Pigment epithelium-derived factor (PEDF) is a serine protease inhibitor (serpin) protein with well established neuroprotective and anti-angiogenic properties. Recent studies have also shown that PEDF enhances renewal of adult subventricular zone (SVZ) neural precursors. In neurosphere cultures prepared from the SVZ of adult mice, we found that addition of recombinant PEDF to the medium enhanced...

In 1949, Victor and Adams observed an alcoholic patient who developed quadriplegia and pseudobulbar palsy, and inability to chew, talk or swallow. A post-mortem confirmed their suspicion of 'a large, symmetrical, essentially demyelinative lesion occupying the greater part of the basis pontis.' This paper follows the historical evolution of central pontine myelinolysis and the changing concepts ...

Introduction Organic osmolytes have been implicated in the pathogenesis of myelinolysis because some of them are accumulated slowly during correction of chronic hyponatremia. I investigated whether there was a topographic correlation between demyelinative lesions and the regional changes of organic osmolytes after rapid correction of chronic hyponatremia. In normal female Sprague-Dawley rats, c...

The spinal nerve roots and dorsal ganglia of 104- to 135-week-old rats with spontaneous radiculoneuropathy were examined by light and electron microscopy. Demyelination was common in myelinated fibers of various diameters of both ventral and dorsal roots. The most striking alteration was wide distention of myelin sheaths, which extended throughout the entire internode. The spaces formed between...

A 25-year-old man suffered from recurrent, mass-like, intracerebral lesions and was diagnosed with tumefactive multiple sclerosis (MS). Both interferon β-1b and oral methotrexate (7.5 mg weekly) could not suppress frequent relapses, but he had been in remission for 8 months by the mitoxantrone treatment. At the age of 27, the patient developed a partial seizure. Brain MRI revealed a lesion in t...

Peripheral neuropathy is a rare manifestation of sarcoidosis, and previous studies have shown axonal degeneration as the main pathology. We herein report three patients with sarcoidosis who presented with multiple mononeuropathy as the initial manifestation. Nerve conduction studies showed prominent multifocal conduction blocks in the intermediate nerve trunk. In all three patients, corticoster...

Introduction Central pontine myelinolysis (CPM) in alcoholics and malnourished patients was first described by Adams, Victor and Mancall in 1959. This commonly fatal demyelinative process that involves the pons almost exclusively, is of unknown aetiology. In the majority of the published cases, CPM was associated with chronic alcoholism, malnutrition and electrolyte abnormalities, particularly ...

The effects of a standard 3 minutes' hyperventilation on the full-field pattern-reversal visual evoked potential (VEP) were studied in 33 normal subjects, 30 definite multiple sclerosis patients and in twenty-five patients with abnormal VEPs due to either tumourous compression of the anterior visual pathways or optic atrophy of other origin. Significantly greater reductions in P100 latency occu...

Since a number of anti-glycosphingolipid (GSL) antibody activities have been demonstrated in patients with various neurological disorders, the presence of common antigens between brain microvascular endothelial cells (BMECs) and the nervous tissues presents a potential mechanism for the penetration of macromolecules from the circulation to the nervous system parenchyma. We first investigated GS...

The diseases of white matter compose essentially three nosological entities of a heterogeneous group. The clinical and neuropathological attention has been directed to the deficiency of myelin in more or less specific patterns and distribution as the primary characteristic, and the unique natural history and the respective cellular pathology as the modifiers which define each disease. Basically...