John T. MacDonald

Intravenous glycerol and mannitol therapy in children with intracranial hypertension

Acute intracranial hypertension may respond to intravenous mannitol, but frequent administration can cause cerebral edema or renal problems. We evaluated the use of 20% glycerol administered intravenously as an alternative to mannitol. Intravenous glycerol and mannitol were equally effective in lowering acute elevations of intracranial pressure. The duration of effect was similar for both agents. Side effects of intravenous glycerol were related to concentration, rate, and frequency of administration. In severe encephalopathies, such as Reye syndrome, we recommend infusions of 20% glycerol or 20% mannitol at a dose of 0.5-1.0 gm per kilogram. Glycerol should be administered in 0.45% or 0.9% saline, no faster than 1.5 ml (3.3 mOsm) per minute.

Self-hypnosis and biofeedback in the management of juvenile migraine

ABSTRACTSelf-hypnosis with and without biofeedback of peripheral temperature was used successfully in treating migraine headaches of 15 children who had not responded well to previous pharmacologic management. Case histories of 3 of these children are presented. Prospective research is needed to det

Susceptibility of Pasteurella haemolytica to the bactericidal effects of serum, nasal secretions and bronchoalveolar washings from cattle

Several isolates of logarithmic-phase organisms of Pasteurella haemolytica were shown to be sensitive to an antibody and complement-mediated killing mechanism in adult bovine serum. Data suggested that the classical complement pathway was important in the induction of bactericidal activity of serum. Sera from calves after colostrum feedings (post-colostral sera) killed only 30% of the bacteria in spite of the presence of high levels of antibodies against P. haemolytica. Addition of post-colostral serum to heat-inactivated adult bovine serum decreased the bactericidal capacity of the latter. It was speculated that this inhibition may have been caused by the presence of blocking antibodies (IgA) found in the post-colostral serum. Undiluted nasal secretions collected from adult cattle were not bactericidal to P. haemolytica. The results also suggest that the bronchoalveolar washings (BAW) from vaccinated calves, in spite of having a high antibody titer, were less bactericidal to P. haemolytica than BAW from sham-vaccinated calves (71.12% vs. 83.12%). The bactericidal factor(s) present in BAW from sham-vaccinated calves was heat stable, not complement dependent, and was not related to lysozyme concentration.

Acute hemiparesis in juvenile insulin‐dependent diabetes mellitus (JIDDM)

Four episodes of acute left hemiparesis occurred in three pediatric-age, insulin-dependent diabetic patients. Each had a concomitant respiratory tract infection and headache, but no obvious signs of systemic hypoglycemia. No cerebral abnormalities were demonstrated by angiography or computerized tomography. The clinical course was benign, with slow but complete resolution of neurologic abnormalities within 8 to 24 hours of onset, and no further neurologic complaints in a 7-to 16-month follow-up.

Paroxysmal Facial Itch: A Presenting Sign of Childhood Brainstem Glioma

Two children with neurofibromatosis and a chief complaint of severe, episodic, unilateral facial itching were found to have brainstem glioma. Initial computerized tomography of the brain was thought to be normal, but the brainstem tumor was subsequently demonstrated on magnetic resonance imaging. The paroxysmal facial itching resolved in both cases after a course of radiation therapy. The pathophysiology of the attacks of facial itching is unknown but must be related to the brainstem glioma. This is the first report of paroxysmal, unilateral facial itching as a presenting sign of childhood brainstem tumor. (J Child Neurol 1988;3:189-192).

Reading acuity in albinism: Evaluation with MNREAD charts

INTRODUCTION The MNREAD reading acuity (RA) charts use continuous-text reading to measure (1) RA, ie, the smallest size of print that the patient can resolve; (2) maximum reading speed (MRS); and (3) critical print size (CPS), ie, the smallest print that the patient can read with maximum speed. This project used the MNREAD charts to evaluate reading performance in children and adults with albinism to determine the smallest print size recommended to facilitate effortless reading. METHODS A total of 63 subjects with albinism were administered the MNREAD test to determine the critical print size for effortless reading. The subjects read continuous, high-contrast text print until they could no longer discern the words. The MRS and CPS were determined with the MNREAD protocol. Presence or absence of nystagmus and strabismus was recorded. Distance visual acuity was measured with ETDRS charts. RESULTS The sample included 25 male and 38 female subjects with a median age of 16 years. Median MRS was 150 words per minute. The mean CPS was 0.84 logMAR, notably larger than the mean RA of 0.53 logMAR. CONCLUSIONS The MNREAD acuity charts can be used to determine functional reading ability and critical print size for effortless reading in individuals with albinism. The amount of enlargement in print size can be used in educational and vocational recommendations to facilitate reading.

Ophthalmoplegia as a sign of metabolic disease in the newborn

Fluctuating ophthalmoplegia in the neonatal period is unusual. We report two infants, one with branched-chain keto-aminoaciduria, the other with nonketotic hyperglycinemia, who demonstrated varying degrees of ophthalmoplegia. In one, the abnormalities of extraocular motility best correlated with elevations of serum leucine. In the other case, increased CSF glycine was implicated. Varying ophthalmoplegia during the neonatal period should alert the clinician to consider an underlying metabolic disorder.

Prolonged but Reversible Respiratory Failure in a Newborn With Prader-Willi Syndrome

A 29-week premature infant with severe central hypoventilation secondary to Prader-Willi syndrome required mechanical ventilation until 55 days of age. This disorder must be considered in the differential diagnosis of a premature infant who does not have primary lung disease but has significant hypotonia because the respiratory failure may require prolonged supportive care. (J Child Neurol 2001;16:153-154).

Neurodevelopment in Children with Albinism

OBJECTIVES To evaluate neurologic development in children with albinism. DESIGN Observational cohort series. PARTICIPANTS AND/OR CONTROLS Seventy-eight children with albinism, ages 4 to 18 years. METHODS Parents completed a developmental questionnaire and were interviewed to evaluate their child for attention deficit hyperactivity disorder (ADHD) utilizing Diagnostic and Statistical Manual IV criteria. Sixty-five children underwent neurologic evaluation of balance and fine and gross motor movements. Results were compared with age-appropriate norms. Standardized reading tests were administered to 44 children. Each of 7 neurodevelopmental parameters were compared in terms of binocular best-corrected visual acuity (BCVA) using the nonparametric Wilcoxon rank-sum test. MAIN OUTCOME MEASURES Seven neurodevelopmental parameters were measured, including onset of walking, tandem gait, repetitive finger movements, sequential finger movements, standing on one foot, hopping on one foot, and throwing a ball overhand. School performance, reading performance, and presence of ADHD were also measured. RESULTS The BCVA ranged from 20/20 to 20/800, with median of 20/150. A diagnosis of ADHD was present in 21.8% and pervasive developmental disorder was noted in three children (3.8%). No significant developmental delays were noted in the majority of children. Motor development was generally within the normal range and unaffected by severity of visual impairment. Parents reported that 82% performed at grade level in math and 74% at grade level in reading. Only 18% scored below average on standardized reading tests. CONCLUSIONS Most children with albinism have normal neurologic development despite visual impairment and increased prevalence of ADHD.

Evaluation of vision-specific quality-of-life in albinism

INTRODUCTION Human albinism is a genetic condition associated with visual impairment that affects many aspects of daily life. Office measurements of visual acuity do not necessarily reflect daily visual function and health status. This study used the National Eye Institute Visual Function Questionnaire (NEI-VFQ) to determine the effect of albinism-associated ophthalmopathy on quality of life (QOL). METHODS We administered the NEI-VFQ, which consists of 25 questions about QOL (VFQ-25) and 14 questions about well-being (VFQ-39), to 44 consenting participants with albinism older than the age of 18 years. RESULTS Nineteen male and 25 female subjects completed the study. Median age was 30.5 years (range, 18-79 years). Mean best-corrected visual acuity was 20/83 (range, 20/20 to 20/320). Forty-eight percent reported that they were currently able to drive with their condition. Participants perceived midscale problems with their general vision (median subscale score 60.0). Visual acuity correlated moderately with overall NEI-VFQ composite scores (r(s) = 0.40, p = 0.01 for VFQ-25 and r(s) = 0.36, p = 0.02 for VFQ-39). Most notable impairment was recorded for distance acuity, vision-specific mental health, and vision-specific role difficulties (VFQ-39 median subscale scores 66.7, 70.0, and 75.0, respectively). Differences by sex were insignificant. Greater ceiling effects were noted for the VFQ-25 than for the VFQ-39. CONCLUSIONS The NEI-VFQ-39 is a method to evaluate self-reported effects of vision-related QOL in albinism and may be used as a baseline for evaluating outcomes in interventional studies in these patients.