Movement disorder following herpes simplex encephalitis.

Two children, an eight-year-old girl and a seven-month-old boy, recovered from herpes simplex encephalitis with minimal neurological residua following acyclovir treatment. Subsequently, they experienced marked deterioration, interpreted as either recrudescent infection or a post-infectious phenomenon. Features of the deterioration included encephalopathy and hyperkinetic movement disorder. MRI studies showed extensive neocortical damage, without involvement of the basal ganglia, thalamus or subthalamic nuclei. With aggressive supportive care, both children made a slow, steady recovery over several months. This supportive care is best provided in a closely supervised interdisciplinary setting.

Holoprosencephaly and interstitial deletion of 2(p2101p2109).

We report on a girl with holoprosencephaly and a small, de novo interstitial deletion of most of band 2(p21). The similarity between the cytogenetic findings and CNS malformations in our patient and those recently reported by Münke et al. [Am J Med Genet 30:929-938, 1988] suggests a phenotypic relationship between deletion of this band and holoprosencephaly.

Plasmapheresis associated with a relapsing course in Guillain-Barré syndrome.

An 11-year-old boy with severe Guillain-Barré syndrome underwent 2 courses of 5 plasmaphereses. During each course his strength and respiratory function improved. Within 3 days of completing each course, his deficits worsened. After a prolonged plateau following the second course of plasmapheresis, he spontaneously improved and continues to recover gradually 11 months following the onset of symptoms. Plasmapheresis appeared to produce a fluctuating course in our patient. Prior to the availability of effective therapeutic intervention for Guillain-Barré syndrome, a natural course, characterized by recurrence of symptoms, predicted poorer recovery. Fluctuations related to plasmapheresis may not carry similar implications; the clinician should consider this phenomenon in assessing prognosis.

Lobar holoprosencephaly presenting as spastic diplegia.

A child with lobar holoprosencephaly presented with spastic diplegia and mild mental retardation, compounded by attention deficits and hyperactivity. His facial features were normal, and except for borderline microcephaly, there were no other predictors of holoprosencephaly. This patient represents the mild end of the spectrum of the holoprosencephaly malformation complex, which seems to be under-represented in the medical literature. Holoprosencephaly does not appear to have been described previously in association with spastic diplegia. This patient also illustrates the value of CT scans of the head of patients with neurological findings, without specific etiology.

Clinical comparison of two electronic spirometers with a water-sealed spirometer.

Two electronic spirometers which use a hot-wire anemometer to measure air flow were clinically compared with a water-sealed spirometer. The forced vital capacity (FVC), the forced expiratory volume in one second (FEV1), the FEV1/FVC%, the mean forced expiratory flow between 200 and 1,200 ml of the FVC, the mean forced expiratory flow during the middle half of the FVC, the mean forced expiratory flow between 75 and 85 percent of the FVC, and the maximum voluntary ventilation were determined for a group of 67 subjects. Techniques are described for connecting the spirometers in series to permit evaluation by human subjects or by syringe injection. High correlation coefficients generally were obtained when comparing the electronic spirometers with the water-sealed spirometer, but the actual range of percent difference was greater than 11 percent in all spirometric tests. The results indicate the need for systematic evaluation of electronic spirometers to characterize their deviation from accented standards. Frequent calibration is necessary to maintain consistent performance.