Israel Alfonso

Focal upper extremity neuropathy in neonates

Focal upper extremity neuropathies are common in neonates. The brachial plexus is the most common site involved. Brachial plexus injuries may involve different structures, thus producing different clinical presentations: complete brachial plexus palsy, Duchenne-Erb palsy, upper-middle trunk brachial plexus palsy, Klumpke palsy, fascicular brachial plexus palsy, and bilateral brachial plexus palsy. The causes of brachial plexus palsy are obstetric injury, intrauterine compression, humeral osteomyelitis, hemangioma, exostosis of the first rib, neck compression, and neoplasm. The differential diagnosis of brachial plexus palsy includes pseudoparesis, amyoplasia congenita, congenita varicella syndrome, and neurological lesions at other neuroanatomical levels. The cause and the degree of injury dictate the prognosis. The prognosis of obstetric brachial plexus injury is usually good.

Spinal cord involvement in encephalocraniocutaneous lipomatosis

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.

Outcome after later combined brachial plexus and shoulder surgery after birth trauma

Of 22 infants aged between 11 and 29 months who underwent a combined reconstruction of the upper brachial plexus and shoulder for the sequelae of a birth injury, 19 were followed up for two or more years. The results were evaluated using a modified Gilbert scale. Three patients required a secondary procedure before follow-up. Three patients had a persistent minor internal rotation contracture. All improved by at least two grades on a modified Gilbert scale.

Cervicothoracic Extradural Arachnoid Cyst: Possible Association With Obstetric Brachial Plexus Palsy

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. (J Child Neurol 2002;17:770—772).

Intravenous Valproate Dosing in Neonates

The loading dosage of intravenous valproate required to achieve a desired serum concentration in neonates is not known. Two neonates with seizures received loading doses of intravenous valproate over 30 minutes. Serum valproate concentrations were measured 45 minutes and 3 hours after initiation of the infusion. Both neonates had received phenobarbital and phenytoin before the loading infusions. In the first patient, a loading dose of intravenous valproate of 10 mg/kg increased the 45-minute postinfusion serum valproate concentration to 41 μg/mL with a 3-hour postinfusion serum valproate concentration of 33 μg/mL. In the second patient, a loading dose of 25 mg/kg increased the 45-minute postinfusion serum valproic acid concentration to 100 μg/mL with a 3-hour postinfusion serum valproic acid concentration of 78 μg/mL. We found that each 1 mg/kg of intravenous valproate increased the 45-minute and 3-hour postinfusion serum valproic acid concentrations by approximately 4 μg/mL and 3 μg/mL, respectively. We suggest that these figures be used to calculate the desirable loading dose of intravenous valproate in neonates until larger studies are conducted. The volume of distribution and the serum clearance of valproate were approximately 0.245 L/kg and 25 mL/h/kg, respectively. (J Child Neurol 2000;15:827-829).

Neoplasm as a cause of brachial plexus palsy in neonates

Two patients with neonatal onset of arm weakness resulting from neoplastic involvement of the brachial plexus who were initially considered to have obstetric brachial plexus palsies are reported. The first patient was a 7-day-old female who presented with a left supraclavicular mass that was first detected at 2 days of age and left proximal arm weakness. The weakness involved the whole arm within 3 days. The mass was a malignant rhabdoid tumor. The second patient was a 28-month-old male who presented with slowly progressive right arm weakness, which began at 3 weeks of age, and episodes of scratch marks on the arm that began at 4 months of age. Magnetic resonance imaging revealed a plexiform neurofibroma of the brachial plexus. The features that are suggestive of a brachial plexus palsy caused by a neoplasm rather than of obstetric brachial plexus palsy include the following: the onset of weakness after the first day of age, with a progressive course; a history of a normal delivery and birth weight; the absence of signs of a traumatic injury or injuries; the appearance before 7 days of age of a growing supraclavicular mass without radiographic evidence of a clavicular fracture; and recurrent scratch marks on the weak arm.

Neurophysiological evaluation of children with traumatic radiculopathy, plexopathy, and peripheral neuropathy.

Neurophysiological evaluation of children with traumatic peripheral nervous system injury is accomplished with electromyography, motor and sensory nerve conduction studies, and somatosensory and motor-evoked potentials. Preoperative neurophysiological tests are performed if motor deficits persist for more than 3 months. Evidence of reinnervation on these neurophysiological tests predates clinical recovery by weeks, hence they help determine the site of the lesion and provide objective measures for selecting candidates for surgical exploration. Intraoperative neurophysiological tests help to identify and confirm the integrity of nerves and to develop the optimal surgical strategy. Postoperative evaluations every 3 to 6 months may determine efficacy of treatment.

Hand function in children with an upper brachial plexus birth injury: results of the nine-hole peg test.

Aim  The aim of this study was to evaluate hand function in children with Erb upper brachial plexus palsy.

Hemispherectomy in Early Infantile Epileptic Encephalopathy

The authors report the successful surgical treatment of early infantile epileptic encephalopathy and suppression burst in 2 children with hemispheric dysplasia. At surgical evaluation, focal epileptic patterns were recorded from the abnormal hemisphere, and ictal single-photon emission computed tomography demonstrated ipsilateral regional hyperperfusion. Functional hemispherectomy was performed after electrocorticography revealed near-continuous spike and slow-wave discharges over the affected hemisphere. Histopathology revealed Taylor-type cortical dysplasia. Postoperatively, both patients are seizure free on antiepileptic drug monotherapy and have shown improved developmental status. Their favorable outcome demonstrates that children with early infantile epileptic encephalopathy due to unilateral malformations of cortical development may benefit from early surgical intervention.

Similar brain spect findings in subclinical and clinical seizures in two neonates with hemimegalencephaly

Brain single-photon emission computed tomography (SPECT) findings during clinical and subclinical seizures were compared in two neonates with hemimegalencephaly. Interictal and ictal brain SPECT were performed in two neonates. The ictal studies were performed during a clinical seizure in one neonate and during a subclinical seizure in another neonate. They revealed similar focal hemispheric hyperperfusion at the electroencephalographic seizure foci in both cases. The similar perfusion patterns imply that clinical and subclinical seizures place similar metabolic demands on the cerebral tissue involved in the generation of electroencephalographic seizures in neonates with cerebral dysgenesis and suggest that clinical and subclinical seizures should be treated similarly in this population.