[Combined spina-bifida clinic].
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Biomedical subjects
Publications and source records attributed to U Wald.
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Several unusual aspects of primary cerebral arachnoid cysts are presented. The optimal management of these lesions is discussed. Attempts at craniotomy and excision of these cysts is not always effective in eliminating the lesion and may provoke intracranial catastrophies. Huge and bilateral cysts are best treated by cystoperitoneal shunting. In general shunting may be preferable to craniotomy and resection of the cyst. An anti-siphon device is recommended to avoid overdrainage of cerebrospinal fluid.
Three children are presented with dermoid cysts of the posterior fossa, one of the most common locations of dermoid. They were initially diagnosed as having abscesses. Clinical presentations included hydrocephalus and recurrent meningitis. A helpful clue for identification is a bony defect of the skull on plain film or on a bone-window at Computed Tomography (CT).
Central neurogenic hyperventilation is a rare but important cause of the frequently observed phenomenon of hyperventilation. Its diagnosis demands that primary respiratory or metabolic causes, as well as cerebrospinal fluid abnormalities, be ruled out. We hereby describe a patient whose attacks of hyperventilation were the presenting sign of her disease and might be consistent with the central neurogenic mechanism. This patient suffered from a medulloblastoma which compressed the pons. After removal of the tumor the hyperventilation attacks ceased.
Based on the successful use of a basement membrane-like extracellular matrix (ECM) as a physiological substrate for the growth of adult rat oligodendrocytes, we have applied the system to oligodendroglia of human origin. ECM is excreted and deposited on a culture dish by endothelia cells. Glial cells isolated from fresh human brains by a Percoll density gradient were maintained on ECM for several weeks in vitro. Cells identified as oligodendrocytes by anti-galactocerebroside (GalC) could be transferred and replated on ECM-coated plates. In one case, GalC-positive cells isolated from a biopsy of an 8-year old girl were able to incorporate [3H]thymidine as revealed by autoradiography. ECM can be used successfully for the growth and in vitro maintenance of human oligodendrocytes.
A 4-year-old girl with a right ventriculoperitoneal shunt presented with a huge subcutaneous fluid collection in the scalp on the right side. There were no neurological symptoms or signs. On CT, a normal-sized ventricular system was seen. The shunt was disconnected distal to the antisiphon device and transmitted fluid into a subgaleal collection, which functioned as a reservoir for excess fluid. The ability of the subgaleal space to serve as a reservoir for cerebrospinal fluid is discussed.
A randomized, placebo-controlled, double-blind and sequentially analysed clinical trial to determine the efficacy of intra-operative parenteral gentamicin and vancomycin (with streptomycin in the irrigating solution) in preventing infection at the operative site following neurosurgical procedures is described. Patients receiving prophylaxis had a significantly (P = 0.046) lower operative site infection rate (2/71 = 2.8%) than those receiving placebo (9/77 = 11.7%). This difference was most apparent during an epidemic, the source of which was not evident. Moreover, a total of 13 infections (two operative site, five pneumonia and six urinary tract) occurred among 12 patients receiving prophylaxis, whereas there was a total of 31 infections (nine operative site, nine pneumonia, 10 urinary tract and three septicaemia) among 24 patients receiving placebo. A smaller quantity of antimicrobial drugs was administered postoperatively to patients receiving prophylaxis (3.96 'antibiotic-days' per patient) than to those receiving placebo (6.87 'antibiotic-days' per patient).
The case of an asymptomatic 7-year-old girl with a hard bulge in the left forehead is presented. The radiological evaluation disclosed a hyperostotic mass with a large intracranial extension. A surgical specimen revealed a meningioma. The presence of a localized cranial bulge as the only sign of an intracranial tumor and the diagnostic value of computerized tomography (CT) are discussed.
Gangliogliomas are rare tumors, primarily seen in patients under the age of 30 years. They occur least commonly in the spinal cord. This report presents a 2 1/2-year-old boy who harbored an intramedullary conus tumor with the light microscopic appearance of a ganglioglioma. The neurobiological behavior of this tumor is yet to be determined.
A vertex epidural hematoma of a 21-year-old man was diagnosed by angiography 1 month after head trauma. Two axial computed tomography scans did not reveal the lesion, whereas a coronal computed tomography scan demonstrated the hyperdense hematoma. The problems of detecting vertex lesions with axial computed tomography scans are discussed.
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A 6-year-old girl with signs of a space occupying lesion had a CT scan compatible with a brain tumor. A preoperative hemogram revealed thrombocytopenia which was later proved to be due to ITP. Intracranial bleeding can rarely be the first manifestation of ITP. Early recognition of this situation aided by serial CT follow-up may lead to an improvement in the handling of these patients.
A patient with acutely elevated intracranial pressure (ICP) and a severe attack of bronchoconstriction is presented. Further investigation after treating the combined emergency situation revealed uncompensated hydrocephalus. Possible mechanisms which might be responsible for the coexisting emergency states are discussed. The urgent need for immediate simultaneous treatment of both disease entities is emphasized.
We report two cases of children, 7 and 14 yr old, in whom prolonged infusion of thiopental sodium (TS) was used to control intracranial hypertension previously unresponsive to conventional therapy. Intracranial hypertension followed removal of a large tumor in 1 case, and trauma in the other. TS was administered at a rate of up to 7 mg/kg . h for 8 days in 1 patient, and up to 12 mg/kg . h for 10 days in the other. Both children regained consciousness and made significant recovery of neurological function. The advantage and the side-effects of the prolonged use of TS for intracranial hypertension are discussed.
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A 6-year-old girl developed generalized seizures followed by coma, five days after surgical removal of a craniopharyngioma. Low serum sodium levels and low serum osmolality with inappropriately high urinary sodium output confirmed the diagnosis of inappropriate antidiuretic hormone (ADH) secretion. Treatment with 3% hypertonic saline solution and repeated doses of furosemide (1 mg/kg) improved her clinical condition; serum sodium levels, however, rose slowly and urinary excretion remained high. Deoxycorticosterone acetate (DOCA), 4 mg/sq m/day, was added to the above regimen. A striking clinical improvement was noted. Serum sodium levels returned to normal with a concomitant sharp decline in urinary sodium output. The clinical course of this patient demonstrates the efficacy of the addition of deoxycorticosterone acetate to hypertonic saline and furosemide in the treatment of severe, life-threatening hyponatremia due to the syndrome of inappropriate antidiuretic hormone secretion.
The distribution of [14C]inulin was measured in isolated spinal cords of larval and feeding stage adult forms of sea lamprey (Petromyzon marinus) and expressed in per cent of total cord wet weight. In larval cord the apparent inulin space reached a plateau value of 32--33% within 2.5. min. This correlates well with electrophysiological experiments in which 10(-7) M tetrodotoxin added to the perfusion fluid blocked the responses of giant interneurons to both intracellular and rostral cord stimulation in 1 to 2 min. Thus the plateau level of inulin space probably represents the extracellular space. [14C]Mannitol did not reach a steady distribution space even after 30 min of incubation. Therefore, mannitol is not an accurate extracellular space indicator in the isolated lamprey spinal cord. The inulin space increased with increasing temperature of incubation. Average inulin spaces for larval spinal cords incubated at 5, 10 and 22 degrees C were approximately 26%, 33% and 42% respectively. The inulin space of isolated adult lamprey spinal cords was about 18--19%. Since in larvae the inulin space did not vary consistently with the sizes (and therefore presumably the ages) of the animals, it is likely that the reduction in inulin space during maturation does not occur gradually during the larval phase, but probably occurs during transformation. The difference between the inulin spaces of isolated larval and adult spinal cords is reflected qualitatively in the electron microscopic appearance of the extracellular space. We conclude that the inulin space in the lamprey spinal cord behaves similarly to the picture of the mammalian brain extracellular space which has emerged in recent years. Because of the rapidity of inulin diffusion in the lamprey cord and the unambiguous time-dependent behavior of the inulin space of the isolated lamprey cord, the latter would seem to be useful model for the extracellular space of the vertebrate central nervous system.
The authors studied 86 consecutive patients admitted to the ICU after severe head trauma. Of these, 19 nondiabetic patients developed persistent nonketotic hyperglycemia (NKH), defined as plasma glucose greater than 270 mg/dl (15 mM/L), 1.7 +/- 1.1 (SD) days after injury. When NKH occurred, all patients were in very deep coma (Glasgow Coma Scale score 3), and when intracranial pressure (ICP) was measured (12 patients), its mean value was 59.8 +/- 20.4 mm Hg. Although 11 of 19 patients had associated diabetes insipidus (DI), NKH was not related to increased fluid and glucose loading. All patients with NKH died (mean time of survival after NKH was 2.1 +/- 1.4 days) in contrast to a 17.1% early mortality rate in patients with severe head injury without NKH. The authors could not show a direct correlation between the height of ICP and the level of blood glucose.