aspartate receptor antagonists, codeine, ibuprofen, lorazepam, certain 5HT1 receptor agonists, and acyclovir, as well as topical administration of benzydamine, diclofenac/diethyl ether, and vincristine. Also, intrathecal administration of lidocaine alone, epidural administration of lidocaine and methylprednisolone, intravenous therapy with lidocaine, subcutaneous injection of Cronassial, and acupuncture did not demonstrate efficacy.

A protein called KDI tripeptide (KDI) is able to block glutamate, thereby preventing apoptosis and even promoting regeneration of cells, according to a report in the July 25 online edition of the Journal of Neuroscience Research. In a previous study, researchers demonstrated that paralyzed rats injected with KDI were able to bear weight and walk again after only three months. “KDI treatment may become the first natural and targeted therapy for people with central nervous system injuries resulting in paralysis and a range of diseases such as Alzheimer’s and amyotrophic lateral sclerosis,” said the researchers. They said human clinical trials are expected to begin as soon as next year.

Combined treatment with neurotrophins and glial-restricted precursor cell grafts can lead to functional recovery from spinal cord injury in rats, according to a study in the July 27 Journal of Neuroscience. Researchers injected glial-restricted precursor cells with retroviruses—some expressing the multineurotrophin D15A—and then transplanted them into the adult rat spinal cord nine days after injury. Six weeks after transplantation, the rats were evaluated using the Basso-Beattie-Bresnahan scale and an electrical current test. Most of the rats that received the D15A-treated glial-restricted precursor cells improved significantly on both tests. The researchers plan to test similar techniques using undifferentiated embryonic stem cells instead of glial-restricted precursor cells.

Researchers have found that fragile X–associated tremor/ataxia syndrome is frequently misdiagnosed, according to a report in the July 26 Neurology. These findings led them to develop guidelines for diagnostic testing for the disorder. Results of their study indicated that 56 people had received 98 prior diagnoses, including parkinsonism and essential tremor, before receiving a diagnosis of fragile X–associated tremor/ataxia syndrome. They said this was partly due to the recently formed definition of fragile X–associated tremor/ataxia syndrome and unfamiliarity with the disorder. They emphasized that men 50 and older who develop unexplained ataxia or who have parkinsonism, tremor, or dementia as well as a family history of developmental delay or autism should undergo testing to check if they have the FMR1 gene related to the syndrome.

Aβ42 is essential for parenchymal and vascular amyloid deposition, according to results of a study in the July 21 Neuron. Researchers created transgenic mice that overproduced either Aβ40 or Aβ42 in the absence of overproduction of amyloid precursor protein. Mice with overexpression of Aβ40 showed little amyloid deposition, while mice with overexpression of Aβ42 showed extensive accumulation of amyloid plaque and resulting neural damage. Also, when Aβ42 mice were crossed with mice producing mutant amyloid precursor protein, a significant increase in amyloid deposition was observed.

Asymptomatic boys with X-linked adrenoleukodystrophy (ALD) who are treated with Lorenzo’s oil have a reduced risk of developing the more severely debilitating form of the disease, cerebral ALD, according to a report in the July Archives of Neurology. Eighty-nine boys with ALD—most of whom were younger than 7 at the beginning of the study—were treated with Lorenzo’s oil and moderate dietary fat restriction. Hexacosanoic acid levels were assessed every month for the first six months and every three to six months thereafter. MRI and neurological examinations were scheduled every six to 12 months. Patients were followed for an average of 6.9 years. Results indicated that 24% of the patients developed MRI abnormalities and 11% developed both neurological and MRI abnormalities. MRI abnormalities were associated with increased hexacosanoic acid levels.

Children with congenital central hypoventilation syndrome (CCHS) show stroke-like damage in regions of the brain that regulate the cardiovascular system, body temperature, and urination, according to a study in the July 11 Journal of Comparative Neurology. Researchers performed MRI on 12 children with CCHS and on 28 controls. CCHS patients showed damaged or maldeveloped tissue in portions of the posterior, mid, and anterior cingulate, as well as the internal capsule, putamen, globus pallidus, and basal forebrain. Deficits were also observed in the cingulum bundle, mid-hippocampus, and ventral prefrontal cortex. The researchers said the findings would allow them to focus on new strategies to treat the injury, such as injecting injured areas with nerve growth factors to stimulate regrowth of lost nerve fibers and recovery of some functions.

Researchers have identified a specific role of the protein p53 in the mitochondria-associated cellular dysfunction and behavioral abnormalities of Huntington’s disease. According to a report in the July 7 Neuron, p53 is increased in the brains of Huntington’s disease patients, with the highest levels in those with the most extensive pathology. They noted that genetic deletion of p53 suppresses damage to neurons in the eyes of fruit flies engineered to have the mutant huntingtin protein and corrects neurobehavioral abnormalities in transgenic mice. The authors suggested that disturbances in p53 might also play a role in Parkinson’s disease and amyotrophic lateral sclerosis.

Functional MRI (fMRI) of the brain reduces the need for invasive testing of patients with seizure disorder who are being considered for surgery, according to a study in the July Radiology. Researchers evaluated the effect of fMRI results on the diagnostic work-up and treatment of 60 patients with seizure disorder and found that five patients were able to avoid a two-stage surgery and received one-stage resection surgery instead. In addition, the extent of surgical resection was altered in another four patients because critical areas near the seizure focus were identified. Overall surgical plans and intraoperative mapping were altered in 42% and 52% of patients, respectively. The team was also able to alter patient and family counseling in 58% of the patients. They noted that as a result of the fMRI findings, 63% of patients were able to avoid further studies.

Patients with untreated obstructive sleep apnea syndrome (OSAS) have normal serum and plasma brain-derived neurotrophic factor (BDNF) levels, while 21 patients treated with continuous positive airway pressure (CPAP) show a rapid decrease in serum and plasma BDNF levels. According to researchers, after one night of CPAP treatment, median serum BDNF concentrations declined from 18.0 ng/mL to 4.1 ng/mL and plasma BDNF concentrations declined from 58.7 pg/mL to 22 pg/ mL. After three months of treatment, BDNF concentrations did not return to baseline values. The researchers suggested that the decrease in serum and plasma BDNF levels might reflect enhanced neuronal demand for BDNF in this condition. Results of the study were published in the August Thorax.

Dysfunction of the neuronal sodium channel SCN1A is associated with familial hemiplegic migraine, according to a study in the July 30 Lancet. Researchers identified a gene locus for familial hemiplegic migraine on chromosome 2q24, revealing a heterozygous missense mutation on the gene SCN1A; this mutation has also been identified as being associated with epilepsy. According to the researchers, the SCN1A mutation was present in three families with familial hemiplegic migraine. These findings provide further evidence of the molecular link between migraine and epilepsy, the researchers said.

The discovery of “amyloid ion channels” may lead to the development of new treatments for Alzheimer’s disease and other degenerative diseases, according to a study in the July 26 Proceedings of the National Academy of Sciences. Researchers examined the structures of several different proteins—including Aβ40, alpha-synuclein, ABri, ADan, serum amyloid A, and amylin—and found that all of the proteins folded into structures resembling ion channels. The investigators commented, “By controlling activity and designing specific drugs to regulate these channels, we might be able to prevent and/ or treat various diseases related to the amyloids.”

NR