Kevin Gordon

Melatonin Appears Ineffective in Children With Intellectual Deficits and Fragmented Sleep: Six "N of 1" Trials

9. Starkman SP, Brown TC, Linell EA: Cerebral arachnoid cysts. J Neuropathol Exp Neurol 1958; 17:484-500. 10. Klein TE, Bernard EM, Gold JWM, Armstrong D: Candidiasis, detection by gas-liquid chromatography of D-arabinitol, a fungal metabolite, in human serum. Science 1979;206:577-580. 11. Wong B, Brauer KL: Entantioselective measurement of fungal D-arabinitol in the sera of normal adults and patients with candidiasis. J Clin Microbiol 1988;26:1670-1674.

Duchenne Muscular Dystrophy—Parental Perceptions

Quality of life and availability of services are important for boys with Duchenne muscular dystrophy (DMD) and their families. Families attending our neuromuscular clinic completed a questionnaire on parental perception regarding the importance of services, health issues, and quality of life issues both “now” and “in the future.” Eighty-nine percent of the families (31/35) completed questionnaires. Services and health issues related to prolonging ambulation were most important, especially for the parents of younger boys. Mental health issues such as social isolation, anger, and depression were very important, particularly for the families of older boys and were anticipated to be more important in the future. Pediatricians should be aware of both the immediate needs of families to meet the physical and emotional challenges of DMD and the increasing requirement to address the social needs of these patients and their families as the boys become older.

Self‐Reported Headache Frequency in Canadian Adolescents: Validation and Follow‐Up

Objective.—To validate our previous estimates of the prevalence of frequent headache and associated factors in a new sample of 12‐ to 13‐year‐old adolescent Canadians, and to explore if estimates of the prevalence of frequent headache and associated factors remain stable within the original cohort after a two‐year interval.

Pseudo-tics in Tourette Syndrome

We describe two patients with Tourette syndrome who also had pseudo-tics. The psychogenic movements resolved in both children when the stressful issues in their lives were addressed. It is important to differentiate psychogenic symptoms from tics and compulsions in children with Tourette syndrome in order to avoid unnecessary medication and allow appropriate therapy. (J Child Neurol 1994;9:50-51).

Families are content to discontinue antiepileptic drugs at different risks than their physicians

Summary: Purpose: To define the risk of seizure recurrence (RSR) that families and physicians would accept before discontinuing antiepileptic drugs (AEDs) for children with controlled epilepsy.

Tay-Sachs disease: B1 variant

This first child of non-Jewish parents had nystagmus at 4 months of age, bilateral cherry-red macular spots at 7 months of age, and hyperacusis at 8 months of age; the patient has deteriorated progressively following a clinical course typical of Tay-Sachs disease B variant. Total beta-N-acetylhexosaminidase assayed with 4-methylumbelliferyl-beta-glucosamine (4 MU GlcNAc) as substrate was within the normal range in plasma and cultured dermal fibroblasts and 2/3 the normal mean in leukocytes. The hexosaminidase A activity, assayed with the same substrate in plasma and cultured fibroblasts, approximated Tay-Sachs disease heterozygote levels; however, the activity of hexosaminidase A assayed with 4 MU Glc NAc-6-sulfate in the plasma, leukocytes, and cultured fibroblasts was less than 8, 2, and 1%, respectively of the control mean. This female infant with the B1 variant of Tay-Sachs disease demonstrated an earlier onset and more rapidly progressive course than was observed in 4 of the 5 previously reported patients with this Tay-Sachs disease variant.

Benign Familial Neonatal Convulsions Are Epileptic

An infant with benign familial neonatal convulsions fortuitously had a clinical seizure during a routine EEG. The seizures had started on day 2 of life, and the EEG recording was performed on day 6. The EEG, although not complete, did show a simultaneous electrographic seizure. Our finding establishes that benign familial neonatal convulsions are indeed epileptic. (J Child Neurol 1991;6:340-342).

Severe Polymorphic Epilepsy of Infancy

4. Hasuo T, Tamura K, Yasumori K, et al: Computed tomography and angiography in MELAS: Report of three cases. Neuroradiology 1987;29:393-397. 5. Galen NB, Lawrence AL: Massive focal swelling as a feature of MELAS. Pediatr Neurol 1988;4:366-369. 6. Suresh K, Patti LP, Margaret EM, et al: Impaired NADH-COQ reductase activity in a child with moyamoya syndrome. Pediatr Neurol 1988;4:241-244. 7. Forster BC, Hubner G, Muller-Hocker J, et al: Mitochondrial angiopathy in a family with MELAS. Neuropediatrics 1992;23: 165-168.