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A 10-year-old boy had a sore throat, followed in 4 weeks by acute rheumatic fever and in 6 weeks by atlanto-axial dislocation. Reduction of the dislocation by means of a halo vest relieved his pain, but the cervical spine remained unstable after 3 months of immobilization and required an occiput-C1-2 fusion and rib graft to stabilize the atlanto-axial joint. This is the eighth reported case of atlanto-axial dislocation associated with acute rheumatic fever. The features of previous cases are summarized and the clinical aspects, mechanisms, diagnosis, and treatment of atlanto-axial dislocation are reviewed.

A 10-year-old girl, one of three affected sisters, with non-ketotic hyperglycinaemia is described. In contrast to other reported cases, the course of the disorder was comparatively mild in this family. The only clinical signs were mental retardation and abnormalities in the EEG; blood glycine levels were 2-3 times normal. In the propositus, the formation of 14CO2 from glycine-1-14C and of FH414CH2OH from glycine-2-14C were impaired, shown by the decreased 14CO2 content of expired air and diminished labelling of carbon 3 of serine. However, the biochemical defect was no less than that seen in patients with much more severe clinical effects.

The water content of the sural nerve of diabetic patients was quantitatively defined by magnetic resonance proton imaging as a putative reflection of activity of the aldose-reductase pathway. Thirty-nine patients were evaluated, comparing group A, symptomatic diabetic men with sensory neuropathy; group B, similarly symptomatic diabetic men treated with aldose-reductase inhibition; group C, neurologically asymptomatic diabetic men; and group D, control nondiabetic men. Marked increase in hydration of the sural nerve was seen in more than half of the symptomatic diabetic patients. Two of 11 neurologically asymptomatic diabetics had increased nerve hydration, suggesting a presymptomatic alteration of the nerve. Symptomatic diabetics treated with aldose-reductase inhibitors had normal nerve water levels. Increased level of peripheral nerve water represents a new finding in diabetes mellitus. It seems to be related to aldose-reductase activity, involved in the development of neuropathy, and similar to events that occur in other target tissue in human diabetes.

The disorders of ocular motility seen in association with brainstem or cerebellar disorders may point to rather specific anatomical or pathological correlations. Pontine gaze palsy reflects involvement of the pontine paramedian reticular formation. Internuclear ophthalmoplegia signifies a lesion in the medial longitudinal fasciculus. Skew deviation may result from a lesion anywhere in the posterior fossa. Ocular bobbing typically results from a pontine lesion. The Sylvian aqueduct syndrome is characteristic of involvement in the upper midbrain-pretectal region, usually a pinealoma. Cerebellar lesions may be manifested by gaze paresis, skew deviation, disturbances of saccadic or smooth pursuit movements, ocular myoclonus, or several characteristic forms of nystagmus. Familiarity with these disorders may be of great help to the physician dealing with a patient with a possible posterior fossa lesion.

A 1.1-kb region of DNA containing the p26 gene of the multicapsid nuclear polyhedrosis virus of Orgyia pseudotsugata (OpMNPV) was sequenced, transcriptionally mapped, and compared to the same region in the MNPV of Autographa californica (AcMNPV). The mRNA start site of the p26 gene occurs about 22 nucleotides downstream from an A/T-rich putative promoter sequence that is highly conserved between AcMNPV and OpMNPV. The p26 mRNA is transcribed through the p26 gene and coterminates with the p10 gene resulting in a mRNA containing copies of both genes. The reading frames of the OpMNPV and AcMNPV p26 genes showed 47% amino acid sequence homology which is clustered in six regions with over 65% amino acid homology. There was a distinct bias toward incorporation of G/C-rich codons in the OpMNPV p26 gene. No DNA homology was observed between the region upstream of the p26 gene in AcMNPV and OpMNPV. In AcMNPV, this region contains the homologous repeated (hr) sequence hr5. Hybridization of a plasmid containing an AcMNPV-repeated sequence (hr5) to Southern blots of the OpMNPV genome indicated that this repeated sequence is lacking in OpMNPV.

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