Images in clinical medicine. Sympathetic-nerve damage in brachial-plexus palsy.
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Biomedical subjects
Publications and source records attributed to Israel Alfonso.
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We report the medication management of electroencephalographic status epilepticus with subtle clinical manifestations in a young infant while simultaneously recording electroencephalographic activity and cerebral regional oxygen saturation (rSO(2)) index using near infrared spectroscopy (NIRS). We found that antiepileptic drugs equally influence the frequency of rSO(2) index fluctuations and electroencephalographic seizures. The purpose of this report is to illustrate the use of NIRS in the medication management of an infant with status epilepticus and subtle or no clinical manifestations; and to suggest that if future studies confirm our finding, NIRS may be reliably used to gauge the effects of antiepileptic medications in similar patients.
The purpose of this report is to discuss the association of brachial plexus palsy and congenital deformations. We reviewed all charts of patients less than 1 year of age with obstetrical brachial plexus palsy evaluated by one of the authors (IA) between January 1998 and October 2005 at Miami Children's Hospital Brachial Plexus Center. Of 158 patients with obstetrical brachial plexus palsy, 7 had deformations (4.4%). Deformations were present in 32% of patients delivered by cesarean section, but in only 2% of patients delivered vaginally. The deformations were ipsilateral, involving the chest in two patients, distal arms in two patients, proximal arm in one patient, ear in one patient, and the leg in one patient. All patients with deformations had unilateral Erb's palsies. None had a history of maternal uterine malformation. Two presumptive mechanisms of injury, one causing the deformation (compressive forces) and one causing brachial plexus palsy at the time of delivery (traction forces), were present in all cases. The higher incidence of deformation in patients with obstetrical brachial plexus palsy born by cesarean sections and the presence of two presumptive mechanisms in all of the cases presented here raises the possibility that fetal deformations are a risk factor for obstetrical brachial plexus palsy.
We describe the brain magnetic resonance imaging (MRI) findings in eight neonates with tuberous sclerosis complex to further delineate the spectrum of characteristic findings in these patients. In addition to the previously described characteristic brain MRI findings in neonates, which included cortical tuber, transmantle dysplasia, subependymal nodules, cerebral subependymal giant cell astrocytomas, white-matter anomalies, and hemimegalencephaly, we found one neonate with cerebellar subependymal giant cell astrocytomas and one patient who had a normal MRI and computed tomographic scan of the brain at 4 days of age but subsequently was found to have cortical tubers and subependymal nodules by MRI.
Eleven children ranging in age from 9 to 21 months underwent late nerve reconstruction for persistent shoulder paralysis following an upper brachial plexus birth injury. Only neurolysis was performed in three patients. Neurolysis and nerve grafting bypassing the neuroma with proximal and distal end-to-side repairs was performed in the other eight. All patients were followed for 2 or more years. Two patients underwent a secondary procedure before their final follow-up evaluation. All infants demonstrated significant improvement when assessed by a modified Gilbert shoulder motion scale.
Obstetrical brachial plexus injury occurs when the forces preventing the stretch of the brachial plexus are overcome by the forces stretching it. This report describes an 8-day-old male delivered by uncomplicated cesarean section with right obstetrical brachial plexus palsy and congenital arm atrophy. The patient had a history of decreased right arm movement detected by fetal ultrasound at 18 to 20 weeks of gestation. The purpose of this article is to report that stretching of brachial plexus at birth sufficient to produce a plexus injury may occur in a patient with a vulnerable plexus even in the absence of traction during delivery.
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Early surgical removal of a dysplastic hemisphere appears to be beneficial for neonates with hemimegalencephaly and medically resistant seizures. We analyzed the changes in the cerebral regional oxygen saturation index in a neonate with tuberous sclerosis and right hemimegalencephaly (1) during seven episodes of right hemisphere electroencephalographic status epilepticus with and without clinical manifestations and (2) after right hemispherectomy. The cerebral regional oxygen saturation index demonstrated marked fluctuations and progressive decline in both hemispheres during the episodes and normal values in the remaining hemisphere after surgery. We speculate that decreased oxygenation of the nonepileptic cerebral hemisphere in patients with hemimegalencephaly and medically resistant seizures can contribute to the production of global neurologic impairments in these patients and that the benefits of early hemispherectomy are due to the improved oxygenation of the nondysplastic hemisphere following surgery.
We describe a neonate with tuberous sclerosis complex and right frontal cortical dysplasia who underwent simultaneous near-infrared spectroscopy and electroencephalography (EEG) during repetitive clinically silent right frontal EEG seizures. The seizures produced a progressive decline in regional oxygen saturation index and wider regional oxygen saturation index fluctuations in the right hemisphere than in the left hemisphere. We conclude that recurrent clinically silent focal EEG seizures in this neonate were associated with lateralizing near-infrared spectroscopy changes suggestive of relative cerebral hypoxia.
Preferential involvement of certain central nervous systems areas by specific viruses provides a valuable guide to the selection of antiviral agents. We report a neonate that developed opsoclonus 7 days prior to the diagnosis of herpes simplex type 2 cerebellitis. The course of the opsoclonus paralleled the clinical course and radiologic evolution of the infection. The purpose of this report is to describe opsoclonus as a possible early sign of herpes simplex type 2 central nervous system infection.
High levels of methemoglobinemia can cause tissue hypoxia and cyanosis. We report the case of a 7-month-old girl with pulmonary dysplasia receiving inhaled nitric oxide who had cyanosis caused by methemoglobinemia after prolonged use of a eutectic mixture of local anesthetics cream.
The association of cervicothoracic extradural arachnoid cysts and obstetric brachial plexus palsy has not previously been reported. We report two patients with this association. The first patient is a 9-month-old boy with left obstetric brachial plexus palsy that developed bilateral leg weakness at 6 months of age owing to compression of the spinal cord by a C6 to T8 left cervicothoracic extradural arachnoid cyst. The second patient is a 3-year-old girl with bilateral brachial plexus palsy and spastic paraparesis who had magnetic resonance imaging at 3 days of age that showed intraspinal cord injury and a cervicothoracic extradural arachnoid cyst compressing the spinal cord. We believe that the association of cervicothoracic epidural arachnoid cysts and obstetric brachial plexus palsy in these patients was causal and recommend that the possibility of a cervicothoracic epidural arachnoid cyst be considered in patients with brachial plexus palsy and evidence of spinal cord injury.
The muscular dystrophies (MDs) are inherited or de novo gene mutation disorders of sarcolemma-associated proteins or nuclear membrane-associated proteins. They are manifested by progressive striated muscle weakness and wasting. Cardiac and central nervous system abnormalities may be present. MD phenotypes vary in terms of defective gene, mode of inheritance, age of onset, distribution of muscle involvement, and severity. Clinical diagnosis is confirmed by direct or indirect mutation analysis and gene product defect detection in frozen muscle samples by immunohistochemistry and Western blot. The majority of the adolescents with MD are already aware of the nature and course of their condition, which adds a significant psychosocial pathology to their physical disability. Management includes: (1). prevention of new cases by preconception counseling, prenatal testing, and neonatal screening; (2). prevention and treatment of skeletal deformities, cardiac and respiratory insufficiencies, and psychological and psychiatric disturbances; and (3). education, counseling, and support to patients, parents, siblings, caretakers, and teachers. Management is better achieved with a multidisciplinary approach. Although there is no cure, oral steroids may preserve transitorily acceptable muscle function. Despite the initial failure with myoblast transfer therapy, advances in molecular genetic and stem cell implants are promising.