Distinct from migraine with aura or hemiplegic migraine, a rare clinical entity of migraine-like headache, transient focal neurologic deficit and CSF pleocytosis (HaNDL) has been known. Although the etiology or pathogenesis is unknown, possibility of viral infection or inflammation has been suggested. We report a 25-year-old man diagnosed as HaNDL with literature review. (J Korean Neurol Assoc 19(3):302~304, 2001)
Adult ; Aphasia ; Headache* ; Humans ; Inflammation ; Leukocytosis* ; Migraine Disorders ; Migraine with Aura ; Neurologic Manifestations*

No abstract available.
Pneumonia*

No abstract available.
Encephalitis* ; Glioma* ; Recurrence*

BACKGROUND AND PURPOSE: Hypertrophic pachymeningitis (HP) is a rare disease caused by autoimmunity in the meninx that causes various neurologic symptoms, including headache, seizures, weakness, paresthesia, and cranial nerve palsies. Although the first-line therapy for HP is steroids, many HP cases are refractory to steroids or recur when the steroids are tapered. Here we report three HP cases that were successfully treated with rituximab (RTX). METHODS: From an institutional cohort recruited from April 2012 to July 2016, three HP cases that were identified to be steroid-refractory were treated with RTX (four weekly doses of 375 mg/m²). Clinical improvement was assessed by the number of relapses of any neurologic symptom and the largest dural thickness in MRI. RESULTS: All three patients were recurrence-free of neurologic symptoms and exhibited prominent decreases in the dural thickness after RTX treatment. No adverse events were observed in the patients. CONCLUSIONS: We suggest RTX as a second-line therapy for steroid-refractory HP. Further studies are warranted to confirm this observation in a larger population and to consider RTX as a first-line therapy.
Autoimmunity ; Cohort Studies ; Cranial Nerve Diseases ; Headache ; Humans ; Magnetic Resonance Imaging ; Meningitis* ; Neurologic Manifestations ; Paresthesia ; Rare Diseases ; Recurrence ; Rituximab* ; Seizures ; Steroids

BACKGROUND: Intracerebral hemorrhage (ICH) is associated with a considerable proportion of stroke and head injuries, but except for supportive care, there is no medical therapy available. Transplantation of human neural stem cells (NSCs) can be used to reduce behavioral deficit in experimental ischemic infarct model. However, effect of stem cell transplantation in experimental intracerebral hemorrhage (ICH) is unknown. We hypothesized that NSCs could migrate and differentiate into neurons or glial cells, and improve functional outcome in ICH. METHODS: Experimental ICH was made by intrastriatal administration of bacterial collagenase in adult rats. Animals were randomized to receive intravenously either immortalized Lac-Z positive human NSCs (5x1 06 in 500microL, n=15) or same volume of saline (n=12) on the following day. Animals were evaluated for 8 weeks after surgery with behavioral test battery. After 8 weeks, animals were sacrificed and the brains were sectioned. Transplanted NSCs were detected by X-gal histochemistry or beta-gal immunohistochemistry, and differentiation of grafted NSCs were evaluated by double labeling of GFAP, NeuN, or neurofilament. RESULTS: Transplanted NSCs migrated to the side of peri-hematomal areas, and differentiated into neurons and astrocytes. NSCs injection group showed improved performances on rotarod test after 2 weeks and on limb placing test after 5 weeks compared with control group (p<0.05) and these effect persisted up to 8 weeks. CONCLUSIONS: Intravenously injected NSCs enter rat brain with ICH, and differentiate into astrocytes or neuronal cell, which lead to functional recovery. These findings show the possibility that NSCs can be used to reduce neurological deficits in the experimental ICH.
Adult ; Animals ; Astrocytes ; Brain ; Cerebral Hemorrhage* ; Collagenases ; Craniocerebral Trauma ; Extremities ; Humans* ; Immunohistochemistry ; Neural Stem Cells* ; Neuroglia ; Neurons ; Rats ; Rotarod Performance Test ; Stem Cell Transplantation ; Stroke ; Transplants

Rheumatoid arthritis (RA) is an autoimmune disease involving the synovial membrane of peripheral joints. T cells specific for self antigens may play a critical role. Identification of T cell receptors (TCR) of such specific T cell clones is very important for treatment, prevention and identification of relevant autoantigens. To identify specific T cells, TCR V beta family repertoire and the clonal expansion of T cells were analyzed in this study. The percentage of V beta 5+ or V beta 8+ cells in the synovial fluid mononuclear cells (SFMCs) was similar to that in the peripheral blood mononuclear cells (PBMCs). However, the percentage of DR+ T cells in the SFMCs was higher (p< 0.01). Analyzing the clonality of T cells in 8 V beta families (V beta 1, V beta 5, V beta 8, V beta 14, V beta 16, V beta 17, V beta 18, V beta 20), clonal expansions in CD8+ T cells from the SFMCs were found more frequently than in the PBMCs. The patterns of clonal expansions were discrepant between the SFMCs and the PBMCs even in the same patient, which suggests several inflamed tissue specific T cell clonal expansions in the SFMCs. These T cell clones might be activated by autoantigens which are not identified yet and responsible for the RA pathogenesis.
Arthritis, Rheumatoid/*metabolism/pathology ; Base Sequence ; Blood Cells/*metabolism ; Clone Cells ; Female ; Human ; Male ; Molecular Probes ; Molecular Sequence Data ; Polymerase Chain Reaction ; Receptors, Antigen, T-Cell/genetics/*metabolism ; Support, Non-U.S. Gov't ; Synovial Fluid/cytology/*metabolism ; T-Lymphocytes/*metabolism

Meningioma is generally regarded as benign and extracranial metastasis of intracranial meningioma is rare. About 60 cases of distant metastasis of meningioma have now been reported. Angioblastic meningioma, although a rare type, shows rapid growth and sarcomatous change frequently, and has higher incidence of metastasis than other types of meningioma. The authors report a case of malignant angioblastic meningioma arisen in left olfactory groove with multiple metastasis to skeletal system. The available literature regarding these tumors is reviewed.
Incidence ; Meningioma* ; Neoplasm Metastasis*

Anti-leucine-rich glioma inactivated-1 (LGI1) limbic encephalitis (LE) is a rare neurological disorder that has a subacute course of progressive encephalopathy and fasciobrachial dystonic seizures. We report a patient with anti-LGI1 LE that presented with atypical manifestations that complicated the diagnosis. A 62-year-old woman presented with a chronic course of memory disturbance and a subsequent relapse with an altered mental status after 10 months. The patient reported frequent chest pain of squeezing and dull nature, typically lasting 10-30 seconds. The chest pain was related to partial seizures, which were confirmed by video-EEG monitoring. Anti-LGI1 antibody was identified in serum and CSF. The patient's symptoms improved by immune modulation treatment. Patients with anti-LGI1 LE can experience atypical partial seizures, and a chronic relapsing course. Clinical suspicions and video-EEG monitoring are helpful for the early diagnosis and effective immune modulation.
Chest Pain ; Diagnosis ; Early Diagnosis ; Female ; Glioma ; Humans ; Limbic Encephalitis* ; Memory ; Middle Aged ; Nervous System Diseases ; Recurrence ; Seizures