• Annals of Clinical Neurophysiology
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• v.4 no.1
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• pp.60-62
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• 2002

Two separate cranial nerve variants of Guillain-Barre syndrome(GBS) have been reported. One is Miller-Fisher syndrome, the other is polyneuritis cranialis. Involvement of the extraocular muscles in variants of GBS is well recognized, but complete external and internal opthalmoplegia is rare. Optic neuritis remains the only consistent, albeit very uncommon, evidence of inflammation of central nervous system myelin in GBS. This propose that GBS is part of a spectrum of central and peripheral inflammation. This case is an unusual clinical variant who had ptosis, opthalmoplegia, areflexia, ataxia, optic neurritis, marked oropharyngeal, and neck and shoulder weakness. This combined regional from is able to misdiagnose initially as botulism or diphtheria and less so, myasthenia. So if we were consider variant from of GBS, it is possible for make a correct diagnosis more easily and treatment without delay.

• Journal of The Korean Ophthalmological Society
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• v.59 no.11
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• pp.1097-1102
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• 2018

Purpose: To report a case of toxic optic neuropathy caused by chlorfenapyr ingestion accompanied by central nervous system involvement. Case summary: A 44-year-old female visited our clinic complaining of reduced visual acuity in both eyes for 7 days. She had ingested a mouthful of chlorfenapyr for a suicide attempt 2 weeks prior to the visit. Gastric lavage was performed immediately after ingestion at the other hospital. Her best-corrected visual acuity was finger count 30 cm in the right eye and hand motion in the left eye. Both pupils were dilated by 5.0 mm and the response to light was sluggish in both eyes. A relative afferent pupillary defect was detected in her left eye. Funduscopy revealed optic disc swelling in both eyes. Magnetic resonance imaging of the brain showed a symmetric hyper-intense signal in the white matter tract including the internal capsule, corpus callosum, middle cerebellar peduncle, and brainstem. The patient was diagnosed with toxic optic neuropathy induced by chlorfenapyr ingestion, and underwent high-dose intravenous corticosteroid pulse therapy. Three days later, the best-corrected visual acuity was no light perception in both eyes. Three months later, optic atrophy was observed in both eyes. Optical coherence tomography revealed a reduction in the thicknesses of the retinal nerve fiber layer and ganglion cell and inner plexiform layer in the macular area. Conclusions: Ingestion of even a small amount of chlorfenapyr can cause severe optic nerve damage through the latent period, despite prompt lavage and high-dose steroid treatment.

• Clinical and Experimental Pediatrics
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• v.52 no.6
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• pp.725-729
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• 2009

Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) with central nervous system (CNS) involvement is usually fatal unless stem cell transplant (SCT) is offered. However, SCT with conventional intensity conditioning is associated with high transplant-related mortality. We describe our experience with unrelated SCTs after reduced-intensity conditioning (RIC) for patients with EBV-HLH with progressive CNS disease. This approach was associated with minimal toxicities and might be an effective option in patients with EBV-HLH with progressive CNS disease. Moreover, the addition of rituximab to RIC appears to be safe and effective in suppressing EBV in the patients with EBV-HLH.

• Journal of Yeungnam Medical Science
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• v.15 no.2
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• pp.371-380
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• 1998

Systemic lupus erythematosus(SLE) is an autoimmune disease which may affect many different organs and disclose various clinical manifestations. Recently central nervous system(CNS) involvement has been recognized as an increasingly significant contributor to morbidity and mortality of SLE. The clinical manifestations of CNS-lupus are highly variable and range from mild cognitive dysfunction, movement disorder, headache, psychosis to life-threatening stroke and coma. Among the neuropsychiatric disorders encountered in patients with SLE, cerebrovascular disease has been a relatively rare complication. The diagnosis and management of CNS-lupus is difficult because of the lack of useful diagnostic methods. If cerebrovascular involvement is suspected, aggressive treatment such as high dose steroid, immunosuppressive therapy, plasma exchange may be required to reduce high mortality rate. We experienced 2 cases cerebrovascular dis eases occurring in SLE patients which presented with various neuropsychiatric manifestations. They were diagnosed as CNS-lupus by neuropsychiatric symptoms, brain MRI, and BEG, and showed good response to high dose steroid pulse therapy.

• Journal of Korean Neurosurgical Society
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• v.30 no.sup1
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• pp.124-127
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• 2001

Langerhans' cell histiocytosis is a rare disease showing proliferation of histiocytes in multiple organ system. Bone lesions are the most common radiolologic manifestation of Langerhans' cell hysticytosis. Extraskeletal sites of Langerhans' cell hystiocytosis involvement include the skin, lymph nodes, thymus, lungs, central nervous system, liver, pancrease, spleen, and bowel. The authors have experienced a case of Langerhans' cell histiocytosis which involved the skull. A 3-year-old female presented with soft tissue mass on right periorbital area. Plain skull X-ray showed punched out bone lesion. Computed tomography showed non-enhancing osteolytic lesion on right frontal skull. Histologic findings of an excised mass revealed pathologic features of Langerhans' cell histiocytosis.

• Clinical and Experimental Pediatrics
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• v.46 no.10
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• pp.1040-1043
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• 2003

Henoch-Shonlein purpura(HSP) is a systemic small-vessel vasculitis that primarily affects the skin, gastrointestinal tract, joints, and kidneys. The nervous system may be involved, less commonly than other organs. When the central nervous system(CNS) was involved, headache, changes in mental status, seizures, and focal neurologic deficits have been reported. Hypertension, uremic encephalopathy, metabolic abnomalities, electrolyte abnormalities, or cerebral vasculitis were suggested as possible causes of the neurologic manifestation. Diagnosis of vasculitic involvement of CNS is difficult. Magnetic resonance imaging of the brain is the modality of choice for the evaluation of the CNS disease. Steroid or plasmapheresis are used in treatment of cerebral vasculitis. We experienced a case of 9-year-old boy who had presented with Henoch-Schonlein purpura nephritis complicating encephalopathy accompanied by hypertension and cerebral vasculitis. Brain MRI showed multiple small nodular-linear pattern enhancing lesions in whole cerebral hemispheres and focal increased T2 signal in the right basal ganglia. We used intravenous immunoglobulin in treatment of cerebral vasculitis. We report this case with a brief review of related literature.

• Annals of Clinical Neurophysiology
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• v.4 no.2
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• pp.119-124
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• 2002

Background : Guillain-$Barr{\acute{e}}$ syndrome(GBS) is a neurologically emergent condition, leading to respiratory insufficiency without an early and appropriate treatment. Thus, the treatment of GBS requires early diagnosis but it is difficult due to the low sensitivity of laboratory tools in the initial stage. Hoffman reflex (H-reflex) and its facilitation by Jendrassik maneuver (JM) are sensitive tools evaluating the central circuit of motor system on the spinal cord level. The aim of this study is to test whether the change of H-reflex and F-wave under the JM is able to detect the early stage of GBS and whether GBS involves the central nervous system (CNS). Material and Methods : All 7 GBS patients who showed normal or nearly normal nerve conduction study were included. The facilitation of H-reflex and changes of F-wave were calculated by measuring the percent difference of H-reflex or F-wave amplitude under JM compared to basal H-reflex of F-wave amplitude. The changes of F-wave and H-reflex in the GBS patients were compared with them of 8 healthy controls. Results : The F-wave amplitudes of both healthy controls and GBS patients did not changed under the influence of JM ($102.4{\pm}24.9%$, $108.7{\pm}29.0%$ respectively). However, the facilitation of H-wave by JM in the GBS patients was absent ($98.8{\pm}5.8%$), even though the H-reflex amplitude I the healthy controls increased under the influence of JM ($124.8{\pm}12.1%$). Conclusions : The loss of H-reflex facilitation in GBS implies that this phenomenon might be the most early change detected in the electrophysiological study and support the early diagnosis, and that GBS might include lesion in the spinal motor CNS.

• Journal of Yeungnam Medical Science
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• v.26 no.2
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• pp.130-136
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• 2009

Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis that typically affects the medium-sized muscular arteries, with occasional involvement of the small muscular arteries. As with other vasculitides, PAN can affect any organ system, including the cardiovascular, gastrointestinal and central nervous systems. The prognosis for patients with untreated PAN is relatively poor, with five-year survival rates of approximately 13 percent. The outcome has improved with proper therapy to approximately 80 percent survival at five years. We report here on a case of a 46 year old man with polyarteritis nodosa and who suffered from pulmonary tuberculosis.

• 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.

• 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.