A 14-day-old neonate was transferred to our university hospital because of respiratory distress and mild disturbance of consciousness. He had no history of abnormal pregnancy or delivery, but had developed apnea at 6 days old. Thereafter, respiratory distress progressed and his condition deteriorated. On admission to our hospital, several vesicles were found on the left upper arm, and moderate hepatomegaly was also present. Herpes simplex virus (HSV) type II genome was detected from serum, spinal fluid, and bone marrow. Laboratory examinations revealed typical abnormalities of disseminated intravascular coagulation, increased levels of serum ferritin, aspartate aminotransferase, and lactate dehydrogenase. Bone marrow aspiration demonstrated activated macrophages and hemophagocytosis. Spinal tap revealed numerous mononuclear cells. Meningitis and virus-associated hemophagocytic syndrome (VAHS) due to systemic HSV type II infection were thus diagnosed. Acyclovir (60 mg/kg/day) and vidarabine were promptly administered. Dexamethasone palmitate and intravenous cyclosporine were also administered for systemic inflammation due to VAHS. Finally, these aggressive therapies rescued the patient without any sequelae. In general, neonatal systemic HSV infection is life-threatening and results in poor intact survival. Our case report suggests that not only antiviral treatment for HSV, but also anti-inflammatory treatment including steroid and cyclosporine should be considered from the early phase of neonatal systemic HSV infection.
To descr ibe the potentially ser ious adver se effects of antiepileptic dr ugs (AEDs) in individuals with severe motor and intellectual disabilities (SMID) and to improve the quality of drug treatment for these patients. Methods: The data of 99 SMID patients with epilepsy staying at an institute for mor e than 1 year were retrospectively reviewed. Each patient was taking 3.4 ± 1.6 (range, 1-8) kinds of AEDs, and clinical seizures were still prevalent in 68 patients. Results: Discontinuation or dose r eduction of AEDs amelior ated agitation and/or sleep disturbance, somnolence, vomiting, appetite loss, and stridor in the corresponding patients, and additionally resolved serious gastrointestinal or cardiorespiratory problems in three patients whose clinical courses were detailed. Patient 1 manifested aerophagia-related chronic distension of the abdomen causing mechanical ileus, requiring repeated intestinal resection. The abdominal distension improved significantly after discontinuation of acetazolamide. Patient 2 experienced progressive motor deterioration during young adulthood, with simultaneous sudden elevation in blood phenytoin level. Although motor disability persisted, resolution of hypoventilation was noted after cessation of phenytoin. Patients 3 had long-term use of carbamazepine and exhibited episodic bradycardia (heart rate as low as 25 bpm) that resolved after drug discontinuation. Conclusions: Patients with SMID sometimes fail to effectively ver balize subjective adver se effects of medications to healthcare providers. The chronic adverse effects of AEDs, including those described in this study, should be broadly evaluated for better patient management.
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