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Electrophysiology of motor pathways for sphincter control in multiple sclerosis.

The central and peripheral motor pathways serving striated sphincter muscle function were studied using cortical and lumbar transcutaneous electrical stimulation, pudendal nerve stimulation and sphincter electromyography in 23 patients with multiple sclerosis (MS), and sphincter disturbance, including incontinence of urine or faeces, urinary voiding dysfunction, or constipation. The central motor conduction time was significantly increased in the MS group compared to controls (p less than 0.05). Damage to both the upper and lower motor neuron pathways can contribute to sphincter disturbance in MS. The latter may be due to coexisting pathology or to involvement of the conus medullaris by MS.

Adult↗

Promoting neurotrophic effects by GPCR ligands.

The neurotrophins-nerve growth factor (NGF), brain-derived neurotrophic factor (BDNF), NT-3 and NT-4-represent a family of proteins essential for neuronal survival and plasticity. Each neurotrophin can signal through two different transmembrane receptors, Trk receptor tyrosine kinases and the p75 neurotrophin receptor, the first member of the TNF receptor superfamily. Neurotrophic factors play an important role in neurodegenerative diseases, as well as neuropsychiatric disorders such as depression, bipolar disease and eating disorders. Indeed, a number of approaches have been taken to use neurotrophins to treat Alzheimer's dementia, amyotrophic lateral sclerosis and peripheral sensory neuropathy. However, many of these clinical trails have failed, due to problems in delivery and unforeseen side effects of neurotrophic factors. An alternative approach is to use ligands in the G protein-coupled receptor (GPCR) family to transactivate trophic activities. We have discovered that treatment with adenosine, a neuromodulator that acts through G protein-coupled receptors, is capable of activating Trk tyrosine kinase receptors. Transactivation of neurotrophic receptors by GPCR ligands raise the possibility that small molecules may be used to elicit neurotrophic effects for the treatment of neurodegenerative diseases. This approach would allow for selective targeting of neurons that express specific G protein-coupled receptors and trophic factor receptors. GPCRs transduce information provided by extracellular signals to modulate synaptic activity and neurotransmission. In addition to the classical G protein signalling, GPCR ligands also activate receptor tyrosine kinases (RTK), including neurotrophin receptors. Activation of Trk neurotrophin receptors can occur by GPCR ligands in the absence of neurotrophins. Adenosine and PACAP (pituitary adenylate cyclase activating polypeptide) induce Trk activation specifically through their respective GPCRs to promote cell survival. Transactivation of Trks by GPCRs has emerged as a new theme in the biology of neurotrophin function. Although the precise role of transactivation is unknown, one possibility is that it adds a safety factor that might protect neurons from death in the absence of neurotrophins. Abnormal activity of the neurotrophin system has been implicated in several psychiatric and neurobiological illnesses. However, the lack of knowledge about the precise site of neurotrophin dysfunction has compromised the ability to improve the efficacy and the safety of drugs used in treatment modalities. If small-molecule GPCR ligands can ameliorate neuronal cell loss through Trk, transactivation may offer a new strategy for promoting trophic effects during neurodegeneration.

Adenosine↗

Tetanus-induced re-activation of evoked spiking in the post-ischemic dentate gyrus.

This study aimed at investigating and influencing the basic electrophysiological functions and neuronal plasticity in the dentate gyrus in freely moving rats at several time-points after global ischemia. Although neuronal death was induced selectively in the cornu ammonis, subfield 1 (CA1)-region of the hippocampus, we found an additional loss of the population spike in the dentate gyrus after stimulation of the perforant path. Input/output-measurements revealed that as early as 1 day post-ischemia population spike generation in the granular cell layer is greatly decreased when compared with pre-ischemic values and to sham-operated animals, despite an apparently intact morphology of granular cells as evidenced by Nissl-staining. In contrast, the synaptic transmission (excitatory postsynaptic field potential) shows no significant difference when comparing values before and after ischemia and ischemic and sham-operated animals. Despite reduced output function, indicated by very small population spike amplitudes, long lasting potentiation can be induced 10 days after ischemia. Surprisingly, even "silent" populations of neurons, which appear selectively post-ischemia and do not show any evoked population spike, can be re-activated by tetanisation which is followed by a normal appearing long-term potentiation. However, this functional recovery seems to be partial and transient under current conditions: population spike-values do not reach pre-ischemic values and return to the low pre-tetanic baseline values the next day. Electrophysiological measurements ex vivo after ischemia indicate that the neuronal dysfunction in the dentate gyrus is not due to locally destroyed structures but that the activity of granular cells is merely suppressed only under in vivo conditions. In summary, global ischemia leaves a neighboring morphologically intact input area, functionally impaired. However, neuronal function can be partially regenerated by electrophysiological tetanic stimulation.

Analysis of Variance↗

Degenerative axonal changes in the hippocampus and amygdala in Parkinson's disease.

The morphological background of cognitive and emotional impairments in Parkinson's disease (PD) has not yet been fully explained. We evaluated the expression of synaptic proteins: alpha- and beta-synuclein, synaptophysin and synaptobrevin and ultrastructural changes of perikaryons and axons in limbic structures at post-mortem from cases of PD to estimate degenerative axonal pathology in the hippocampus and amygdala [corrected]. Limbic structures (enthorinal cortex, hippocampus, and amygdala) are essential for the cognitive processes and emotional behaviour. We found that presynaptic axon pathology is mostly connected with hippocampal CA2-3 and dentate hilar regions as well as with the cortical and medio-central complexes of amygdala. Heterogeneous immunoreactivity of alpha-synuclein and diversified ultrastructure of Lewy bodies (LBs) and Lewy neurites (LNs) indicate their consecutive developmental stages. We observed an excessive perineuroneal expression of synaptophysin in the dentate hilar region in all PD cases, except one. This suggests that the dysfunction of synapses in this region may result from axonal pathology. Our study indicates a relation between cognitive and behavioural symptomatology in PD and alpha-synuclein dependent axonal pathology in the hippocampus and amygdala.

Aged↗

Glutamate receptor ligands.

Glutamate acts through a variety of receptors to modulate neurotransmission and neuronal excitability. Glutamate plays a critical role in neuroplasticity as well as in nervous system dysfunctions and disorders. Hyperfunction or dysfunction of glutamatergic neurotransmission also represents a key mechanism of pain-related plastic changes in the central and peripheral nervous system. This chapter will review the classification of glutamate receptors and their role in peripheral and central nociceptive processing. Evidence from preclinical pain models and clinical studies for the therapeutic value of certain glutamate receptor ligands will be discussed.

Animals↗

Immediate recovery of cognitive functions and resolution of fatigue by treatment with weak electromagnetic fields in a patient with multiple sclerosis.

Cognitive deficits are common among patients with multiple sclerosis (MS). The pathogenetic mechanisms underlying the cognitive impairment in MS are unknown and there is presently no effective therapeutic modality which has shown efficacy in improving cognitive deficits in MS. A 53 year old college professor with a long history of secondary progressive MS experienced, over the preceding year, noticeable deterioration in cognitive functions with difficulties in short and long term memory, word finding in spontaneous speech, attention and concentration span. Unable to pursue his academic activities, he was considering early retirement. Mental examination disclosed features of subcortical and cortical dementia involving frontal lobe, left hemispheric and right hemispheric dysfunction. Almost immediately following the extracerebral application of AC pulsed electromagnetic fields (EMFs) of 7.5 picotesla intensity and a 4-Hz sinusoidal wave, the patient experienced a heightend sense of well being, which he defined as enhancement of cognitive functions with a feeling "like a cloud lifted off my head." He reported heightend clarity of thinking and during the application of EMFs he felt that words were formed faster and he experienced no difficulty finding the appropriate words. His speech was stronger and well modulated and he felt "energized" with resolution of his fatigue. There was improvement in manual dexterity and handwriting and testing of constructional praxis demonstrated improvement in visuospatial, visuoperceptive and visuomotor functions. It is suggested that some of the cognitive deficits associated with MS, which are caused by synaptic disruption of neurotransmitter functions, may be reversed through pulsed applications of picotesla range EMFs.

Cognition Disorders↗

The differentiation of peripheral effector neuron failure from acute brain stem dysfunction in a critically ill patient.

A patient appeared to be in coma following pneumococcal meningitis, an intracerebral haemorrhage, and a cardiac arrest. Late in the course of his illness neurophysiological investigations confirmed a proposal that he also had a fulminant acute demyelinating polyneuropathy which, for a period of five days, rendered the patient completely unresponsive. The patient recovered and is now working without any disability.

Adult↗

Uncovering the genetic architecture of ME/CFS: a precision approach reveals impact of rare monogenic variation.

BACKGROUND: Myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS) is a disabling and heterogeneous disorder lacking validated biomarkers or targeted therapies. Clinical variability and elusive pathophysiology hinder progress toward effective diagnostics and treatment. Core symptoms include persistent fatigue, post-exertional malaise, unrefreshing sleep, cognitive dysfunction, and pain. We tested whether an individualized, “n-of-1” genomic and transcriptomic framework combined with comprehensive, participant-informed phenotyping could reveal molecular signatures unique to each patient. METHODS: Clinical-grade whole-genome sequencing was conducted in 31 affected individuals from 25 families, with RNA-seq performed on a subset (16 affected, 7 unaffected) using blood samples. Machine-learning assisted variant triage, transcript-aware damage prediction, and expert review identified pathogenic or likely pathogenic variants in 8 of 25 probands (32%) and 12 of 31 affected individuals (39%). RESULTS: Findings revealed marked genetic heterogeneity, including large-effect rare and more common variants. Implicated pathways included ATP generation, oxidative phosphorylation, fatty acid oxidation; regulation of glycolysis, amino acid and lipid turnover; ion and solute homeostasis; synaptic signaling, excitability, oxygen transport, and muscle integrity, resilience, and post-exertional recovery; previously implicated processes. Plausible modifiers influencing disease onset, severity, and relapsing–remitting patterns and possibly explaining intrafamilial variability and inconsistent findings across studies, were also identified. Despite gene-level diversity, downstream effects converged on impaired energy production, reduced stress resilience, and vulnerability to post-exertional metabolic failure; disruptions consistent with core ME/CFS symptoms of exertional intolerance, cognitive fog, and fatigue. CONCLUSIONS: Our findings support the hypothesis that at least a subset of ME/CFS cases represent distinct molecular disorders that converge on shared physiological pathways. Validation in larger, more diverse cohorts will be essential to test this hypothesis and establish generalizability, but increase size alone is unlikely to resolve causation in a disorder defined by rarity, heterogeneity, and molecular complexity. We suggest that progress will require experimental designs that integrate individual-level genomic data with deep, participant-informed deep phenotyping, capturing the combined effects of rare and common variants and environmental modifiers on disease expression and progression. We believe that an individualized precision medicine framework will uncover molecular drivers and modifiers of ME/CFS previously obscured by heterogeneity, enabling biologically informed stratification, improved trial design, biomarker discovery, and targeted interventions in this historically neglected condition.

Humans↗

Altered GABAergic neurotransmission in mice lacking dopamine D2 receptors.

The levels of glutamic acid decarboxylase (GAD) were strongly increased in the cortex and the striatum in dopamine D2 receptors null (D2R-/-) mice, which show a significant locomotor impairment. In this study, the effects of different GABAergic drugs on locomotor activity were analyzed in D2R-/- mice. After administering muscimol (1 mg/kg), a GABA(A) receptor agonist, the D2R-/- mice showed increased locomotor activity up to 200%. When the muscimol dose was increased (4-6 mg/kg), the D2R-/- mice exhibited seizure-like behavior, and the electroencephalographic (EEG) recordings during these behaviors showed a high amplitude rhythmic epileptiform activity in these mice. In situ hybridization showed that after injecting muscimol in the D2R-/- mice, the expression of enkephalin and immediate early gene, NGFI-A, was closely regulated with the locomotor activity regulated by GABAergic stimulation. These results suggest that the absence of D2R alters the GABAergic neurotransmission, specifically on GABA(A)-receptor mediated signaling, and stimulating the GABA(A) receptor can reverse the dysfunction of GABAergic inhibition in the motor circuits in the basal ganglia.

Animals↗

A mouse model of episodic ataxia type-1.

Episodic ataxia type-1 (EA1) is a dominant human neurological disorder characterized by stress-induced attacks of ataxia. EA1 is caused by mutations in the voltage-gated potassium channel Kv1.1, and affected individuals are heterozygous. Here we introduced the V408A EA1 mutation into mice using homologous recombination. In contrast to Kv1.1 null mice, homozygous V408A/V408A mice died after embryonic day 3 (E3). V408A/+ mice showed stress-induced loss of motor coordination that was ameliorated by acetazolamide, a carbonic anhydrase inhibitor that minimizes EA1 symptoms in human patients. We made electrophysiological recordings from cerebellar Purkinje cells in both V408A/+ mice and their wild-type littermates. V408A/+ mice showed a greater frequency and amplitude of spontaneous GABAergic inhibitory postsynaptic currents (IPSCs) than did wild type; however, the amplitude or frequency of miniature IPSCs and the basket cell firing frequency did not differ between groups. The stress-induced motor dysfunction in V408A mice is similar to that of family members harboring the EA1 allele, and our findings suggest that these behavioral changes are linked to changes in GABA release.

Acetazolamide↗

Imaging sympathetic innervation.

The autonomic innervation of the heart modifies most cardiac functions. Especially the heart rate and the force of contraction of myocytes are modulated by the autonomic nervous system. A number of specific neurotransmitters interact with receptors on post- and presynaptic binding sites regulating the complex electromechanical system of the heart. Disturbances at this interaction result in a variety of cardioneuropathies. The clinical manifestations can be mild and may only consist of sporadic arrhythmias without hemodynamic effects. In some cases however the autonomic dysfunction may be severe, e.g. in the acute phase of the Guillain-Barré syndrome and in advanced diabetic neuropathy. At present, the only available techniques to visualise and quantitate disbalanced innervation of the myocardium are scintigraphic modalities as single photon emission tomography (SPECT) and positron emission tomography (PET) with appropriate radiopharmaceuticals. These methods are reviewed with respect to their possible clinical application and to future developments.

Autonomic Nervous System Diseases↗

Rethinking the excitotoxic ionic milieu: the emerging role of Zn(2+) in ischemic neuronal injury.

Zn(2+) plays an important role in diverse physiological processes, but when released in excess amounts it is potently neurotoxic. In vivo trans-synaptic movement and subsequent post-synaptic accumulation of intracellular Zn(2+) contributes to the neuronal injury observed in some forms of cerebral ischemia. Zn(2+) may enter neurons through NMDA channels, voltage-sensitive calcium channels, Ca(2+)-permeable AMPA/kainate (Ca-A/K) channels, or Zn(2+)-sensitive membrane transporters. Furthermore, Zn(2+) is also released from intracellular sites such as metallothioneins and mitochondria. The mechanisms by which Zn(2+) exerts its potent neurotoxic effects involve many signaling pathways, including mitochondrial and extra-mitochondrial generation of reactive oxygen species (ROS) and disruption of metabolic enzyme activity, ultimately leading to activation of apoptotic and/or necrotic processes. As is the case with Ca(2+), neuronal mitochondria take up Zn(2+) as a way of modulating cellular Zn(2+) homeostasis. However, excessive mitochondrial Zn(2+) sequestration leads to a marked dysfunction of these organelles, characterized by prolonged ROS generation. Intriguingly, in direct comparison to Ca(2+), Zn(2+) appears to induce these changes with a considerably greater degree of potency. These effects are particularly evident upon large (i.e., micromolar) rises in intracellular Zn(2+) concentration ([Zn(2+)](i)), and likely hasten necrotic neuronal death. In contrast, sub-micromolar [Zn(2+)](i) increases promote release of pro-apoptotic factors, suggesting that different intensities of [Zn(2+)](i) load may activate distinct pathways of injury. Finally, Zn(2+) homeostasis seems particularly sensitive to the environmental changes observed in ischemia, such as acidosis and oxidative stress, indicating that alterations in [Zn(2+)](i) may play a very significant role in the development of ischemic neuronal damage.

Animals↗

[Pathophysiologic basis of the treatment of neurogenic pain].

According to the International Association for the Study of Pain (IASP) neuropathic pain is "pain initiated or caused by a primary lesion or dysfunction or transitory perturbation in the peripheral or central nervous system". Neuropathic pain is usually classified according to the etiology, location of the lesion, and pain characteristics--individual symptoms and signs, but also according to the possible mechanisms involved. Identifying the underlying pain mechanisms during the diagnosis becomes essential for treatment strategies. The clinical picture of neuropathic pain is similar in many cases, and clinical features include: ongoing spontaneous or evoked pain in an area with sensory loss, positive sensory symptoms such as allodynia and hyperalgesia, wind-up pain following repetitive stimulation, referred pain and abnormal sympathetic activity. The understanding of the mechanisms underlying neuropathic pain has increased over the last decade. The primary pathophysiologic mechanisms that produce pain are: nociceptor sensitization, nerve trunk inflammation, sympathetic nervous system involvement, ectopic neuronal discharges, pathologic synaptic reorganization--neuroplasticity and central sensitization. In most clinical features, there is a complex interaction that involves peripheral and central nervous system rather than a single mechanism. Because numerous mechanisms are implicated, the traditional approach to pain control using single drug therapy may not be most effective, and therapeutic combinations are a better choice. Neuropathic pain is poorly responsive to conventional analgesics. In spite of a variety of drug classes used to treat neuropathic pain including antidepressants, anticonvulsants, antiarrhythmics, opioids, local anesthetic blockers, neuropathic pain remains difficult to treat. The possibility to select specific drugs and treatments for the individual patient lies in elucidating the relationships between clinical neuropathic states and underlying pathophysiologic changes. Progress in defining the mechanisms involved in neuropathic pain, based on further clinical studies and fundamental investigations, will improve therapeutic management of neuropathic pain.

Animals↗

Widespread gamma-secretase activity in the cell, but do we need it at the mitochondria?

gamma-Secretase cleavage of the amyloid precursor protein already subjected to a prior beta-secretase cleavage generates beta-amyloid (Abeta) peptide fragments, which are major constituents of the amyloid plagues found in Alzheimer's disease brain tissues. gamma-Secretase activity and components of the gamma-secretase complex are found in the endoplasmic reticulum-Golgi intermediate compartment, the Golgi, the trans-Golgi network, the plasma membrane, the endosomal-lysosomal system and recently, the mitochondria. Abeta fragments have been shown to be neurotoxic, leading to mitochondrial dysfunction and enhanced apoptotic cell death. However, if Abeta fragments are indeed detrimental to neurons, the widespread presence of enzymatic activity that would result in their generation in the cell appears to make little sense. The presence of a gamma-secretase complex in the mitochondrion, an organelle that is particularly susceptible to Abeta toxicity, is even more puzzling. Emerging evidence suggests that both secreted and intracellular Abeta fragments have endogenous functions. Also, while the fibrillogenic Abeta1-42 is clearly neurotoxic, the more abundant and soluble Abeta1-40 is an antioxidant and could potentially be neuroprotective in several ways. A "physiological" amount of Abeta1-40 production by cellular gamma-secretase activity may be part of the neuron's natural counter against oxidative damage, in addition to endogenous roles in neuronal survival and modulation of synaptic transmission. In any case, whether Abeta is produced locally in the mitochondria and the function for mitochondrial Abeta, if produced, is yet unclear.

Alzheimer Disease↗

Striatal D2 receptors and LTD: yes, but not where you thought they were.

D1 and D2 dopamine receptors are expressed in disjoint subsets of striatal projection neurons, the direct and indirect pathways, respectively. This differential distribution of receptors forms the basis for explanations of many aspects of basal ganglia function and dysfunction, but it seems incompatible with some other important properties of striatal neurons. In this issue of Neuron, Wang et al. discover the mechanism of D2 sensitivity of long term depression at synapses on the striatal projection neuron. They show that D2 dependence of LTD does not depend on dopamine receptors of on the projection cell but is mediated by dopamine-induced changes in release of acetylcholine by interneurons that contact projection cells of both types.

Acetylcholine↗

Neuroendocrine evaluation of 5-HT1A function in male alcoholic patients.

BACKGROUND: Preclinical evidences support the hypothesis of a serotonergic dysfunction in alcohol preference. In human, studies have demonstrated a serotonergic hypoactivity in alcoholism. However, little is known about the role of 5-HT1A receptors. METHODS: We assessed the hormonal (prolactin and cortisol) responses to flesinoxan (a highly potent and selective 5-HT1A agonist) in 12 male inpatients meeting DSM-IV criteria for alcohol dependence, 3 weeks after the last reported use of alcohol and antidepressants. These patients were compared to 10 male controls. RESULTS: There was a highly significant difference between alcoholic patients and controls for the area under the curve relative (AUCr) values of prolactin responses. AUCr values of cortisol responses to flesinoxan showed a trend towards lower values in alcoholics compared to controls. CONCLUSION: These results support the implication of the serotonergic system, and particularly a decreased sensitivity of post-synaptic 5-HT1A receptors, in alcoholism.

Adult↗

Neuropharmacological studies with SPECT in neuropsychiatric disorders.

The last decade saw a rapid development of single photon emission computed tomography (SPECT) from a tool to assess cerebral blood flow to the study of specific neurotransmitter systems. Because of the relatively long half-life of SPECT radioisotopes, it is practical to measure the availability of neuroreceptors and transporters in conditions approaching equilibrium. The cost-efficiency of SPECT allowed studies in relatively large samples of patients with various neuropsychiatric disorders. We have applied this approach in studies of dopaminergic, serotonergic, and muscarinergic neurotransmission in patients with dementia, extrapyramidal disorders, schizophrenia, and alcoholism. No simple associations were observed between a single defect in one neurotransmitter system and a certain neuropsychiatric disease. Instead, complex dysfunction of several neurotransmitter systems in multiple, partially connected brain circuits have been implicated. Treatment effects also have been characterized. Microdialysis and neurotransmitter depletion studies showed that most radioligands and endogenous neurotransmitters compete for binding at receptors and transporters. Future research directions include the assessment of endogenous neurotransmitter concentrations measured by depletion studies and of genetic effects on neuroreceptor and transporter expression.

Brain↗

Evidence that antioxidants prevent the inhibition of Na+,K(+)-ATPase activity induced by octanoic acid in rat cerebral cortex in vitro.

The objective of the present study was to investigate the in vitro effects of octanoic acid, which accumulates in medium-chain acyl-CoA dehydrogenase (MCAD) deficiency and in Reye syndrome, on key enzyme activities of energy metabolism in the cerebral cortex of young rats. The activities of the respiratory chain complexes I-IV, creatine kinase, and Na+,K(+)-ATPase were evaluated. Octanoic acid did not alter the electron transport chain and creatine kinase activities, but, in contrast, significantly inhibited Na+,K(+)-ATPase activity both in synaptic plasma membranes and in homogenates prepared from cerebral cortex. Furthermore, decanoic acid, which is also increased in MCAD deficiency, and oleic acid strongly reduced Na+,K(+)-ATPase activity, whereas palmitic acid had no effect. We also examined the effects of incubating glutathione and trolox (alpha-tocopherol) alone or with octanoic acid on Na+,K(+)-ATPase activity. Tested compounds did not affect Na+,K(+)-ATPase activity by itself, but prevented the inhibitory effect of octanoic acid. These results suggest that inhibition of Na+,K(+)-ATPase activity by octanoic acid is possibly mediated by oxidation of essential groups of the enzyme. Considering that Na+,K(+)-ATPase is critical for normal brain function, it is feasible that the significant inhibition of this enzyme activity by octanoate and also by decanoate may be related to the neurological dysfunction found in patients affected by MCAD deficiency and Reye syndrome.

Animals↗