Creutzfeldt-Jakob disease(CJD) is characterized clinically by rapidly progressive dementia with pyramidal, extrapyramidal, and cerebellar symptoms and signs, and histologically by spongiform change, neuronal loss and reactive gliosis. We have experienced 2 cases of CJD. Case 1 was a 36-year-old male who had suffered from myoclonus and cerebellar symptoms including sluggish speech, gait and balance disturbance. Case 2 was a 70-year-old female who had showed cognitive dysfunction, ataxic gait and disturbance of extraocular movement. Both patients, underwent brain biopsy. Case 1 revealed marked cortical atrophy, 2mm in thickness, with neuronal loss and astrocytic proliferation extending into white matter. The spongiform change, made up of many small, usually rounded or oval, vacuoles was noted mainly in the neuropil. Case 2 revealed remarkable spongiform change throughout the cortex and cytoplasmic vacuoles compressing the nuclei of neuronal cells were numerous. Neuronal loss and gliosis were also found without considerable change in the white matter. On double immunostaining against GFAP and PrP(Prion Protein), there was a weak positive reaction for PrP in the perinuclear cytoplasm in case 1, and a strongly positive reaction in case 2. The electron microscopic examination showed numerous membrane-bound vacuoles in neuropil and perikarya of neurons. The majority of the vacuoles were multiseptated by thin membranous structures. They demonstrated curled, or disrupted membrane, that had foldings and protrusions into the vacuolar clear spaces. There were neither identifiable virus-like particles nor amyloid deposition.
Female ; Male ; Humans

Polyarteritis nodosa (PAN) is a chronic inflammatory disorder characterized by a necrotizing vasculitis of small and medium-sized artery, which can involve several organs, such as, the kidney, skin, peripheral nerves, muscle, heart, and gastrointestinal tract. In addition to systemic involvement of the classical PAN, a localized disease of the gallbladder, uterus, testis, and skin has been reported. In particular, a limited involvement of the breast is an unusual manifestation of PAN and usually runs a favorable prognosis when compared with the classical PAN. We describe a 64-year-old female patient who had a limited disease of PAN on both the breasts. She presented with mass-like lesions on both the breasts. After surgical excision of the breast lesions, she has been doing well with only a low dosage of prednisolone.
Arteries ; Breast* ; Female ; Gallbladder ; Gastrointestinal Tract ; Humans ; Kidney ; Middle Aged ; Myocardium ; Peripheral Nerves ; Polyarteritis Nodosa* ; Prednisolone ; Prognosis ; Skin ; Testis ; Uterus ; Vasculitis

Polymyositis is one form of inflammatory myopathy. In some patients, this disease does not entirely respond to conventional initial therapy with glucocorticoid, methotrexate and azathioprine. Multiple options exist for treating these patients, but only intravenous immune globulin has been subjected to a randomized clinical trial. We report here on a case of polymyositis that did not respond to multiple drug therapy, but it did respond to tacrolimus. After treatment with tacrolimus, the patient's disease has been well controlled for many years.
Azathioprine ; Humans ; Immunoglobulins, Intravenous ; Methotrexate ; Myositis ; Polymyositis ; Tacrolimus

A 49-year-old male presented with chills and a fever. Five years previously, he underwent ascending aorta and aortic arch replacement using the elephant trunk technique for DeBakey type 1 aortic dissection. The preoperative evaluation found an esophago-paraprosthetic fistula between the prosthetic graft and the esophagus. Multiple-stage surgery was performed with appropriate antibiotic and antifungal management. First, we performed esophageal exclusion and drainage of the perigraft abscess. Second, we removed the previous graft, debrided the abscess, and performed an in situ re-replacement of the ascending aorta, aortic arch, and proximal descending thoracic aorta, with separate replacement of the innominate artery, left common carotid artery, and extra-anatomical bypass of the left subclavian artery. Finally, staged esophageal reconstruction was performed via transthoracic anastomosis. The patient's postoperative course was unremarkable and the patient has done well without dietary problems or recurrent infections over one and a half years of follow-up.
Abscess ; Aorta ; Aorta, Thoracic ; Brachiocephalic Trunk ; Carotid Artery, Common ; Chills ; Drainage ; Elephants ; Esophagus ; Fever ; Fistula* ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Subclavian Artery ; Transplants*

Eccrine angiomatous hamartoma (EAH) is a rare benign disease that is characterized by an abnormal proliferation of eccrine glands and vascular elements. It is generally congenital, but it can appear before puberty. It usually presents as a single plaque or nodule, but multiple patch-like lesions are also possible. EAH is mostly asymptomatic, but it is sometimes associated with pain or hyperhidrosis. It generally does not require aggressive treatment, but the lesion can be excised due to pain, enlargement and cosmetic reasons. A 3-week-old Korean female presented with a hemorrhagic skin lesion on the right foot since birth. There was no specific birth history. The lesion first appeared on the third toe of the right foot and quickly spread to almost half of the right foot. Histopathology examination revealed acanthosis in the epidermis and a proliferation of eccrine ducts, glands and capillaries. The eccrine glands were immunohistochemically-positive for carcinoembryonic antigen.
Capillaries ; Carcinoembryonic Antigen ; Cosmetics ; Eccrine Glands ; Epidermis ; Female ; Foot ; Hamartoma ; Hemorrhage ; Humans ; Hyperhidrosis ; Parturition ; Puberty ; Reproductive History ; Skin ; Toes

Transposition of the island pedicle flap is a versatile method of mobilizing tissue from a relatively distant reservoir and moving it across intervening tissue. Advantages are the similar color and texture to those of the affected area, the minimal donor scar, the increased viability with local blood supply, and the potential to camouflage scars into natural skin lines or cosmetic units. In a 73-year-old woman, the lesion of a basal cell carcinoma on her left nasal ala was removed by Mohs micrographic surgery and the resultant defect was reconstructed by transposition of the island pedicle flap. In our case, there were no significant complications and the final result was satisfactory in both functional and cosmetic aspects. Through our experience, we can confirm that transposition of the island pedicle flap is another alternative for the reconstruction of nasal alar defects.
Aged ; Carcinoma, Basal Cell ; Cicatrix ; Female ; Humans ; Mohs Surgery ; Skin ; Tissue Donors

No abstract available.

Arytenoid dislocation is an unusual complication of endotracheal intubation. We reported a case of a 48-year-old female with arytenoid dislocation after uneventful endotracheal intubation, which was successfully treated with arytenoid reduction. The patient complained of persistent hoarseness until the fourth day after an uneventful gynecologic surgery under general anesthesia. On laryngoscopic examination, paralyzed left vocal cord with minimal arytenoid movement was observed. An anteromedial dislocation of the left arytenoid cartilage was suspected and surgical reduction was performed by the laryngologist. The hoarseness was immediately resolved after surgical intervention. Anesthesiologists should be careful not to cause laryngeal trauma in anesthetized patients. In addition, early diagnosis and prompt surgical reduction are essential for a better prognosis for arytenoid dislocation.
Anesthesia, General ; Arytenoid Cartilage ; Dislocations* ; Early Diagnosis ; Female ; Gynecologic Surgical Procedures ; Hoarseness ; Humans ; Intubation ; Intubation, Intratracheal* ; Middle Aged ; Prognosis ; Vocal Cords

OBJECTIVE: We investigated the norepinephrine transporter (NET) expression in normal and pre-eclamptic placentas and analyzed the invasion activity of trophoblastic cells based on norepinephrine (NE)-NET regulation. METHODS: NET and NE expression levels were examined by western blot and enzyme-linked immunosorbent assay, respectively. Trophoblast invasion activity, depending on NE-NET regulation, was determined by NET-small interfering RNA (siRNA) and NET transfection into the human extravillous trophoblast cells with or without NE treatment and invasion rates were analyzed by zymography and an invasion assay. RESULTS: NET mRNA was expressed at a low level in pre-eclamptic placentas compared with normal placentas and NE concentration in maternal plasma increased significantly in pre-eclamptic women compared to normal pregnant women (p<0.05). NET gene upregulation and NE treatment stimulated trophoblast cell invasion up to 2.5-fold (p<0.05) by stimulating matrix metalloproteinase-9 activity via the phosphoinositol-3-kinase/AKT signaling pathway, whereas NET-siRNA with NE treatment reduced invasion rates. CONCLUSION: NET expression is reduced by inadequate regulation of NE levels during placental development. This suggests that a complementary balance between NET and NE regulates trophoblast cell invasion activities during placental development.
Blotting, Western ; Enzyme-Linked Immunosorbent Assay ; Female ; Humans ; Matrix Metalloproteinase 9 ; Norepinephrine ; Norepinephrine Plasma Membrane Transport Proteins ; Placenta ; Placentation ; Plasma ; Pre-Eclampsia ; Pregnant Women ; RNA ; RNA, Messenger ; RNA, Small Interfering ; Transfection ; Trophoblasts ; Up-Regulation

No abstract available.
Arachnoid* ; Hypoglossal Nerve Diseases* ; Hypoglossal Nerve*