Nonconvulsive status epilepticus, which constitutes about 25% of all cases of status, has been subdivided into generalized nonconvulsive(absence) status and complex partial status. We report a case of complex partial status epilepticus in a 3-year-old male patient with altered mental function lasting about 24 hours. The electroencephalographic finding was compatible with complex partial status and the outcome of treatment is excellent, so far.
Child, Preschool ; Humans ; Male ; Status Epilepticus*

Serial Brain MRI was performed on a seventeen-year-old girl with Kearns-Sayre syndrome. At the age of 11, she complained bilateral ptosis. Two years later, bilateral blepharoplasty was done and brain MRI was taken. T2-weighted MRI sequence showed high signal intensity areas in the brainstem, thalamus and white matter of the cerebrum and cerebellum bilaterally. Four years later, chronic progressive external ophthalmoplegia developed and serial MRI and proton MRS were taken. Follow-up MRI showed similar but slightly progressed findings compared with previous films. The proton MR spectroscopic imaging demonstrated focal localization of abnormally increased lactate content in the involved area of the brain.
Blepharoplasty ; Brain ; Brain Stem ; Cerebellum ; Cerebrum ; Female ; Follow-Up Studies* ; Humans ; Kearns-Sayre Syndrome* ; Lactic Acid ; Magnetic Resonance Imaging* ; Magnetic Resonance Spectroscopy ; Ophthalmoplegia, Chronic Progressive External ; Protons ; Thalamus

A case of transient neonatal diabetes mellitus combined with congenital adrenal hyperplasia(CAH) is described. A female infant was born by cesarean delivery due to fetal distress, she had sunken eyeball and anterior fontanelle, large protruded tongue and thin subcutaneous tissues. She had large clitoris and progressive pigmentation on whole body was observed since 10th day of birth. Hyperglycemia and glycosuria was noted at 3rd day of birth. Level of insulin and C-peptide was 3.0 mU/L and 0.35 ng/mL respectively. Serum ACTH was 870.4 pg/mL and 17-hydroxyprogesterone was increased to 20,000 ng/dL. Serum Na was 124 mEq/L, K 5.6 mEq/L. Abdominal MRI showed no abnormality. Chromosomal study showed 46,XX. Genetic analysis with polymorphic DNA markers for chromosome 6 showed paternal uniparental isodisomy at D6S276, D6S1704 and DNA analysis of CYP 21 gene showed mutation at P435S. She required insulin therapy for 8 months after birth. Hydrocortisone and florinef was needed for the control of CAH.
17-alpha-Hydroxyprogesterone ; Adrenal Hyperplasia, Congenital* ; Adrenocorticotropic Hormone ; C-Peptide ; Chromosomes, Human, Pair 6 ; Clitoris ; Cranial Fontanelles ; Diabetes Mellitus* ; DNA ; Female ; Fetal Distress ; Genetic Markers ; Glycosuria ; Humans ; Hydrocortisone ; Hyperglycemia ; Infant ; Insulin ; Magnetic Resonance Imaging ; Parturition ; Pigmentation ; Subcutaneous Tissue ; Tongue ; Uniparental Disomy

No abstract available.

We treated two patients of measles postinfectious encephalomyelitis with intravenous methylprednisolone pulse therapy(1 g/1.73 m2/day for 5 days) 38 hours and 5 days respectively after the onset of neurologic symptoms. Despite extensive white matter involvement shown in MRI and severe clinical symptoms, the patients recovered from their neurologic symptoms dramatically following intravenous methylprednisolone pulse therapy. Because acute postinfectious encephalomyelitis has been postulated to be immunologically mediated disease, instead of direct viral invasion, ACTH and dexamethasone are widely used and the outcome is variable. This case report of successful recovery from fulminant ADEM with pulse intravenous methylprednisolone therapy, although uncontrolled, suggests that this therapeutic regimen should be studied in other cases.
Adrenocorticotropic Hormone ; Dexamethasone ; Encephalomyelitis, Acute Disseminated* ; Humans ; Magnetic Resonance Imaging ; Measles* ; Methylprednisolone* ; Neurologic Manifestations

Percutaneous balloon angioplasty has been reported to be useful in the treatment of intracranial atherosclerotic arterial stenosis. However, arterial dissection with increased risk of acute closure and stroke has limited its widespread implementation. Stenting of the intracranial vasculature recently has been shown to be feasible in a variety of circumstance. However, stenting of middle cerebral artery has been limited because of difficulty with tracking stents across the carotid siphon. We report a case of successful percutaneous stenting of a symptomatic middle cerebral artery stenosis using a balloon-expandible flexible coronary stent.
Angioplasty ; Angioplasty, Balloon ; Constriction, Pathologic* ; Middle Cerebral Artery* ; Stents* ; Stroke

Protrusion of coil loop(s) and subsequent occlusion of the parent artery is one of the dreadful complications during endovascular coil embolization of cerebral aneurysm. Although protrusion of one or two coil loops may not cause adverse events and can be ignored in many instances, it can also compromise the parent artery in some cases with or without thrombus formation. We report a case of rescue balloon reposition of the protruding coil mass back into the aneurysm and recanalization of parent artery during embolization of the anterior communicating artery aneurysm.
Aneurysm ; Arteries ; Embolization, Therapeutic ; Humans ; Intracranial Aneurysm* ; Parents ; Thrombosis

OBJECTIVE: To evaluate the prevalence of incidentally found unruptured intracranial aneurysms (UIAs) on the brain MR angiography (MRA) from a community-based general hospital. MATERIALS AND METHODS: This was a prospectively collected retrospective study, carried out from January 2004 to December 2004. The subjects included 3049 persons from a community-based hospital in whom MRA was performed according to a standardized protocol in an outpatient setting. Age- and sex-specific prevalence of UIAs was calculated. The results by MRA were compared with intra-arterial digital subtraction angiography (DSA) findings. RESULTS: Unruptured intracranial aneurysms were found in 137 (5%) of the 3049 patients (M:F = 43:94; mean age, 60.2 years). The prevalence of UIAs was 5% (n = 94) in women and 4% (n = 43) in men, respectively (p = 0.2046) and showed no age-related increase. The most common site of aneurysm was at the distal internal carotid artery (n = 64, 39%), followed by the middle cerebral artery (n = 40, 24%). In total, 99% of aneurysms measured less than 12 mm, and 93% of aneurysms measured less than 7 mm. Direct comparisons between MRA and DSA were available in 70 patients with 83 UIAs; the results revealed two false positive and two false negative results. CONCLUSION: This community-hospital based study suggested a higher prevalence of UIAs observed by MRA than previously reported. These findings should be anticipated in the design and use of neuroimaging in clinical practice.
Adolescent ; Adult ; Aged ; Aged, 80 and over ; Aneurysm, Ruptured/diagnosis ; Angiography, Digital Subtraction ; Cerebral Angiography ; Female ; Humans ; Incidental Findings ; Intracranial Aneurysm/*diagnosis/radiography ; *Magnetic Resonance Angiography ; Male ; Middle Aged ; Young Adult

Neuroleptic malignant syndrome (NMS) is a rare but potentially lethal outcome caused by sudden discontinuation or dose reduction of dopaminergic agents. We report an extremely rare case of NMS after deep brain stimulation (DBS) surgery in a cerebral palsy (CP) patient without the withdrawal of dopaminergic agents. A 19-year-old girl with CP was admitted for DBS due to medically refractory dystonia and rigidity. Dopaminergic agents were not stopped preoperatively. DBS was performed uneventfully under monitored anesthesia. Dopaminergic medication was continued during the postoperative period. She manifested spasticity and muscle rigidity, and was high fever resistant to anti-pyretic drugs at 2 h postoperative. At postoperative 20 h, she suffered cardiac arrest and expired, despite vigorous cardiopulmonary resuscitation. NMS should be considered for hyperthermia and severe spasticity in CP patients after DBS surgery, irrespective of continued dopaminergic medication.
Anesthesia ; Cardiopulmonary Resuscitation ; Cerebral Palsy* ; Deep Brain Stimulation* ; Dopamine Agents ; Dystonia ; Female ; Fever ; Globus Pallidus* ; Heart Arrest ; Humans ; Muscle Rigidity ; Muscle Spasticity ; Neuroleptic Malignant Syndrome ; Postoperative Period ; Young Adult

No abstract available.
Asphyxia* ; Brain* ; Humans ; Infant* ; Magnetic Resonance Imaging* ; Tomography, Emission-Computed, Single-Photon*