Interictal and ictal SPECT in a neonate with hemimegalencephaly.
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Biomedical subjects
Publications and source records attributed to I Alfonso.
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Coma is differentiated from sleep by the absence of a normal arousal response and from death by the presence of heart beats and the absence of brain death criteria. Most causes of coma are readily diagnosed and treated. Others require a test whose results are not immediately available, transportation or a risky procedure and empirical treatment has to be considered. In addition to treating the cause of coma, treatment of the systemic and neurological causes of secondary brain damage is paramount.
Unfortunately, in spite of the advances in foetal and perinatal medicine in the last twenty years, the incidence of cerebral palsy has remained unchanged (1.5-2.5 per 1000 live births). It has even possibly risen slightly in premature babies of low birth weight, in parallel with the increased survival of these babies. In spite of modern techniques of rehabilitation, 25% of these patients cannot walk and 35% are mentally retarded. This costs society 5,000 million dollars annually, not counting the loss of opportunity and the emotional and economic burden imposed on these families. The development of new preventive measures such as the use of antagonists of the cortical excitatory amino acids (which when in excess may cause irreversible cerebral damage in cases of hypoxic-ischaemic encephalopathy of the new born). Intramuscular botulinum toxin and continuous intrathecal baclofen seem to promise a reduction in the incidence and functional incapacity of cerebral palsy.
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Conventional computerized cranial tomography by single photon emission (TLCESF) is a nuclear medicine technique which makes use of a radioactive chemical complex to obtain a qualitative image of cerebral, cerebellar and brain-stem vascularization. This technique shows the changes in vascularization which occur between (decrease) and during (increase) the clinical events of paroxysmal epilepsy (ECP) in the area of origin. This technique is indicated in neonates with ECP which does not disappear on etiological treatment, where a non-epileptic cause cannot be ruled out on clinical grounds and where there are no associated encephalographic epileptic changes during the ECP.
Two children developed severe cognitive and behavioral deterioration suggestive of a degenerative disease while being treated with sodium valproate for idiopathic, localization-related epilepsy with centrotemporal spikes. Magnetic resonance imaging revealed marked central and generalized cortical and cerebellar atrophy. In both patients, clinical symptoms and signs cleared in a few weeks following valproate withdrawal. The magnetic resonance imaging appearance improved within 3 months in 1 of the patients and normalized in both after 6 and 12 months. No metabolic changes were associated with the clinical or imaging abnormalities. Although the mechanism of this rare idiosyncratic complication of valproate therapy is unknown, we advocate discontinuing valproate therapy in all epileptic patients with neuromental deterioration or brain atrophy of unknown etiology.
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We report a case of linear nevus sebaceous syndrome with large areas of calcification involving multiple layers of the posterior aspects of both eyes. This patient had a normal neurological examination despite dysplastic brain changes. We discuss and review the multiple clinical presentation and embryopathogenesis of this disorder.
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Encephalocraniocutaneous lipomatosis is a rare hamartomatosis involving the craniofacial region and the central nervous system. The most prominent clinical features are large areas of scalp alopecia, soft subcutaneous craniofacial masses, lipomas, connective tissue nevi of the eyelids and surrounding areas, pterygium-like choriostoma of the ocular conjunctiva, mental retardation, motor deficit, and seizures. Of the eight patients reported previously, three had spinal cord evaluations and two had evidence of lipomatosis. We report the third patient with this association, review the literature of encephalocraniocutaneous lipomatosis, and stress the importance of spinal cord evaluation during the newborn period.
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INTRODUCTION AND DEVELOPMENT: Evoked potentials evaluation of children with central nervous system developmental disorders o dysfunctions is better than clinical and imaging assessment for determination of: 1) conceptional age, 2) auditory and visual threshold, 3) integrity of sensory pathways, 4) neurologic motor outcome, and 5) course and evaluation of treatment of neurometabolic disorders. CONCLUSIONS: However, it is not the same for predicting or making the diagnosis of the majority of primary developmental disorders (autism, receptive and expressive language disorders, learning disability, attention deficit disorders and Gilles de la Tourette syndrome.
INTRODUCTION: A literature review using the term Ohtahara syndrome and early infantile epileptic encephalopathy revealed 51 cases. DEVELOPMENT: The characteristics of these cases were: 1) early onset of seizures; 2) tonic seizures; 3) poor response to treatment; 4) mental retardation; 5) poor prognosis; 6) burst-suppression EEG pattern; 7) evolution to West syndrome, and 8) multiple causes. These characteristics are non specific. No cause was found in most cases. The onset of seizures was between 7 hours and 86 days of age. The most frequent brain imaging abnormality was diffuse atrophy. ACTH was effective in a few cases and hemispherectomy was successful in one case. CONCLUSION: We believe that Ohtahara syndrome and early myoclonic encephalopathy are the same entity.
INTRODUCTION: Continuous display four channels EEG monitoring and near infrared spectroscopy (NIRS) are relatively new and valuable techniques used for continuous brain monitoring in the neonatal period. Supporting the value of continuous display four channels EEG monitoring in the evaluation of neonates with paroxysmal motor events is a study recently conducted at Miami Children s Hospital. DEVELOPMENT: In this study 30 events were captures in 88 hours of simultaneous continuous display four channels EEG monitoring and continuous video EEG telemetry recording in five patients. Fourteen of the events were consider epileptic and 16 were considered non epileptic after evaluation of the continuous display four channels EEG monitoring printed epochs. RESULTS: The continuous video EEG telemetry confirmed the diagnosis for all the events. Several studies attest to the usefulness of NIRS in neonates and in older children with pathologies similar to those seeing in neonates. CONCLUSIONS: 1. NIRS is the best method to monitor regional cerebral oxygen saturation; 2. Regional cerebral oxygen saturation values are primarily those of the venous circulation in the subcortical white matter below the sensor; 3. Values of regional oxygen saturation should be considered not independently but only relative as a change over time; 4. Decreased regional cerebral oxygen saturation may be a sign of impending or established hypoxemia or hypotension, increased cerebral metabolic demands, or a cerebral oxygenation or perfusion problem that involve only brain, and 5. NIRS should be used in conjunction with arterial oxygen saturation and pressure monitoring (to detect hypoxemia and hypotension), with EEG to detect increased cerebral metabolic demands (electroencephalographic seizures) and with anterior cerebral artery Doppler ultrasound to detect perfusion (decreased flow velocity) and oxygenation (constant flow velocity) problems that involve only the brain.