1,722,338 research outputs found
Role of EMG in congenital hypotonia with favorable outcome
No full textBackground: Since hypotonia is the phenotype of several clinical conditions that do not always lead to a favorable outcome, prompt diagnosis is important. Congenital Hypotonia with Favorable Outcome (CHFO), an underestimated condition, should be rapidly differentiated from other more serious hypotonic states by means of simple, effective and only slightly invasive instrumental diagnostic examinations. Aim: We analyzed the electromyographic data of a group of patients with CHFO and compared them with data taken from their muscle biopsies to evidence the utility of the electromyographic study (EMG) in this condition. Methods: We performed EMG, nerve conduction study (NCS) and muscle biopsy on 41 subjects with a diagnosis of CHFO (age range 9 months to 12 years, mean age, 6.7 years). Results: No specific EMG findings were observed. Muscle biopsy was normal in all subjects, and we obtained concordance between biopsy and EMG in 85% of the subjects (35/41). Conclusion: A normal EMG examination helps investigators to exclude several neurological diseases characterized by hypotonia and it can provide valuable complementary information to confirm the clinical diagnosis of CHFO. © Mattioli 1885
The independent role of neonatal seizures in epilepsy and other long-term neurological outcomes
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Acute motor neuropathy after intrathecal treatment with methotrexate: A case report
No full textIntrathecal therapy with methotrexate and cytosine-arabinoside is a therapeutic strategy for maintaining remission in patients with acute lymphoblastic leukemia, that is associated with neurological side effects are described. We report a 4-year-old girl who became tetraplegic after the last dose of intrathecal therapy. Cerebrospinal fluid showed elevated protein level and nerve conduction study revealed a motor neuronopathy. Spine magnetic resonance imaging revealed gadolinium enhancement of the anterior roots of the cauda equina. We describe a myelopathy confined to the anterior horns and anterior spinal nerve roots without sensory involvement caused by intrathecal methotrexate. The pathogenesis of this selective involvement remains uncertain. © 2007 IOS Press. All rights reserved
Acute symptomatic neonatal seizures in preterm neonates: etiologies and treatments
No full textAcute symptomatic neonatal seizures in preterm newborns are a relevant clinical challenge due to the presence of many knowledge gaps. Etiology-wise, acute symptomatic seizures have an age-specific epidemiology, with intraventricular hemorrhage and its complications representing the first cause in extremely and very preterm neonates, whereas other etiologies have similar occurrence rates as in full-term infants. Specific treatment strategies for the premature neonates are not yet available. Studies suggest a similarly low response rate with even more unfavorable prognosis than in full-term infants. Pharmacodynamic and pharmacokinetic changes are likely under way during the preterm period, with the potential to affect both effectiveness and safety of antiepileptic drugs in these patients. However, due to the lack of clear evidence to guide prioritization of second-line drugs, off-label medications are frequently indicated by review papers and flow-charts, and are prescribed in clinical practice. We therefore conclude by exploring potential future lines of research
Monitoring of newborns at high risk for brain injury
Full text linkDue to the increasing number of surviving preterm newborns and to the recognition of therapeutic hypothermia as the current gold standard in newborns with hypoxic-ischaemic encephalopathy, there has been a growing interest in the implementation of brain monitoring tools in newborns at high risk for neurological disorders. Among the most frequent neurological conditions and presentations in the neonatal period, neonatal seizures and neonatal status epilepticus, paroxysmal non-epileptic motor phenomena, hypoxic-ischaemic encephalopathy, white matter injury of prematurity and stroke require specific approaches to diagnosis. In this review we will describe the characteristics, aims, indications and limitations of routinely available diagnostic techniques such as conventional and amplitude-integrated EEG, evoked potentials, cranial ultrasound and brain MRI. We will conclude by briefly outlining potential future perspectives from research studies
Metabolic Disorders Following Kidney Transplantation
No full textSeveral metabolic processes are altered by kidney transplantation (KT), mainly due to a mechanism of overlapping between preexisting favoring conditions and side effects of immunosuppressive agents. Among the metabolic alterations commonly observed after KT, diabetes mellitus, dyslipidemias, and uric acid metabolism represent the most important ones, mainly due to the clinical impact they may present not only in relation to graft function but also in terms of patient survival. The most recent (as of 2017) national guidelines, randomized controlled trials, and metaanalyses on these arguments are reported in this chapter, with the intent to better clarify the therapeutic strategies to adopt in case of metabolic disorder after KT
Diagnosis and management of acute seizures in neonates.
No full textSeizures in newborns are the most frequent manifestation of an acute neurologic dysfunction, but sometimes they can be the early onset of an epileptic encephalopathy, mainly of genetic origin. When neonatal seizures are suspected, a video-encephalographic (EEG) recording is fundamental for correct interpretation of the clinical phenomena to avoid unnecessary anticonvulsant therapy. After a diagnosis of seizures, continuous monitoring is necessary to evaluate seizure burden, to diagnose status epilepticus, to monitor efficacy of anticonvulsant medications, and to form a differential diagnosis with the uncoupling phenomenon. Although the outcome of newborns depends mostly on the underlying etiology, the presence of seizures seems to have a negative prognostic value, especially if the seizures are prolonged. The most common antiepileptic drugs are only partially effective. Phenobarbital is the most used first-line drug with no differences between preterm and full-term newborns. Moderate to severe abnormal background EEG activity and electrographic-only seizures seem to be associated with a lack of response to phenobarbital. Moreover, seizures management in newborns has not significantly changed if the etiology is acute symptomatic. However, evidence of promising therapeutic approaches for neonatal epilepsy encephalopathies is growing. An accurate and prompt diagnosis of seizures is required for early management to avoid the potential detrimental effects of seizures on the immature brain
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