• Clinical and Experimental Pediatrics
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• v.58 no.9
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• pp.358-361
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• 2015

Hemophagocytic lymphohistiocytosis (HLH) is characterized by fever, splenomegaly, jaundice, and pathologic findings of hemophagocytosis in bone marrow or other tissues such as the lymph nodes and liver. Pleocytosis, or the presence of elevated protein levels in cerebrospinal fluid, could be helpful in diagnosing HLH. However, the pathologic diagnosis of the brain is not included in the diagnostic criteria for this condition. In the present report, we describe the case of a patient diagnosed with HLH, in whom the brain pathology, but not the bone marrow pathology, showed hemophagocytosis. As the diagnosis of HLH is difficult in many cases, a high level of suspicion is required. Moreover, the pathologic diagnosis of organs other than the bone marrow, liver, and lymph nodes may be a useful alternative.

• Parasites, Hosts and Diseases
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• v.51 no.3
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• pp.335-341
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• 2013

Balamuthia mandrillaris is one of the 4 amebas in fresh water and soil that cause diseases in humans. Granulomatous amebic encephalitis (GAE), caused by B. mandrillaris, is a rare but life-threatening condition. A 4-year-old, previously healthy, Thai girl presented with progressive headache and ataxia for over a month. Neuroimaging studies showed an infiltrative mass at the right cerebellar hemisphere mimicking a malignant cerebellar tumor. The pathological finding after total mass removal revealed severe necrotizing inflammation, with presence of scattered amebic trophozoites. Cerebrospinal fluid (CSF) obtained from lumbar puncture showed evidence of non-specific inflammation without identifiable organisms. A combination of pentamidine, sulfasalazine, fluconazole, and clarithromycin had been initiated promptly before PCR confirmed the diagnosis of Balamuthia amebic encephalitis (BAE). The patient showed initial improvement after the surgery and combined medical treatment, but gradually deteriorated and died of multiple organ failure within 46 days upon admission despite early diagnosis and treatment. In addition to the case, 10 survivors of BAE reported in the PubMed database were briefly reviewed in an attempt to identify the possible factors leading to survival of the patients diagnosed with this rare disease.

• Korean Journal of Veterinary Pathology
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• v.2 no.2
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• pp.117-125
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• 1998

Pathological studies were performed on the five piglets experimentally infected with Aujeszky's disease virus(pseudorabies), NYJ isolate, isolated from the naturally infected pigs in Korea: two piglets were inoculated intramuscularly, two piglets intranasally, and one piglet subcutaneously at the dose of 1$m\ell$ per animal with the 105.5 $TCID_50$/0.1ml titer. Clinical signs included dyspnea, high fever(＞$41^{\circ}C$), anorexia, vomiting, diarrhea or constipation, ataxia, circling movement, posterior paralysis, intermittent convulsion, and coma followed by death although some variations by age and inoculated routes were observed. Gross features included multiple necrotic foci in the liver, congestion and hemorrhage in the lymph nodes and spleen, petechial hemorrhage in the kidney, hemorrhagic pneumonia, marked meningeal congestion, severe sub meningeal hemorrhage in the spinal cord, excessive cerebrospinal fluid retention, and muscular necrosis at the inoculated area. Microscopically, non suppurative meningoencephalitis with gliosis and perivascular cuffing in CNS, ganglioneuritis, necrohemorrhagic splenitis, necrotic hepatitis, tonsillitis and rhinitis, hemorrhagic or interstitial pneumonia, and non-suppurative myositis in the injected area were observed. Eosinophilic intranuclear inclusion bodies were found in a variety of tissues the including the liver, kidney, adrenal gland, spleen, lymph nodes, tonsil, and lung. Ultrastructurally, virus particles were confirmed in nucleus and cytoplasms of pneumocytes around the necrotic areas.

• Annals of Clinical Neurophysiology
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• v.8 no.1
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• pp.58-62
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• 2006

Background: Acute transverse myelitis(ATM) is a group of disorders characterized by focal inflammation of the spinal cord and resultant neural injury. It can be diagnosed by Transverse Myelitis Consortium Working Group(TMCWG) criteria. But there are some cases which were not satisfied with idiopathic ATM criteria, both clinically and radiologically, especially in acute stage. So we analyzed 27 cases retrospectively, which were diagnosed as idiopathic ATM. Methods: All the records of the patients at Gil Medical Center with a diagnosis of idiopathic ATM from 2001 to 2005 were reviewed. And clinical manifestations including neurological examination, radiologic features and cerebrospinal fluid (CSF) findings were analyzed. Results: Among the patients(20 men and 7 women; mean age, 45.3 years), 11 cases could not be diagnosed as idiopathic ATM according to the TMCWG criteria ; 6 cases did not have well marginated upper sensory level and 5 cases were not satisfied with spinal cord inflammation. Conclusions: Although most cases of suspected idiopathic ATM were suitable for TMCWG criteria, some cases were not satisfied with this diagnostic criteria, especially in acute stage. Subsequent study might be needed to evaluate the reliability and clinical application of the criteria.

• Korean Journal of Microbiology
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• v.37 no.4
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• pp.289-293
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• 2001

Incidence of infectious viruses is ensuing throughout the world and threatening the health of children as well as adults. The outbreaks of viral diseases of alimentary tract in Pusan from 1998 to 2000 were detected. Viruses were isolated from stool specimens, cerebrospinal fluid and throat swabs from suspicious patients and confirmed by cell culture, latex agglutination test, indirect immunofluorescent test and electron microscopic observation. The average isolation rate was 12.5% from the suspected specimens. From this work, 2 cases of enteric adenoviruses, 23 cases of echovirus, 31 cases of coxsackivirus 36 cases of rotavirus, 45 cases of SRSV, and 7 cases of poliovirus were detected. The major serotypes of coxsackievirus were B2, B3, B4, B6 and echovirus of serotypes 6, 9, 11, 25, and 30 were examined. Two cases of enteric adenovirus type 41 were also confirmed. The incidence of SRSV was mostly concentrated between December through following March, April through October with echovirus and coxsackievirus, and January through April with rotavirus, respectively. Electron micrograph of negative-stained viruses showed typical appearance with 30-80 nm in diameter.

• Journal of Veterinary Clinics
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• v.27 no.4
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• pp.440-444
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• 2010

A 7-year-old neutered male Shih Tzu dog weighing 5.2 kg was presented because of severe neurological signs (paddling, unconsciousness, blindness, seizure). ECG revealed accelerated idioventricular rhythm (AIVR) with ~140 bpm ventricular rate. Based on magnetic resonance imaging (MRI) study and cerebrospinal fluid (CSF) analysis, the neurological condition was tentatively diagnosed as disseminated granulomatous meningoencephalomyelitis (GME). The neurological signs were managed with steroids, anticonvulsive drugs, diuretics and antibiotics with 20% mannitol infusion. The rhythm disturbance (AIVR) was managed with oral medication of mexiletine. Because the heart rhythms were unstable without anti-arrhythmic therapy in spite of improvement of clinical signs after emergency treatment for neurological problems, the anti-arrhythmic therapy was maintained till the neurological signs were abolished.

• Journal of Veterinary Clinics
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• v.27 no.4
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• pp.462-467
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• 2010

A Maltese (5-years old, castrated male) and a Yorkshire Terrier (8-years old, intact female) were referred to Seoul National University Hospital for Animals with ataxia, circling, depression and anorexia. It was suspected to meningoencephalitis of unknown etiology (MUE) based on neurological signs, magnetic resonance imaging findings, cerebrospinal fluid analysis and canine distemper virus antigen test. Immunosuppressive dose of corticosteroid was initially chosen to treat in two dogs. However, the clinical signs were relapsed and side effects had arisen including polyuria, polydipsia, calcinosis cutis, liver enzyme elevation, weight gain and abdominal distension. Combination therapy of cytosine arabinoside (CA) and prednisolone (PDS) was tried. With this combination, the clinical signs were resolved and the side effects were alleviated. The dose of PDS was tapered to 0.5 mg/kg q48h in one case and the PDS was discontinued in the other case. These cases suggested that combined CA and PDS therapy may be significantly useful for the treatment of MUE uncontrolled by PDS alone.

• Radiation Oncology Journal
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• v.38 no.1
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• pp.26-34
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• 2020

Purpose: Intensity-modulated radiotherapy (IMRT) allows for more precise treatment, reducing unwanted radiation to nearby structures. We investigated the safety and feasibility of IMRT for anaplastic ependymoma patients below 3 years of age. Materials and Methods: A total of 9 anaplastic ependymoma patients below 3 years of age, who received IMRT between October 2011 and December 2017 were retrospectively reviewed. The median equivalent dose in 2 Gy fractions was 52.0 Gy (range, 48.0 to 60.0 Gy). Treatment outcomes and neurologic morbidities were reviewed in detail. Results: The median patient age was 20.9 months (range, 12.1 to 31.2 months). All patients underwent surgery. The rates of 5-year overall survival, freedom from local recurrence, and progression-free survival were 40.6%, 53.3%, and 26.7%, respectively. Of the 9 patients, 5 experienced recurrences (3 had local recurrence, 1 had both local recurrence and cerebrospinal fluid [CSF] seeding, and 1 had CSF seeding alone). Five patients died because of disease progression. Assessment of neurologic morbidity revealed motor dysfunction in 3 patients, all of whom presented with hydrocephalus at initial diagnosis because of the location of the tumor and already had neurologic deficits before radiotherapy (RT). Conclusion: Neurologic morbidity is not caused by RT alone but may result from mass effects of the tumor and surgical sequelae. Administration of IMRT to anaplastic ependymoma patients below 3 years of age yielded encouraging local control and tolerable morbidities. High-precision modern RT such as IMRT can be considered for very young patients with anaplastic ependymoma.

• Journal of Veterinary Clinics
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• v.37 no.5
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• pp.255-260
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• 2020

The purpose of this retrospective study was to describe the characteristics of magnetic resonance imaging (MRI) findings in dogs with meningoencephalitis of unknown etiology (MUE), and to evaluate the usefulness of meningeal enhancement. Thirty-two dogs were included in MUE group on the basis of clinical signs, MRI findings and cerebrospinal fluid (CSF) results, and for comparison of the meningeal enhancement, twenty-three dogs with normal MRI, normal CSF and no clinical sign were included in the control group. The evaluated MRI findings included lesion site, lesion number, signal intensity of each MRI sequence, mass effect, perilesional edema, contrast enhancement, and meningeal enhancement. The MUE was most frequently associated with multiple lesions (50%) with perilesional edema (72%) in forebrain (66%) that were hyperintense (92%) in T2W and FLAIR images. Of the meningeal enhancement, there was no significant difference between the control group and the MUE groups in the pachymeningeal enhancement. However, leptomeningeal (or both) enhancement was found relatively high proportion in the MUE group than in the control group (P < 0.001, Odd ratio = 10.26), and based on this result, leptomeningeal (or both) enhancement is considered to be significant finding for indicating MUE.

• Clinical and Experimental Pediatrics
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• v.55 no.8
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• pp.301-305
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• 2012

Nonketotic hyperglycinemia (NKH) is a rare inborn error of amino acid metabolism. A defect in the glycine cleavage enzyme system results in highly elevated concentrations of glycine in the plasma, urine, cerebrospinal fluid, and brain, resulting in glycine-induced encephalopathy and neuropathy. The prevalence of NKH in Korea is very low, and no reports of surviving patients are available, given the scarcity and poor prognosis of this disease. In the current study, we present a patient with NKH diagnosed on the basis of clinical features, biochemical profiles, and genetic analysis. Magnetic resonance spectroscopy (MRS) allowed the measurement of absolute glycine concentrations in different parts of the brain that showed a significantly increased glycine peak, consolidating the diagnosis of NKH. In additional, serial MRS follow-up showed changes in the glycine/creatinine ratios in different parts of the brain. In conclusion, MRS is an effective, noninvasive diagnostic tool for NKH that can be used to distinguish this disease from other glycine metabolism disorders. It may also be useful for monitoring NKH treatment.