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A. Leland Albright and Susan S. Ferson

✓ Since its introduction in the late 1980s, intrathecal baclofen (ITB) therapy has become the standard treatment for severe generalized spasticity and dystonia in children. Treatment with ITB decreases spasticity in the upper and lower extremities and has been associated with improved function and decreased musculoskeletal contractures. In addition, ITB decreases generalized secondary dystonia and has been associated with improved comfort and ease of care in approximately 85% and with improved function in approximately 33% of patients. Continued effectiveness of ITB in treating spasticity has been observed for up to 17 years, and its effectiveness in treating dystonia has been observed for up to 10 years. Although ITB therapy is frequently associated with complications such as infections, catheter malfunctions, and cerebrospinal fluid leaks, the benefits of therapy appear to outweigh the risks. Additional investigation is needed to determine the effects of ITB on other movement disorders such as athetosis and chorea.

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A. Leland Albright and Susan S. Ferson

Object

The aim of this study was to evaluate the use of intraventricular baclofen (IVB) for the treatment of severe generalized secondary and heredodegenerative dystonia.

Methods

Nine children and 1 adult with severe dystonia unresponsive to multiple oral medications were treated with IVB. Intraventricular catheters were positioned endoscopically in the third ventricle.

Results

Eight of the 10 patients responded to IVB; their mean dystonia scores on the Barry-Albright dystonia scale decreased from 23 to 8. The 2 patients who did not respond had not responded to previous high doses of intrathecal baclofen. No adverse side effects related to IVB occurred. One child developed a pump infection that required pump removal, 1 developed a CSF infection that cleared after antibiotic administration, and 1 developed ventriculomegaly that required a shunt placement.

Conclusions

Intraventricular baclofen is an effective method of infusing baclofen to treat severe, generalized secondary dystonia, and, at times, heredodegenerative dystonia. The site of baclofen's activity when treating dystonia may be at the cortical level, and intraventricular infusion may result in higher baclofen concentrations over the cortex than intrathecal infusion. Additional studies are necessary to determine whether IVB is effective at lower doses than those used with intrathecal baclofen administration.

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A. Leland Albright, Susan S. Ferson and Humphrey Okechi

Object

The authors undertook this study to determine white blood cell (WBC) counts in CSF obtained from lateral ventricles and myelomeningoceles (MMCs) in infants in a developing country at the time of their initial presentation for medical evaluation.

Methods

CSF was aspirated from the lateral ventricles and from MMC sacs of 100 consecutive infants at Kijabe Hospital, Kijabe, Kenya. Peripheral blood WBC counts and CSF WBC counts were determined in the laboratory. CSF with WBC counts of 5 cells/mm3 or greater was cultured.

Results

The mean WBC count in ventricular CSF was 16 cells/mm3, with a median and mode of 0 cells/mm3. The mean WBC count of CSF in MMC sacs was 141 cells/mm3 (median 15 cells/mm3). No child had both a positive culture from ventricular CSF and a negative culture from MMC CSF. There was no correlation between age at presentation and WBC counts in the MMCs. Infants younger than 8 days old were as likely to have high WBC counts in CSF from their MMC sacs as were older children; 7 of 12 infants with 500 WBCs or more in CSF from their MMCs were younger than 8 days old. Only 5 of 58 CSF specimens from MMC sacs with 5 or more WBCs/mm3 had positive bacterial cultures, which may be a reflection of CSF specimen processing rather than of true culture negativity.

Conclusions

CSF from ventricular fluid of infants presenting with MMCs infrequently has high WBC counts, so infrequently that it does not need to be evaluated routinely. CSF in MMC sacs often has high WBC counts that suggest the presence of bacterial infection. In developing countries where culture reliability is questionable, intravenous administration of antibiotics before MMC closure for infants with high MMC WBC counts may diminish postoperative meningitis/ventriculitis.

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Grace M. Thiong’o, Susan S. Ferson and A. Leland Albright

OBJECTIVE

The objective of this study was to review treatment options for infants with hydranencephaly and to consider the pros and cons of each treatment modality.

METHODS

This paper is a review of hydranencephaly as well as a retrospective analysis evaluating the outcomes of 52 infants with hydranencephaly who were treated at the Kijabe Hospital, Kijabe, Kenya, in one of four ways: ventriculoperitoneal shunt (VPS) insertion, endoscopic choroid plexus coagulation (CPC), open choroid plexectomy (CPlx), and palliative care. The primary outcome measure was control of head size, with the aim of improving patient care. One-year mortality was a secondary outcome.

RESULTS

Of the 52 patients analyzed, 11 underwent VPS insertion, 17 CPC, 14 CPlx, and 10 were treated palliatively. Head size was controlled at the 3-month evaluation interval in 5 of 7 infants treated with VPS, 10 of 16 of those treated with CPC, 6 of 9 of those treated with CPlx, and 1 of 4 treated palliatively. The number of infants in each category with complete follow-up data that were needed to analyze change in head size was lower than the total number of patients included in each category. Mortality at 1 year of age was 9 of 11 in the VPS group, 14 of 17 in the CPC group, 6 of 14 in the CPlx group, and 7 of 10 in the palliative group.

CONCLUSIONS

Head size decreased by 1 cm or more in similar proportions (62%–71%) of infants with hydranencephaly who were treated by VPS insertion, CPC, and CPlx, and progressed in those who received palliative care. Mortality at 1 year of age was similar in infants treated by a VPS, CPC, and palliative care (70%–82%), but lower (43%) in those treated with CPlx.