Posterior pituitary ectopy in children with idiopathic growth hormone deficiency.

AIMS: To evaluate the underlying pathogenesis in children with pituitary hormone deficiency by means of high resolution MRI of the brain. PATIENTS/METHODS: Thirty-seven children with short stature and isolated GH deficiency (IGHD, n = 17) or multiple pituitary hormone deficiency (MPHD, n = 20) were subjected to an MRI of the brain at the age of 1.0-17.3 years. The anatomic condition of the hypothalamo-pituitary area was analyzed and the height of the pituitary gland was measured and compared to the data of age-matched healthy subjects. RESULTS: Seventy percent of the patients had a characteristic anomaly: the adenohypophysis was hypoplastic, the infundibulum was absent and the posterior pituitary lobe was ectopic at the bottom of the median eminence. The height of the anterior pituitary was significantly reduced in these patients (1.9 +/- 0.1 mm; mean +/- SD) when compared to age-matched healthy controls (4.1 +/- 0.8 mm, p<0.001) or hypopituitary patients with a normal MRI (4.3 +/- 0.8 mm). MPHD was found in 62% of patients with the pituitary anomaly whereas only 27% of children with a normal MRI had MPHD (p<0.05). CONCLUSIONS: The pathogenesis of the pituitary anomaly is unknown; a disorder during embryonal development or perinatal events have been discussed as causal factors. MRI should have a prominent position in the work-up of hypopituitary children. When an anatomical malformation is visualized by MRI, the diagnostic terminology should be adapted accordingly.

Standardized assessment instruments for minimally-responsive, brain-injured patients.

Among the most significant advances in the care and rehabilitation of severely brain injured, minimally-responsive patients (MRP) has been the development of standardized assessment scales. Currently available instruments include the Coma/Near Coma Scale (CNC), Coma Recovery Scale (CRS), Sensory Stimulation Assessment Measure (SSAM), and the Western Neuro Sensory Stimulation Profile (WNSSP). Each scale is reviewed in terms of content, psychometric properties, and clinical attributes. Data is then presented comparing converted, percentile admission scores for the CRS, WNSSP, and CNC in a group of ten MRP at Rancho Levels II-IV, with a mean age of 31 years and mean time from injury of 37.5 days. Admission CNC and CRS scores tended to group in the middle range, while WNSSP scores tended to group in the lower quartile. This suggests the potential concern for 'floor effect' with the WNSSP. At admission, none of the three scales was able to effectively distinguish between dichotomized outcome variables: disposition (home vs. nursing home), advancement to active rehabilitation, or discharge Functional Independence Measure score (> 80 vs. < 80). The data indicate the CRS demonstrates moderately strong relationships with both the CNC and WNSSP. Full delineation of how these scales relate to one another awaits both cross-sectional and longitudinal analyses in larger samples and should include the SSAM.