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A. Leland Albright


The purpose of this study of infants with myelomeningoceles was to evaluate the effect of stimulation of the distal spinal cord, proximal to the neural placode, on functioning lower-extremity muscles distal to the placode.


In 13 infants with lumbosacral myelomeningoceles, the lateral spinal cord was stimulated, with a monopolar probe, between its exit from the spinal canal and the neural placode. Responses of functional muscles in the lower extremities were monitored with a neural integrity monitor.


Stimulation of the lateral spinal cord resulted in no contraction of lower-extremity muscles in 7 of 13 infants in whom no nerves exited the cord between the spinal canal and the neural placode. In those with exiting nerves in that location, stimulation of the cord resulted in the contraction of functional muscles. Distal cordectomies at the junction between the spinal cord and the neural placode, distal to any nerve roots exiting from the cord, resulted in no loss of movement.


Monopolar stimulation of the lateral spinal cord does not seem to cause contraction of functional lower-extremity muscles unless nerve roots to the lower extremities exit the cord distal to the spinal canal but before its junction with the neural placode. Muscle contraction in cases in which nerve roots exit the spinal cord between the canal and the placode may be caused by the spread of current from the monopolar stimulator.

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A. Leland Albright

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A. Leland Albright


This study was designed to evaluate the clinical and histopathological effects of long-term (3-month) intra-ventricular baclofen (IVB) infusion in beagle dogs.


Catheters were inserted stereotactically into the lateral ventricles of nine dogs and were connected to implanted Synchromed II pumps. Saline solution (control animals) and IVB (experimental animals) were infused continuously. The IVB dosages ranged from 100 to 1000 μg/day.


An IVB infusion of 135 μg/day or less throughout a 3-month period was associated with no adverse side effects in any animal. Infusion of 150 μg/day produced overt seizures in one dog and produced adverse side effects in another; a reduction in the dosage given to these animals to 135 μg/day was tolerated with no adverse effects. Infusion of IVB at a dosage of 250, 500, or 1000 μg/day caused lethal toxicity within the first 4 days of infusion. Histopathological specimens obtained at necropsy revealed no ventricular or subependymal changes in animals receiving an IVB dosage of 135 μg/day or less.


Intraventricular baclofen infusion in beagles has dose-related toxicity; however, no clinical or neurological toxicity was evidenced at clinically tolerated dosages (≤ 135 μg/day) throughout 3 months of infusion.

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A. Leland Albright

Intraventricular baclofen (IVB) infusion has been used recently to treat secondary dystonia. Techniques of catheter implantation have not been thoroughly described. The object of this study was to describe a technique developed in the past 6 years to implant intraventricular catheters for baclofen infusion. Thirty-one patients underwent endoscopic placement of intraventricular catheters for IVB, primarily patients whose anatomy made implantation of intrathecal catheters difficult or inappropriate. Using the technique described, catheters were successfully inserted into the desired ventricular location in each patient. Catheters remained in the desired location in 29 of 31 cases. Catheters can be inserted into the cerebral ventricles for infusion of baclofen. The technique described herein has been associated with few complications.

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A. Leland Albright

Object. The author conducted a retrospective review of 19 cases in which he resected thalamic tumors between 1986 and 2001.

Methods. The median age of the children was 8 years. Five different routes were used to resect the tumors; two tumors were resected via two approaches. The extent of resection was evaluated by postoperative imaging. Gross-total resections were performed in six cases and resections of greater than 90% of the tumor were conducted in 10 others; 90% or more of the tumor was resected in 84% of the cases. Seven tumors were low-grade gliomas and 12 were high grade. There was one postoperative death, and two children suffered permanent morbidity. Five of seven children with low-grade tumors are alive 2 to 12 years postoperatively; three of 11 children with high-grade tumors are alive at 2, 3, and 16 years, respectively.

Conclusions. Treatment of pediatric thalamic tumors must be individualized, with consideration given to the tumor's location, enhancement, and margins as well as the published data about the correlation between extent of resection and prognosis.

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A. Leland Albright

✓ Two children with generalized dystonia were given continuous intraventricular baclofen (IVB) infusions. The first was a 12-year-old girl with mixed dystonia and spasticity caused by methylmalonic aciduria whose spinal anatomy precluded administration of intrathecal baclofen (ITB). The second was an 11-year-old boy whose dystonia was associated with cerebral palsy and had not improved significantly in response to conventional ITB treatment. The girl improved dramatically with IVB; the boy experienced no greater improvement from IVB than from ITB. No adverse effects were noted in either child during 2 to 6 months of infusion. These are the first reports of the use of IVB in this clinical setting. The results warrant additional investigation.

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Paul A. Gardner and A. Leland Albright

✓Anterior sacral meningocele (ASM) is a relatively rare, congenital disorder. Usually it presents sporadically, but there are case reports of hereditary ASMs and evidence of a dominant mode of inheritance. In this article the authors describe a case illustrating the hereditary nature of ASM and present available literature on the disease.

The authors present the case of a 19-month-old boy in whom an ASM was diagnosed during a workup for constipation. The child's 31-year-old mother had been treated for the same condition 20 years earlier, when she had presented with back pain. These cases are discussed in the context of previous reports of similar cases.

There are several case reports in the literature in which an ASM occurred as a familial, isolated disorder (in the absence of other caudal abnormalities or syndromes). The condition is reported more commonly in women, but it is unclear whether this is a true difference in prevalence or a diagnosis or reporting bias. A review of the literature indicates an autosomal-dominant inheritance with variable penetrance and presentation.

Anterior sacral meningoceles can be hereditary. Given the potential complications of the disease if left untreated and the simplicity of screening—obtaining an abdominal radiograph and the patient's clinical history—we recommend screening of immediate family members of affected individuals. Surgical treatment is recommended if an ASM is discovered.

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A. Leland Albright and Ryland P. Byrd

✓ Gross and histological pathology of skull sutures affected by craniosynostosis was evaluated. Nineteen sutures were examined: 14 sagittal, three coronal, and two metopic. Sutures were removed en bloc, examined grossly, then sectioned perpendicular to the axis of the suture and examined microscopically. Foci of dural invagination into the sutures occurred in only four of the 19 specimens. In areas of maximum clinical abnormality, there was no microscopic evidence of the suture. The suture adjacent to the fused portion was narrowed by encroaching calvaria, and suture farther away was normal. Suture ossification was never multifocal. In infants less than 1 year old, the extent of suture fusion did not correlate with age. It is concluded that craniosynostosis is characterized by the progressive obliteration of cranial sutures by fusion of adjacent cranial bones, and that changes in the skull base angles are secondary to suture obliteration.