Biomedical subjects
Publications and source records attributed to F W Newell.
Linear bead-like pigmentation in choroidal folds and diffuse nonspecific retinal pigment epithelial proliferation are seen in long-study choroidal folds. In addition there may be linear streaks of scleral staining.
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Forty-four patients with periorbital cellulitis or abscess were admitted to the hospital for treatment in a five-year period. Twenty-five patients were less than 5 years old; nine were more than 20 years old. Skin infection or trauma, upper respiratory tract infection, and sinusitis were the most common predisposing causes. Staphylococcus aureus was cultured in 13 patients and Haemophilus influenzae in nine patients. Ampicillin and methicillin were the chief antibiotics used. Ten patients required surgical drainage.
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External ophthalmoplegia, retinal pigmentary degeneration, and heart block constitute the Kearns-Sayre syndrome. Skeletal muscle weakness, deafness, ataxia and endocrine disturbances also may occur. We examined 15 members in two generations of a family with autosomal dominant Kearns-Sayre syndrome. Seven had external ophthalmoplegia, six had electrocardiographic abnormalities, six had limb weakness nad six patients were normal. A deltoid muscle biopsy specimen from one patient contained typical "ragged-red fibers," abnormal lipid accumulation, and mitochondria increased in size and number, containing inclusions. The study demonstrated: (1) the marked variability in genetic expression; (2) the need to examine family members to discover asymptomatic patients and to establish an otherwise unrecognized hereditary pattern; and (3) the absence of specific and consistent biochemical abnormalities.
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Four patients in three different families had a form of autosomal recessive incomplete achromatopsia not previously described. The visual acuity was 6/18 to 6/60 (20/60 to 20/200) with minimal ophthalmoscopic abnormality and normal fluorescein angiogram. The photopic electroretinographic responses were present in all four patients; the fusion rate of 60 Hz was only slightly subnormal. The high-intensity scotopic response was subnormal. The patients failed color screening plates and accumulated over 400 errors with scotopic axis on the Farnsworth-Munsell 100-hue test. The Rayleigh match was abnormal, displaced toward the red primary, but with normal luminance. The photopic luminous efficiency function was similar to that of the deuteranope. Color matching revealed a trichromatic form of color vision mediated by long wavelength and short wavelength cones, and a rhodopsin receptor.
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Dizygotic twins developed a progressive neurologic disorder at age 6 months. When examined at age 7 1/2 years each had spastic quadriparesis and dystonia. Neither had ever spoken a complete sentence. The fundi showed bone spicule formation, a conspicuous choroidal circulation, and a striking accumulation of yellowish-white globular masses of varying sizes and shapes. Because our patients developed both the pigmentary degeneration and clinical signs of Hallervorden-Spatz syndrome at a much younger age than patients without retinopathy, we believe this case demonstrated a distinct nosologic entity.
Nabilone is a synthesized crystalline benzopyran that resembles the cannabinols but is not a tetrahydrocannabinol. Oral administration of 0.5 to 2 mg to patients with open-angle glaucoma reduced the intraocular pressure from 10% to 54% with an average of 28%. Administration of a topical ophthalmic nabilone solution (0.1 mg/drop) to both eyes of adult albino rabbits (1.5 to 3.5 kg) lowered the IOP an average of 25%. The peak action of the nabilone administered in this manner was 60 minutes, with a return to normal IOP by 180 minutes. Tolerance developed in rabbits after one week of topical administration.
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