Handbook of clinical neurology
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Obstetrical brachial plexus palsy is considered to be the result of a trauma during the delivery, even if there remains some controversy surrounding the causes. Although most babies recover spontaneously in the first 3 months of life, a small number remains with poor recovery which requires surgical brachial plexus exploration. Surgical indications depend on the type of lesion (producing total or partial palsy) and particularly the nonrecovery of biceps function by the age of 3 months. ⋯ Clinical manifestations at birth are directly correlated with the anatomical lesion. Finally, operative procedures are considered, including strategies of reconstruction with nerve grafting in infants and secondary surgery to increase functional capacity at later ages. However, normal function is usually not recovered, particularly in total brachial plexus palsy.
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Genetic deficiencies of lysosomal catabolic pathways lead to storage disorders with multiple organ abnormalities or to degeneration of purely nervous structures. Krabbe disease and metachromatic leukodystrophy are caused by metabolic errors concerning lipids of neural membranes. They are characterized by demyelination of the central nervous system and, variably, the peripheral nerves. ⋯ In a rare variant, multiple sulfatases are deficient. Stem cell transplantation may prevent disease progression in selected cases. Enzyme replacement is being evaluated, and gene therapies are being developed.
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In neuromuscular disorders beginning in childhood, the involvement of respiratory muscles is common. Deterioration of respiratory function occurs insidiously, contributes to significant morbidity, and is often responsible for mortality. ⋯ The key to care of respiratory problems in neuromuscular disorders is a preventive approach. Careful monitoring of symptoms, regular assessment of pulmonary function, appropriate presurgical management, and aggressive treatment of respiratory infections must be considered a standard of care.
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There are many children with intractable epilepsy who do not respond to anticonvulsant medications yet are not candidates for resective epilepsy surgery. For these children and more, nonpharmacologic therapies can be very helpful. ⋯ Neurostimulation, using electricity to abort seizures, includes vagus nerve stimulation only at this time. However, other treatments such as deep brain stimulation and cortical responsive stimulation (NeuroPace) are under active development.
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Magnetic resonance imaging (MRI) has become the standard of care for the evaluation of different neurological disorders of the brain and spinal cord due to its multiplanar capabilities and excellent soft tissue resolution. With the large and increasing population of patients with implanted deep brain stimulation (DBS) devices, a significant proportion of these patients with chronic neurological diseases require evaluation of their primary neurological disease processes by MRI. ⋯ These include the potential for induction of electrical currents or heating in DBS devices, which can result in neurological tissue injury, magnetic field-induced device migration, or disruption of the operational aspects of the devices. In this chapter, we review the basic physics of potential interactions of the MRI environment with implanted DBS devices, summarize results from phantom studies and clinical series, and discuss present recommendations for safe MRI in patients with implanted DBS devices.