[Long-term results after velopharyngoplasty]. / Langzeitergebnisse nach Velopharyngoplastik bei Patienten mit Spaltbildungen des Gaumens.

BACKGROUND: Children with cleft palate frequently demonstrate speech and resonance disorders following primary cleft repair. In some patients no improvement can be achieved with conservative therapy and a velopharyngoplasty (VPP) may be indicated. This study was performed to evaluate the long-term results after VPP. MATERIAL AND METHODS: Twenty-six patients were followed up on an average 9 years after VPP (mean age: 16+/-5 years). Apart from the phoniatric-pedaudiologic findings, the speech intelligibility and nasality were judged by speech pathologists and by laymen. The extent of the speech handicaps was evaluated with a questionnaire. The nasalance was measured with NasalView. The vowels, two test sentences, and three reading texts (LT(1-3)) were used as test materials. RESULTS: The phoniatric-pedaudiologic examination revealed a clear improvement of hearing, language, and speech function. Compared to the previous results, decreased nasality and improved ability in articulation were detected. The judgment of laymen was also positive. The speech intelligibility was mostly evaluated as favorable. A speech handicap was present in only a few patients. The evaluation using NasalView showed significantly increased nasalance values for the LT(2) ( p=0.030). Moreover there were lower nasalance values for all the reading texts compared to the control group (LT(1) p=0.257, LT(2) p=0.408, LT(3) p=0.187). CONCLUSION: A clear improvement can be achieved with VPP in patients with a high degree of therapy-resistant nasality. In many cases even normal colloquial language is possible. The evaluation of nasalance has proved to be successful for rating surgical outcome.

Linuron sorption-desorption in field-moist soils.

Pesticide sorption or binding to soil is traditionally characterized using batch slurry techniques. The objective of this study was to determine linuron sorption in field-moist or unsaturated soils. Experiments were performed using low-density (i.e., 0.25 g mL(-)(1)) supercritical carbon dioxide to remove linuron from the soil water phase, thus allowing calculation of sorption coefficients (K(d)) at low water contents. Both soil water content and temperature influenced sorption. K(d) values increased with increased water content, if less than saturated. K(d) values decreased with increased temperature. K(d) values for linuron sorption on silty clay and sandy loam soils at 12% water content and 40 degrees C were 3.9 and 7.0 mL g(-)(1), respectively. Isosteric heats of sorption (DeltaH(i)) were -41 and -35 kJ mol(-)(1) for the silty clay and sandy loam soils, respectively. The sorption coefficient obtained using the batch method was comparable (K(f) for sandy loam soil = 7. 9 microg(1)(-)(1/)(n)() mL(1/)(n)() g(-)(1)) to that obtained using the SFE technique. On the basis of these results, pesticide sorption as a function of water content must be known to more accurately predict pesticide transport through soils.

Effect of treatment with amifostine used as a single agent in patients with refractory anemia on clinical outcome and serum tumor necrosis factor alpha levels.

Amifostine increases in vitro burst-forming unit-erythroid and colony-forming unit-granulocyte/granulcoyte-macrophage cultured from bone-marrow cells from patients with myelodysplastic syndrome (MDS). Several small clinical studies give divergent informations about the potential of amifostine as single agent to improve hematopoiesis in MDS patients. In these studies, patients with refractory anemia (RA), RA with excess of blasts (RAEB), and RAEB in transformation (RAEB-T) were analyzed together, resulting in response rates varying from 8% to 30%. The present multi-center study evaluated whether treatment with amifostine is of clinical benefit in patients with RA who are transfusion dependent. The effect on transfusion frequency as well as on platelets and absolute neutrophil count (ANC) was examined in 14 patients with RA [median age 67 years (55-72 years), male:female 9:5]. Four treatment cycles were planned, each consisting of intravenous amifostine at 200 mg/m2/day three times per week followed by a 2-week interval. Since tumor necrosis factor (TNF) alpha is a main suppressive cytokine for hematopoiesis in RA patients, serum samples for analyzing endogenous levels of TNF alpha were collected prior to the study and after four treatment cycles. In three patients (21%), reduced transfusion requirement with prolongation of the transfusion interval from 4 weeks to 8 weeks (two patients) and 4 weeks to 6 weeks was seen. An increase in ANC from 400/microliter to 2600/microliter and 200/microliter to 3400/microliter was observed in two patients. Platelets increased from 129,000/microliter to 277,000/microliter in an additional patient. In one patient, disease progression from RA to RAEB was observed. Serum TNF alpha levels were increased in MDS patients compared with normal controls (18.8 pg/ml vs 9.1 pg/ml), and there was no change during the treatment with amifostine (17.5 pg/ml). In conclusion, treatment with amifostine as a single agent was of limited benefit in patients with RA. The serum TNF alpha levels were unchanged during treatment with amifostine in RA patients.

Retrospective study of Creutzfeldt-Jakob disease in Belgium: neuropathological findings.

Creutzfeldt-Jakob disease (CJD) is a spongiform encephalopathy that affects about 1 in 10(6) inhabitants in most countries. Recently, a new variant of CJD has been linked to the epidemic of bovine spongiform encephalopathy. Therefore, vigilance concerning the disease's incidence has been increased. We conducted a comprehensive, nation-wide and retrospective study. In 79 Belgian autopsies, we found the characteristic triad of spongiosis, neuronal loss and reactive gliosis. The occipital cortex was most affected, while the cerebellum was mostly spared. Immunohistochemistry was performed using hydrated autoclave pretreatment and several monoclonal antibodies directed against the prion protein. We identified prion-immunoreactive patterns and locations reflecting the important heterogeneity, independently of the antibody that was used. Granular prion immunoreactivity was observed in astrocytes. We studied the regional intensity of the prion immunostaining and determined that the frontal cortex with 95% positive immunoreactivity was best suited for a biopsy. We studied the disease duration in sporadic CJD patients who showed neuropathological lesions of other neurodegenerative disorders (such as Alzheimer's disease). The study shapes the framework in which a prospective neuropathological registry will be able to function.

Early thalamic and cortical hypometabolism in adult-onset dementia due to metachromatic leukodystrophy.

A case of early-onset adult dementia with family history of dementia is reported, characterised by neuropsychological deficits, suggesting frontal involvement, with mild non specific white matter abnormalities on CT scan. Familial Alzheimer's disease was suspected but the neuropathological diagnosis on brain biopsy was metachromatic leukodystrophy. 18FDG-PET revealed a very peculiar pattern of metabolic impairment in thalamic areas, in medial and frontopolar regions, and in occipital lobes. Neuropsychological follow-up showed relatively stable difficulties of long-term memory and signs of frontal lobe dysfunction, similar to those observed in subcortical dementias. MRI subsequently showed periventricular leukoencephalopathy. The brain metabolic pattern observed in that case of metachromatic leukodystrophy was quite different from that reported in other types of dementia.

Sporadic Creutzfeldt-Jakob disease. A clinico-neuropathological analysis of nine definite cases.

The authors have analyzed clinico-neuropathologically nine cases of the definite sporadic form of Creutzfeldt-Jakob disease (CJD). All cases were female, with mean age of 62.7 years. Eighty-nine percent of the patients exhibited prodromal and initial psychiatric symptoms; definite signs of dementia, and myoclonus were present in 100% of cases. The EEG was abnormal in all cases and pseudoperiodic paroxysms were present in 56% of the patients. Their evolution time ranged from 3 to 19 months. Neuropathologically, brain and cerebellar atrophy, spongiosis, astrocytosis and neuronal loss were present in 100% of the patients. In 5 (56%) of these 9 cases, prion protein (PrP) amyloid plaques were detected in the cerebellum, by optical- and electronmicroscopy. There was a positive correlation between the number of plaques and the evolution time. The authors outline the similarities of their cases in the elderly with the new variant of CJD described in young people.

Enzymes of the purine nucleotide cycle in muscle of patients with exercise intolerance.

The activities of adenylosuccinate synthetase, adenylosuccinate lyase, and adenosine monophosphate deaminase were measured in muscle from patients suffering from fatigue and cramps following exercise. Results denote the existence of secondary deficiencies of adenylosuccinate synthetase and/or adenylosuccinate lyase in subjects with congenital or acquired myopathies. They also suggest that searches are warranted for primary deficiencies of adenylosuccinate synthetase as a cause of exercise intolerance.

Diagnostic and microsurgical presentation of intracranial angiolipomas. Case report and review of the literature.

Angiolipomas (ALs) are hamartomas composed of abnormally differentiated vessels and mature adipose tissue. Although they are most commonly found in peripheral tissues, ALs sometimes grow in the spinal epidural space. Intracranial ALs (ICALs) are rare: only seven cases have been reported in the literature. The authors describe the case of a 70-year-old woman who presented with ocular symptoms from a clinically and radiologically progressing parasellar ICAL. The radiological as well as the microsurgical findings are illustrated and compared with the seven previously published cases. The most frequent location of ALs is the skull base, especially the parasellar region. Other ICALs were diagnosed as components of cerebral arteriovenous malformations and were not symptomatic by themselves. Neuroradiological studies of ICALs usually demonstrate the characteristics of both adipose and vascular tissues. However, a review of the literature shows that the diagnosis had not been suspected preoperatively in any of the cases. Operative descriptions emphasize that most neurosurgeons were caught off guard by the profuse bleeding and the unusual relationship of this unexpected lesion to the cavernous sinus, so that removal was rarely complete. The authors conclude that preoperative diagnosis of ICALs is achievable based on magnetic resonance analysis, which should help optimize the microsurgical management of these lesions.

Acute corticosteroid myopathy in intensive care patients.

Several recent studies have attributed the occurrence of acute myopathy in intensive care unit patients to the combination of corticosteroids and neuromuscular junction blocking agents (NMBAs) used for mechanical ventilation. We present 4 patients who developed acute myopathy after administration of high doses of glucocorticoids during sedation with propofol without any NMBAs. All patients had elevated creatine kinase levels. Electrophysiological studies indicated normal motor and sensory nerve conduction velocities but reduced motor nerve response amplitudes. Needle electromyography identified abnormal spontaneous activity; motor unit potentials were polyphasic of low amplitude and short duration, characteristic of a myopathic process. Muscle biopsy demonstrated a prominent acute necrotizing myopathy in all 4 patients with a loss of thick filaments. Our observations support glucocorticoids rather than NMBAs as the main offending drug in acute corticosteroid myopathy. The predisposing factor should be the hypersensitivity of paralyzed muscles to corticosteroids regardless of the drug inducing paralysis: NMBAs or propofol.

Regional methionine and glucose uptake in high-grade gliomas: a comparative study on PET-guided stereotactic biopsy.

UNLABELLED: Gliomas are regionally heterogeneous tumors. The local relationship between histologic features and radiotracer uptake evaluated by PET should therefore influence analysis and interpretation of PET results on gliomas. This study explored this local relationship as a result of PET guidance of stereotactic biopsies. METHODS: Local histology was confronted to the regional uptake of 18F-2-fluoro-2-deoxy-D-glucose (18F-FDG) and 11C-methionine (11C-MET) in 14 patients with high-grade glioma diagnosed during a procedure of PET-guided stereotactic biopsies. We analyzed the uptake of both tracers in regions of interest centered on the stereotactic coordinates of 93 biopsy samples. RESULTS: A semiquantitative analysis revealed a significant regional correlation between 11C-MET and 18F-FDG uptakes. Uptake of both tracers was significantly higher on the site of tumor samples showing anaplastic changes than in the rest of the tumor. Presence of necrosis in anaplastic areas of the tumor significantly reduced the uptake of 11C-MET. CONCLUSION: PET with 11C-MET and 18F-FDG may help to evaluate, in vivo, the metabolic heterogeneity of human gliomas. Anaplasia is a factor of increased uptake of both tracers, but microscopic necrosis in anaplastic areas influences their uptake differently. This finding probably relates to the differences in tracer uptake by non-neoplastic components of necrotic tumors. These results underline the complementary role of 18F-FDG and 11C-MET for the study of brain tumors and favors their use for stereotactic PET guidance of diagnostic or therapeutic procedures.

Late effects of X irradiation on regulation of cerebral blood flow after whole-brain exposure in rats.

Hemodynamic parameters such as total cerebral blood volume (total CBV), cerebral parenchymal blood volume (CBV), cerebral blood flow (CBF) and cerebral blood velocity index were measured in rats 6, 12 and 18 months after single exposures of brain to 5, 10, 15 and 20 Gy X rays for total CBV, CBF and blood velocity index, and only 20 Gy for CBV. Total CBV and blood velocity index were determined by a noninvasive blood dilution method using [99mTc]pertechnetate and CBF by [131I]iodoantipyrine brain extraction. The CBV was obtained from both parenchymal plasma and erythrocyte volumes measured in isolated brain by 125I-labeled serum albumin and 51Cr-labeled erythrocytes, respectively. Neither the dose nor the time after irradiation influenced total CBV. Nevertheless, CBV decreased slightly while CBF decreased strongly at 12 and 18 months after 20 Gy. In contrast, the blood velocity index increased progressively at 12 and 18 months after 15 Gy and at all times after 20 Gy. According to the coexistence in irradiated brains of a remodeling with microvascular occlusions and dilated abnormal vessels, this lowered CBF can be explained by the smaller number of open capillaries and a "steal phenomenon" through low-resistance channels developed in the parenchymal and extraparenchymal vasculatures. Such a "steal phenomenon" is also supported by the response of the blood velocity index, which appears to be the earliest sensitive index for the detection of hemodynamic changes with respect to time (6 months) and dose of radiation (15 Gy).

Glucocorticoid-induced long-term remission in primary cerebral lymphoma: case report and review of the literature.

We report a 25-year old immunocompetent woman with a high grade primary non-Hodgkin's lymphoma of the central nervous system (PNHL-CNS) in whom the administration of dexamethasone alone during three months produced a complete clinical and radiological response lasting over four years. If complete remission of PNHL-CNS induced by glucocorticoids are well known, the opportunity to observe glucocorticoid-induced remission for a long period of time without radio- and chemotherapy is rare. Only nine other cases of PNHL-CNS with complete remission induced by glucocorticoids lasting from 6 to 60 months, were found in the literature and are summarized here. Duration of glucocorticoids therapeutic effect in PNHL-CNS is probably underestimated. Glucocorticoids cannot be recommended as sole initial treatment for PNHL-CNS. However, we suggest standard therapies to be delayed in those patients responding completely to glucocorticoids where radio- and chemotherapy should be contraindicated (kidney, liver, bone marrow failure, pregnancy).

Regional glucose metabolism and histopathology of gliomas. A study based on positron emission tomography-guided stereotactic biopsy.

BACKGROUND: Positron emission tomography (PET) with 18F-2-fluoro-2-deoxy-D-glucose (FDG) is widely applied to the study of gliomas. The histology of most gliomas is regionally heterogeneous. The relationship between histologic features and glucose metabolism evaluated by PET with FDG may therefore vary within the limits of the tumor. PET with FDG integrated in the planning of stereotactic brain biopsy allows precise comparison between local FDG uptake and histology. Using this approach, the authors investigated whether glucose metabolism of gliomas is related to anaplasia, and whether PET with FDG detects metabolic heterogeneity that parallels histologic heterogeneity of gliomas. METHODS: A total of 161 biopsy samples collected from 20 PET-guided procedures performed in patients with gliomas (8 low grade astrocytomas, 8 anaplastic astrocytomas, 1 anaplastic oligoastrocytoma, and 3 glioblastomas) were analyzed for the presence or absence of 8 histologic features. Stereotactic coordinates were used to calculate the metabolic rate of glucose (MRGlu) in the region of each biopsy sample. Gray and white matter MRGlu were used to define four metabolic grades that were compared with local histology. RESULTS: The difference in MRGlu expressed as micromoles per 100 g per minute was highly significant between anaplastic and nonanaplastic samples; the median +/- quartile deviation was 23 +/- 16 in anaplastic samples and 18 +/- 5 in nonanaplastic samples (P < 0.005). Even more significant differences were found when MRGlu was normalized to the cortex or to the white matter. Metabolic grades were different in anaplastic and nonanaplastic samples (P < 0.0001). Approximately 75% of samples metabolically graded 3 or 4 demonstrated signs of anaplasia, compared with 10% of samples graded 0 or 1. CONCLUSIONS: FDG uptake in gliomas is anatomically heterogeneous and is regionally related to the presence of anaplasia.

[Consensus report: tissue handling in suspected Creutzfeldt-Jakob disease and other spongiform encephalopathies (prion diseases) in the human. European Union Biomed-1 Concerted Action]. / Konsensusbericht: Gewebsbehandlung bei Verdacht auf Creutzfeldt-Jakob- Krankheit und andere spongiforme Enzephalopathien (Prionen-Krankheiten) des Menschen. European Union Biomed-1 Concerted Action.

Despite many sensational and intimidating reports in the mass media, transmissible spongiform encephalopathies (prion diseases) are not contagious in the usual sense. Successful transmission requires both specific material (an affected individual's tissue, from or adjacent to CNS) and specific modes (mainly penetrating contact with the recipient). Nevertheless, specific safety precautions are mandatory to avoid accidental transmission and to decontaminate any infectivity. The autopsy is essential for definite diagnosis of these disorders. Recommendations are given here for safe performance of the autopsy, for neuropathology service and appropriate decontamination; they are based on the current literature and on precautions taken in most laboratories experienced in handling tissue from transmissible spongiform encephalopathies. In essence, special care must be taken to avoid penetrating wounds, possible contamination should be kept to a minimum, and potentially infectious material must be adequately decontaminated by specific means. The full English text of this Consensus Report was published in Brain Pathology 5: 319-322 (1995).

[Internuclear bilateral pseudo-ophthalmoplegia and dermatomyositis]. / Pseudo-ophthalmoplégie internucléaire bilatérale et dermatomyosite.

A 60 year-old woman complaining of diplopia presents an ocular motility disturbance mimicking internuclear ophthalmoplegia. Idiopathic dermatomyositis is diagnosed by the help of clinical, biological, electrophysiological and histological data. The outcome is favorable under corticotherapy. Ocular muscle involvement is rare in dermatomyositis. An overlap syndrome with another auto-immune disorder like myasthenia should be excluded in this kind of manifestation.

Intracranial facial nerve rhabdomyoma. Case report.

Nerve rhabdomyomas are exceedingly rare benign tumors of the peripheral nerves consisting of well-differentiated striated muscle fibers admixed with parental nerve fibers. Only one case of intracranial nerve rhabdomyoma has been described, which affected the trigeminal nerve. This report presents the detailed neuropathological description of a nerve rhabdomyoma arising in the schwannian portion of the facial nerve root in a 41-year-old Caucasian man. The nerve fibers were arranged chaotically as in a traumatic neuroma. Because of the intimate intermingling of this slow-growing tumor with the parental nerve fibers, complete excision should be avoided.