Progressive Supranuclear Palsy (PSP) is a rare degenerative disease showing supranuclear ophthalmoplegia, rseudobulbar palsy and several parkinsonian features. This is , as far as the author's knowledge, the first case in this country. The patient is a 55 year old female with slowly progressive disturbances in walking, swallowing, speech and behaviors. The most characteristic finding is the prominent external ophthalmoplegia, especially vertical and downward gaze, which can be easily overcome by the oculocephalic reflexes (Doll's eye movements). The dyspagia, dysarthria and emotional lability may suggest the pseudobulbar palsy. General hyper-reflexia and Babinski sign are noted. There are several features of parkinsonism, but the rigidity is more severe in the trunk than in the extremities and the posture is rather hyperextended than scooped. There is no tremor. The disease is slowly progressive and the medical treatment for Parkinson's disease is not effective.
Deglutition ; Dysarthria ; Extremities ; Female ; Humans ; Middle Aged ; Ophthalmoplegia ; Paralysis ; Parkinson Disease ; Parkinsonian Disorders ; Posture ; Pseudobulbar Palsy ; Reflex ; Reflex, Babinski ; Supranuclear Palsy, Progressive* ; Tremor ; Walking

In reflex epilepsy, seizures triggered by eye closure are uncommon but interesting. The clinical significance and the mechanisms of triggering epileptiform discharges on closing on closing the eyes are not clearly defined yet. Here, five cases are described in which the epileptiform discharges provoked by voluntary closing the eyes. The clinical manifestations are variable in these cases and this electroencephalographic abnormality is not specific for any kind of seizures.
Epilepsy, Reflex ; Seizures

Inflammatory pseudotumor is an uncommon nonneoplastic lesion of unknown etiology that occurs most commonly in the lung. However, it also occurs in diverse extrapulmonary locations: abdomen, retroperitoneum, pelvis, heart, head and neck, upper respiratory tract, trunk, bladder, and extremities. The extrapulmonary inflammatory pseudotumor is often larger, less-well circumscribed and multinodular. Proximity of the tumor to vital structures or involvement of vital organs compromises the opportunity for complete resection; thus higher recurrence rates are often reported even after surgical treatment. The authors report a case of inflammatory pseudotumor originating from common carotid artery in a 42-year-old female patient with a rapidly growing neck mass, treated by en bloc resection of inflammatory pseudotumor and a long segment of common carotid artery followed by polytetrafluoroethylene(PTFE) graft interposition.
Abdomen ; Adult ; Carotid Arteries* ; Carotid Artery, Common ; Extremities ; Female ; Granuloma, Plasma Cell* ; Head ; Heart ; Humans ; Lung ; Neck ; Pelvis ; Recurrence ; Respiratory System ; Transplants ; Urinary Bladder

We have previously shown that the inhibition of fibroblast growth factor (FGF) signaling induced endodermal gene expression in the animal cap and caused the expansion of the endodermal mass in Xenopus embryos. However, we still do not know whether or not the alteration of FGF signaling controls embryonic cell fate, or when FGF signal blocking is required for endoderm formation in Xenopus. Here, we show that FGF signal blocking in embryonic cells causes their descendants to move into the endodermal region and to express endodermal genes. It is also interesting that blocking FGF signaling between fertilization and embryonic stage 10.5 promotes endoderm formation, but persistent FGF signaling blocking after stage 10.5 restricts endoderm formation and differentiation.
Animals ; Endoderm/drug effects/embryology/*metabolism ; Fibroblast Growth Factors/antagonists & inhibitors/genetics/*physiology ; Gene Expression Regulation, Developmental/drug effects ; In Situ Hybridization ; Pyrroles/administration & dosage/pharmacology ; Receptors, Fibroblast Growth Factor/genetics/physiology ; Reverse Transcriptase Polymerase Chain Reaction ; Signal Transduction/drug effects ; Xenopus Proteins/antagonists & inhibitors/genetics/*physiology ; Xenopus laevis/embryology/genetics/*physiology

PURPOSE: Infliximab is currently used for the treatment of active Crohn's disease (CD). We aimed to assess the efficacy and safety of infliximab therapy and to determine the predictors of response in Korean patients with CD. MATERIALS AND METHODS: A total of 317 patients who received at least one infliximab infusion for active luminal CD (n=198) and fistulizing CD (n=86) or both (n=33) were reviewed retrospectively in 29 Korean referral centers. Clinical outcomes of induction and maintenance therapy with infliximab, predictors of response, and adverse events were evaluated. RESULTS: In patients with luminal CD, the rates of clinical response and remission at week 14 were 89.2% and 60.0%, respectively. Male gender and isolated colonic disease were associated with higher remission rates at week 14. In week-14 responders, the probabilities of sustained response and remission were 96.2% and 93.3% at week 30 and 88.0% and 77.0% at week 54, respectively. In patients with fistulizing CD, clinical response and remission were observed in 85.0% and 56.2% of patients, respectively, at week 14. In week-14 responders, the probabilities of sustained response and remission were 94.0% and 97.1%, respectively, at both week 30 and week 54. Thirty-nine patients (12.3%) experienced adverse events related to infliximab. Serious adverse events developed in 19 (6.0%) patients including seven cases of active pulmonary tuberculosis. CONCLUSION: Infliximab induction and maintenance therapy are effective and well tolerable in Korean patients with luminal and fistulizing CD. However, clinicians must be aware of the risk of rare yet critical adverse events.
Colonic Diseases ; Crohn Disease* ; Humans ; Infliximab* ; Male ; Phenobarbital ; Referral and Consultation ; Retrospective Studies ; Tuberculosis ; Tuberculosis, Pulmonary