Myasthenia gravis(MG) is an autoimmune disorder characterized by the presence of anti-acetylcholine receptor antibody and weakness of voluntary muscles. The pathogenesis of MG is decreased numbers of acetylcholine receptors at postsynaptic membranes of neuromuscular junctions. It has been reported that MG often coexists with other autoimmune disorders. This is a case report of systemic lupus erythematosus coexisting with MG in a 23 year old female patient presenting with dysarthria, dysphagia, and limb weakness. We report the case with relevant literature review.
Deglutition Disorders ; Dysarthria ; Extremities ; Female ; Humans ; Lupus Erythematosus, Systemic* ; Membranes ; Muscle, Skeletal ; Myasthenia Gravis* ; Neuromuscular Junction ; Receptors, Cholinergic ; Young Adult

PURPOSE: To evaluate the usefulness of transperineal sonography of the prostate in patients with previousabdominoperineal resection. MATERIALS AND METHODS: Transperineal prostatic sonograms were obtained from ninepatients with abdominoperineal resection. The shortest distance between the probe and the prostate gland, itsvolume, and its internal and external structures were evaluated for image quality, shape, border, and the degreeof distortion in internal echo. Because the number of patients was small, statistical analysis was not performed. RESULTS: Transperineal sonography of the prostate in eight patient was obtained at ease, except that one of ninepatients was not able to continue the procedure because of severe tenderness of the perineum and poor cooperation.Acceptable images of the prostate gland and their adjacent structures were obtained in one patient with less than1cm between the probe and prastate gland, three of four with between 1cm and 1.5cm, and one of three with morethan 1.5cm. CONCLUSION: In patients with previous abdominoperineal resection, transperineal sonography of theprostate may be a good imaging modality.
Humans ; Perineum ; Prostate*

OBJECTIVE: To investigate the autoantibody profile and its clinical association in patients with systemic lupus erythematosus. METHODS: The frequency and clinical correlation of autoantibodies were studied in 73 patients with systemic lupus erythematosus who have been followed in Seoul National University Hospital. Double immunodiffusion, ELISA and immunoblot were used for the detection of autoantibodies. RESULTS: The frequency of each autoantibody measured by double immunodif fusion was as follows; anti-Ro 53.4%, anti-La 11.0%, anti-Sm 20.5%, anti-U1 RNP 20.5%. The frequency of each autoantibody by ELISA was as follows; anti-Ro 69.9%, anti-La 27.4%, anti-Sm 54.8%, anti-Ul RNP 68.5%, anti-dsDNA 72.6%, anti-cardiolipin 47.2% (IgG 43.1?0, igM 15. 3%). The frequency of each autoantibody by immunoblot was as follows; anti-Ro 15.1?0, anti-La 42. 5%, anti-Sm 46. 6%, anti-U1 RNP 42. 5%. anti-ribosomal P(P0) 27.4%. Anti-Ro was associated with decreased frequency of nephrotic syndrome. Anti-U1 RNP was associated with increased frequency of malar rash, Raynaud phenomenon and decreased frequency of nephritis. Patients with both anti-Ro and anti La had more frequent serositis than those with anti-l~o only. Patients with both anti-Sm and anti-U1 RNP had less frequent thrombocytopenia than those with anti-U1 RNP only. And patients with anti-Sm and anti-dsDNA had more frequent arthritis than those with only one of both antibodies. There was a positive correlation of autoantibody titers between anti-Ro and anti-La, anti-Sm and anti-U1 RNP, anti-dsDNA and anti-cardiolipin(IgG). Taking the result of immunoblot as a standard, both of double immunodiffusion and ELISA showed low sensitivity but high specficity for anti La. As for anti-Sm and anti-U1 RNP, double immunodiffusion showed low sensitivity but high specificity, whereas ELISA showed high sensitivity but low specificity. CONCLUSIONS: In our study, some autoantibodies (anti-Ro, anti-U1 RNP) were associated with certain clinical manifestations while others not. Immunoblot being used as a standard method, ELISA showed higher sensitivity but lower specificity for anti-La, anti-Sm and anti-U1 RNP compared with immunodiffusion. It is recommended that in interpretating the laboratory findings of these autoantibodies these parameters of each method should be considered.
Antibodies ; Arthritis ; Autoantibodies ; Blotting, Western* ; Enzyme-Linked Immunosorbent Assay* ; Exanthema ; Humans ; Immunodiffusion* ; Immunoglobulin M ; Lupus Erythematosus, Systemic ; Nephritis ; Nephrotic Syndrome ; Raynaud Disease ; Sensitivity and Specificity ; Seoul ; Serositis ; Thrombocytopenia

Sjogren's syndrome is one of chronic inflammatory autoimmune diseases involving exocrine gland. Involvement of central nerve system was rarely reported. We report a case of primary Sjogren's syndrome presenting as transverse myelitis in 37-year-old female who complained of tingling sense in both legs and voiding difficulty. Magnetic resonance imaging shows high signal intensity with cord swelling from C6 to T7 in T2 weighted image. The patient received intravenous methylprednisolone (1,000 mg/day) for 10 days followed by prednisolone and intravenous monthly cyclophosphamide with subsequent clinical improvement.
Adult ; Autoimmune Diseases ; Cyclophosphamide ; Exocrine Glands ; Female ; Humans ; Leg ; Magnetic Resonance Imaging ; Methylprednisolone ; Myelitis, Transverse* ; Prednisolone ; Sjogren's Syndrome*

Scleroderma (SSc) renal crisis has been reported to be associated with anti-RNA polymerase I and III (RNAP I/III) antibodies in Caucasians and the Japanese. However, no report is available for Korean SSc patients. Here, we describe the case of a 65-yr-old female SSc patient who developed renal crisis and whose serum contained anti-RNAP I/III antibodies. She was finally diagnosed as having diffuse cutaneous SSc based on skin thickening proximal to the elbows and knees. Sudden hypertension, oliguria, and pulmonary edema were features of her renal crisis. Despite the use of captopril and adequate blood pressure control, her renal function deteriorated. Subsequent renal biopsy findings showed severe fibrinoid necrosis with luminal obliteration in interlobar arteries and arterioles consistent with SSc renal crisis. Serum anti-RNAP I/III antibodies were detected by radioimmunoprecipitation. This is the first report of a renal crisis in a Korean SSc patient with RNAP I/III antibodies.
Scleroderma, Systemic/*complications/enzymology/*immunology ; RNA Polymerase III/*immunology ; RNA Polymerase I/*immunology ; Korea ; Kidney Failure, Acute/*complications/enzymology/*immunology ; Humans ; Female ; Critical Care ; Autoantibodies/*blood ; Aged

Transcervical resection of endometrium is under-utilized in the treatment of dysfunctional uterine bleeding, uterine myoma and menorrhagia. The procedure is similar to transurethral resection of prostate in men with a possibility of substantial absorption of irrigation fluid. Absorption of a large volume of fluid can cause excessive intravascular volume, hyponatremia, cerebral edema and death. Severe hyponatremia leading to extrapontine myelinolysis is an extremely rare complication of this procedure. So, We report a case of developed extrapontine myelinolysis after hysteroscopic myomectomy which, however, showed complete recovery.
Absorption ; Brain Edema ; Endometrium ; Female ; Humans ; Hyponatremia ; Leiomyoma ; Male ; Menorrhagia ; Metrorrhagia ; Myelinolysis, Central Pontine ; Transurethral Resection of Prostate

Systemic sclerosis is a rare, autoimmune, connective tissue disorder which involves skin and other internal organs. However, central nervous system involvement including seizure, is a very rare event. This 29-year-old woman experienced repeated episodes of typical generalized tonic clonic seizure. T2 weighted and flare images of magnetic resonance image showed a mass-like high signal intensity lesion in the left temporal lobe. Atypical spikes and waves were present in a eclectroencephalogram. Her seizure episodes were controlled with anticonvulsants, corticosteroids and immunosuppresants. We report a rare case of systemic sclerosis which showed central nervous system involvement.
Adrenal Cortex Hormones ; Adult ; Anticonvulsants ; Central Nervous System ; Connective Tissue ; Female ; Humans ; Magnetic Resonance Imaging ; Scleroderma, Diffuse* ; Scleroderma, Systemic ; Seizures* ; Skin ; Temporal Lobe

Central venous cannulation via an internal jugular vein has become a common procedure in the management and monitoring of severely ill patients. Although complications such as carotid artery puncture or pneumothorax have been reported, hemothorax is relatively uncommon. We describe a case of hydrohemothorax and subclavian artery laceration which occurred during right internal jugular vein cannulation. A 44-year-old female patient was admitted for laparoscopic adrenalectomy under general anesthesia. For central venous pressure monitoring, central venous cannulation performed. However, dilator overinsertion injured the right subclavian artery. This led to a massive ipsilateral hydrohemothorax requiring thoracotomy for subclavian artery repair. This case suggests that central venous cannulation should be done carefully and improves awareness of the potential for dilator induced injury.
Adrenalectomy ; Adult ; Anesthesia, General ; Carotid Arteries ; Catheterization* ; Central Venous Pressure ; Female ; Hemothorax ; Humans ; Jugular Veins* ; Lacerations* ; Pneumothorax ; Punctures ; Subclavian Artery* ; Thoracotomy

OBJECTIVE: To investigate the clinical characteristics of relapsing polychondritis (RP) in Korea. METHODS: We retrospectively reviewed 16 patients with RP who were diagnosed at Seoul National University Hospital and Gyeongsang National University Hospital in the period from July 1987 to August 2003. RESULTS: There were 9 males and 7 females, the mean age was 45.4+/-12.8 years. The frequency of clinical manifestation was as follows; auricular chondritis (81.3%), nasal chondritis (68.8%), ocular inflammation (56.3%), respiratory tract involvement (56.3%), arthritis (50%) and audio-vestibular damage (25%). Westergren erythrocyte sedimentation rate (ESR) was elevated in 53.3% and antinuclear antibody (ANA) was positive in 20% of study patients. Rheumatoid factor was negative in all cases. CONCLUSION: Clinical manifestations were comparable to the reports of Western countries. However, the frequency of arthritis was lower in Korean patients with RP than Western reports.
Antibodies, Antinuclear ; Arthritis ; Blood Sedimentation ; Female ; Humans ; Inflammation ; Korea* ; Male ; Polychondritis, Relapsing* ; Respiratory System ; Retrospective Studies ; Rheumatoid Factor ; Seoul

Relapsing polychondritis is a rare autoimmune disease of unknown etiology characterized by recurrent inflammation of cartilaginous structures of the ears, nose, respiratory tract and joints. Relapsing polychondritis may affect proteoglycan-rich tissues, such as the eyes, aorta, heart, and skin. Several cardiovascular complications have been reported in patients with relapsing polychondritis. The most common findings of cardiovascular manifestations are aortic or mitral regurgitation and aortic aneurysm. Aortitis is a very rare complication of relapsing polychondritis. We report a case of 48-year-old man who had relapsing polychondritis with aortitis mimicking Takayasu's arteritis.
Aorta ; Aortic Aneurysm ; Aortitis ; Autoimmune Diseases ; Ear ; Heart ; Humans ; Inflammation ; Joints ; Middle Aged ; Mitral Valve Insufficiency ; Nose ; Polychondritis, Relapsing* ; Respiratory System ; Skin ; Takayasu Arteritis* ; Vasculitis*