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The ketogenic diet (KD) is an established, effective nonpharmacologic treatment for intractable childhood epilepsy. The KD is provided differently throughout the world, with occasionally significant variations in its administration. There exists a need for more standardized protocols and management recommendations for clinical and research use. In December 2006, The Charlie Foundation commissioned a panel comprised of 26 pediatric epileptologists and dietitians from nine countries with particular expertise using the KD. This group was created in order to create a consensus statement regarding the clinical management of the KD. Subsequently endorsed by the Practice Committee of the Child Neurology Society, this resultant manuscript addresses issues such as patient selection, pre-KD counseling and evaluation, specific dietary therapy selection, implementation, supplementation, follow-up management, adverse event monitoring, and eventual KD discontinuation. This paper highlights recommendations based on best evidence, including areas of agreement and controversy, unanswered questions, and future research.

STENOSIS of the aqueduct of Sylvius is a common cause of human hydrocephalus, occurring most often in infancy but also in childhood and in adult life. In a small proportion of cases, aqueductal narrowing occurs in a genetically determined pattern1 or complicates purulent meningitis and neoplastic disease. In most cases, however, the etiology of the stenosis cannot be determined. Recently, in rodents experimentally infected with human myxoviruses aqueductal stenosis and hydrocephalus were found to develop as delayed sequelae of the acute viral infection.2 Furthermore, by the time these abnormalities became apparent, there was no longer any evidence of infection.3 The . . .

An improved method for the determination of arylsulfatase A, based on the ether solubility of 4-nitrocatechol, has been established. The method has been applied to the diagnosis of metachromatic leukodystrophy and can identify the carrier state. The method is also useful in organ surveys for the enzyme.

Argininosuccinicaciduria is a rare genetic error of the Krebs-Henseleit urea cycle due to the absence or deficiency of the enzyme argininosuccinate lyase and is associated with mental retardation, seizures, ataxia, and hepatomegaly. Hyperammonemia may or may not be present, but blood urea levels are always within normal limits. Abnormally fragile hair has been detected in about half of the reported cases, and in some, a morphologic hair alteration termed trichorrhexis nodosa has been observed. 1 The nature of the hair defect has not been extensively studied, and tensile strength and other physical properties have been measured in only a single case, to our knowledge. 2 Amino acid analysis of acid hydrolysates of our patient's hair revealed a reduction in cystine content to 50% of the control level, concomitant with a similar degree of reduction of values for tensile strength and Young's modulus. No relationship between the urea cycle block, the

Hypokalemic, 1 hyperkalemic, 2 and normokalemic 3 types of familial periodic paralysis have been reported. We studied a woman whose clinical syndrome and serum potassium changes were compatible with the hyperkalemic type of paralysis. In contrast to most of the previously described patients, her family did not demonstrate the pedigree pattern of an autosomal dominant gene with complete or almost complete penetrance. 2 In addition, she had a low serum calcium and carbon dioxide combining power during an attack. Only one other report describes hypocalcemia during an attack. 4 Our patient may represent a heretofore unreported variant of familial periodic paralysis or, possibly a similar but new metabolic defect. Report of Case A 35-year-old, married, Caucasian female secretary complained of episodic weakness and paralysis of her legs and arms. These attacks began when she was 18 months old. A typical attack was characterized by gradual weakness of the arms and