Therapeutic hypothermia with the use of intracranial pressure monitoring for acute disseminated encephalomyelitis with brainstem lesion: a case report.

Acute disseminated encephalomyelitis confined to the brainstem is associated with poor prognosis. We describe a case of a 10-year-old boy with acute disseminated encephalomyelitis in the brainstem that developed after influenza A infection. A 10-year-old boy presented with fever and prolonged disturbance of consciousness and was admitted to our hospital. Magnetic resonance imaging (MRI) of the midbrain, with T2-weighted and fluid-attenuated inversion recovery images, suggested acute disseminated encephalomyelitis accompanied by a brainstem lesion. Lumbar puncture showed pleocytosis and increased protein content, including myelin basic protein, interleukin-6, and immunoglobulin G, all suggestive of acute disseminated encephalomyelitis. Treatments such as methylprednisolone pulse therapy, intravenous immunoglobulin, and therapeutic hypothermia were performed. Although the patient presented with anisocoria with increased intracranial pressure monitoring during hypothermia, prompt therapy with d-mannitol and dopamine was effective. Our case results suggest that hypothermia could be included in the choice of therapy for acute disseminated encephalomyelitis with brainstem lesions.

Assessment of contractility and myocardial function in small and appropriate for gestational age premature neonates using the stress-velocity relationship and tissue Doppler imaging immediately after birth.

Our aim was to determine whether small for gestational age (SGA) neonates exhibit reduced left ventricular contractility and diastolic dysfunction, through the assessment of tissue by Doppler imaging and investigation of the stress-velocity relationship (SVR). The study subjects were limited to 37 neonates just after birth. These patients were grouped into 14 SGA neonates with birth weights below the 10th percentile (aged 29-36 weeks, birth weight 860-2066 g) and 23 appropriate for age (AGA) neonates with birth weights between the 10th-90th percentile (aged 20-36 weeks, birth weight 954-2945 g). Although there were no significant differences in the SVR or diastolic function between the SGA and AGA neonates, wall stress correlated significantly and inversely with birth weight in all subjects with a higher E/Ew ratio by tissue Doppler imaging. In conclusion, there were no significant differences in cardiac function between SGA and AGA neonates. By contrast, the relationship between wall stress, diastolic dysfunction, and birth weight may be due to physiological alterations in neonates, including those with low birth weights, just after birth.

Systemic inflammatory response syndrome and prolonged hypoperfusion lesions in an infant with respiratory syncytial virus encephalopathy.

Respiratory syncytial virus (RSV) is a cause of neurological complications in infants. We report a rare case of RSV encephalopathy in an infant who presented with poor sucking and hypothermia at 17 days of age after suffering from rhinorrhea and a cough for several days. After hospitalization, the patient presented with stupor and hypotonia lasting for at least 24 h, and was intubated, sedated, and ventilated for treatment of pneumonia. These symptoms led to diagnosis of pediatric systemic inflammatory response syndrome (SIRS) caused by RSV infection. High-dose steroid therapy was combined with artificial ventilation because the initial ventilation therapy was ineffective. Interleukin (IL)-6 levels in spinal fluid were markedly increased upon admission, and serum IL-6 and IL-8 levels showed even greater elevation. The patient was diagnosed with RSV encephalopathy. On day 5, high signal intensity in the bilateral hippocampus was observed on diffusion-weighted magnetic resonance imaging (MRI). On day 14, the patient presented with delayed partial seizure and an electroencephalogram showed occasional unilateral spikes in the parietal area, but the hippocampal abnormality had improved to normal on MRI. (99m)Tc-labeled ethylcysteinate dimer single-photon emission computed tomography (SPECT) on day 18 showed hypoperfusion of the bilateral frontal and parietal regions and the unilateral temporal region. SPECT at 3 months after onset still showed hypoperfusion of the bilateral frontal region and unilateral temporal region, but hypoperfusion of the bilateral parietal region had improved. The patient has no neurological deficit at 6 months. These findings suggest that RSV encephalopathy with cytokine storm induces several symptoms and complications, including SIRS and prolonged brain hypoperfusion on SPECT.

Relationship between Aortic Intima-media Thickening, Serum IGF-I and Low-density Lipoprotein Particle Diameter in Newborns with Intrauterine Growth Restriction.

Much epidemiological evidence has linked low birth weight with late cardiovascular risk. In order to investigate the effect of intrauterine growth restriction (IUGR) on early atherosclerosis in the fetus, we measured aortic wall thickness (abdominal aortic intima-media thickness: aIMT) by ultrasonography in 15 neonates with IUGR and in 31 neonates considered to be appropriate for gestational age (AGA). Furthermore, we evaluated the relationship between aIMT, serum insulin-like growth factor-I (IGF-I) and low-density lipoprotein (LDL) particle size to investigate the possible effect of these atherosclerosis-related factors on the early atherosclerosis process. The results showed that the mean aIMT was significantly greater in the IUGR neonates than in the AGA neonates (least squares mean ± SE, 537 ± 24.8 vs. 471 ± 17.0 µm, p=0.037). The serum IGF-I levels were lower in the IUGR neonates than in the AGA neonates (27.9 ± 4.3 vs. 42.7 ± 2.9 ng/ml, p=0.009). A significant negative correlation was observed between aIMT and IGF-I in the IUGR neonates (r=-0.646, p=0.009); however, a positive correlation was observed between aIMT and IGF-I (r=0.416, p=0.020) in the AGA neonates. There appeared to be no relationship between aIMT and LDL particle diameter. Atherogenic small, dense LDL was not detected in the IUGR infants. In conclusion, neonates with IUGR have significant aortic thickening with decreased IGF-I, suggesting that prenatal events might predispose them to later cardiovascular risk.

[Long-term remission in an acute monoblastic leukemia patient with down syndrome after cord blood transplantation].

A 4-year-old boy with Down syndrome (DS) was diagnosed as having acute monoblastic leukemia (AML-M5a). Leukemic cells were CD33+, CD56+ and CD4+, with t(9;11) on cytogenetic analysis and MLL gene rearrangement. After 2 courses of induction therapy using an AML 99-Down protocol failed to obtain complete remission, the patient received cord blood transplantation from an HLA-matched donor (CBT) following a conditioning regimen comprising total body irradiation and cyclophosphamide. Only cyclosporin A was used for graft-versus-host disease prophylaxis. Stem cell transplantation may not be indicated for AML patient with DS in first remission, who display a high rate of life-threatening and fatal toxicity on therapy. This patient remained well controlled in complete remission for 4 years, representing a rare case of DS with chemotherapy-resistant AML successfully treated with a CBT.