K. Wirtavuori

Aspartylglucosaminuria: radiologic course of the disease with histopathologic correlation

Twelve living patients (aged 19 months to 32 years) with aspartylglucosaminuria were examined by magnetic resonance imaging (MRI), and the magnetic resonance (MR) images of 16 healthy volunteers (aged 4 to 32 years) were used as controls. One patient was examined twice. Postmortem MRI and histopathologic analysis were done on the brains of four additional adult patients. Signal intensities determined quantitatively on T2-weighted images differed significantly between patients and controls, being higher from the white matter (P < .0002) and lower from the thalami (P < .03) in the patients. The generally increased signal intensity of the white matter was most obvious in the young patients, with many focal areas of very high signal intensity in the subcortical white matter. The subcortical white matter showed a somewhat increased signal intensity even at the age of 32 years. In two of the four postmortem MR images, the distinction between the gray and white matter was still poor. At histopathologic analysis, the basic cortical cytoarchitecture was generally preserved but most neurons contained vacuoles, which were also found in the neurons of the deep gray matter. In two of the four autopsy cases the white matter showed diffuse pallor of myelin staining and some gliosis. Thus aspartylglucosaminuria is primarily a gray-matter disease also affecting white matter by delaying myelination. (J Child Neurol 1997;12:369-375).

18q− Syndrome: Brain MRI shows poor differentiation of gray and white matter on T2‐weighted images

To study brain MRI findings in patients with 18q− syndrome and to correlate these findings with the results of the molecular breakpoint analysis.

Cisatracurium during halothane and balanced anaesthesia in children

Cisatracurium, 51W89, is one of the ten stereoisomers of Tracrium® which, unlike atracurium, has been reported to have a lack of histamine mediated cardiovascular effects at doses as high as 8×ED95 in adults. We compared the time‐course of neuromuscular effects of 80 μg·kg−1 or 100 μg·kg−1 cisatracurium during N2O‐O2‐halothane or N2O‐O2‐opioid anaesthesia, respectively, in 32 children 2–12 years old. Neuromuscular function was monitored by evoked adductor pollicis EMG. Even‐numbered patients (n=16) were allowed to obtain full spontaneous recovery of neuromuscular function and odd‐numbered patients (n=16) received neostigmine 45 μg·kg−1 together with glycopyrrolate at the time of 25% EMG recovery. Data are expressed as median with 10th to 90th percentile range. Cisatracurium had an onset time (time from administration to maximal effect) of 2.2 (1.7–3.8) or 2.3 (1.8–4.9) min, a clinical duration (time to 25% EMG recovery) of 34 (22–40) or 27 (24–33) min, and a spontaneous 25–75% recovery time (time from 25 to 75% EMG recovery) of 11 (9–13) or 11 (7–12) min during halothane or balanced anaesthesia, respectively (NS). Train‐of‐four ratio recovered to 0.70 in 2.5 (1.8–3.0) or 3.2 (2.1–4.3) min following neostigmine during halothane or balanced anaesthesia, respectively (NS). Changes in blood pressure or heart rate following cisatracurium were negligible. We regard cisatracurium as a safe and promising intermediate duration muscle relaxant the effects of which can easily be reversed with neostigmine.

The safety and efficacy of cisatracurium 0.15 mg.kg−1 during nitrous oxide–opioid anaesthesia in infants and children

We studied the neuromuscular and cardiovascular effects of a single, rapidly administered intravenous dose of cisatracurium 0.15 mg.kg−1 in 27 infants (aged 1–23 months) and 24 children (aged 2–12.5 years). After midazolam premedication, anaesthesia was induced and maintained with thiopental and alfentanil in addition to nitrous oxide in oxygen. Neuromuscular function was monitored by evoked adductor pollicis electromyography. At least 15 min after intubation, each patient received cisatracurium 0.15 mg.kg−1 over 5 s. Complete neuromuscular blockade was produced by this dose in all but one infant. The mean (SD) onset time of maximal blockade was more rapid in infants [2.0 (0.8) min] than in children [3.0 (1.2) min], p = 0.0011. The clinical duration of action of cisatracurium (recovery of evoked response to 25% of control) was significantly longer in infants [43.3 (6.2) min] than in children [36.0 (5.4) min], p < 0.0001. Once neuromuscular function started to recover, the rate of recovery was similar in both age groups. Changes in blood pressure and heart rate after the administration of cisatracurium were negligible in both age groups. Cisatracurium, at a dose of 0.15 mg.kg−1, was effective and well tolerated in infants and children.

Tracheal intubating conditions and pharmacodynamics following cisatracurium in infants and children undergoing halothane and thiopental-fentanyl anesthesia

Background:  The aims of the present study were to determine the tracheal intubating conditions, onset time, duration of action, and hemodynamic responses following the administration of cisatracurium 0.15 mg·kg−1 to infants and children.

Brain Magnetic Resonance Imaging of Siblings from Families with Two or More Children with Learning or Intellectual Disabilities and Need for Full-Time Special Education

Background: Several factors are involved in determining a childs need for special education (SE). Thus, the value of brain magnetic resonance imaging (MRI) for subjects with learning and intellectual disabilities is uncertain. Purpose: To evaluate the usefulness of MRI in the diagnostic process of siblings with learning and intellectual disabilities and need for full-time SE. Material and Methods: Altogether, 119 siblings (mean age 11.9 years) from families in which two or more children attended/had previously attended full-time SE underwent prospective brain MRI. SE grouping included three levels, from specific learning disabilities (level 1) to global intellectual disabilities (level 3). Forty-three controls (level 0, mean age 12.0 years) attended mainstream education groups. Signal intensity and structural abnormalities were analyzed, and areas of the cerebrum, posterior fossa, corpus callosum, vermis and brain stem, and diameters of the corpus callosum were measured. In analyses, all area measurements were calculated in proportion to the total inner skull area. Results: Abnormal finding in MRI was more common for siblings (n=62; 52%) in SE (58% for level 3; 49% for level 2; 35% for level 1) than for controls (n=13; 16%). The siblings showed enlarged supra- (P<0.001) and infratentorial (P=0.015) cerebrospinal fluid (CSF) spaces and mild corpus callosum abnormalities (P=0.003) compared to controls. Siblings in SE had smaller inner skull area than controls (P<0.001). Further, the relative area of the mesencephalon (P=0.027) and the diameter of the body of the corpus callosum (P=0.015) were significantly smaller than in controls. In binary logistic regression analysis, enlarged supratentorial CSF spaces increased the probability of SE (odds ratio 4.2; P=0.023). Conclusion: Subjects with learning and intellectual disabilities commonly have more MRI findings than controls. Enlarged supratentorial CSF spaces were a frequent finding in siblings in full-time SE.