Search Results

You are looking at 1 - 10 of 51 items for

  • Author or Editor: A. Leland Albright x
Clear All Modify Search
Full access

A. Leland Albright

OBJECT

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.

METHODS

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.

RESULTS

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.

CONCLUSIONS

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.

Restricted access

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.

Restricted access

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.

Restricted access

A. Leland Albright

Restricted access

A. Leland Albright

Object

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

Methods

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.

Results

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.

Conclusions

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.

Restricted access

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.

Full access

A. Leland Albright and Humphrey Okechi

Object

The purpose of this study was to evaluate the effect of distal cordectomies on motor function in the lower extremities of infants with lumbosacral myelomeningoceles.

Methods

Medical records were reviewed in 32 infants with lumbosacral myelomeningoceles who were 1 day to 7 months old, who had some lower-extremity function preoperatively, and who were treated by distal cordectomies, dividing the cord between its exit from the intact spinal canal and the neural placode. Neurological function was evaluated before and after operation by therapists who were unaware of the surgical technique.

Results

Neurological function was unchanged after operation in 29 of 32 cases. For the others, hip flexion, foot plantar flexion, and toe movement were lost in 1 case each.

Conclusions

The majority of infants treated by distal cordectomies retain preoperative motor function after operation.

Restricted access

A. Leland Albright and Peter A. Lee

✓ Five children, three girls and two boys, were treated for precocious puberty secondary to hypothalamic hamartoma by resection of the hamartoma. The patients' ages at onset of pubertal development ranged from 6 to 19 months. The hamartomas ranged in size from 6 to 10 mm; four were pedunculated, one was sessile, and all were located below the tuber cinereum. The hamartomas were excised via a right subtemporal approach, with transection at the inferior surface of the hypothalamus; two were adherent posteriorly to the basilar artery and brain stem, and the adhesions were divided. Postoperatively, three children exhibited a transient oculomotor paresis and one other child required eye-muscle surgery. The symptoms and signs of precocious puberty completely regressed postoperatively in all patients. Preoperative hormone assays of testosterone, luteinizing hormone, and follicle-stimulating hormone were within the pubertal range in all five children; postoperative assays fell to prepubertal levels. The children have been followed for 0.5 to 10.5 years (mean 5.0 years) postoperatively, without evidence of recurrence of precocious puberty. One child has begun spontaneous puberty at a normal age. It is concluded that complete resection of hypothalamic hamartomas causing precocious puberty is curative.

Restricted access

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.