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It should be noted that the spasticity is occasionally asymmetrical, and the gait may have an ataxic component This neurologic picture, in mild form and without adrenal insuf ciency, is also the manner in which the disease may present in female carriers of the gene abnormality (see below) Like adrenoleukodystrophy, AMN is typically inherited as an X-linked, male-speci c trait However, we have encountered a large family with adrenomyeloneuropathy in which males and females are both affected in a pattern that suggests dominant inheritance The VLCFAs are modestly elevated in affected individuals, and there is no evidence of cerebral involvement Moser and colleagues, using clinical and biochemical criteria, have identi ed the following subtypes of ALD: 1 A progressive degeneration of cerebral white matter in young males, often with cortical blindness the classic type, accounting for half of all cases (Fig 37-10) 2.

An intermediate form in juvenile or young adult males with cerebral and spinal involvement (5 percent of cases) 3 A progressive spinal cord tract degeneration in adult males (25 percent of cases) 4 A chronic mild, nonprogressive spastic paraparesis in heterozygous female carriers (10 percent of cases) 5 Familial instances of Addison disease without neurologic involvement in males (10 percent) 6 Possibly, in male infants, a form originating at birth (eg, Zellweger disease) Illustrating the variability in presentation among kindreds, Marsden and colleagues and, subsequently, Kobayashi and coworkers described a familial spinocerebellar syndrome, and Ohno and associates have reported a sporadic instance of adrenoleukodystrophy presenting as olivopontocerebellar atrophy Moser found cerebral forms alone in 30 percent, adrenomyeloneuropathy alone in 20 percent, and combined childhood cerebral and myelopathic forms in the remaining half.

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Female Heterozygotes Neurologic manifestations have been said to develop in up to 50 percent of female carriers, but in our experience with siblings of affected patients, the gure has been lower The onset of a spastic paraparesis tends to occur later in life, usually in the third or fourth decade, and progression tends to be slow, but an explosive onset has been reported (see Chap 44) As already mentioned, multiple sclerosis is the main consideration in differential diagnosis, particularly since 20 percent of heterozygotes have.

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Real Application Clusters was a big step forward for the Oracle database. With RAC, you could combine multiple physical database servers together to act like a single database, offering a new way to deploy and scale database servers. Automatic Storage Management, or ASM, extends a similar capability for storage. ASM was introduced with Oracle Database 10 g, and provides a way to combine multiple disks into larger logical units, reducing storage costs and simplifying management. In addition, like RAC, ASM delivers additional benefits, such as improving performance and availability and simplifying a number of key management tasks.

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