Post t-PA transfer to hub improves outcome of moderate to severe ischemic stroke patients.

Background And Purpose: Telemedicine offers rural hospitals the ability to treat acute ischemic stroke on site with intravenous tissue plasminogen activator (t-PA). Most patients are subsequently transferred to a hub hospital with a primary stroke center for post t-PA care. There is little evidence that such transfer is beneficial.

β-Propeller protein-associated neurodegeneration: a new X-linked dominant disorder with brain iron accumulation.

Neurodegenerative disorders with high iron in the basal ganglia encompass an expanding collection of single gene disorders collectively known as neurodegeneration with brain iron accumulation. These disorders can largely be distinguished from one another by their associated clinical and neuroimaging features. The aim of this study was to define the phenotype that is associated with mutations in WDR45, a new causative gene for neurodegeneration with brain iron accumulation located on the X chromosome.

Hemiparkinsonism after unilateral traumatic midbrain hemorrhage in a young woman.

The relationship between head trauma and parkinsonism has been debated since James Parkinson's first description of the shaking palsy in the late 19th century. We observed in our outpatient clinic a young woman in whom hemiparkinsonism developed within 3 weeks of sustaining closed head trauma with loss of consciousness. The patient had a discrete unilateral midbrain hemorrhage on head MRI which involved the contralateral substantia nigra.

Exome sequencing reveals de novo WDR45 mutations causing a phenotypically distinct, X-linked dominant form of NBIA.

Neurodegeneration with brain iron accumulation (NBIA) is a group of genetic disorders characterized by abnormal iron deposition in the basal ganglia. We report that de novo mutations in WDR45, a gene located at Xp11.23 and encoding a beta-propeller scaffold protein with a putative role in autophagy, cause a distinctive NBIA phenotype.

Peripheral nervous system pathology in fragile X tremor/ataxia syndrome (FXTAS).

Fragile X tremor/ataxia syndrome (FXTAS) occurs in individuals with moderate CGG expansion of the fragile X mental retardation 1 (FMR1) gene and is associated with intranuclear inclusions in neurons and astrocytes. Although the neuropathologic findings in the brain and spinal cord were described, pathological features in the peripheral nervous system were not reported. Here, we report on novel neuropathological findings in the peripheral nervous system and especially in autonomic ganglia at autopsy in a man with FXTAS.

Episodic nausea and abdominal sensations as sole manifestations of simple partial seizures.

In adults, seizures manifesting with abdominal complaints are usually associated with complex partial or secondary generalized seizures. Also, seizure periodicity is not expected in postmenopausal women. We encountered a 72-year-old woman with episodic nausea and abdominal pain that usually occurred with predictable regularity.

Fragile X premutation in a woman with cognitive impairment, tremor, and history of premature ovarian failure.

Few women with Fragile X tremor ataxia syndrome (FXTAS) have been reported. They have milder manifestations at a later age than men. This gender difference may be related to the X inactivation pattern in women.

Neurologic complications of gastric bypass surgery for morbid obesity.

Background: The number of bariatric procedures is rapidly growing as the prevalence of obesity in the USA is increasing. Such procedures are not without complications, and those affecting the nervous system are often disabling and irreversible. We now describe our experience with these complications and review the pertinent literature.

Tiagabine-induced non-convulsive status epilepticus in a patient without history of epilepsy.

Cases of non-convulsive status epilepticus (NCSE) induced by tiagabine (TGB) were occasionally reported. Almost all had a prior history of epilepsy. We describe here, the clinical and EEG findings in a patient, without history of seizures, who after the start of TGB developed NCSE.

Multiple myeloma invasion of the central nervous system.

Background: Although neurologic manifestations often complicate the course of patients with multiple myeloma (MM), direct central nervous system invasion is rare.

Objective: To describe the neurologic symptoms and signs, imaging, cerebrospinal fluid findings, and the clinical course of patients with central nervous system myeloma invasion, all of whom had leptomeningeal myelomatosis.

Design And Participants: Review of 23 patients with MM and leptomeningeal myelomatosis proven by malignant plasma cells in their cerebrospinal fluid.

Acute bilateral inferior cerebellar infarction in a patient with neurosyphilis.

Background: Bilateral simultaneous infarction in the territories of the posterior inferior cerebellar arteries (PICAs) is rare but was recently reported with increasing frequency, probably because of the wider availability of magnetic resonance imaging. The cause of these infarcts is believed to be atherosclerotic or embolic occlusion of a dominant PICA, which perfused the territories of the medial branches of both PICAs.

Results: We encountered a patient with simultaneous infarction in the territories of the medial branches of both PICAs.

Myeloma of the central nervous system: strong association with unfavorable chromosomal abnormalities and other high-risk disease features.

Involvement of the central nervous system (CNS) by multiple myeloma, defined by the detection of malignant plasma cells in the cerebrospinal fluid in the presence of suggestive symptoms, is considered extremely rare. We present the characteristics of 25 such patients (18 previously reported) intended to receive high-dose treatment at the University of Arkansas over the last 12 years; an extensive review of the published literature since 1968, including 71 patients, is also presented. In both patient groups, high tumor burden was overwhelmingly present while circulating plasma cells were detected in a significant proportion of cases.

Albuterol improves response to levodopa and increases skeletal muscle mass in patients with fluctuating Parkinson disease.

Animal studies indicate that beta(2)-adrenergic receptor agonists enhance transport of levodopa across the blood-brain barrier. Preliminary studies showed improved response to levodopa in patients with Parkinson disease (PD) who were given albuterol as adjunctive therapy. Beta(2)-adrenergic agonists may offer additional benefits to PD patients via their skeletal muscle anabolic effects, particularly those who experience decreased muscle strength and weight loss.

VEGF mRNA expressed in microvessels of neonatal and adult rat cerebral cortex.

We measured mRNA levels of vascular endothelial growth factor (VEGF) and its Flk-1/KDR receptor in isolated cerebral cortical microvessels and in the cerebral cortex of neonatal (1 week) and adult (11 week) rats using reverse transcription-polymerase chain reaction (RT-PCR). Cerebral microvessels were isolated by density centrifugation, mesh filtration and passage through glass bead columns. The dominant cell types in this preparation are endothelial cells and pericytes.

Myeloma of the central nervous system: association with high-risk chromosomal abnormalities, plasmablastic morphology and extramedullary manifestations.

Involvement of the central nervous system (CNS) by multiple myeloma, as defined by the detection of malignant plasma cells in the cerebrospinal fluid in the presence of suggestive symptoms, is considered extremely rare. We report on the characteristics of 18 such patients diagnosed and treated at the University of Arkansas over the last 10 years for an overall incidence of approximately 1%. Their evaluation revealed association of CNS involvement with unfavourable cytogenetic abnormalities (especially translocations and deletion of the chromosome 13), high tumour mass, plasmablastic morphology, additional extramedullary myeloma manifestations and circulating plasma cells.

β-Adrenergics enhance brain extraction of levodopa.

We sought to determine whether beta-adrenergic agonists enhance the brain extraction of L-dopa and L-leucine. Systemic administration of beta-adrenergic agonists increase brain concentrations of L-dopa and other large neutral amino acids (LNAA) in rats and monkeys and may improve symptoms and reduce daily L-dopa requirement in patients with Parkinson's disease. Cerebral blood flow (CBF) using [3H]nicotine and the extraction fraction of 14C-labeled L-dopa or L-leucine were measured simultaneously in various brain regions of conscious rats using the dual-isotope indicator fractionation technique after intraperitoneal administration of isoproterenol (a peripheral nonselective beta-adrenergic agonist), or clenbuterol (a beta2-adrenergic agonist that crosses the blood-brain barrier), or beta-adrenergic agonist preceded by nadolol (a peripheral nonselective beta-adrenergic antagonist), or saline vehicle.

Endothelial activation following prolonged hypobaric hypoxia.

Prolonged exposure to low oxygen may induce adaptive changes which can be either beneficial or deleterious to cell survival. We examined the effect of prolonged moderate hypobaric hypoxia on CNS endothelial cell (EC) function. Exposure to hypoxia resulted in expression of EC activation markers, the cell surface adhesion proteins intracellular adhesion molecule-1 and E-selectin.

Nervous system dysfunction in Waldenström's macroglobulinemia: response to treatment.

A patient presented with a peripheral neuropathy and was found to have Waldenstrom's macroglobulinemia with high serum titers of antibodies to myelin-associated glycoprotein. He developed parkinsonism that was poorly responsive to levodopa. He failed conventional therapy and was treated with autologous bone marrow transplantation, which resulted in improvement of the neuropathy but not his parkinsonism.