Primary intradural chordoma: report on three cases and review of the literature.

OBJECTIVE: Chordomas are rare malignant bone tumors of the skull base or sacrococcygeal region. They derive from notochordal remnants and usually have a chronic progressive course. Even rarer, intradural chordomas with a better biological behavior have also been reported. We present 3 further primary intradural extraosseous chordomas with a favorable clinical outcome. CLINICAL PRESENTATION: Two patients, a 38-year-old man and a 44-year-old woman, presented with neck pain. In these, intradural extraspinal tumors within intervertebral foramina were found. Both tumors were totally removed and the patients have been free of disease for 7 years and 1 year, respectively. The other patient, a 76-year-old man suffered from an unspecific gait disorder and diplopia as a result of a prepontine space-occupying lesion. In this case, only an incomplete tumor resection was possible but progression has not occurred for 5 years. MATERIALS AND METHODS: Paraffin blocks from all cases were examined with classical histopathological stainings and immunohistochemistry for pancytokeratin, CK7, CK8/18, CK19, EMA, CEA, vimentin, S100, aktin, desmin, GFAP, CD117, PDGF-receptor alpha and beta, collagen-type-IV, p63, and Ki67. Fluorescent in situ hybridization was used to exclude EWS translocation. RESULTS: All cases showed the typical histological picture with physaliphorous cells in a myxoid matrix and the characteristic immunohistochemical profile with positivity for vimentin, pancytokeratin, CK19, EMA, and S100. Staining for P63 and type IV collagen was consistently negative. Myxoid extraskeletal chondrosarcoma was excluded by in-situ-hybridization of the EWS gene. CONCLUSION: Considering our cases in context with so far published literature, we conclude that intradural chordomas are rare and in this location usually have a better prognosis compared to classical intraosseous chordomas.

Chondroid tumors arising from the meninges--report of 2 cases and review of the literature.

Chondroid tumors are rare intracranial tumors usually arising from the base of the skull. We present 2 cases of intracranial cartilaginous tumors with unusual location. In case 1, a 19-year-old woman, a chondroma of the falx cerebri with extensive secondary ossification was diagnosed. In case 2, a 30-year-old woman, a low-grade chondrosarcoma was resected from the right frontal lobe. Both patients showed an uneventful clinical course without evidence of disease 4.5 and 6 years after total extirpation. Our cases show that chondromas and low-grade chondrosarcomas of the dura and meninges usually occur in young adults with a good prognosis after complete extirpation.

[The cervicogenic headache from the spiral surgery point of view--a pilot study]. / Der zervikogene Kopfschmerz aus wirbelsäulenchirurgischer Sicht. Eine Pilotstudie.

In a retrospective analysis of 299 consecutive patients with degenerative cervical spine disease the incidence of cervicogenic headache and the results of conservative and operative therapy have been investigated. Cervicogenic headache was noted in 117 of 299 patients (40%). 73 of those patients were treated conservatively. The cervicogenic headache resolved in 10% or improved in 27% and remained unchanged in 63% of the patients. 44 of these patients were operated by ventral discectomy, which was indicated in case of neurological deficit and painful shoulder-arm syndrome refractory to conservative treatment. In none of these patients the operation was indicated by the cervicogenic headache. In the postoperative follow-up with a mean of 6 months the headache resolved in 20%, improved in 60% and remained unchanged in 20% of the patients. These results suggest the existence of a cervicogenic headache, i.e. a headache due to disorders of the cervical spine, and the chance of successful operative treatment.

Repeated sleep deprivation once versus twice a week in combination with amitriptyline.

OBJECTIVE: Although a combination of antidepressive pharmacotherapy with repeated sleep deprivation therapy has proved to be an effective and easily applied treatment strategy, no systematic study investigating the most favourable interval between the sleep deprivation trials is available. METHOD: In a cross-over design, 44 patients with a major depressive episode (ICD-10) were randomly allocated to 4 weeks treatment with amitriptyline (150 mg/d) in combination with either late sleep deprivation (of the second half of the night, LSD) twice weekly during week 1 and 2 followed by LSDs once weekly during week 3 and 4 or vice versa. Two hypotheses were tested: Does LSD twice weekly during week 1 and 2 accelerate the clinical response compared with LSD once weekly? Does LSD twice weekly during week 3 and 4 improve the response rate compared with LSD once weekly? RESULTS: According to both observer rating (Hamilton Rating for Depression, 21-item version) and self-rating (Visual Analogue Mood Scale), no significant differences could be ascertained between the groups compared at any time of the investigation. With respect to drop-outs and immediate LSD effects there is a trend for patients undergoing twice weekly LSDs followed by once weekly LSD trials to have a more favourable treatment course than the control group. CONCLUSION: The hypotheses tested were answered in the negative. "High dose" LSD administration does not prove to be clearly superior to "low dose" LSD. This finding is in line with most of the corresponding data on antidepressive pharmacotherapy.

Chronic encapsulated intracerebral hematoma--a well-defined disease. Report on two cases and review of the literature.

Chronic encapsulated intracerebral hematoma (CEIH) is a rare disease which is believed to be caused by angiographically negative vascular malformations. CEIH has the following characteristic findings: 1. It affects all age groups 2. Clinical symptoms progress slowly after sudden onset. Often there is a latency of months or years 3. There is no correlation with arterial hypertension 4. Imaging reveals a typical fibrous capsule with enclosed blood contents and signs of recurrent bleedings 5. Cavernoma was identified histologically as the cause of bleeding in 30% of cases. 6. All patients had a primary diagnosis of intracerebral tumor. To the best of our knowledge, 27 cases have been reported in the literature. We now add two cases, one of which is the first in the available literature which was not operated and could be followed by imaging.

Ascending central nervous spreading of a spinal astrocytoma.

A 43-year-old man died from the complications of astrocytoma metastasis. He first noticed symptoms of a lumbar disc prolapse in 1979. In 1987 a pilocytic astrocytoma (grade I) of the spinal cauda was removed. In 1989 a tumor recidivation at the same site was partially removed. Histology showed a grade II astrocytoma. Two months later the patient developed symptoms of increased intracerebral pressure. CSF cytology showed polymorphic giant tumor cells with hyperchromatic nuclei and a glioblastoma of the cerebral ventricles was diagnosed. The patient died from cardiovascular complications. The post-mortem investigation revealed an astrocytoma of the conus medullaris with an anaplastic ventral area (grade IV). This area was inaccessible to the biopsy. It is believed that tumor metastases from anaplastic parts spread along the spinal cord and brainstem and finally invaded the brain and cerebral ventricles.

Magnetic source localization and morphological changes in temporal lobe epilepsy: comparison of MEG/EEG, ECoG and volumetric MRI in presurgical evaluation of operated patients.

Is MEG source analysis able to precisely locate the primary focal epileptic activity? 22 patients with pharmacoresistant temporal lobe epilepsy were recorded during presurgical evaluation simultaneously with multichannel MEG/EEG and invasive (subdural) electrodes to evaluate the increase of information gained by MEG concerning the localization of focal epileptic activity and lesions. With this systematic study it should become clearer how often MEG can establish a diagnostic bridge between function and morphology. In addition, MEG localization accuracy of focal epileptic activity was to be validated empirically by invasive EEG recordings and postsurgical outcome. Spikes in the MEG were used for magnetic source localization, and the result was combined with magnetic resonance imaging (MRI). All patients definitely suffered from temporal lobe epilepsy and revealed a structural abnormality in MRI. 17 patients with lesions in the temporal lobe were operated meanwhile and became markedly improved or seizure free. In 7 of 8 patients with a tumor and validated operation outcome, a very close correlation of the 3D-magnetic source localization and the border of the tumor in the brain was found (distance less than 10 mm). In 8 of 9 patients with a temporal/hippocampal atrophy and validated operation outcome, dipoles of epileptiform activity were located within the atrophic lobe.(ABSTRACT TRUNCATED AT 250 WORDS)

[Medulloblastomas--the results after postoperative radiotherapy with and without adjuvant chemotherapy]. / Medulloblastome--Ergebnisse nach postoperativer Radiotherapie mit und ohne adjuvante Chemotherapie.

Between 1975 and 1991, 40 patients with newly diagnosed medulloblastoma were treated at the authors' institutions. After aggressive surgical resection 39/40 (98%) received craniospinal radiation therapy with a local boost to the posterior fossa and other macroscopically involved areas. A group of 29 patients was treated with adjuvant chemotherapy. The five-year actuarial survival and event-free survival were 75% and 65%, respectively. Survival was significantly better for patients treated after 1981 as compared to those treated between 1975 and 1980 (p = .02). Younger age (two to four years) was associated with a better prognosis (p = .02). The extend of resection, Chang-stage, radiation dose to posterior fossa and the use of chemotherapy did not significantly impact on survival and relapse-free survival.

Brain-stem lesions in the course of a presurgical re-evaluation by foramen-ovale electrodes in temporal lobe epilepsy.

Two of 4 patients who were re-evaluated by means of foramen-ovale electrodes in the course of presurgical monitoring suffered a brain-stem lesion. Only 1 of these 2 patients already had a temporal resection. None of 55 patients who underwent this procedure for the first time had such a complication. We conclude that this electrode technique is not indicated to evaluate patients a second time, even if no surgical resection has been performed between the 2 recording sessions.

Ictal and interictal activity in partial epilepsy recorded with multichannel magnetoelectroencephalography: correlation of electroencephalography/electrocorticography, magnetic resonance imaging, single photon emission computed tomography, and positron emission tomography findings.

Ictal and interictal epileptic activity was recorded for the first time by multichannel magnetoencephalography (MEG) in three patients with partial epilepsy. Pre- and intra-operative localization of the epileptogenic region was compared. The interictal epileptic activity was localized at the same region of the temporal or frontal lobe as the ictal activity. Main zones of ictal activity were shown to evolve from the tissue at the centers of interictal activity. Pre- and intra-operative electrocorticography (ECoG) as well as postoperative outcome confirmed localization in the temporal and frontal lobe. Results also correlated with findings from scalp EEG, interictal and ictal single photon emission computed tomography (SPECT), positron emission tomography (PET), and magnetic resonance imaging (MRI). Combined multichannel MEG/EEG recording permitted dipole localization of interictal and ictal activity.

Serum prolactin concentrations and epilepsy. A study which compares healthy subjects with a group of patients in presurgical evaluation and circadian variations with those related to seizures.

In 20 healthy subjects (10 female and 10 male) and 17 patients undergoing presurgical epilepsy evaluation with intracranial EEG electrodes, circadian variations of serum prolactin (PRL) were measured. A comparison between the peak values found in normals with the postictal rises in patients, led us to consider 700 microU/ml to be the threshold of diagnostic value and the observed rises above this level to be all induced by seizures. In order to assess the clinical value of this threshold, PRL was measured postictally in a further 30 patients with epilepsy and in 11 patients with psychogenic seizures. In none of the latter group did PRL rises exceed 700 microU/ml, while they did so in 39% of the complex partial seizures and in 80% of the tonic-clonic seizures. There was no significant difference with respect to sex (a rise over 700 microU/ml in 42% in male and in 55% in female patients). Based on the findings in 17 patients investigated by means of intracranial electrodes, we were not able to establish different criteria for different focus localisations: in 66% of both temporal as well as frontal lobe seizures the 700 microU/ml level was exceeded. As a trend, in the period preceding an epileptic seizure we found a slightly decreasing PRL level, whereas in healthy persons the PRL concentrations gradually increased in the 40 minutes before the maximum spontaneous peak was reached.

Diagnosis of moyamoya disease with additional renal artery stenosis by colour coded Doppler sonography.

Moyamoya disease is a rare vascular anomaly of the cerebral arteries. The etiology of the disease has not yet been clearly identified. We report the noninvasive diagnosis of Moyamoya disease in a patients with a very early onset of symptoms in infancy. The diagnosis was made by colour coded Doppler sonography and confirmed by angiography at the age of 6 months, following two episodes of cerebral infarction. A bilateral encephalodurosynangiosis was performed at the age of 7 months with subsequent slight improvement of the neurological deficits. Colour Doppler sonography revealed early vascularisation from the fascia temporalis graft into the arachnoid space. At the age of 10 months the patient developed arterial hypertension caused by left renal artery stenosis. Our case suggests, that in infancy Moyamoya disease can be suspected noninvasively by colour Doppler sonography of the cerebral arteries. Patients should be carefully screened for possible extracranial arterial stenoses which may develop in the course of time. Encephalodurosynangiosis seems to be a good therapeutic option for patients with severe neurological symptoms.

Malignant tumors of the anterior cranial skull base.

Malignant tumors of the anterior cranial skull base are still a challenge for radical surgical treatment. Several different techniques and approaches have been developed over the years and the results, with mortality rates over 50%, are still not encouraging. Here we present our results of an interdisciplinary, one-stage, neurohino transfronto-transbasal surgical approach in twelve patients with such tumors. The long-term survival rate in our patients is now 83% with a mean postoperative follow-up of 19.3 months. Two patients died due to early recurrencies and metastasis after 10 and 13 months after initial treatment and postoperative irradiation. Another five patients with a follow-up of 34.2 months have had tumor recurrencies diagnosed at 19.5 months postoperatively. These patients have survived their recurrent tumor for 14.7 months. Five patients, 41% of the whole group, have been living without evidence of tumor recurrence for between 2 and 35 months.

[Ictal and interictal 99mTc-HMPAO-SPECT studies in temporal lobe epilepsy with unilateral EEG focus]. / Iktuale und interiktuale 99mTc-HMPAO-SPECT Untersuchungen bei Temporallappenepilepsien mit unilateralem EEG-Fokus.

Ictal and interictal SPECT measurements using 99mTc-HMPAO are helpful in localising an epileptic focus. 24 patients (12 women and 12 men; mean age 30 years) suffering from temporal lobe epilepsies with unilateral EEG foci were examined. In all patients a presurgical evaluation including sphenoidal and foramen oval electrodes was carried out. The analysis of SPECT measurement was done visually and semi-quantitatively, comparing the data with findings in healthy persons (10 women and 10 men; mean age 24 years). The etiology of the epilepsy was confirmed by histological investigation in 16 cases, and by CT and MRI in 8 cases. The findings were gliosis in 6 cases, astrocytoma in 3 cases, cysts in 4 cases, and angioma in 3 cases. In 8 cases no pathological findings could be detected. In 46% of cases interictal SPECTs exhibited a focal hypoperfusion corresponding with the EEG-focus. In ictal SPECTs this could be found in 75% of cases. In all 10 patients without morphological findings or with gliosis (proved by histological investigations) a hyperperfusion coincident with the EEG-focus appeared in ictal SPECTs.

The neocortico to mesio-basal limbic propagation of focal epileptic activity during the spike-wave complex.

In order to localize epileptogenic electrophysiological sources, a multichannel MEG system was used in 3 patients with partial epilepsy during presurgical evaluation. MEG and EEG (including scalp, sphenoidal and intracranial foramen ovale electrodes) were recorded simultaneously during a period of intensive video-EEG monitoring in order to observe single spontaneous spikes. In addition to MRI, SPECT and PET investigations were performed. Electrical activity subsequent to the activity of the epileptic focus could be localized by the MEG after noise reduction using a temporal correlation technique. Simultaneous registration of the magnetic field and the electrical field showed that the source of the primary focal epileptic activity (first period during the total spike wave complex where a dipolar magnetic field pattern is found) is localized in neocortical lateral regions, whereas another focal epileptic activity in a later phase of propagation occurs in temporal mesial regions. In 1 patient (case 1) the primary focal epileptic activity was localized in the surrounding neocortical tissue of an angioma and the middle and inferior temporal gyrus. The second phase of propagation is localized in temporo-basal-mesial regions, including para- and hippocampal structures. The latest center of activity occurred in posterior parts of the gyrus cinguli. In 2 other patients, the primary focal epileptogenic activity was localized at the insula and also spread into temporal basal mesial regions. A multi-modal approach to research of focal epilepsy, combining metabolic, electrical potential, magnetoencephalographic and morphological data, recorded by non-invasive techniques, offers new perspectives for the detection of involved brain regions. The 3-D and time-resolved localization of focal epileptic activity, correlated with the individual anatomy of the human brain, may improve the determination of neuronal populations involved in the individual epileptogenic process, especially in the interaction between temporal or extratemporal neocortex and limbic system.