Dyke-Davidoff-Masson syndrome is a rare clinical syndrome consisting of hemiatrophy of cerebral hemisphere, enlargement of ventricles and prominent pneumatization of petrous pyramid of the temporal bone. Its clinical manifestations are hemiparesis, several types of seizure, mental retardation and other neurologic deficits. We experienced a case of Dyke-Davidoff-Masson syndrome in a four months old boy who visited to our department due to right side upper extremity weakness and infantile spasm. The brain magnetic resonance imaging showed marked hemiatrophy of left cerebral hemisphere and enlargement of left lateral ventricle. So with the review of literatures, we report a case of Dyke-Davidoff-Masson syndrome with infantile spasm.
Brain ; Cerebrum ; Humans ; Infant ; Infant, Newborn ; Intellectual Disability ; Lateral Ventricles ; Magnetic Resonance Imaging ; Male ; Neurologic Manifestations ; Paresis ; Petrous Bone ; Seizures ; Spasms, Infantile* ; Temporal Bone ; Upper Extremity

No abstract available.

BACKGROUND: Calcinosis cutis may occur when connective tissue is abnormal (dystrophic), or where calcium or phosphate levels in the blood are high(metastatic); alternatively, there may be no obvious underlying cause(idiopathic). The exact incidence of calcinosis cutis in dermatologic patients is not well-kniown and the pathomechanism of it remains to be elucidated. OBJECTIVE: This study was performed to analyze the incidence, clinical and histopathological features of calcinosis cutis. METHODS: We studied 72 patients with calcinosis cutis who visited our department between January 1985 and December 1996. The patients were classified into 3 types (dystrophic, metastatic, and idiopathic) and were analyzed clinically and histopathologically. Results : The results were summerized as follows; 1. There were 60 cases(83.3%) and 12 cases(16.7%) of the dystrophic and idiopathic types, respectively but the metastatic type was not observed. The frequency of dystrophic calcification in individual disorders was 88.2% in pseudoxanthoma elasticum, 66.7% in trichilemmal cyst and dermatomyositis, and 64.6% in pilomatrichoma. 2. In the idiopathic type, it was common in females aged over 50 years and the mean duration of lesions was 2.8+/-2.0 years. The predilection site was the flank(5 cases) and most of the lesions(7 cases) showed skin-colored nodules 3. Histopathologically, calcified material of the idiopathic type was located predominently in the dermis(10 cases) and was present as large masses surrounded by a foreign body reaction. 4. In the dystrophic type, calcified deposits were noted predominently within the shadow cells of pilomatricoma, in the degenerated elastic fibers of pseudoxanthoma elasticum, and in the keratinized area of basal cell carcinoma, squamous cell carcinoma, trichilemmal cyst, and epidermal cyst. Foreign body reactions with giant cells and mononuclear cell infiltrations were often found around large deposits of calcium. Conclusion : The incidence of calcinosis cutis in dermatologic patients is relatively low, but a variety of disorders can be associated with cutaneous calcification. Therefore, dermatologists should be familiar with the different forms of cutaneous calcification and the dermatoses that manifest them.
Calcinosis* ; Calcium ; Carcinoma, Basal Cell ; Carcinoma, Squamous Cell ; Connective Tissue ; Dermatomyositis ; Elastic Tissue ; Epidermal Cyst ; Female ; Foreign Bodies ; Foreign-Body Reaction ; Giant Cells ; Humans ; Incidence ; Pilomatrixoma ; Pseudoxanthoma Elasticum ; Skin Diseases

Septo-optic dysplasia, as first described by de Morsier, is a rare developmental anomaly of absent or hypoplastic septum pellucidum, a primitive optic vesicle, and dysplasia of the optic nerve, chiasm, and optic tracts. It is a common cause of hypopituitarism in children and has variable hypothalamic-pituitary dysfunction. We experienced a case of septo-optic dysplasia in 5 month old female baby who presented with developmental delay and seizure. A magnetic resonance imaging of the brain showed absence of the septum pellucidum and the fundoscopic examination disclosed the absence of left optic disc and atrophy of right optic disc.
Atrophy ; Brain ; Child ; Female ; Humans ; Hypopituitarism ; Infant ; Magnetic Resonance Imaging ; Optic Nerve ; Seizures ; Septo-Optic Dysplasia* ; Septum Pellucidum ; Visual Pathways

Multiple endocrine neoplasi~a type 3 is a rare, heritable or sporadic, multiple neoplastic disorder that is charracterized by thyroid medullary careinoma, pheochromocytoma, multiple mucosal neuroma, and marfanoid habitus. The most important disorder of the syndrome is mucosal neuroma that is an early diagnostic sign of multiple endocrine neoplasia type 3. Early diagnosis of MEN type 3 determines prognosis of the disease. We present a case of multiple endocrine neoplasia type 3 of the sporadic pattern in a 27-year-old man who had typical medullary thyroid carcinoma, mucosal neuroma, marfanoid habitus, and megacolon.
Adult ; Early Diagnosis ; Humans ; Male ; Megacolon ; Multiple Endocrine Neoplasia* ; Neuroma* ; Pheochromocytoma ; Prognosis ; Thyroid Gland ; Thyroid Neoplasms

A 26-year-old Korean man had an erythematous scaly plaque on his right thigh for fifteen years. He was diagnosed with chronic eczema and treated with topical steroid, but no clinical improvement was obtained for 15 years. Laboratory studies including complete blood cell count, urinalysis, liver and renal function test, and peripheral blood smear were normal. A biopsy specimen showed hyperkeratosis, acanthosis, and mononuclear cellular infiltration in the epidermis. These cells had a perinuclear halo and showed a Pautrier's microabscess like configuration. In the dermis, there was a band like infiltration of inflammatory cells. Infiltrates of the epidermis were negative for leukocyte common antigen(CD45) and positive for Pan T cell(CD45RO). He was treated with topical PUVA twice a week for twelve weeks. Clinical and pathological improvements were obtained. We propose PUVA may be a useful therapeutic modality to treat Woringer Kolopp disease.
Adult ; Biopsy ; Blood Cell Count ; Dermis ; Eczema ; Epidermis ; Humans ; Leukocytes ; Liver ; Pagetoid Reticulosis* ; Thigh ; Urinalysis

We report the clinical evolution of two male patients with generalized granulama annulare(GGA) who were controlled with hydroxychloroquine. In the first case, a 3-year-old boy was treated with systemic steroid, and in the second case, a 68-year-old man was treated with prednisolone, niacin, and retinoic acid. But, remission and recurrence of the skin lesions were repeated. A good clinieal response was achieved in both cases after administration of hydroxychloroquine without side effects or any signs of recurrence. Hydroxychloroquine may be helpful to treat the GGA that have side effects to the systemic corticosteroid or resistence to other therapeutic modalities.
Aged ; Child, Preschool ; Granuloma Annulare* ; Granuloma* ; Humans ; Hydroxychloroquine* ; Male ; Niacin ; Prednisolone ; Recurrence ; Skin ; Tretinoin

Tumors of the lung and bronchi containing cartilage were known by a variety of names, chondroma, adenochondroma, chondromatous hamartoma and mixed tumor. This variation in nomenclatures explain the difference of illustration on the nature of these tumor. The concept pulmonary harmatomas are benign neoplasm and not developmental malformations, has gained wide acceptance in recent years. We have experienced four cases of intrapulmonary hamartoma which were all discovered during routine chest film check up for certificate of health and evaluation of other disease. One case is added further detailed histologic examination by electron microscopy. The age at time of the detection were 53 (male), 23 (male), 39 (female), and 56 (female) years old. The mean size is 4.3x3.7x3.4 cm. The locations were three left upper lobes and one right upper lobe. Lobectomy and wedge resecions were done. Cut surface showed promiment lobular structures, papillary configuration and multiple cleft like spaces. Predominant cellular components were cartilage but fat tissue in one of the four cases. Microscopic findings showed abundant hyaline cartilages bearing lobular configuration and overlying pseudostratified ciliated columnar and cuboidal epithelium. Fibromyxoid and undifferentiated cells were seen in myxoid and fatty tissue. Electron microscopic findings revealed stellate, undifferentiated mesenchymal cells bearing collagen formation, stellate smooth muscle and transition areas between undifferentiated mesenchymal cells and mature cartilage. Epithelial components were similar to terminal bronchiole and alveolar epithelium. These findings suggest the concept that intrapulmonary hamartoma represent a histologic specturm of benign mesenchymal neoplasms, which originate in peribronchial connective tissue.
Female ; Male ; Humans ; Hamartoma

No abstract available.
Dimethylformamide* ; Liver Diseases* ; Liver*

No abstract available.