Delayed Post-Hypoxic Leukoencephalopathy Induced by an Overdose with Fentanyl Patches: A Case Report

Fentanyl intoxication has occasionally been reported since fentanyl patches became available for medical use. Delayed post-hypoxic leukoencephalopathy is a complication of hypoxia. However, its neuropsychiatric symptoms can be delayed, and it can progress to leukoencephalopathy even after full recovery from coma due to acute intoxication. Herein, we report a case of fentanyl intoxication in a 65-year-old man who was presented to the hospital because of unconsciousness for 13 hours after using ten fentanyl patches simultaneously. Initial brain CT findings were non-specific. Twenty days later, delayed neuropathologic symptoms manifested, and MRI showed bilaterally symmetrical, heterogeneous, confluent high signal intensities on T2-weighted/fluid attenuated inversion recovery MRI in the cerebral white matter with diffusion restriction. This was followed by rapid exacerbation of neuropathological symptoms with diffuse severe cerebral atrophy over 1 year.

Brain MRI Findings of the Cri-Du-Chat Syndrome: A Case Report and Summary

Cri-du-chat syndrome is a rare genetic disorder in which the patient presents with a characteristic high-pitched monotonous cry and recurrent aspiration pneumonia, attributed to abnormalities in the larynx, epiglottis, and nervous system. The most prominent brain MRI findings are the presence of pontine and cerebellar hypoplasia, which primarily involve posterior cranial fossa structures. Although atrophy of supratentorial structures were also a common radiological finding, it was considered to be a secondary change due to pontine hypoplasia. Here, we present the case of a three-month-old patient presenting with cri-du-chat at our institution. The patient also showed the presence of prominent pontine hypoplasia similar to previously reported cases; however, contrary to other cases, there was a general delayed myelination of brain instead of decreased myelination of anterior limb of internal capsule. Since the larynx, pons, and cerebellum all originated from similar notochord level, which suggests anomaly in early stage of development, laryngeal, and brain anomaly characteristically observed in the cridu- chat syndrome.

Tissue-specific activation of mitogen-activated protein kinases for expression of transthyretin by phenylalanine and its metabolite, phenylpyruvic acid

Phenylketonuria is an autosomal recessive disorder caused by a deficiency of phenylalanine hydroxylase. Transthyretin has been implicated as an indicator of nutritional status in phenylketonuria patients. In this study, we report that phenylalanine and its metabolite, phenylpyruvic acid, affect MAPK, changing transthyretin expression in a cell- and tissue-specific manner. Treatment of HepG2 cells with phenylalanine or phenylpyruvic acid decreased transcription of the TTR gene and decreased the transcriptional activity of the TTR promoter site, which was partly mediated through HNF4alpha. Decreased levels of p38 MAPK were detected in the liver of phenylketonuria-affected mice compared with wild-type mice. In contrast, treatment with phenylalanine increased transthyretin expression and induced ERK1/2 activation in PC-12 cells; ERK1/2 activation was also elevated in the brainstem of phenylketonuria-affected mice. These findings may explain between-tissue differences in gene expression, including Ttr gene expression, in the phenylketonuria mouse model.

Protective Effect of Recombinant Adeno-Associated Virus 2/8-Mediated Gene Therapy from the Maternal Hyperphenylalaninemia in Offsprings of a Mouse Model of Phenylketonuria

Phenylketonuria (PKU) is an autosomal recessively inherited metabolic disorder caused by a deficiency of phenylalanine hydroxylase (PAH). The accumulation of phenylalanine leads to severe mental and psychomotor retardation, and the fetus of an uncontrolled pregnant female patient presents with maternal PKU syndrome. We have reported previously on the cognitive outcome of biochemical and phenotypic reversal of PKU in a mouse model, Pah(enu2), by the AAV serotype 2-mediated gene delivery of a human PAH transgene. However, the therapeutic efficacy had been limited to only male PKU mice. In this study, we generated a pseudotyped recombinant AAV2/8-hPAH vector and infused it into female PKU mice through the hepatic portal vein or tail vein. Two weeks after injection, complete fur color change to black was observed in female PKU, as in males. The PAH activity in the liver increased to 65-70% of the wild-type activity in female PKU mice and to 90% in male PKU mice. Plasma phenylalanine concentration in female PKU mice decreased to the normal value. In addition, the offsprings of the treated female PKU mice can rescue from the harmful effect of maternal hyperphenylalaninemia. These results indicate that recombinant AAV2/8-mediated gene therapy is a potential therapeutic strategy for PKU.

A Case of Lipoid Pneumonia Caused by Aspiration of Laxatives / 소아알레르기및호흡기학회지

Lipoid pneumonia is chronic, interstitial, proliferative inflammation resulting from aspiration of lipoid material. Mineral oil is a hydrocarbon that physicians often use to treat chronic constipation in children and adults. Mineral oil may not elicit a normal protective cough reflex and may impair mucociliary transport. We experienced a case of exogenous lipoid pneumonia caused by aspiration of mineral oil given as a laxatives confirmed by fiberoptic bronchoscopy with bronchoalveolar lavage and bronchial biopsy in a 9-month-old boy with chronic cough and radiologic evidence of parenchymal lung disease.We reported this case with a brief review of related literatures.

Primary Non-Hodgkin's Bone Lymphoma in a Child: A Case Report

Non-Hodgkin's bone lymphoma is rare, accounting for only 3-4% of all primary malignant bone tumors. The femur, tibia and humerus are most frequently affected. Most lesions are located in the medul-lary cavity of the diaphysis, or in the metaphysis adjacent to the diaphysis and the majority of patients are between 20 and 40 years old. Intermittent localized pain, dull and aching, and not relieved by rest, is present in almost all patients. Classically, the lesion begins in the bone marrow as a permeative or moth-eaten lytic process. The treat-ment of choice for primary non-Hodgkin's bone lymphoma is radiation therapy and adjuvant chemotherapy. The prognosis for this tumor is better than that for the majority of other primary malignant bone tumors, and the five-year survival rate is approximately 48%. We report a case of primary non-Hodgkin's bone lymphoma in a 9-year-old boy, describing the MR and plain film imaging findings.

MR Findings of Kallmann Syndrome

Kallman syndrome is characterised by hypogonadotrophic hypogonadism and anosmia. T1 weighted MR coronal images show the bilateral absence or hypoplasia of olfactory bulbs, tracts, and sulci. We report the MR findings of Kallmann syndrome and revew the literature.

MR Imaging of Cerebral Aspergillosis in an Infant with Normal-Immunity: A Case Report

Cerebral aspergillosis is a rare condition, and like other opportunistic fungal infections, it most commonly occurs in immunocompromised patients. Because of the increasing use of chemotherapy in organ transplanta-tion, cases involving neoplasms, corticosteroid therapy, and cases of lymphoma and leukemia, the incidence of fungal infections in the brain has recently increased. Cerebral aspergillosis in an infant with normal immunity is a very rare condition, and has not been reported in Korea. We report the MR findings of this condition in an infant with normal immunity.

Imaging Findings of Cerebellopontine Angle Cistern Melanocytoma: A Case Report

Because melanocytes are found throughout the leptomeninges, primary melanotic pigmented tumors such as pigmented meningioma, malignant melanoma, melanoblastosis, and melanocytoma can arise from this region. Melanocytomas have been described in the literature as isodense or hyperdense compared with brain parenchyme, as seen on noncontrast-enhanced CT, and as showing relatively homogeneous enhancement. MR imaging demonstrates a high signal on T1-weighted images, and an iso to hypointense signal on T2- weighted images. We report the imaging findings of a melanocytoma that showed hyperdense attenuation on precontrast CT with homogeneous enhancement and hyperintense signal intensity on T1-weighted images and dark signal intensity enhancement on T2-weighted images, and also review the literature.

CT Findings in Differential Diagnosis of Pelvic Inflammatory Bowel Mass and Tuboovarian Abscess

PURPOSE: To evaluate the CT findings which may help differentiate pelvic inflammatory bowel mass(IBM) fromtubo-ovarian abscess(TOA). MATERIALS AND METHODS: Twenty-five patients with histologically confirmed TOA(n=14),periappendiceal abscess(n=9), an abscess caused by diverticulitis(n=1), and by ulcerative colitis(n=1) wereevaluated. For TOA, age distribution ranged only from the 3rd to the 5th decade, but for IBM, the range was the 2nd to 8th decade with highest frequency during the 3rd-4th decade. CT findings were retrospectively analysed forbilaterality, internal septa, anterior displacement of the mesosalpinx, and perirectal and mesenteric fat. RESULTS: Mesenteric fat infiltration was detected in all 11 cases of pelvic IBM, but in only two of 14 TOA cases(p<0.05).Anterior displacement of the mesosalpinx was observed in two of 11 pelvic IBM cases and in nine of 14 TOAcases(P<0.05). There were no significant difference in bilaterality, internal septa, or perirectal fatinfiltration. CONCLUSION: Mesenteric fat infiltration was the most reliable finding in differentiating pelvic IBMform TOA. Anterior displacement of the mesosalpinx, and age distribution were also helpful in differentiating thetwo disease groups.

Differential Diagnosis of Vertebral Lesions with paraspinal Mass with MRI

PURPOSE: To assess the characteristic features of MR findings which would be useful for the differentiation of various spinal diseases involving paraspinal soft tissue mass. MATERIALS AND METHODS: We retrospectively reviewed MR findings in 31 cases(M:F=20:11) of spinal disease in which paraspinal mass was involved. The breakdown of cases was as follows : spinal tuberculosis, 12; spinal metastasis, 13; multiple myeloma, 3; pyogenic spondylitis, 2; spinal aspergillosis; 1. RESULTS: The pattern of bone marrow invasion in spinal metastasis, multiple myeloma, spinal tuberculosis and aspergillosis was mixed ; focal, homogeneously diffuse and inhomogeneously patterns were seen. Pyogenic spondylitis showed inhomogeneously diffuse invasion; an intravertebral abscess was seen in the only five cases of spinal tuberculosis. Vertebral posterior compartment invasion was observed in seven cases of spinal tuberculosis, two of multiple myeloma, the one case of spinal aspergillosis and in all 13 cases of spinal metastasis. This and multiple myeloma showed no disc space invasion, in any case, but all cases of infectious spondylitis showed such invasion. Peripheral rim-enhancement in the paravertebral mass was seen in 11 cases of spinal tuberculosis, one case of pyogenic spondylitis and the case of aspergillosis. Bilobate anterior epidural mass was noted in 60% of spinal tuberculosis cases, 36% of spinal metastasis and one case of pyogenic spondylitis. CONCLUSION: MR findings of spinal disease involving a paraspinal soft tissue mass were useful for differentiation.

CT Findings in Differential Diagnosis of Benign and Malignant Parotid Tumors

PURPOSE: To evaluate CT findings which may help differentiate benign from malignant parotid tumors. MATERIALS AND METHODS: The CT findings of seventy-one cases with surgically-proven parotid tumors were retrospectively analysed for size, location, margin, internal density, adjacent tissue plane and lymphadenopathy. RESULTS: The margin of the mass was smooth and sharp in most benign tumors (89.5%), and irregular or indistinct in twelve which were malignant (75%, p<0.01). With regard to internal density, 70.2% of benign tumors were homogeneous (similar to muscle) and 81.3% of malignant tumors were heterogeneous (p<0.01). When analysing low density patterns within the mass, focal low densities in benign tumors (11/17) and diffuse or scattered multifocal low densities in those which were maligant (8/13) were frequently seen. Three malignant tumors invaded adjacent muscles, the parapharyngeal space, and bones, each in one case, and twelve malignant and one benign tumor infiltrated the adjacent fascia or subcutaneous fat layer. In five patients with a malignant tumor, obliteration by the mass of the fat plane between the mastoid tip and styloid process was noted, suggesting facial nerve invasion, while in three cases of malignancy, lymphadenopathy greater than 1cm was seen. CONCLUSION: In differentiating malignant and benign parotid tumors, the presence of irregular or indistinct margin of the mass, and invasion of adjacent structures, are important. Lymph node enlargement greater than 1cm and diffuse internal low densities, which may suggest necrosis or cystic change were also helpful in differential diagnosis.

Correlation between MR Imaging and Histology of Intracranial Meningioma

PURPOSE: The magnetic resonance(MR) appearance of 23 biopsy-proven meningiomas were retrospectively evaluated and correlated with histologic pattern ;fibroblastic, transitional, syncytial, angioblastic. MATERIALS AND METHODS: Twenty three patients with biopsy-proven meningiomas were examined with MR imaging over a 3 years period. Images were assessed for average T1, T2 intensity score(1-5), degree of edema, calcification, cyst formation or necrosis, and dural tail sign. RESULTS: Tl-weighted images showed only one significant correlation between fibroblastic type and angiobiastic type (p<0.04). Angioblastic type was more hypointense than gray matter(1.83/isointense to gray 3) on T1-weighted image. Syncytial and angioblastic tumors had average T2 intensity scores much higher than those of fibroblastic and transitional types. All three cases of angioblastic tumors had shown severe edema. Calcified lesions were much more common in transitional and fibroblastic types than in syncytial and angioblastic types. CONCLUSION: MRI is a helpful diagnostic imaging modality with good correlation between its MR findings and histologic patterns.

Neurosonographic Abnormality; Periventricular Echodensities and Intraventricular Hemorrhage: Usefulness in Predicting Neurodevelopmental Outcome in Very-Low-Birth-Weight, Preterm Infants

Serial neurosonographic examinations are routinely performed at frequent intervals during nursery course of all preterm infants of very low-birth-weight who are admitted to the intensive care nursery of Presbyterian Medical Center from November 1, 1990 to July 30, 1992. After discharge, the following survivors who had received periodic, serial scanning by meas of cranial ultrasonography were longitudinally observed in an interdisciplinary neurodevelopmental follow-up program to a mean corrected age of 13 months. Neurodevelopmental outcome was assessed by means of Vojta's postural reaction and other neurological examinations. The results are as follows: 1) The incidence of PV-IVH in the study was 79%. 2) According to Papile's grading system of PV-IVH, gradel was 20%, gradell was 46%, gradelll was 19%, and grade IV was 13%. 3) The risk factors associated with PV-IVH were birth weight, gestational age, apgar score, ventilator care, RDS, and sepsis. 4) The mortality of PV-IVH was 20% for gradel, 19% for gradell, 44% for gradelll, and 67% for grade lV. 5) According to relationship between PV-IVH and neurodevelopmental outcome, in two of the four subjects with grade lll PV-IVH, moderate/severe CCD was developed. 6) According to relationship between PVE with cysts and nuerodevelopmental outcome, moderate/severe PVE with periventricular cysts larger than 3mm in diameter was associated with development of severe CCD.

Neurosonographic diagnosis of periventricular-intraventricular hemorrhage in low birth weight infants

Periventricular-intraventricular hemorrhage (PV-IVH)is one of the most important neurologic lesion of the low birth weight infants. Serial neurosonographic exeaminations were performed in 113 low birth weight infants who were admitted to the neonatal intensive care unit of Presbyterian Medical Center from November 1, 1990to July 31, 1991. The results were summarized as follows: 1) The incidence of PV-IVH in the study was 54% 2) According to Papile's grading system of PV-IVH, grade I was 32.8%, grade II was 45.9%, grade IIIwas 11.5% and grade IV was 9.8%. 3) The onset of PV-IVH was within the first 7 days of life in 82%. 4) Poor activity, apnea, bradycardia and hypotension were statistically significant clinical findings associated with PV-IVH(P<0.05). 5) The risk factors associated with PV-IVH were gestational age, birth weight, hyaling membrane disease, patent ductus arteriosus and artifical ventilation. 6) The mortality of PV-IVH was 0% for grade I, 10.7% for grade II,42.9% for grade III and 83.3% for gradeIV.

Neurosonographic evaluation and follow-up study of GMH/IVH in infants with less than 2500mg

GMH/IVH(Germinal martrix-Intraventricular hemorrhage ) is an inportant factor that influences on the mortality rate of low-birth-weight infants. The real-time high resolution sonography with a mechanical sector scanner is a convenient and useful method for the detection and follow-up study of intracranial hemorrhage in low-birth-weight infants. Authors analysed 112 cases of neurosonographic findings in low-birth-weight infants, weighing less than 2,500gm. The incidence of GMH/IVH was 54.5%. The severity of GMH/IVH was classified into 4 grades(I-IV) and their percentages were 41%, 41% 8.1%, and 9.9%, respectively. The onset of GMH/IVH was within the first week after birth in 75.4% of cases. The overall mortality rate of low-birth-weight infants with GMH/IVH was 18% (4% for grade I, 12% for grade II, 40% for grade III and 83% for grade IV). In the follow-up study of 61 cases, complete absorption was seen in 25 cases, rebleeding in 5 cases, cystic change in 24 cases, ventriculomegaly in 9 cases and hydrocephalus in 7 cases. The incidence of GMH/IVH in neonates with pathologic lung conditions was 82.5% and that with normal lung conditions was 39%. In conclusion, sonography is very useful in the diagnosis and follow-up of GMH/IVH in low-birth-weight infants. Our study is the first step in the further study of the relationship between GMH/IVH and neuromotor outcome.

Computed tomographic findings of maxillary sinus cancer

No abstract available.

Computerized tomographic evaluation of intracranial metastases

In a study of intacranial metastases, 46 cases having satisfactory clinical, operative and histological proofswere analyzed by computerized tomography at Presbyterian Medical Center from May, 1982 to February, 1986. Theresults were as follows: 1. The male to female ratio of itracranial metastases were 67:33. The 5th decade group(34.8%) was the most prevalent age group, followed by the 6th decade(21.7%) and 7th decade(21.7%). 2. The numberof lesions was found to be: single-25 cases(54.3%); multiple-21 cases(45.7%). 3. The source of intracranialmetastases found to be: lung 15 cases(32.6%); unknown 12 cases(26.0%); chorioca 3 cases(6.5%); liver 3cases(6.5%); liver 3 cases(6.5%); stomach 2 cases(4.3%); parotid, breast, kidney, prostate, melanoma, rectal ca.rhabdomyosarcoma, nasal ca. lymphoma, testicular ca, cervix, each 1 case(2.2%). 4. The locations of theintracranial metastases were as follows: Cerebral hemisphere 37.7% in parietal region Cerebra hemisphere 15.9% inin frontal region Cerebral hemisphere 13.4% in occipital region Cerebra hemisphere 10.5% in temporal regionCerebellar hemisphere 3.2% Cerebellopontine angle 3.2% Intraventricular 4.8% Meninges 4.8% Skull vault 6.5% 5.Peritumor edema was found to be: Grade II-17 cases(37.0%): Grade III-14 cases(30.4%); Grade I-8 cases(17.4%);Grade 0–7 cases(15.2%) in that order. 6. The chief complaints of intracranial metastases on admission, were asfollows: Headache 30 cases(65.2%); Vomiting 11 casees(23.9%); deteriorated mental state 10 cases(21.6%);Hemiplegia 7 cases(15.2%); visual disturbance 6 cases(13.0%); hemiparesis 4 cases(8.7%); seizure 4 cases(8.7%);other symptoms were less frequent. 7. On pre-contrast scan, hyperdense lesions were present in 18 cases(39.1%);hypodense lesions in 15 cases(32.6%); mixed density in 8 cases(17.4%); isodenisty was present in 5 cases(10.9%).On post-contrast scan, ring enhancement was seen in 19 cases(41.3%); nodular enhancement in 17 cases(37%); mixedring-nodular enhancement in 8 cases(17.4%); only hypodense 2 cases(4.3%).

Computerized tomographic evaluation of primary brain tumors

In a study of primary brain tumors 104 cases having satisfactory clinical, operative and histological proofswere analyzed by computerized tomography at Presbyterian Medical Center from May, 1982 to April, 1985. The resultswere as follows: 1. The male to female ratio of primary brain tumor was 54:46. 2.The 2nd decade group (26%) wasthe most prevalent age group, followed by the 5th decade(16.3%), 1st decade(14.4%), 3rd decade(12.5%), 4thdecade(11.5%), 6th decade(10.6%), 7th decade(8.7%) in that order. 3. The incidence of primary brain tumors was found to be: glioma 64 cases(61.6%) (among the GM, the most frequent 17 cases(16.3%), followed by meningioma 12cases (11.5%), pituitary adenoma 10 cases (9.6%), craniopharyngioma 6 cases(5.8%), pinealoma and germinoma 3cases(2.9%) respectively, and dermoid cyst 2 cases(1.9%) in that order. 4. The locations of the primary braintumors were as follows: cb. hemisphere(49%) of these 24.5% in parietal region, 11.9% in temporal region, 9.7% infrontal region, 3.0% in occipital region: Juxtasella area(16.3%), cerebellar hemisphere(8.7%), Parapineal andintraventricle(7.7%) respectively, cerebello-pontine angle area(5.8%), vermis and 4th ventricular region(4.8%). 5.There were no remarkable differences in the findings of pre-and post-contast CT scanning of primary brain tumorscompared with others.

Radiological evaluation of pulmonary metastasis

158 cases of pulmonary metastasis having satisfactory histological proof of the primary malignant tumor, the frequency and pattern of which were analysed by conventional radiologically. The results are as follows: 1. The frequency of pulomonary metastasis according to the primary sites was 24%(38/158) hepatobiliary system,19.6%(31/158) gastrointestinal tract, 18.9%(30/158) uterine cervix and 12%(13/158) head and neck. 2. Pulmonary metastatic pattern showed nodular type 74% (117/158), granular type 10.1%(16/158), streaky type 9.5%(15/158),hilar enlargement 3.8%(6/158), patchy type 1.9% (3/158) and others 0.6%(1/158). Of all these type, nodular type was predominant. 3. The nodular type of pulmonary metastasis was classified into less than 10 nodules56.4%(66/17), 10-50 nodules 25.6%(30/117), more than 50, 17.9%(21/117), less than 1cm 12.8%(15/117) and more than5cm in 5.2%(1/117) of the cases. 4. The size of nodule was 1-3cm 36.6%(43/117), 2-3cm 20.5%(24/117), less than 1cm12.8%(15/117) and more than 5cm in 5.2%(1/117) of the cases. 5. Most cases of pulmonary metastasis involved bothlung fields 69.2%(110/58). Others were right lung field only 17.7% (28/158), left lung only 12.7%(20/158). 6. Theages between 40 and 70 represented 79.8%(126/158) of the cases and the raio of male and female was about 1.5:1.