X-linked adrenoleukodystrophy presenting as neurologically pure familial spastic paraparesis.

The familial spastic paraplegias (FSPs) are heterogeneous neurologic disorders that are known to occur clinically as "pure" or "complicated" forms. Although some of the complicated FSPs have been linked to specific metabolic defects, the pure forms of this disorder remain idiopathic and are considered to be primary system degenerations. We report the case of a 28-year-old man who has evidenced a neurologically pure spastic paraparesis since age twenty-five. Consistent with this diagnosis were the findings of magnetic resonance imaging, which revealed atrophy of the thoracic spinal cord without evidence for white matter disease in the cerebrum, cerebellum, or brainstem. His 32-year-old brother has also evidenced progressive spastic paraparesis since age 30, but his case is confounded by a severe head injury at age 24 that caused a skull fracture and a focal demyelinating lesion of the right frontal lobe. Both patients have had hypogonadism, requiring treatment with testosterone, since age 20. Measurement of plasma levels of very long-chain fatty acids (VLCFA) revealed that both brothers had concentrations diagnostic of adrenoleukodystrophy; their mother had plasma VLCFA levels in the heterozygous range. We conclude that neurologically pure FSP can be an early manifestation of adrenoleukodystrophy and that levels of plasma VLCFA should be determined for all cases of FSP in which X-linked inheritance appears tenable. These considerations may have bearing on the ongoing linkage studies for these disorders.

Concentration of antibacterial agents in rat bone. A comparative study of gentamicin, ampicillin and cephaloridine.

The concentrations of Gentamicin, Ampicillin and Cephaloridine in thigh rat bone were studied. Measurements were done after a 24 h administration for 3 different times. Measurable quantities in the bone for the three antibiotics were detected. The best levels in relation with the M.I.C. of 4 microorganisms occurred in Cephaloridine (and in measurements of serum and bone (microgram/g). The concentration in the serum does not represent concentration in the bone. The most convenient parity was for Cephaloridine. The elimination of the drug in the bone was impressive after 2h. The slower fall occurred in Cephaloridine. Ampicillin had the same level in 4 and 6h. Gentamicin and Cephaloridine had failed to present measurable quantities in some of the animals of the last measurement.