Annals of neurology
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Annals of neurology · Aug 2009
Inhibitory actions of the gamma-aminobutyric acid in pediatric Sturge-Weber syndrome.
The mechanisms of epileptogenesis in Sturge-Weber syndrome (SWS) are unknown. We explored the properties of neurons from human pediatric SWS cortex in vitro and tested in particular whether gamma-aminobutyric acid (GABA) excites neurons in SWS cortex, as has been suggested for various types of epilepsies. ⋯ SWS cortical neurons have a relatively depolarized resting membrane potential and spontaneously fire action potentials that may contribute to increased network excitability. In contrast to previous data depicting excitatory and proconvulsive actions of GABA in certain pediatric and adult epilepsies, GABA plays mainly an inhibitory and anticonvulsive role in SWS pediatric cortex.
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Annals of neurology · Jul 2009
Activation of metabotropic glutamate receptor 5 improves recovery after spinal cord injury in rodents.
Activation of metabotropic glutamate receptor 5 (mGluR5) has neuroprotective properties in vitro and has been reported to limit postischemic lesion volume in vivo. Previously, mGluR5 has been identified on microglia in vitro, but the effects of mGluR5 activation on inflammation in vivo or on recovery after spinal cord injury is unknown. ⋯ These data demonstrate that mGluR5 activation can reduce microglial-associated inflammation, suggesting that the protective effects of mGluR5 agonists may reflect this action. Ann Neurol 2009;66:63-74.
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Annals of neurology · Jun 2009
ReviewIs reflex sympathetic dystrophy/complex regional pain syndrome type I a small-fiber neuropathy?
Neurologist S. Weir Mitchell first described "causalgia" following wartime nerve injury, with its persistent distal limb burning pain, swelling, and abnormal skin color, temperature, and sweating. Similar post-traumatic symptoms were later identified in patients without overt nerve injuries after trauma. ⋯ CRPS symptoms likely reflect combined effects of axonal degeneration and plasticity, inappropriate firing and neurosecretion by residual axons, and denervation supersensitivity. The resulting tissue edema, hypoxia, and secondary central nervous system changes can exacerbate symptoms and perpetuate pathology. Restoring the interest of neurologists in RSD/CRPS should improve patient care and broaden our knowledge of small-fiber functions.
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Annals of neurology · Jun 2009
Multicenter Study Comparative StudyNegative fluid-attenuated inversion recovery imaging identifies acute ischemic stroke at 3 hours or less.
To evaluate the use of fluid-attenuated inversion recovery (FLAIR) imaging as surrogate marker of lesion age within the first 6 hours of ischemic stroke. ⋯ "mismatch" between positive DWI and negative FLAIR allows the identification of patients that are highly likely to be within the 3-hour time window. Within the first 6 hours of stroke, the sensitivity of FLAIR sequences for acute ischemic lesions increases with time from symptom onset elapsing, approximating 100% after 3 to 6 hours.
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Annals of neurology · Jun 2009
Case Reports Comparative StudyA novel Nav1.7 mutation producing carbamazepine-responsive erythromelalgia.
Human and animal studies have shown that Na(v)1.7 sodium channels, which are preferentially expressed within nociceptors and sympathetic neurons, play a major role in inflammatory and neuropathic pain. Inherited erythromelalgia (IEM) has been linked to gain-of-function mutations of Na(v)1.7. We now report a novel mutation (V400M) in a three-generation Canadian family in which pain is relieved by carbamazepine (CBZ). ⋯ Our results demonstrate a normalizing effect of CBZ on mutant Na(v)1.7 channels in this kindred with CBZ-responsive inherited erythromelalgia. The selective effect of CBZ on the mutant Na(v)1.7 channel appears to explain the ameliorative response to treatment in this kindred. Our results suggest that functional expression and pharmacological studies may provide mechanistic insights into hereditary painful disorders.