• Journal of Korean Neurosurgical Society
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• v.51 no.3
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• pp.170-172
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• 2012

Central nervous system (CNS) sparganosis is a rare parasitic infestation caused by ingestion of the raw or inadequately cooked snakes or frogs. Sparganum is well known for its ability of migrating though the tissue, therefore, it can cause various neurological symptoms if it involves neurological systems. A 51-year-old male patient visited our department of neurosurgery complaining of the motor weakness and radiating pain on both upper extremities over 4 months. He had a history of ingesting raw snakes untill his late twenties. The magnetic resonance (MR) images of. cervical spine revealed an intramedullary ill-defined enhancing lesion with the aggregated cysts in the upper cervical spinal cord. Under presumptive diagnosis of sparganosis, we took brain MR image. The brain MR images revealed the signal change in right fronto-temporallobe suggesting the trajectory of parasitic migration via ventricular systems. He underwent a midline myelotomy and granuloma removal followed by the posterior laminoplasty. Pathologic findings showed inflammatory changes and necrosis with keratinized tissue suggesting the CNS sparganosis. We report an uncommon case of CNS sparganosis migrated from the brain to the spinal cord with literature review.

• Journal of Korean Neurosurgical Society
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• v.52 no.4
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• pp.325-333
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• 2012

Objective : The indications and optimal surgical treatments for intracranial cysts are controversial. In the present study, we describe long-term clinical and neuroimaging results of surgically treated intracranial cysts in children. The goal of this study is to contribute to the discussion of the debate. Methods : This study included 110 pediatric patients that underwent surgeries to treat intracranial cysts. Endoscopic cyst fenestrations were performed in 71 cases, while craniotomies and cyst excisions (with or without fenestrations) were performed in 30 patients. Cystoperitoneal shunts were necessary for nine patients. Long-term results were retrospectively assessed with medical and neuroimaging records. Results : Clinical and radiological improvement was reported in 87.3% and 92.8% of cases, respectively, after endoscopic neurosurgery, and in 93.3% and 100% using open microsurgery whereas 88.9% and 85.7% after shunt operation. There were no statistical differences in clinical outcomes (p=0.710) or volume reductions (p=0.177) among the different surgeries. There were no mortalities or permanent morbidities, but complications such as shunt malfunctions, infections, and subdural hematomas were observed in 56% of the patients that had shunt operations. A total of 13 patients (11.8%) underwent additional surgeries due to recurrences or treatment failures. The type of surgery performed did not influence the recurrence rate (p=0.662) or the failure rate (p=0.247). Conclusion : Endoscopic neurosurgeries are less invasive than microsurgeries and are at least as effective as open surgeries. Thus, given the advantages and complications of these surgical techniques, we suggest that endoscopic fenestration should be the first treatment attempted in children with intracranial cysts.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.32 no.1
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• pp.81-85
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• 2010

Multiple jaw cysts are one of the most constant features of the basal cell nevus syndrome. Basal cell nevus syndrome is inherited as an autosomal dominant trait with variable expressiveness. This syndrome comprises a number of abnormalities such as multiple nevoid basal cell carcinomas of the skin, skeletal abnormalities as bifid rib and fusion of vertebrae, central nervous system abnormalities as mental retardation, eye abnormalities with multiple jaw cysts. The odontogenic keratocysts in patients with this syndrome are often associated with the crowns of unerupted teeth and huge size; on radiographs they may mimic dentigerous cysts. The most important feature of the cyst is its extraordinary recurrence rate. Since recurrence may be long delayed in this lesion, follow-up of any case of odontogenic keratocyst with roentgenograms and clinical examination of basal cell carcinoma are essential for at least five years after surgery. We report the result of 7-year follow up after cyst enucleation associated with basal cell nevus syndrome with the literature of review.

• Journal of Genetic Medicine
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• v.14 no.2
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• pp.67-70
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• 2017

Down syndrome (DS, trisomy 21) is associated with neuroanatomical abnormalities, including choroid plexus cysts and various types of brain tumors. Trisomy 21 is associated with oncogenic factor, especially in brain tumor. The brain of DS patients had a smaller volume of gray and white matter and an unbalanced cerebellum volume, indicating a smaller volume overall than normal. We report a case of a DS male patient who had an incidentally discovered neuroglial cyst in left cerebellar vermis. He visited our hospital with gait disturbance and fatigue. But, the neurologic exam was normal. To the best of our knowledge, this is the first reported case of a neuroglial cyst in a trisomy 21 patient. As the developmental mechanisms of a cyst and the choroid plexus are related, more research is needed.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.28 no.2
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• pp.136-140
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• 2002

Basal cell nevus syndrome is inherited as an autosomal dominant trait with variable expressivity. This syndrome comprises a number of abnormalities such as multiple nevoid basal cell carcinomas of the skin, skeletal abnormalities as bifid rib and fusion of vertebrae, central nervous system abnormalities as mental retardation, eye abnormalities, and multiple odontogenic kerato cysts. In 1960, Gorlin and Goltz first described the features of this disease as constituting a true syndrome; since then, it has been realized that it is much more complex and encompassing than initially thought. This patient has many symtoms of basal cell nevus syndrome. - we has known multiple jaw cysts through panorama and facial computed tomography. He has hyperchromatism on basal cell through skin biopsy. In ophthalmologic consult, he has blindness on right. On his past medical history, amputation was done on his toes for polyductalism. - So we report with literature reviews

• Investigative Magnetic Resonance Imaging
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• v.13 no.1
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• pp.54-62
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• 2009

Purpose : Craniospinal neurenteric (NE) cysts are rare developmental non-neoplastic cysts of the central nervous system with diverse MR imaging findings. The purpose of this study was to evaluate various MR imaging findings of intracranial and intraspinal NE cysts. Materials and Methods : We retrospectively reviewed the MR imaging findings of six NE cysts that were confirmed by pathology. We evaluated anatomic location, signal intensity, size and enhancement pattern of NE cysts. Results : Two intracranial lesions were located extra-axially in the cerebellopontine angle and quadrigeminal cisterns. Three spinal lesions were intraduralextramedullary cysts, located ventral to the spinal cord, but one thoracic lesion was an intramedullary cyst. The signal intensity of the cysts was hyperintense on T1-weighted images as compared with the cerebrospinal fluid (CSF) for two intracranial lesions and one cervical lesion. In addition, all intracranial lesions showed diffusion restriction. For the remaining three spinal lesions, the signal intensity was nearly the same as the signal intensity of the CSF as seen on both T1- and T2-weighted images. On contrast-enhanced studies, two intracranial cysts showed a small nodular enhancement and one thoracic spinal lesion showed rim enhancement. Conclusion : NE cysts have various locations, signal intensities, and possible focal nodular or rim enhancement. Therefore, NE cysts can be included in the differential diagnosis of various craniospinal cystic or tumorous cystic lesions.

• 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.

• Journal of Korean Neurosurgical Society
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• v.38 no.6
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• pp.478-480
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• 2005

The authors describe a case of intracranial epidermoid cyst with fourth ventricle involvement. A 43-year-old woman presented with progressive aggravating headache and cerebellar syndrome due to a large tumor in the fourth ventricle, that extended into the left side of foramen of Luschka. The histopathological workup following gross total removal of tumor had revealed the typical features of an epidermoid cyst. Four months after surgery, the cerebellar sings recovered to normal with a remarkable improvement of headache. Epidermoid cysts involving the fourth ventricle have been known to be one of the most rare diseases in the central nervous system. This rare case is discussed and a review of the relevant literature is presented.

• Journal of Korean Neurosurgical Society
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• v.62 no.6
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• pp.723-726
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• 2019

We report two rare cases of spontaneously regressed Rathke's cleft cyst (RCC). A 52-year-old woman presented with headache. A pituitary hormone study was normal. Brain magnetic resonance imaging (MRI) showed a $0.45-cm^3$ cystic sellar lesion. The cyst was hyperintense on T1-weighed imaging and hypointense on T2-weighted imaging without rim enhancement, comparable to a RCC. Six months later, brain MRI showed no change in the cyst size. Without any medical treatments, brain MRI 1 year later revealed a spontaneous decrease in cyst volume to $0.05cm^3$. A 34-year-old woman presented with headache and galactorrhea lasting 1 week. At the time of the visit, the patient's headache had disappeared. Her initial serum prolactin level was 81.1 ng/mL, and after 1 week without the cold medicine, the serum prolactin level normalized to 11.28 ng/mL. Brain MRI showed a RCC measuring $0.71cm^3$. Without further treatments, brain computed tomography 6 months later showed a spontaneous decrease in cyst volume to $0.07cm^3$. Another 6 months later, brain MRI revealed that the cyst had remained the same size. Neither patient experienced neurological symptoms, such as headache or visual disturbance, during the period of cyst reduction. The RCCs in both patients underwent spontaneous regression without any medical treatment during a period of 6 months to 1 year. Although spontaneous regression of a RCC is rare, it is still possible and a sufficient follow-up period should be considered.

• Asian Pacific Journal of Cancer Prevention
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• v.16 no.5
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• pp.1977-1980
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• 2015

Objective: The Von Hippel-Lindau syndrome (VHLD), an inherited neoplastic syndrome predisposing to central nervous system hemangioblastoma (CNS), pheochromocytoma (PCC), renal cell carcinoma(RCC), retinal hemangioma (RA) and renal cysts, is caused by mutations or deletions of the VHL tumor-suppressor gene. To assess VHL genotype-phenotype correlations with function of pVHL a gene mutation analysis of members in a Chinese family with non-syndromic PCCs and individuals with apparently sporadic pheochromocytoma (ASP) was performed. Materials and Methods: DNA samples of 20 members from the Chinese family with non-syndromic PCCs and 41 patients with ASP were analyzed by polymerase chain reaction and direct sequencing, confirmed by Taqman probe. Results: Three novel mutations (H125P, 623(^TTTGTtG) and R120T) were identified in the Chinese family and in 3 among 41 ASP patients. The mutations were all located in exon 2 of VHL gene encoding ${\beta}$-domain of pVHL. The tumor type in H125P carriers and R120T carriers was VHL type 2C. And 623(^TTTGTtG) carriers presented VHL type 2B or type 2C. Conclusions: VHL gene abnormalities were identified in the Chinese family with non-syndromic PCCs and patients with APS, resulting in dysfunction of pVHL. H125P and R120T could be associated with VHL type 2C, while 623(^TTTGTtG) might be linked with VHL type 2B or type 2C. Not only is the genetic analysis helpful for early diagnosis and treatment of patients with VHLD, it is also benefitial for research intoVHLD pathogenesis.