Handbook of clinical neurology
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Tourette syndrome is a neuropsychiatric disorder characterized by motor and vocal tics, often associated with behavioral disorders, with typical onset in early childhood. In most patients, the symptoms decrease spontaneously when adulthood is reached, or can be treated with behavioral therapy or medication. Only a small proportion of patients are candidates for surgical treatment. ⋯ Current knowledge of cortical-basal ganglia-thalamocortical circuits provides explanations for the beneficial effects of DBS on tics. Inclusion and exclusion criteria have been formulated to identify good candidates for DBS. Because of the small number of patients, there is a strong need for multicenter double-blind trials with standard protocols.
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Deep brain stimulation for seizures has been applied to cerebellum, caudate, locus coeruleus, subthalamic nucleus, mammillary bodies, centromedian thalamus, anterior nucleus of thalamus, hippocampus and amygdala, hippocampal commissure, corpus callosum, neocortex, and occasionally to other sites. Animal and clinical studies have primarily investigated seizure prevention and, to a lessersmaller extent, seizure interruption. No studies have yet shown stimulation able to cure epilepsy. ⋯ We do not know the mechanisms, the best stimulation parameters, the best patient population, or how to predict benefit in advance. We do not know why benefit of neurostimulation for epilepsy seems to increase over time or whether there are long-term deleterious effects. All of these questions may be answerable with a combination of laboratory research and clinical experience.
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Skin biopsy with a 3mm disposable circular punch is easy to perform and allows, after proper processing, the visualization of epidermal, dermal, and sweat gland nerve fibers. A technique of sampling the epidermis alone by applying a suction capsule, the "blister" technique, has also been developed. It is most common to stain immunohistochemically for the pan-axonal marker protein gene product 9.5 (PGP 9.5), an ubiquitin C-terminal hydroxylase. ⋯ In several hereditary neuropathies intraepidermal nerve fiber density may be reduced but other abnormalities can also be demonstrated in dermal myelinated fibers. Some small swellings and varicosities may be present in the distal leg skin biopsy of healthy individuals but large axonal swellings are considered as evidence of a pathological process affecting the normal structure of nerves. The indirect immunofluorescence technique with confocal microscopy provides the opportunity to study the complex structure of sensory receptors and cutaneous myelinated fibers and the innervation of sweat glands, arrector pilorum muscles, and vessels.
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This chapter describes the clinical presentation, diagnosis, and treatment of patients with both aseptic meningitis and encephalitis. It also addresses the major causes of aseptic meningitis. ⋯ Aseptic meningitis, on the other hand, is typically a benign childhood infection requiring supportive care alone. It also reviews available clinical decision rules that may assist the clinician in distinguishing which children with aseptic meningitis are at very low risk of bacterial meningitis using predictors available at the time of clinical presentation.
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To most doctors, brachial and lumbosacral plexopathies are known as difficult disorders, because of their complicated anatomy and relatively rare occurrence. Both the brachial, lumbar, and sacral plexuses are extensive PNS structures stretching from the neck to axillary region and running in the paraspinal lumbar and pelvic region, containing 100000-200000 axons with 12-15 major terminal branches supplying almost 50 muscles in each limb. The most difficult part in diagnosing a plexopathy is probably that it requires an adequate amount of clinical suspicion combined with a thorough anatomical knowledge of the PNS and a meticulous clinical examination. ⋯ The most common cause of brachial plexopathy is probably neuralgic amyotrophy and the most common cause of lumbosacral plexopathy is diabetic amyotrophy. Traumatic and malignant lesions are fortunately rarer but just as devastating. This chapter provides an overview of both common and rarer brachial and lumbosacral plexus disorders, focusing on clinical examination, the use of additional investigative techniques, prognosis, and treatment.