• Title/Summary/Keyword: Central nervous system involvement

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Multiple Hemangioblastomas on Cerebellum and Spinal Cord in a Patient of Von Hippel-Lindau Disease - A Case Report - (본 히펠 린다우병 환자에서 소뇌와 척수에 동시에 발생한 혈관아세포종 - 증례보고 -)

  • Yoon, Chang Sik;Ha, Young Soo;Park, Chong Oon;Hyun, Dong Keun
    • Journal of Korean Neurosurgical Society
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    • v.30 no.8
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    • pp.1023-1027
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    • 2001
  • Hemangioblastomas are rare benign tumor of the central nervous system that commonly occur in the posterior fossa around the 4th ventricle. In case of von Hippel-Lindau disease, hemangioblastomas involve multiple regions such as cerebellum, spinal cord and brainstem but, rarely show simultaneous involvement of cerebellum and spinal cord. We have experienced a case of multiple hemangioblastomas that were located at the cerebellum, cervical cord and conus medullaris and also had multiple lesions that a part of von Hippel-Lindau disease ; retinal angioma, syringomyelia, multiple cyst on kidney and pancreas, renal cell carcinoma on left kidney. Hemangioblastomas on cerebellum and spinal cord were removed totally, retinal angioma was treated with laser photocoagulation and renal cell carcinoma was also totally excised. The authors report a case of von Hippel-Lindau disease had multiple located hemangioblastomas on cerebellum, cervical cord and conus medullaris with review of literature.

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Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids with a Significant Elevation of β-2 Microglobulin Levels

  • Fujisawa, Naoaki;Oya, Soichi;Mori, Harushi;Matsui, Toru
    • Journal of Korean Neurosurgical Society
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    • v.58 no.5
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    • pp.487-490
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    • 2015
  • Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a relapsing-remitting disorder for which steroid administration is a key to control the progression. CLIPPERS can exhibit radiological features similar to malignant lymphoma, whose diagnosis is confounded by prior steroid administration. We report a case of CLIPPERS accompanied by abnormal elevation of ${\beta}-2$ microglobulin in the cerebrospinal fluid (CSF). A 62-year-old man started to experience numbness in all fingers of his left hand one year ago, which gradually extended to his body trunk and legs on both sides. Magnetic resonance imaging demonstrated numerous small enhancing spots scattered in his brain and spinal cord. CSF levels of ${\beta}-2$ microglobulin were elevated; although this often indicates central nervous system involvement in leukemia and lymphoma, the lesions were diagnosed as CLIPPERS based on the pathological findings from a biopsy specimen. We emphasize the importance of biopsy to differentiate between CLIPPERS and malignant lymphoma because the temporary radiological response to steroid might be the same in both diseases but the treatment strategies regarding the use of steroid are quite different.

Racemose Cysticercosis in the Cerebellar Hemisphere

  • Kim, Sang-Wook;Kim, Moon-Kyu;Oh, Sae-Moon;Park, Se-Hyuck
    • Journal of Korean Neurosurgical Society
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    • v.48 no.1
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    • pp.59-61
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    • 2010
  • Neurocysticercosis is the most common parasitic disease of the central nervous system in humans, caused by infection of the larval stage of the pork tapeworm, Taenia solium. However, cerebellar involvement is rarely reported. We report of a case of racemose cysticercosis in the cerebellar hemisphere. A 44-year-old man presented with headache and dizziness. Magnetic resonance imaging showed hydrocephalus and an ill-defined, multicystic cerebellar mass with hypersignal on T2-weighted images, hyposignal on T1-weighted images and rim enhancement after gadolinium injection. The patient underwent endoscopic third ventriculostomy and the cyst resection was done through a craniotomy. In surgical field, cysts were conglomerated in a dense collagen capsule that were severely adherent to surrounding cerebellar tissue, and transparent cysts contained white, milky fluid. Histological findings confirmed the diagnosis of cysticercosis. He received antiparasitic therapy with praziquantel after surgery. Racemose cysticercosis is rare in the cerebellar hemisphere but neurocysticercosis should be taken into consideration as a differential diagnosis of multiple cystic lesions in the cerebellum.

Ambulatory Anesthesia Management of the Patient with Adrenoleukodystrophy for Dental Treatment -A Case Report- (부신백질이영양증 환자의 치과치료를 위한 외래전신마취 -증례 보고-)

  • Seo, Kwang-Suk;Bak, So-Yeon;Shin, Teo-Jeon;Kim, Hyun-Jeong
    • Journal of The Korean Dental Society of Anesthesiology
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    • v.10 no.1
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    • pp.45-49
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    • 2010
  • Adrenoleukodystrophy (ALD) is a rare X-linked genetic disorder associated with various central nervous system problems and adrenal insufficiency. It is common in childhood and symptoms include loss of previously acquired neurological abilities, seizures, ataxia, Addison's disease as well as degeneration of visual and auditory function. These children frequently require anesthesia during imagining procedure such as MRI or during some surgical procedures like gastrostomy. There is special need for careful management because of numerous anesthetic challenges like difficulty in cooperation, seizure disorders, life-threatening airway obstruction, copious oral secretion and possibility of aspiration. In addition, adrenal involvement and hypofunction must be considered for safe anesthesia management. We report a successful anesthetic management in a patient with ALD for dental procedures.

Isolated Weakness of Radial-side Fingers Due to a Small Cortical Infarction (국소 뇌경색에 의한 요골측 손가락 마비)

  • Hwang, Kyoung Jin;Park, Key-Chung;Chang, Dae-Il;Yoon, Sung Sang
    • Investigative Magnetic Resonance Imaging
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    • v.18 no.4
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    • pp.362-365
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    • 2014
  • Predominant involvement of a particular group of fingers due to a central nervous system lesion has been described as pseudoperipheral palsy. Two patients visited our hospital with isolated weakness of a particular group of fingers due to small cortical infarctions. A 51-year-old woman suddenly developed weakness in her left index and middle fingers. The brain MRI showed a small infarct in the right frontal cortex. A 67-year-old man was sudden difficulty using his chopsticks and had weakness in his right thumb and index finger. The brain MRI showed a small infarct in the left precentral cortex.

BEHCET DISEASE WITH SUPERIOR VENA CAVA THROMBUS IN A PATIENT PRESENTING DELAYED FACIAL WOUND HEALING : A CASE REPORT (단순안면열상환자에서 발생한 창상치유지연 및 상대정맥혈전증을 동반한 베체트병 : 증례보고)

  • Choi, Sun-Mi;Ha, Joo-Hyo;Kim, Sun-Ae;Choi, Mi-Ra;Jung, Su-Jin;Lee, Hyen-Soo;Yang, Soo-Nam
    • Maxillofacial Plastic and Reconstructive Surgery
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    • v.30 no.2
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    • pp.198-204
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    • 2008
  • Behcet Disease is a multisystem inflammatory disorder of unknown orign. It is characterized by recurrent oral ulcer, genital ulcer, skin lesions and ocular inflammation, and which may involve the joints, skin, central nervous system and gastrointestinal tract. Because Behcet Disease dose not have any specific symptoms and laboratory findings, the diagnosis is made on the basis of the criteria proposed by the the International Study Group for Behcet Disease. Behcet Disease is affecting both arteries and veins, and clinically manifest large vessel involvement occurs in between 7 and 49% of patients. Superior vena cava thrombosis is a rare but well-recognized manifestation of Behcet disease. We report a case of a Behcet Disease with superior vena cava thrombosis in a patient presenting delayed facial wound healing.

Enterovirus 71 infection and neurological complications

  • Lee, Kyung Yeon
    • Clinical and Experimental Pediatrics
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    • v.59 no.10
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    • pp.395-401
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    • 2016
  • Since the outbreak of the enterovirus 71 (EV71) infection in Malaysia in 1997, large epidemics of EV71 have occurred in the Asia-Pacific region. Many children and infants have died from serious neurological complications during these epidemics, and EV71 infection has become a serious public health problem in these areas. EV71 infection causes hand, foot and mouth disease (HFMD) in children, and usually resolves spontaneously. However, EV71 occasionally involves the central nervous system (CNS), and induces diverse neurological complications such as brainstem encephalitis, aseptic meningitis, and acute flaccid paralysis. Among those complications, brainstem encephalitis is the most critical neurological manifestation because it can cause neurogenic pulmonary hemorrhage/edema leading to death. The characteristic clinical symptoms such as myoclonus and ataxia, cerebrospinal fluid (CSF) pleocytosis, and brainstem lesions on magnetic resonance imaging, in conjunction with the skin rash of HFMD and the isolation of EV71 from a stool, throat-swab, or CSF sample are typical findings indicating CNS involvement of EV71 infection. Treatment with intravenous immunoglobulin and milrinone are recommended in cases with severe neurological complications from EV71 infection, such as brainstem encephalitis. Despite the recent discovery of receptors for EV71 in human cells, such as the scavenger receptor B2 and P-selection glycoprotein ligand 1, it is not known why EV71 infection predominantly involves the brainstem. Recently, 3 companies in China have completed phase III clinical trials of EV71 vaccines. However, the promotion and approval of these vaccines in various countries are problems yet to be resolved.

A Case of Laryngeal Inflammatory Myofibroblastic Tumor (후두에 발생한 염증성 근섬유모세포종 1 례)

  • Park, Sang Gyu;Kim, Yeseul;Woong, Jun Hyun;Song, Chang Myeon
    • Korean Journal of Head & Neck Oncology
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    • v.35 no.2
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    • pp.71-75
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    • 2019
  • Inflammatory myofibrolastic tumor (IMT) is a rare borderline neoplasm. It frequently occurs in the lung but occasionally occurs in extrapulmonary sites such as the genitourinary tract, gastrointestinal tract, breast, salivary glands, sinonasal tract, orbit, and the central nervous system. Laryngeal involvement of IMT is very rare. A 61-year-old woman who complained of hoarseness persisting for 3 months visited our hospital. Laryngoscopy showed an elevated lesion in the right true vocal cord. Incisional biopsy was confirmed as larygeal inflammatory myofibrolastic tumor. We performed a transoral excision with CO2 LASER under suspension examination. Regional recurrence or distant metastasis was not observed after 9 months of follow-up. Herein we report a case of larygeal inflammatory myofibrolastic tumor that was treated with surgery alone, with a literature review.

Weight status in survivors of childhood acute lymphocytic leukemia in South Korea: a retrospective descriptive study

  • Yeongseon Kim;Kyung-Sook Bang
    • Child Health Nursing Research
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    • v.29 no.4
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    • pp.280-289
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    • 2023
  • Purpose: This study investigated weight status in survivors of childhood acute lymphocytic leukemia (ALL) and identified related factors. Methods: A retrospective review of the electronic medical records of survivors of childhood ALL (n=230) was conducted. We analyzed the survivors' characteristics, including sex, age, weight status at diagnosis, central nervous system involvement, risk classification, length of treatment, radiation therapy, and hematopoietic stem cell transplantation. Analysis of variance and the chi-squared test were applied to investigate influencing factors. Results: The weight status distribution was as follows: 23 individuals (10.0%) were classified as underweight, 151 individuals (65.7%) were healthy weight, and 56 individuals (24.3%) were overweight/obese. Age at diagnosis (F=10.03, p<.001), weight status at diagnosis (x2=43.41, p<.001), and risk classification (F=10.98, p=0.027) showed significant differences among the weight status groups. Survivors who were older at diagnosis and those in the very high-risk category had a higher likelihood of experiencing underweight status during their survivorship, while survivors who were overweight/obese at diagnosis were more likely to remain overweight/obese at the time of survival. Conclusion: Considering the potential health implications related to an unhealthy weight status in survivors of ALL, it is imperative to undertake early identification and implement interventions for at-risk individuals.

A Case of Pulmonary Sarcoidosis Combined with Neurosarcoidosis (신경유육종증이 병발한 폐유육종증 1예)

  • Park, Byung Hoon;Park, Seon Cheol;Shin, Sang Yun;Jeon, Han Ho;Jung, Kyung Soo;Chung, Woo Young;Byun, Min Kwang;Moon, Ji Ae;Kim, Young Sam;Kim, Se Kyu;Chang, Joon;Kim, Sung Kyu;Park, Moo Suk
    • Tuberculosis and Respiratory Diseases
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    • v.62 no.6
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    • pp.549-553
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    • 2007
  • Sarcoidosis is a multi-systemic syndrome of an unknown etiology, and it is characterized by the formation of multiple noncaseating granulomas that disrupt the architecture and function of the tissues in which they reside. The most commonly affected organs are lung, skin and lymph nodes. Overt clinical involvement of the nervous system is uncommon and this occurs in about 5% of all patients during the course of their disease. The most common manifestations are granulomatous leptomeningitis, cranial nerve palsy, electrolyte or other endocrinologic abnormalities, but isolated memory impairment is a rare manifestation. This is a case of 59 years-old male with recent memory impairment, and he was previously diagnosed with pulmonary sarcoidosis by transbronchial lung biopsy. The brain MRI imaging revealed the leptomeningeal and parenchymal involvement of sarcoidosis. He was treated with high dose corticosteroid and his memory function was improved to nearly a normal level. We report here on a case of successful treatment of pulmonary sarcoidosis combined with neurosarcoidosis with using high dose corticosteroid, and the patient presented with recent memory impairment.