Sex-linked hereditary bilateral anophthalmos. Pathologic and radiologic correlation.

A 27-year-old man had X-linked true anophthalmos. No evidence of optic globe, nerves, or chiasm was found. Rudimentary structures suggesting optic tracts were present. Lateral geniculate nuclei were present but gliotic. Calcarine cortex was thinner but had usual lamination. The normal patches of cytochrome oxidase activity in layers II and III of visual cortex were absent.

Recurrent encephalomyelitis associated with incontinentia pigmenti.

A 4-year-old girl developed progressive obtundation following an upper respiratory tract illness. Physical, cerebrospinal fluid, computed tomographic, electroencephalographic, and evoked response findings were compatible with disseminated encephalomyelitis. Skin lesions indicative of incontinentia pigmenti were confirmed by biopsy. Transient loss of suppressor T cells was observed. Prior history revealed that at 6 months of age a similar episode of acute central nervous system deterioration had occurred. Recurrent encephalomyelitis does occur with incontinentia pigmenti. Transient loss of suppressor T cells suggests that this is an immune-mediated process.

Sources of information used in diagnosing childhood learning disabilities.

One hundred nineteen questionnaires in which board certified/eligible child neurologists were asked to rank the diagnostic importance of 8 sources of information commonly used in the assessment of children with learning disabilities were analyzed by the Wilcoxon matched-pairs signed ranks test. All pairwise comparisons of the 8 sources revealed significant differences (P less than or equal to .033) except for psychologic reports (mean rank +/- 1 S.D. = 2.09 +/- 1.14) and medical histories (2.26 +/- 1.45) which were rated equal (P = .397) and, compared to the other sources, diagnostically most useful (P less than .0001). Analysis of the 6 remaining sources demonstrated the following: teachers' reports (3.39 +/- 1.35) were deemed more helpful than the mental status examination (3.92 +/- 1.61; P = .033) and questionnaires distributed to parents and teachers (5.05 +/- 2.11) were more useful than findings on the remainder of the neurologic examination (5.81 +/- 1.46; P = .012). Social service reports (6.45 +/- 1.32) took precedence over soft signs (7.12 +/- 1.05; P = .001) which were considered the least diagnostically helpful. Analysis of variance revealed that this rank order was relatively independent of the responders' age, type of practice, years elapsed since completion of training and percentage of professional time spent examining pediatric patients. Information derived from outside sources (collective mean rank +/- 1 S.D. = 4.25 +/- 0.9) contributed significantly more to the diagnosis of learning disabilities than to information elicited directly by the examining neurologists (4.77 +/- .88; P = .002).(ABSTRACT TRUNCATED AT 250 WORDS)

Mental status examination of children with learning problems.

Questionnaires were submitted to 163 board-certified/eligible child neurologists to determine whether they employed a consistent approach to the mental status examination of children with learning problems and whether this approach appropriately emphasized the assessment of higher and related cortical functions. The responders' frequency of testing in 6 major categories of mental status function was independent of their age, sex, board-certified/eligible status, type of practice, and years elapsed since completion of training. The results of the entire study group and comparisons among demographic subgroups demonstrated a progressive decline in testing frequency with increasing complexity of mental status function. Child neurologists' approach to the mental status examination was remarkably uniform across a wide range of demographic variables. Higher and related cortical functions are tested significantly less often in children with learning problems than are other more elementary categories of mental status function; therefore, the importance of the mental status examination in this context must be questioned. It is likely that the diagnosis ascribed to a child with learning problems is based on findings other than those provided by the mental status examination.

Ontogenesis of lumbar spinal somatosensory evoked potentials after posterior tibial nerve stimulation in the rat.

Although data are available regarding lumbar spinal somatosensory evoked potentials (SSEPs) after posterior tibial nerve stimulation in the mature rat, age-related changes spanning the period of early development have not been defined. We obtained lumbar spinal SEPs after posterior tibial nerve stimulation in 6 cohorts of animals (N = 36) ranging in age from weanling (15 days) to early adulthood (110 days) by recording from needle electrodes placed in the L1-2 and L5-6 interspinous ligaments. Absolute latencies of the major wave form components at the two recording sites declined rapidly until the mid-juvenile period (36 days) and more slowly thereafter. Mean peripheral conduction velocities (+/- S.D.) increased from 11.06 (+/- 0.02) to 33.22 (+/- 3.55) m/sec and mean central conduction velocities (+/- S.D.) increased from 6.27 (+/- 1.07) to 23.64 (+/- 3.84) m/sec from 15 to 110 days respectively. The linear relationships of central and peripheral conduction velocities to both age and weight as defined by standard regression were highly significant. No sex differences were noted for peripheral velocities at all ages studied. Central velocities revealed significant sex differences at 110 days but not earlier. This study demonstrates that lumbar spinal SEPs after posterior tibial nerve stimulation undergo a predictable evolution which can be represented by a simple cable model of a lengthening myelinated pathway.