• Journal of Korean Neurosurgical Society
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• v.39 no.6
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• pp.438-442
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• 2006

The sylvian aqueduct syndrome is a global rostral midbrain dysfunction induced by a transtentorial pressure gradient through the aquaeductus. Several months after ventriculoperitoneal shunt, a patient with hydrocephalus began experiencing a constellation of midbrain dysfunction symptoms, including bradykinesia, medial longitudinal fasciculus syndrome, third nerve palsy, and mutism. These were indicative of cerebral aqueduct syndrome. In addition, the patient showed posture-dependent underdrainage or overdrainage. All symptoms were resolved after distal catheter elongation and floating cranioplasty. We present a case of reversible parkinsonism, which developed in a patient with shunted hydrocephalus and aqueductal stenosis, and discuss the diagnosis and treatment of the sylvian aqueduct syndrome. We also review the literature to address problems of drainage and potential treatment modalities.

• Clinical and Experimental Pediatrics
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• v.56 no.7
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• pp.298-303
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• 2013

Purpose: The purpose of this study was to evaluate short-term thyroid dysfunction and related risk factors in pediatric patients who underwent hematopoietic stem cell transplantation (HSCT) during childhood. Methods: We studied 166 patients (100 boys and 66 girls) who underwent HSCT at the Catholic HSCT Center from January 2004 through December 2009. The mean age at HSCT was $10.0{\pm}4.8$ years. Thyroid function of the patients was tested before and during 3 months of HSCT. Results: Out of 166 patients, 165 (99.4%) underwent allotransplantation. Acute graft-versus-host disease (GVHD, grades II to IV) developed in 76 patients. Conditioning regimens before HSCT include total body irradiation (n=57), busulfan (n=80), and reduced intensity (n=29). Forty-five (27.1%) had thyroid dysfunction during 3 months after HSCT (29 euthyroid sick syndrome [ESS], 6 subclinical hyperthyroidism, 4 subclinical hypothyroidism, 3 hypothyroxinemia, 2 overt hyperthyroidism, and 1 high $T_4$ syndrome). In a univariate logistic regression analysis, age at HSCT (P=0.002) and acute GVHD (P=0.009) had statistically significant relationships with thyroid dysfunction during 3 months after HSCT. Also, in a univariate logistic regression analysis, ESS (P=0.014) showed a strong statistically significant association with mortality. Conclusion: In our study 27.1% patients experienced thyroid dysfunction during 3 months after HSCT. Increase in age and acute GVHD may be risk factors for thyroid dysfunction during 3 months after HSCT. There was a significant association between ESS and mortality.

• Annals of Clinical Neurophysiology
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• v.24 no.2
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• pp.84-89
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• 2022

Vestibular dysfunction has rarely been reported in MELAS syndrome. A 40-year-old male with long-term diabetes and hearing loss experienced a stroke-like episode with hemisensory disturbance and lactic acidosis. Brain MRI showed temporo-parieto-occipital cortical lesions, and a final diagnosis was made of MELAS syndrome with the mitochondrial 3243A>G mutation. Neuro-otologic evaluations revealed anterior-canal-sparing bilateral impairments of the vestibulo-ocular reflex in the video head impulse test and no caloric paresis. This unique pattern of vestibular dysfunction may aid in diagnosing MELAS syndrome.

• Sleep Medicine and Psychophysiology
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• v.17 no.2
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• pp.69-74
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• 2010

Obstructive sleep apnea syndrome is associated with significant cardiovascular morbidity and increased mortality. However, it was controversial whether obstructive sleep apnea syndrome could cause pulmonary hypertension. The controversy was resolved by several studies that have shown pulmonary hypertension in 20% to 40% of patients with obstructive sleep apnea syndrome without underlying other cardiopulmonary diseases and reductions in pulmonary arterial pressure in patients with obstructive sleep apnea syndrome after treatment with nocturnal continuous positive airway pressure. Recent studies provide strong evidence for endothelial dysfunction in obstructive sleep apnea syndrome and pulmonary hypertension. Endothelin-1 is a 21 amino acid peptide with diverse biologic activity such as highly potent vasoconstrictor and mitogen regulator that may play a key role in obstructive sleep ap-nea syndrome and pulmonary hypertension. Continuous positive airway pressure therapy is moderately effective in reducing pulmonary arterial pressure. Further researches are needed to assess the therapeutic efficacy of pharmacologic therapy with agents that inhibit the action of endothelin-1 in obstructive sleep apnea syndrome patients with pulmonary hypertension.

• Journal of mucopolysaccharidosis and rare diseases
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• v.1 no.2
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• pp.35-39
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• 2015

Sleep problems occur frequently among patients with Prader-Willi syndrome (PWS). The most common problem is excessive daytime sleepiness (EDS) that are closely related to of sleep-related breathing disorder (SRBD) such as obstructive sleep apnea (OSA) and congenital hypoventilation syndrome. Obesity, craniofacial dysmorphism and muscular hypotonia of patients with PWS may increase the risk of SRBD. Sleep apneas can interrupt the continuity of sleep, and these disruptions result in a decrease in both the quality and quantity of sleep. In addition to SRBD, other sleep disorders have been reported, such as hypersomnia, a primary abnormality of the rapid eye movement (REM) sleep and narcolepsy traits at sleep onset REM sleep. Patients with PWS have intrinsic abnormalities of sleep-wake cycles due to hypothalamic dysfunction. The treatment of EDS and other sleep disorders in PWS are similar to standard treatments. Correction of sleep hygiene such as sufficient amount of sleep, maintenance of regular sleep-wake rhythm, and planned naps are important. After comprehensive evaluation of sleep disturbances, CPAP or surgery should be recommended for treatment of SRBD. Remaining EDS or narcolepsy-like syndrome are controlled by stimulant medication. Bright light therapy might be beneficial for disturbed circadian sleep-wake rhythm caused by hypothalamic dysfunction.

• Childhood Kidney Diseases
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• v.2 no.1
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• pp.90-94
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• 1998

Hinman syndrome is a condition representing urinary voiding dysfunction in the neurologically intact child. The syndrome is probably caused by acquired behavioral and psychosocial disorders manifested by bladder and/or bowel dysfunction mimicking neurologic disease. Clinically, the symptom complex may include day and night time enuresis, encopresis, constipation, and recurrent urinary tract infections. Cystoscopy frequently demonstrates normal vesicourethral anatomy. Voiding films usually demonstarate a carrot-shaped proximal urethra with a persistent narrowing at the external sphincter. The bladder is large and often appears trabeculated with a thickened wall and significant postvoid residual. A 13-year-old male child was admitted due to fever, urinary tract infection, enuresis and flank pain. His neurologic examination was normal. Renal sonograms showed moderate hydronephrosis. Voiding cystourethrograms showed a huge, trabeculated bladder without vesicourethral reflux and urethral valves. No abnormal findings was found in spinal MRI.

• Journal of Korean Academy of Oral and Maxillofacial Radiology
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• v.5 no.1
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• pp.33-37
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• 1975

The author has observed 64 films from M. P. D. syndrome cases taken in Dept. of Radiology, the Infirmary of Dental College, Seoul National University in 1974, and obtained the following results: 1. In M. P. D. syndrome, the ratio between the female and the male were 2:l. 2. The prevalenting age were aged 20, 30, 10, and 40 in decreasing order. 3. The incidence was 21.8% in the left side, 29% in the right, and 21% was bilaterally, which show the right most frequent. 4. The roentgenograms revealed variable findings in each case, but there were not recognized any significant differences.

• Clinical and Experimental Pediatrics
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• v.59 no.sup1
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• pp.125-128
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• 2016

Harlequin syndrome, which is a rare disorder caused by dysfunction of the autonomic system, manifests as asymmetric facial flushing and sweating in response to heat, exercise, or emotional factors. The syndrome may be primary (idiopathic) with a benign course, or can occur secondary to structural abnormalities or iatrogenic factors. The precise mechanism underlying idiopathic harlequin syndrome remains unclear. Here, we describe a case of a 6-year-old boy who reported left hemifacial flushing and sweating after exercise. He had an unremarkable birth history and no significant medical history. Complete ophthalmological and neurological examinations were performed, and no other abnormalities were identified. Magnetic resonance imaging was performed to exclude lesions of the cerebrum and cervicothoracic spinal cord, and no abnormalities were noted. His final diagnosis was classic idiopathic harlequin syndrome. Herein, we report the first pediatric case of idiopathic harlequin syndrome in Korea.

• Journal of Korean Medical classics
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• v.22 no.1
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• pp.103-109
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• 2009

It was described in "Somun Jogyeongron" that Eum(Yin)-Yang' deficiency, excess and exuberance. According to "Somun Jogyeongron", It was known that the syndrome of Yang deficiency and exuberance is belong to external contraction, Eum deficiency and exuberance is belong to internal damage. The syndrome of Yang deficiency is belong to Gyejitang[contraction of wind] which constitutional weakness as the main etiological factor of deficiency conditions. The syndrome of Yang exuberance is belong to Mahwangtang[cold damage] which constitutional strong as the main etiological factor of exuberance conditions. Eum deficiency is so dysfunction of the spleen and stomach that Eum fluid and essence is not engender, distributed. So the dysfunction of spleen and stomach makes dampness-heat obstruction and then makes Internal heat at last. The syndrome of Eum deficiency is applicable to bojungikgitang. As contrasted with Eum deficiency, Eum exuberance is occurred cold-dampness obstruction, which we call 'Eum exuberance'. The syndrome of Yang exuberance is applicable to Ijungtang. In the light of "Somun Jogyongron", We explain that the syndrome of Yin deficiency and Yin exuberance, which was caused by dampness-heat, cold-dampness obstruction and internal damage based on disorder of the spleen and stomach.

• Pediatric Infection and Vaccine
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• v.19 no.2
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• pp.79-83
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• 2012

Reye syndrome is a rapidly progressive encephalopathy with hepatic dysfunction, which often begins several days after apparent recovery from a viral illness, especially varicella or influenza A or B. Salicylate use was identified as a major precipitating factor for the development of Reye syndrome. With the recommendation to avoid use of salicylates in children, Reye syndrome has virtually disappeared in recent years. We report a case of Reye syndrome in a 5-month-old infant who had been treated with intravenous immunoglobulin and aspirin under the diagnosis of Kawasaki disease, and showed symptoms of sudden onset of irritability, rigidity, decreased activity, vomiting, poor appetite, lethargy, liver dysfunction without jaundice, coagulopathy, and hyperammonemia.