Calciphylaxsis is a rare and life-threatening condition of progressive cutaneous necrosis secondary to calcification of small and medium-sized blood vessels. It is seen almost exclusively in patients with end-stage renal disease, in a setting of secondary hyperparathyroidism and hypercalcemia resulting in metastatic vascular calcification, We experienced a case of calciphylaxsis manifested with chara- cteristic cutaneous lesions that began as tender violaceous livedoid discolorations and subsequent ulceration on the proximal aspect of the extremities and on the lower part of the abdomen. Histologically, calcification is found in small and medium-sized blood vessels in the deep dermis and subcutaneous tissue. The patient had been treated for hypercalcemia, not complicating renal failure, but the cause of hypercalcemia was not found. However, the hypercalcemia was contolled and the skin lesions also improved after oral prednisolone therapy. Our case is a rare presentation of calciphylaxsis in the absence of renal failure, reversibly improved.
Abdomen ; Blood Vessels ; Dermis ; Extremities ; Humans ; Hypercalcemia ; Hyperparathyroidism, Secondary ; Kidney Failure, Chronic ; Necrosis ; Prednisolone ; Renal Insufficiency ; Skin ; Subcutaneous Tissue ; Ulcer ; Vascular Calcification

Epilepsy is one of the most common neurological problems in pregnancy. For the majority of women, pregnancy proceeds without any apparent difficulties but there is growing evidence of an increased risk of major malformations and later cognitive problems in children exposed to antiepileptic drugs in utero. Updated evidence from several prospective pregnancy registries suggests an increased risk of major malformations with valproic acid compared with other antiepileptic drugs, becoming more evident as doses exceed 1,000 mg/day. The effects of polytherapy appear to carry greater risks compared with monotherapy. Limited data exist for the newer AEDs except for lamotrigine. Although most women with epilepsy have no change in seizure frequency, seizures, especially generalized tonic-clonic seizures can produce adverse effects on mother and fetus. Data about the risk associated with seizures in pregnancy are limited. The pregnancy registry will be performed in Korea to assess the relative risk of major congenital malformation from in utero exposure to antiepileptic drug and to analyze seizure control and treatment in pregnant women with epilepsy.
Anticonvulsants* ; Child ; Epilepsy ; Female ; Fetus ; Humans ; Korea ; Mothers ; Pregnancy* ; Pregnant Women ; Registries ; Seizures* ; Valproic Acid

No abstract available.
Diagnosis* ; Electroencephalography* ; Epilepsy*

A Case of Fibromuscular dysplasia involoving the left internal carotid and middle cerebral rtery in a previously healthy man is presented. Symptoms were characteristic of cerebral schemia in the territory that the left middle cerbral artery supplies. This case suggests that The fibromuscular dysplasia is one of the causes provoking cerebral infarction.
Arteries ; Cerebral Infarction* ; Equipment and Supplies ; Fibromuscular Dysplasia*

The prognosis of epilepsy has improved considerably, and about 80% of patients can now be expected to achieve complete seizure control with antiepileptic drug treatment. It is important to understand that the response to individual drugs may vary considerably in relation to the seizure type or epilepsy syndrome, so that optimization of treatment requires careful individualization of the dosage, and that rational polytherapy should be performed in consideration of the mechanisms of action of antiepileptic drugs, adverse effects, and drug interactions. Although newly developed antiepileptic drugs have some advantages in terms of adverse effects and pharmacokinetics compared with traditional antiepileptic drugs, they have demonstrated no significant difference in efficacy in comparative studies. The purpose of this chapter is to review the basic principles, which should guide the optimal treatment with antiepileptic drugs in patients with epilepsy.
Anticonvulsants ; Drug Interactions ; Epilepsy* ; Humans ; Pharmacokinetics ; Prognosis ; Seizures

Churg-Strauss syndrome is one of idiopathic, systemic necrotizing vasulitides and characterized by a history of asthma or allergic rhinitis, eosinophilia of peripheral blood and variable symptoms of the systemic vasculitis. This is a case report of classical Churg-Strauss syndrome in a 6- year-old male who has the multiple mononeuropathy as a symptom of the systemic vasculitis. The sural nerve biopsy showed a few myelin digestion chambers suggesting axonal degeneration which was not suspected on the electrophysiological study, and the specimen of right gastrocnemius muscle revealed the typical pathological findings of eosinophilic infiltration, extravascular granuloma and necrotizing small vessel vasculitis. The therapeutic response of cyclophosphamide looked better than the oral prednisolone treatment only.
Asthma ; Axons ; Biopsy ; Churg-Strauss Syndrome* ; Cyclophosphamide ; Digestion ; Eosinophilia ; Eosinophils ; Granuloma ; Humans ; Male ; Mononeuropathies ; Muscle, Skeletal ; Myelin Sheath ; Prednisolone ; Rhinitis ; Sural Nerve ; Systemic Vasculitis ; Vasculitis

No abstract available.
Anticonvulsants*

Epilepsy is one of the most common chronic neurologic conditions. Pharmacologic therapy is by far the most common approach, with the other modalities typically limited to patients with pharmacoresistant epilepsies. A host of new antiepileptic drugs (AEDs) have been introduced over the last 20 years. The AEDs including the conventional ones are more or less equally effective in patients with partial epilepsy. Therefore, relative efficacy is not a useful factor in selecting a particular drug. A conventional AED, valproic acid is regarded as having superior efficacy than the other broad-spectrum AEDs including new ones in patients with generalized epilepsy. However, it can have considerable side effects, such as reproductive dysfunction and teratogenicity to young women with epilepsy. One of the clearest advantages of many new AEDs over the conventional ones has been their more favorable pharmacokinetic and drug-drug interaction profiles compared with the conventional ones involved in the cytochrome P450 enzymatic system, which may change the levels of other antiepileptic and nonantiepileptic drugs, and endogenous substances. Many new AEDs have unique mechanisms of action and slightly better tolerability than the conventional ones. Several new AEDs can allow young women with epilepsy, particularly those with idiopathic generalized epilepsy, to avoid valproic acid treatment. Furthermore, the new AEDs may provide a modest but positive effect in seizure control, particularly as an add-on treatment. The greater variety of AEDs allows better patient tailoring according to patient's characteristics and contributes to improvement in quality of life.
Anticonvulsants ; Cytochrome P-450 Enzyme System ; Drug Interactions ; Epilepsies, Partial ; Epilepsy ; Epilepsy, Generalized ; Female ; Humans ; Quality of Life ; Seizures ; Valproic Acid

A 57 year-old patient developed transient blindness and impending syncope whenever he tumed his head to the right side. The angiography showed severe focal luminal narrowing of the right vertebral artery at the level of C5-C6 interspace with head tumed to the right, but showed normal filling of the right vertebral artery in the straight position. The distal cervical portion of the left vertebral artery was hypoplastic We believe that symptoms of this patient are due to the right vertebral artery compression produced by osteophyte formed at uncinate portion of the vertebrae on head tuming to the right. This patient also experienced transient global amnesia for about four hours after the right vertebral arteriography performed with head tumed to the right.
Amnesia, Transient Global* ; Angiography ; Blindness ; Head ; Humans ; Middle Aged ; Osteophyte ; Phenobarbital ; Spine ; Spondylosis* ; Syncope ; Vertebral Artery ; Vertebrobasilar Insufficiency*

No abstract available.
Orbit* ; Vascular Malformations ; Visual Acuity*