• The Korean Journal of Pain
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• v.18 no.1
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• pp.85-88
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• 2005

Encephalitis is known as a rare complication of varicella zoster virus (VZV) reactivation. It is usually regarded as a complication of a cutaneous infection in patients with impaired cellular immunity. The reported incidence of herpetic motor involvement range between 0.5 and 31%, but is possibly more frequent as the weakness is readily obscured by pain. A 53-years-old woman, who presented with severe shoulder pain, fever, headache and seizure, which developed the day after skin eruptions, also developed motor paresis 7 days after the seizure. Her cerebrospinal fluid (CSF) was VZV-Polymerase chain reaction (PCR) negative, but VZV specific IgG antibody positive, and her brain MRI was found to be normal. With the early diagnosis and proper treatment, such as intravenous administration of acyclovir, stellate ganglion block and Yamamoto New Scalp Stimulation (YNSS), the patient completely recovered, without psychoneurological sequelae. Herein, we present this case, with a discussion of the relevant literature on the incidence, pathophysiology, diagnosis and management of central nervous system VZV involvement.

• Brain Tumor Research and Treatment
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• v.6 no.2
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• pp.73-77
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• 2018

Germinoma is the most common type of intracranial germ cell tumors (GCTs). Pineal gland and suprasellar region are the most frequent sites of central nervous system (CNS) involvement. Intracranial masses caused by Langerhans cell histiocytosis (LCH) mimics features of CNS GCTs. LCH frequently involve spine and is the most common cause of vertebra plana in children. A 15-year-old boy presented with progressing symptoms of polydipsia, polyuria, general headache, nausea and severe back pain. Brain MRI showed brain tumor with simultaneous involvement of suprasellar region and pineal gland. An excisional biopsy of suprasellar mass was done. The pathologic assessment confirmed the diagnosis of germinoma. Patient's treatment continued accordingly. A spine MRI, done due to persistent backache, showed a vertebra plana. We reevaluated the primary diagnosis suspecting LCH. Germinoma of CNS was confirmed and a biopsy of vertebral lesion resulted in hemangioma. Thus we report a case of CNS germinoma with co-occurrence of vertebra plana. We emphasized the importance of histopathologic diagnosis of pineal/suprasellar masses and primary investigation of other CNS regions including spine for possible metastasis or comorbidities.

• Childhood Kidney Diseases
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• v.28 no.1
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• pp.44-50
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• 2024

Systemic lupus erythematosus (SLE) is a chronic autoimmune inflammatory disease that affects multiple organs. More than half of the patients with SLE have kidney involvement, and up to 10% of patients with lupus nephritis develop end-stage renal disease (ESRD). Central nervous system (CNS) involvement in SLE occurs in 21% to 95% of patients. Severe neurological manifestations such as seizures, cerebrovascular disease, meningitis, and cerebrovascular accidents can develop in childhood-onset SLE, but cerebral infections, such as brain abscess and hemorrhage, are seldom reported in lupus nephritis, even in adults. Here, we report a rare case of childhood-onset SLE with ESRD, cerebral abscess, and hemorrhage. A 9-year-old girl diagnosed with lupus nephritis was administered high-dose steroids and immunosuppressant therapy to treat acute kidney injury (AKI) and massive proteinuria. The AKI deteriorated, and after 3 months, she developed ESRD. She received hemodialysis three times a week along with daily peritoneal dialysis to control edema. She developed seizures, and imaging showed a brain abscess. This was complicated by spontaneous cerebral hemorrhage, and she became unstable. She died shortly after the hemorrhage was discovered. In conclusion, CNS complications should always be considered in clinical practice because they increase mortality, especially in those with risk factors for infection.

• Journal of Korean Neurosurgical Society
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• v.55 no.1
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• pp.57-60
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• 2014

Rhabdoid tumor of the kidney (RTK) is a rare malignancy in infancy. Central nervous system involvement in RTK is already known. However, solitary spinal metastasis in RTK has been hardly reported. The authors report a case of metastatic RTK to spine causing paraplegia in an 8-month-old girl. Since the patient was young, the diagnosis of spine metastasis was delayed until paraplegia was seen after radical nephrectomy. Thorough neurological examination should be performed for early diagnosis of spinal metastasis in young patients with RTK. If there are any abnormal signs in neurologic examination, magnetic resonance images of brain and spine are recommended.

• Journal of Korean Neurosurgical Society
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• v.57 no.1
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• pp.65-67
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• 2015

Cavernous hemangiomas were first reported in 1929 by Globus and Doshay, and are defined as benign vascular structures developed between the neural tissues occurring in the central nervous system, consisting of a dilated vascular bed. Cavernous hemangiomas comprise nearly 5-12% of all spinal vascular malformations; however, existence in the epidural space without bone involvement is rare. Only 4% of all cavernous hemangiomas (0.22/1.000.000) are purely epidural cavernous hemangiomas. In this case report, we removed a hemorrhagic thoracic mass presenting with progressive neurological deficits in a 55-year-old male patient. We found this case to be appropriate for presentation due to the rare occurrence of this type of cavernous hemangioma.

• The Korean Journal of Cytopathology
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• v.4 no.2
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• pp.156-159
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• 1993

Cryptococcosis is a systemic mycosis that most often involves the central nervous system, followed by subclinical or symptomatic pulmonary involvement. We describe a case of unsuspected pulmonary cryptococcosis in a 69-year-old woman, diagnosed by sputum cytology. Sputum smears revealed numerous yeast like organisms typical of Cryptococcus neoformans measuring from 7 to 17 um in diameter. Mucicarmine, PAS, and methenamine sliver stains showed positive reaction of capsule and cell wall of Cryptococcus neoformans.

• Investigative Magnetic Resonance Imaging
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• v.22 no.3
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• pp.187-193
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• 2018

Sweet's syndrome also known as acute neutrophilic dermatosis is a multisystem inflammatory disorder characterized by fever, malaise, leukocytosis, and skin lesions. Sweet's syndrome affects multiple organs though only rarely does it affect the central nervous system (CNS) when it does it is called Neuro-Sweet disease (NSD). We report on a case study of a biopsy-proven NSD in a 50 year old man. Serial magnetic resonance imaging (MRI) showed repeated CNS involvement of Sweet's syndrome after a respiratory tract infection preceded it. On the MRI, T2 hyperintense lesions occurred at multiple sites and disappeared after steroid therapy.

• 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 Genetic Medicine
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• v.18 no.1
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• pp.16-23
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• 2021

GLB1-related disorders comprise two phenotypically unique disorders: GM1 gangliosidosis and Morquio B disease. These autosomal recessive disorders are caused by b-galactosidase deficiency. A hallmark of GM1 gangliosidosis is central nervous system degeneration where ganglioside synthesis is highest. The accumulation of keratan sulfate is the suspected cause of the bone findings in Morquio B disease. GM1 gangliosidosis is clinically characterized by a neurodegenerative disorder associated with dysostosis multiplex, while Morquio B disease is characterized by severe skeletal manifestations and the preservation of intelligence. Morquio B disease and GM1 gangliosidosis may be on a continuum of skeletal involvement. There is currently no effective treatment for GLB1-related disorders. Recently, multiple interventions have been developed and there are several ongoing clinical trials.

• Journal of Genetic Medicine
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• v.19 no.2
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• pp.57-62
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• 2022

Systemic autoinflammatory diseases (SAIDs) are characterized by unprovoked inflammatory episodes such as recurrent/periodic fever, serositis, skin lesions, abdominal symptoms, arthritis/arthralgia, and central nervous system involvement. Genetic diagnosis of SAIDs has been challenging because disease manifestations overlap among themselves and with other immunological disease categories, such as infection and autoimmune diseases. However, the advent of next-generation sequencing (NGS) technologies and expanding knowledge about the innate immunity and inflammation have made the routine genetic diagnosis of SAIDs possible. Here, we review the recurrent/periodic fevers, other recently identified autoinflammatory diseases, and type I interferonopathies, and discuss the clinical usefulness of NGS targeted sequencing for SAIDs, and recent advance of understandings for this heterogeneous disease group as for underlying primary immunodeficiency.