Idiopathic REM Sleep Behavior Disorder in Young Adults and Quantitative Analysis of Polysomnography

Rapid eye movement sleep behavior disorder (RBD) is a sleep disorder characterized by loss of muscle atonia during REM sleep associated with dream enactment, which usually start at the age of older than 50. RBD in elders are known to be developing symptoms of neurodegenerative disorders in the course of disease. However, the pathophysiology and prognosis of "early-onset" idiopathic RBD is unclear. Several existing standard value about REM sleep without atonia (RSWA) of RBD patients was established with "late-onset" RBD for those diagnosed at age 50 and greater, RSWA metrics in "early-onset" RBD patients diagnosed prior to age 50 years have not been previously described. Therefore, we report here two patients who were diagnosed as "early-onset" idiopathic RBD, and performed quantitative RSWA scoring. We suggested that "early-onset" idiopathic RBD has different etiologies the various characteristics of RSWA than adults with RBD.

Neurosyphilis Presenting with Multiple Cranial Neuropathy

Neurosyphilis can present with various clinical symptoms. We report a patient with multiple cranial neuropathy that, mimicked tuberculosis, but was finally diagnosed as neurosyphilis. A 38-year-old man was admitted due to subacutely progressive hearing loss. Brain MRI revealed multiple dural enhanced masses on contrast-enhanced T1 weighted images. Brain biopsy of the dural enhanced lesion revealed chronic granulomatous inflammation with coagulation necrosis, compatible with syphilitic gumma. Serum venereal disease research laboratory and fluorescent treponemal antibody absorption test results were positive. Treatment with penicillin resolved the patient's symptom.

Nationwide surveillance of acute interstitial pneumonia in Korea / 소아과

PURPOSE: Acute interstitial pneumonia (AIP) is a rare disease, but its prognosis is fatal because of lack of efficient treatment modality. Recently, it has been reported that there was epidemic AIP in Korea. This study aims to investigate the past and current status of AIP in Korea. METHODS: We performed a nationwide survey and a prospective study. From August 6 to 15, 2008, a questionnaire survey was conducted to identify the prevalence, local distribution, and response to current treatments. The questionnaire was answered by pediatrician working in 23 referral centers in Korea. In addition, 5 referral centers in Seoul performed a preliminary prospective observational study by obtaining clinical data and specimens from appropriate patients. The Korea Centers for Disease Control and Prevention analyzed the samples for possible pathogens. RESULTS: The survey showed 78 AIP cases had occurred and 36 patients had died. Lung biopsy was performed only on 20 patients. In 2008, 9 AIP cases developed. In a prospective study, 9 (M:F=5:4) patients developed AIP in spring and 7 (78%) died, with the mean rate of death occurring 46 days after diagnosis. Human corona virus 229 E, cytomegalovirus, influenza A virus, influenza B virus, and parainfluenza virus were isolated from the respiratory specimens. CONCLUSION: This study showed nationwide prevalence of AIP in Korea. In addition, because of the high mortality rate and rapid progress, pediatricians need to be aware of the disease. Further studies and a nationwide network are required for reducing the morbidity and mortality rates related to AIP.

Neonatal Hearing Screening in Neonatal Intensive Care Unit Graduate

PURPOSE: Hearing loss is one of the common birth defects in humans, with a reported prevalence of 1-3 per 1000 newborns. We investigated the incidence of hearing loss and evaluated the use of neonatal hearing screening test in neonatal intensive care unit (NICU) graduates who are at greater risk for hearing loss than normal newborns. METHODS: The neonates admitted to the NICU of Asan Medical Center from 1 March, 2003 to 30 March, 2008 who were available for follow-up were included. Those who failed the first auditory brainstem response prior to discharge were retested with the stapedial reflex test, auditory brainstem response and tympanometry in the Otolaryngology department. RESULTS: Of 2,137 neonates, 2,000 (93.5%) neonates were tested prior to discharge. Sixty-seven neonates (3.4%) failed the first newborn hearing screening test. Of 67 infants, 52 infants were retested for a second hearing test. Excluding 10 infants (19.2%) who were lost during follow-up, 16 infants were confirmed to have hearing impairment of which 12 and 4 infants had unilateral and bilateral hearing loss, respectively. Of 16 infants, 5 did not meet the criteria set by the Korean National Health Insurance Corporation. CONCLUSION: The prevalence of hearing impairment in NICU graduates is about 0.8%, excluding those who were lost for follow up, necessitating a systemic and effective hearing assessment program among these high risk infants and more generous national insurance coverage.