Current opinion in neurology
-
To highlight recent advances in understanding the clinical manifestations and molecular genetics of myotonic syndromes, with particular emphasis on the myotonic dystrophies. ⋯ It now appears likely that myotonic dystrophy is the first instance of a genetic disease in which the harmful effect of a mutation involves the production of a pathogenic RNA. However, the exact mechanism is not understood, and it is unclear whether this RNA-mediated disease process is also responsible for the manifestations of myotonic dystrophy in non-muscle tissues.
-
Mitochondrial dysfunction has gained considerable interest as a potential cause of epileptic seizures and therapy-resistant forms of severe epilepsy. Impairment of mitochondrial function has recently been observed in the seizure focus of human and experimental epilepsy. ⋯ Since mitochondrial oxidative phosphorylation provides the major source of adenosine triphosphate in neurons, and mitochondria participate in cellular Ca2+ homeostasis they can modulate neuronal excitability and synaptic transmission. Furthermore, mitochondria are intimately involved in pathways leading to the neuronal cell death characteristic for the areas of epileptogenesis.
-
Now nearly 5 years post-approval, vagus nerve stimulation has emerged as a major non-pharmacological treatment for epilepsy. The place of vagus nerve stimulation among antiepileptic drugs and other surgical therapies is still evolving. This review evaluates the role of vagus nerve stimulation in light of recently published research of its mechanism(s) of action, long-term efficacy, safety and tolerability, and application to other disorders besides epilepsy.
-
Curr. Opin. Neurol. · Apr 2002
ReviewEpilepsy surgery within the temporal lobe and its short-term and long-term effects on memory.
In patients with pharmacologically intractable, complex-partial seizures, surgical excision of the involved temporal lobe may have significant therapeutic benefit. Resection of mesial structures of the temporal lobes, however, entails a significant risk of decreased memory function. Recent advances in the assessment of memory changes following temporal lobectomy surgery emphasize the complexity of subjective ratings of memory functioning in this population. ⋯ Functional magnetic resonance imaging appears to provide significant information regarding hemispheric representation of language in the temporal lobe epilepsy patient, and the use of this technique to predict memory status following surgery appears promising. Clinical studies involving patients who had temporal lobectomy surgeries have also revealed changes in emotional learning related to the degree of amygdala involvement. Moreover, there is increasing evidence to suggest that differential changes in emotional learning occur among patients with right versus left temporal lobe resections.
-
Delirium is the presenting feature in a few stroke patients, but can complicate the clinical course of acute stroke in up to 48% of cases. Old age, extensive motor impairment, previous cognitive decline, metabolic and infectious complications, and sleep apnoea are all predisposing conditions for delirium. Patients with delirium have longer hospitalizations and a poorer prognosis, and are at increased risk of developing dementia. The identification of the patients at risk and non-pharmacological preventative interventions are the key measures in the management of delirium.