Secondary amyloidosis as a life-ending event in multifocal motor neuropathy.

Multifocal motor neuropathy (MMN) is a disorder of peripheral nerve often associated with a high monosialoganglioside (GM1) antibody and multifocal conduction block. It has a chronic, indolent course with involvement of predominantly peripheral motor nerves, usually in an asymmetric fashion. There have been few reported cases of progression to frank quadriplegia. Secondary amyloidosis refers to the deposition of amyloid in various tissues due to an underlying chronic inflammatory state. We report the first case, to our knowledge, of a patient with MMN associated with high titer of GM1 antibody who developed acute paraplegia with both cranial nerve and worsening sensory involvement associated with multiorgan compromise due to a secondary amyloidosis involving the myocardium.

Schizophrenia and N-methyl-D-aspartate receptors: evolutionary adaptations from malfunctioning molecules?

In the history of evolution, biologic organisms have formed traits with both adaptive and sometimes maladaptive significance to their surrounding environments. The sickle cell mutation genes conferring resistance to malarial infection in human heterozygotes is an example of how certain genetic abnormalities can serve adaptive significance to an organism. Schizophrenia and the "Odyssyian personality" have often been cited as a neuropsychiatric correlate for evolutionary adaptive benefit in an organism. This article re-examines the possibility that schizophrenia-related genes can serve a beneficial adaptive role and that altered function of the N-methyl-D-aspartate receptor is the molecular correlate of such adaptive significance.

Long-term potentiation and N-methyl-D-aspartate receptors: foundations of memory and neurologic disease?

Understanding the physiology of learning and memory is one of the great challenges of neuroscience. The discovery in recent years of long-term potentiation (LTP) of synaptic transmission and the elaboration of the mechanisms involved, in particular the NMDA receptor, offers the prospect not only of improving our understanding of normal memory storage and retrieval, but may also yield insights about various neurological and psychiatric clinical disorders. In this review, we begin by examining the different forms, properties, and methods of inducing LTP, followed by a description of molecular mechanisms thought to underlie the phenomenon. Molecular structure of the receptor is discussed, along with the roles of Ca2+ second messenger systems, synaptic morphology changes, and retrograde messengers in LTP. Finally, implications of the NMDA receptor and LTP in learning, memory, and certain clinical conditions such as epilepsy, Alzheimer's disease, and schizophrenia are discussed.

Effects of amygdala, hippocampus, and periaqueductal gray lesions on short- and long-term contextual fear.

The effects of amygdala, hippocampus, and periaqueductal gray (PAG) lesions on contextual fear conditioning in rats were examined. Freezing behavior served as the measure of conditioning. Unlesioned control animals showed reliable conditional freezing in the testing chamber when observed both immediately and 24 hr after footshocks. In contrast, rats with amygdala or ventral PAG lesions exhibited a significant attenuation in freezing both immediately and 24 hr after the shocks. Dorsal PAG lesions had no effect on freezing at either time. Animals with hippocampal lesions displayed robust freezing behavior immediately following the shock, even though they showed a marked deficit in freezing 24 hr after the shock. These results indicate that there are anatomically dissociable short- and long-term conditional fear states.