Infantile hemiplegia is the end state of various pathological conditions affecting the cerebral hemisphere before or during birth, or in first few years of life. In 1950, Krynauw first reported that total hemispherectomy could be carried out with beneficial results for infantile hemiplegia with uncontrollable convulsion, temper tantrum and mental deficiency. Hemispherectomy is not suitable for all cases of infantile hemiplegia, but is seems clear that it should be undertaken in any case of established infantile hemiplegia with fits which cannot be controlled by drugs, or in which there is backwardness or deterioration in the patient's intellectual or emotional state. We have recently experienced a 8-year-old boy who was suffering left hemiplegia with intractable epilepsy. Both carotid angiogram and pneumoencephalogram showed right cerebral hemiatrophy. After right hemispherectomy intractable seizure was controlled and personality disorder disappeared completely. But left hemiplegia wasn't definitely improved.
Cerebrum ; Child ; Epilepsy* ; Hemiplegia* ; Hemispherectomy* ; Humans ; Intellectual Disability ; Male ; Parturition ; Personality Disorders ; Seizures

If in the aneurysmal rupture patients the brain metabolic parameters obtained from blood chemistry were significant and useful in clinical practice, it cannot be overstimated. Hansdorfer er al.(1973) reported that lactate, pyruvate, uric acid and alpha-HBDH of central venous blood obtained form the patients with brain contusion in basal metabolic state were significantly increased and they were useful in evaluating the prognosis of the patients. Zooping(1970) and Broderson(1974) also had tried to evaluate the prognosis and brain metabolic status of the comatous patients with blood gas analysis and CSF biochemistry. They encouraged us to estimate lactate and lactate/pyruvate ratios of canine jugular venous blood combined with gas analysis. Complete cerebral bloody-ischemia similar with initial stage of aneurysmal rupture was induced by the instanteneous elevation of intracranial pressure 30 mmHg above systemic arterial pressure by infusion of blood and mock CSF mixture into the cisterna arrest or pulmonary hypertension were discarded. At the end of the 5 minutes ischemic period, the needle tip which was inserted in to cisterna magna was removed without decreasing intracranial pressure. At 3 hours, 24 hours, 48 hours and 72 hours after ischemic period jugular venous and arterial blood were sampled for determination of lactate and pyruvate, and blood gas analysis. The following results were obtained. ie ; 1. Both lactate and pyruvate of canine jugular venous blood were increased from 3 hours and reached peak level at 24 hours after insult. Standard value of lactate and pyruvate were 1.416mM and 0.075mM and peak values were 2.429 and 0.165mM(P<0.05). 2. The more severe the neurological deficits of the animals, the highest levels of lactate and pyruvate were observed. 3. The lactate concentrations in 48 hours and 72 hours sample slopped down from 24 hours peak level but were significantly higher than those of standard. 4. Pyruvate returned to the normal range within 48 hours after insult. 5. L/P ratios were not changed significantly until 48 hours after insult but steeply elevated in 72 hours sample. 6. In gas analysis all the animals show respiratory alkalosis after insult. 7. In arterial boundary zones multiple focal ischemia were found in necropsy which was thought as reflecting no-reflow phenomenon. We concluded that elevation of lactate and pyruvate in early stage must be due to the hyperventilation after insult and lactate of late stage reflected CSF lactic acidosis.
Acidosis, Lactic ; Alkalosis, Respiratory ; Aneurysm ; Animals ; Arterial Pressure ; Biochemistry ; Blood Gas Analysis ; Brain ; Brain Injuries ; Chemistry ; Cisterna Magna ; Humans ; Hypertension, Pulmonary ; Hyperventilation ; Intracranial Pressure ; Ischemia ; Lactic Acid* ; Needles ; No-Reflow Phenomenon ; Prognosis ; Pyruvic Acid ; Reference Values ; Rupture ; Uric Acid

BACKGROUND: Spinal anesthesia has several advantages in cesarean section. Those are rapid induction, complete analgesia, profound muscle relaxation, low failure rate and systemic toxicity. But the determination of anesthetic level is more difficult in spinal anesthesia than in epidural anesthesia. So we would like to determine the appropriate dosage of 0.5% hyperbaric bupivacaine and the effects of addition of intrathecal fentanyl. METHODS: Sixty full term parturiants scheduled for cesarean section were randomly received 0.5% hyperbaric bupivacaine 8, 10, 12 mg intrathecally. Analgesia, sensory and motor blockade, muscle relaxation, hypotension and side effects were assessed. We compared these results with the previous study that was done with the same dosages mixed with 10 microgram fentanyl. RESULTS: The maximum level of block was higher in 12 mg group (T2) then 8 and 10 mg group (T3). 5 parturients showed more than T1 sensory block in 12 mg group (n=20). 4 parturients did not reach T4 in 8 mg group (n=20). 7, 4 and 0 parturients complained pain during operation in each 8, 10, 12 mg group (p=0.012 between 8 and 12 mg group, p=0.062 between 10 and 12 mg group). Addition of 10 microgram fentanyl showed better analgesia and less shivering. CONCLUSION: Intrathecal 0.5% hyperbaric bupivacaine 12 mg showed complete analgesia for all parturients but the tendency of high spinal anesthesia. Analgesia was inadequate in 8 mg group. Addition of 10 microgram fentanyl showed better analgesia and less shivering.
Analgesia ; Anesthesia, Epidural ; Anesthesia, Spinal* ; Bupivacaine* ; Cesarean Section* ; Female ; Fentanyl* ; Hypotension ; Muscle Relaxation ; Pregnancy ; Shivering

Segmental neurofibromatosis is a rare disorder characterized by cafe-au-lait spots and neurofibrobmas, or only neurofibroma, limited to one region of the body. Disease-associated systemic involvement is uncommon. Most patients with segmental neurofibromatosis do not have a family history of neurofibromatosis. In Korea, there have been 3 reported cases of segmental neurofibromatosis. None of them had caf6-au-fait spots, systemic involvement, or family history. This report describes a case of segmental neurofibromatosis in a 25-year-old woman who had Becker's nevus-like cafe-au-lait spots and this was linked to the presence of Fanconi's syndrome in a second degree relative of the patient.
Adult ; Cafe-au-Lait Spots ; Female ; Humans ; Korea ; Neurofibroma ; Neurofibromatoses* ; Neurofibromatosis 1

Cryptococcosis is the most common form of fungal infection of the central nervous system, which is classified as meningeal, embolic and perivascular type. Cryptococcal meningitis and meningo-encephalitis are not infrequent, but the only 40 cases of localized torula granuloma in the central nervous system had been reported in the worldwide literature until 1973 since the first reports of LeCount and Myers in 1907. Our new case reported here, a thirty-eight tear old female patient was admitted to our department because of abruptly appeared headache with nausea and vomiting, drowsy mental state and left hemiparesis. On admission the left hemiparesis and remarkable meningeal irritation signs were found. Chest film and skull films showed no evidence of abnormality. The right carotid angiogram revealed the shifting of the anterior cerebral artery to the left side with concomittant U-loop widening (Fig-1), Sylvian triangle displaced posteriorly, closed carotid siphon(Fig. 2) and abnormal draining vessels with staining on the frontal base of cerebrum (Fig. 1,2,3,4). Examination of the cerebrospinal fluid showed remarkable polymorphonuclear leucocyte predominant pleocytosis, elevated protein and normal sugar content. And negative smear for cryptococci and other microorganisms. A right frontal craniotomy and brain exploration exposed grayish gelatinous mass of about 3.5cm in diameter, which was partially removed. Microscopic findings were acute and chronic inflammatory changes in different stages with various types of giant cells and cryptococci (Fig. 5,6). On the third postoperative day, the patient died. Neither amphotericin-B nor 5-Fluorocytosine was administered because preoperative diagnosis was tumor, and postmortem examination couldn't be done.
Anterior Cerebral Artery ; Autopsy ; Brain ; Central Nervous System ; Cerebrospinal Fluid ; Cerebrum ; Craniotomy ; Cryptococcosis ; Cryptococcus* ; Diagnosis ; Female ; Flucytosine ; Gelatin ; Giant Cells ; Granuloma* ; Headache ; Humans ; Leukocytosis ; Meningitis, Cryptococcal ; Nausea ; Paresis ; Skull ; Thorax ; Vomiting

PURPOSE: To evaluate the effectiveness and safety of treating orbital lesions with gamma knife radiosurgery (GKS). METHODS: Between April 2004 and January 2006, ten patients who had orbital tumors or vascular lesions and who underwent GKS were included in this retrospective study. RESULTS: Ten patients with orbital lesions were treated with GKS. The group of orbital lesions consisted of 5 meningiomas, 2 schwannomas, 1 cavernous hemangioma, 1 arteriovenous fistula, and 1 adenoidcystic carcinoma of the lacrimal gland. The most common symptom was proptosis. The tumors were located at the orbital apex in eight patients, and five of these patients were treated with fractionated stereotactic radiosurgery. The mean cumulative marginal dose was 17.0 Gy (12-20 Gy), and the mean cumulative maximal dose was 30.8 Gy (16.2-40.4 Gy). The follow-up period ranged from 3 to 25 months (mean 13.9 months). During the follow-up period, magnetic resonance imaging revealed a decrease of tumor volume in 3 patients with symptomatic improvement. In two patients, tumor volume increased. No radiation-induced optic neuropathy, retinopathy, or cataract was observed in any of the 10 patients during the follow-up period. CONCLUSIONS: Gamma knife radiosurgery is an effective and relatively safe treatment when orbital lesions have a high risk of neurosurgical deficits with surgery, when they recur after incomplete resection, or when complete removal of tumor is impossible.
Arteriovenous Fistula ; Cataract ; Exophthalmos ; Follow-Up Studies ; Hemangioma, Cavernous ; Humans ; Lacrimal Apparatus ; Magnetic Resonance Imaging ; Meningioma ; Neurilemmoma ; Optic Nerve Diseases ; Orbit ; Radiosurgery ; Retrospective Studies ; Tumor Burden

PURPOSE: To determine the efficacy of frontalis sling operation with silicone rods in patients with compromised corneal protective mechanisms. METHODS: The authors retrospectively studied 6 consecutive patients (7 eyelids) with severe blepharoptosis with poor ocular motility who had undergone frontalis sling operations using silicone rods. RESULTS: Preoperative diagnoses included third nerve palsy in 4 patients, double elevator palsy in 1 patient, and suspicious oculopharyngeal muscular dystrophy in 1 patient. With a mean follow up of 27.7 months, a good final lid height was achieved in all 7 eyelids. Mild exposure keratopathy occurred postoperatively in 3 patients. During the follow-up period, no other significant complications, such as extrusion of the sling or infection, occurred. CONCLUSIONS: Silicone rods are effective and safe materials for use in frontalis suspension in treating blepharoptosis in patients with inadequate or absent Bell's phenomenon, resulting in poor eye protective mechanisms associated with an increased incidence of corneal exposure.
Blepharoptosis ; Elevators and Escalators ; Eye ; Eyelids ; Follow-Up Studies ; Humans ; Incidence ; Muscular Dystrophy, Oculopharyngeal ; Oculomotor Nerve Diseases ; Paralysis ; Retrospective Studies ; Silicones

The salient pathological findings following acute blunt cord trauma, now documented by a number of investigators, are hemorrhagic necrosis of central gray matter and increased catecholamine, especially dopamine concentration of the traumatized cord level, which have progressive and, according to some authors, reversible nature. But paraplegia and disappearence of sensory evoked potential (SEP) are the obvious clinical conditions appeared immediately after blunt cord trauma which are sudden events not progressive ones. These phenomena are clearly results of neuronal conduction block of white matter at the traumatized cord level. Dohrmann et al. demonstrated in their electron microscopic studies that the myelinated nerve fibers of the traumatized cord level with 300 gm-cm force are resemble those of a control animal until 15 to 20 minutes after impact. At this point, we can imagine 2 phase of pathophysiological process of acute blunt cord trauma, ie ; 1. Initial alterations of normal cord physiology. 2. Secondary or aggrevating processes to the initial alterations of normal physiology. There are a number of reports and arguements about so called secondary aggrevating processes but neither explanations nor experimental studies about those immediate pathologic clinical phenomena. As the initiation of the investigations about the immediate physiological alterations, we planned this experiment to observe radioactive potassium uptake of the white matter of the blunty traumatized spinal cord. At first, we carried dorsal laminectomy out on the T2 and T6 level of adult Mongoreal dogs and injected K42 Cl, 500 micro Ci into the right atrium via catheterized antecubital vein. At 6 seconds after injection of K42, 5cc bolus of saturated KCI was shot intravenously via same route to produce instant cardiac arrest. As rapidly as possible, the cord of T2 and T6 were excised and dissection of white matter was done which was facilitated by use of loupe. After tissue weighing, tissue indicator was counted with Well-type Scintillating Counter. As a result, the radioactivity of T6 white matter (cpm/mg) was 77% (SD=2.04) of that of T2, which was essentially same as the regional blood flow rates measured by Bingham et al. with fractional indicator technique. Secondly, we elicited seizures on the animals by electrical stimuli delivered to the electrode attached on the bifrontal scalp. Seizure duration was 3 minutes and at the time of termination of tonic phase 500 micro Ci of K42 Cl was injected. 6 seconds later cardiac arrest was produced and radioactivity of the white matter T2 and T6 were measured with same way described above. Radioactivity of the white matter of T2 and T6 were measured with same way described above. Radioactive potassium uptake of the white matter of T2 and T6 were 57.82% (SD=2.62) and 61.38% (SD=3.69) to those of control animal. This result can be explained as a greatly increased K+ ion concentration in the interstitial space of white matter during the convulsive seizure. Next time, the animals were laminectomized on the level of T2 and T6, and 500 gm-cm force was impacted on the T6 exposed cord while T2 segment served as non-traumatized control. 15 minutes after impact, 500 micro Ci of K42 was injected and at 6 seconds after injection cardiac arrest was produced. And radioactivity of white matter was measured. The K42 uptake of the T6 white matter was 171%(SD=24) to that of T2, which was thought as a reflextion of increases regional blood flow. Alteration of blood-brain barrier resistance must be influenced upon the K+ ion uptake but they may be masked by increased blood flow, which was supported by a biphasic curve obtained from time-lag experiments, ie; decreased uptake in 1 1/2 hour group, increased uptake in 2 1/2 hour group and approach to the base line in the 4 hour group. Another experimental study to discover so called immediate physiological changes will be presented in the next report.
Adult ; Animals ; Blood-Brain Barrier ; Catheters ; Dogs ; Dopamine ; Electrodes ; Evoked Potentials ; Heart Arrest ; Heart Atria ; Humans ; Laminectomy ; Masks ; Necrosis ; Nerve Fibers, Myelinated ; Neurons ; Paraplegia ; Physiology ; Potassium ; Radioactivity ; Regional Blood Flow ; Research Personnel ; Scalp ; Seizures ; Spinal Cord Injuries* ; Spinal Cord* ; Veins

Letterer-Siwe disease is a one of Langerhans cell histiocytosis and characterized by proliferation of Langerhans cells. It's clinical features are onset of infancy, hemorrhagic crusted papules and petechiae, hepatomegaly, lymphadenopathy, localized bone defect, and fatal outcome. We report a 38-year-old woman with hepatomegaly, lymphadenopathy, scaly papules and petechiae on the trunk. The eletronmicroscopy showed a tennis racquet shaped Birbeck granules in cytoplasm of histiocyte. Systemic treatment with etoposide, cyclophosphamide, prednisone, and vincristine was effective.
Adult* ; Cyclophosphamide ; Cytoplasm ; Etoposide ; Fatal Outcome ; Female ; Hepatomegaly ; Histiocytes ; Histiocytosis, Langerhans-Cell* ; Humans ; Langerhans Cells ; Lymphatic Diseases ; Prednisone ; Purpura ; Tennis ; Vincristine

BACKGROUND: An intrathecal injection of a small-dose local anesthetic and an opioid using combined spinal-epidural anesthesia (CSEA) technique is a new trend in regional anesthesia for cesarean section. Prophylactic epidural injection may be useful to complete the new regimen. METHODS: Spinal anesthesia via the CSEA technique was performed with 6 mg 0.5% hyperbaric bupivacaine plus 20ng fentanyl in 75 parturients undergoing cesarean section. group S (study, n = 38) received an epidural injection of 10 ml of 0.25% bupivacaine 5 min after intrathecal injection, and was compared with group C (control, n = 37) in sensory levels and Bromage scores, incidences of side effects such as hypotentsion, pain and discomfort, nausea and vomiting, dizziness, pruritus and shivering. Recovery times from sensory and motor block and the duration of postoperative analgesia were assessed between the groups. RESULTS: Sensory block levels were higher (but remained Analgesia ; Anesthesia* ; Anesthesia, Conduction ; Anesthesia, Spinal ; Bupivacaine* ; Cesarean Section* ; Dizziness ; Female ; Fentanyl ; Humans ; Incidence ; Injections, Epidural ; Injections, Spinal ; Nausea ; Pregnancy ; Pruritus ; Shivering ; Vomiting