Porencephaly is relatively rare condition defined by an defect or a defect or cavity in the cerebrum owing to a developmental malformation or to a destructive lesion. Fory-five porencephaly patients diagnosed by Brain CT were clinically analyzed and the following results were obtained. 1) By the age group presenting initial symptoms, the peak incidence was from 1 month to below 3 years old. 2) In initial symptoms, seizure, spastic weakness, headache were showed in order of frequency. But 7 cases (15.5%) were asymptomatic. 3) The latency of diagnosis after presenting initial symptoms from the symptom onset time to 10 years. 4) The subsequent symptoms were as follows: spastic weakness, speech disturbance, gait disturbance, mental retardation, sensory loss and seizure showed independently or combined. 5) As etiologic factor, 21 cases (46.7%) were congenital, 16 cases (35.5%) were post-traumatic or post-operative and 8 cases (17.8%) were perinatal. 6) The prognosis was seen various from mild to severe. Out of 45 cases, 29 cases (64.4%) were no complications. But the prognosis in patients with post-traumatic or postoperative etiological factors was poor. With the advent of brain CT and the resultant capability of detecting structural defect and cerebral lesions responsible for epilepsy or focal neurologic signs, porencephaly was seen to be readily recongizable by CT examination. Since porencephaly is a significant contributor to the spectrum of CNS lesion and benign condition, ist recognition is important in determining prognosis and therapy.
Brain ; Cerebrum ; Child, Preschool ; Diagnosis ; Epilepsy ; Gait ; Headache ; Humans ; Incidence ; Intellectual Disability ; Muscle Spasticity ; Neurologic Manifestations ; Prognosis ; Seizures

Hailey-Hailey disease(benign familial chronic pemphigus) is a rare autosomal dominant disorder characterized by blisters at sites of friction such as the neck, axillae and groin which are caused by suprabasal epidermal acantholysis. We report two cases of Hailey-Hailey disease in the one family. One of the two cases has asymptomatic multiple longitudinal white bands in the fingernails associated with typical skin lesions. The nail lesions have not been described until reported by Burge in 1992 and it may be a characteristic finding in Hailey-Hailey disease.
Acantholysis ; Axilla ; Blister ; Friction ; Groin ; Humans ; Nails ; Neck ; Pemphigus, Benign Familial* ; Skin

OBJECTIVE: The study was performed to evaluate whether thallium reinjection (RI) distinguishes viable from nonviable myocardium among myocardial segments which showed persistent perfusion defect (PD) in patients with acute myocardial infarction (AMI). MATERIALS AND METHODS: We studied 22 patients underwent PTCA after AMI. SPECT was performed in all patients using dipyridamole stress- 4 hour redistribution (RD) followed by 24 hour RI protocols. Dysfunctional segs were classified into 5 groups: 1) normal, 2) reversible, 3) mild to moderate PD, 4) severe PD and 5) reverse redistribution (RR). All patients underwent follow up echocardiography after 4 months to assess regional wall motion (WM) improvement such as a criteria of viable myocardium. RESULTS: A total of 127 segs with abnormal WM was analyzed. Of 74 segs with PD, 17 (23%) showed enhanced uptake after 24 hour RI. Five of 17 segs (29%) with PD that responded to RI with enhanced thallium uptake showed WM improvement. WM improvement were seen in the 24 of 57 segs (42%) not responding to RI. All four segs (100%) with RR that responded to RI showed improvement. WM improvement were not seen in the 5 of 8 segs (71%) with RR not responding to thallium RI. Eleven (73%) of 15 segs with mild-moderate PD after RI showed improvement, but 33% of segs with severe PD after RI did not showed improvement. Segs with mild-moderate PD after RI and fill in after RI showed improvement in comparison to segs with severe PD after RI(p<0.05). CONCLUSIONS: These data indicate that because only small proportion of PD showed further perfusion improvement after RI and predictive value by the uptake after RI was low, there was limited role of RI after myocardial infarction. Usefulness of RI could be found in segs showing RR responding to RI in AMI reflects viable myocardium.
Dipyridamole ; Echocardiography ; Follow-Up Studies ; Humans ; Myocardial Infarction* ; Myocardium ; Perfusion ; Thallium ; Tomography, Emission-Computed, Single-Photon

Kaposis sarcoma is a multicentric neoplastic vascular tumor involving the skin or internal organs. We report a case of classic Kaposis sarcoma in a 69-year-old male who had positive serum cytomegalovirus antibodies and disseminated multiple erythematous to purplish colored confluent papules and edema on both lower legs. The histopathological finding showed abnormally proliferated and dilated vessels, vascular spaces, spindle cells, and extravasated erythrocytes. He was treated with radiotherapy, but died due to ventricular tachycardia.
Aged ; Antibodies ; Cytomegalovirus ; Edema ; Erythrocytes ; Humans ; Leg* ; Male ; Radiotherapy ; Sarcoma, Kaposi* ; Skin ; Tachycardia, Ventricular

The monoclonal antibody Ki-1(CD30) was first described in 1982 and was reported to react selectively with Reed-Sternberg cells in Hodgkins disease, highly activated B and T cells and large cell lymphomas of both T-and B-cell origin. Lymphomatoid papulosis(LyP) is characterized by recurrent erythematous papules or nodules that undergo spontaneous healing with hyperpigmentation and scarring. Histologically, two major types of LyP can be distinguished: type A is characterized by the presence of variable numbers of Ki-1+ large, atypical lymphocytes with some Reed-Sternberg like cells and type B is characterized by Ki-1- atypical cerebriform mononuclear cells similar to those in mycosis fungoides. We report a case of Ki-1+ lymphomatoid papulosis which was histopathologically recognized as a type B lesion but represented a Ki-1 positive reaction.
B-Lymphocytes ; Cicatrix ; Hodgkin Disease ; Hyperpigmentation ; Lymphocytes ; Lymphoma ; Lymphomatoid Papulosis* ; Mycosis Fungoides ; Reed-Sternberg Cells ; T-Lymphocytes

We report two cases of recurrent extramarnmary Pagets disease after wide local excision. Both cases involved the scrotum and the penoserotal junction, respectively. On histopathological examination, we could observed many Paget cells confined to the epidermis and the hair follicle, but not invading the underlying dermis in both cases. There was no evidence of internal malignancy. Both cases were treated with wide local reexcision.
Dermis ; Epidermis ; Hair Follicle ; Paget Disease, Extramammary* ; Scrotum

We report a case of Darier's disease with typical cutaneous and nail lesions in a 50-year-old male patient. He has brownish hyperkeratotic papules on the ant.chest, back, both axilla and groins and verrucous surfaced hypertrophic plaques on the scalp, forehead and both postauricular areas, which were accompanied by a severe itching sensation and malodor. Also he has finger nail lesions such as subungual hyperkeratotic papules, distal wedge shaped notching and multiple longitudinal red lines on the nail plates. Histopathologic features taken from the inguinal area revealed hyperkeratosis, suprabasal clefts and lacuna, protruded villi lined with one layer of basal cells into the cavity and dyskeratotic cells such as corps ronds in the epidermis. Hyperkeratotic papules on the ant.chest, back, both axilla and groins disappeared after systemic treatment withe a low dose of oral etretinate but hypertrophic plaques on the scalp and both postauricular areas still remained clinically and histopathologically. We then the treated postauricular hypertrophic plaques with CO2 laser vaporization and severe malodor with oral isotretinoin and it resulted in a marked improvement.
Acitretin* ; Axilla ; Darier Disease* ; Epidermis ; Etretinate* ; Fingers ; Forehead ; Groin ; Humans ; Isotretinoin* ; Lasers, Gas* ; Male ; Middle Aged ; Pruritus ; Scalp ; Sensation ; Volatilization*

The Ehlers-Danlos syndromes(EDS) are a heterogenous group of inherited connective tissue disorders characterized clinically by skin fragility, skin hyperextensibility, joint hypermobility, and excessive bruising. At least 11 different subtypes of EDS have been classified based on genetic, biochemical, and clinical characteristics. We report a 25-year-old man with EDS type II who presented mild extensibility of skin and joint, skin fragility, and "cigarette paper" like atrophic scar. The EDS type II is more common than other different subtypes. While it may present with subtle clinical features, recognition is important so that good advice can be given to reduce the risks associated with certain careers and sports and to prevent the potential complications of pregnancy and surgery.
Adult ; Cicatrix ; Connective Tissue ; Ehlers-Danlos Syndrome ; Humans ; Joint Instability ; Joints ; Molecular Biology ; Pregnancy ; Skin ; Sports

We successfully closed the isolated patent ductusarteriosus(PDA) in 2 female patients using the Rashkind Double-Disc Occlusion system. nonsurgical closure of PDA usin plug was introduced by Porstmann in 1968 through transfemoral artery catheter and using umbrella by Rashkind in 1977 througth venous site. There has been continued improvement and simplification of the equipment as well as in the implantation technique. Nowaday, a multicenter study was conducted to test the safety and effectiveness of this interventioal method in States. Successful closure rate was high but some problems were reported such as embolization, abnormal deployment and residual shunt. Residual shunt was visualized by aortography in our both cases immediately after intervention but continuous murmur could not be heard in both cases. Oxygen step-up disappeared immediately after occluder implantation in both cases. Complete occlusion was expected with thrombus in near future.
Aortography ; Arteries ; Catheters ; Ductus Arteriosus, Patent* ; Female ; Humans ; Oxygen ; Thrombosis

Most of the macular hole retinai detachinents are limited to the perimacular area. Therefore, we must look for the peripheral retinal hole when the macular hole retinal detachment extends beyond equator. And the principle of operation should be on the peripheral retinal hole rather than macular hole. Authors studied the clinical feature of macular hole retinal detachment associated with the peripheral retinal hole. Among the 39 macular hole retinal detachment patients, peripheral hole was found in 12 patients (30.7%). Sex distribution was the same and mean age was 55.9 years old. Retinal detachment was not diagnosed for more than I month in 7 cases (58.4%). Nine cases (75.0%) were extensive retinal detachments extending beyond 3 quardrants. Nine cases (75.0%) had one peripheral hole and most of peripheral holes were found on the ternporal side. Associated diseases were cataract (5 cases, 38. 5%) and high myopia (5 cases, 38.5%). In 4 cases (33.3%) hole was found on the opposite side. Among the 12 macular hole retinal detachment patients, 11 operations were performed with the technique of gas injection and scleral buckling which lead to successful attachment in 5 cases (41.7%). Among the 7 cases in which reoperations were needed, 3 cases (42.9%) were recurred in 3 months. In 8 cases (66.7%) there were improvements of post-operative visual acuity.
Cataract ; Humans ; Myopia ; Retinal Detachment* ; Retinal Perforations* ; Retinaldehyde* ; Scleral Buckling ; Sex Distribution ; Visual Acuity