Primary granulomatous hypophysitis not responsive to pulsed high dose prednisolone therapy: case report.
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Adolescent↗
Biomedical subjects
J Schramm
Publications and source records attributed to J Schramm.
Significance of factors contributing to surgical complications and to late outcome after elective surgery of cerebral arteriovenous malformations.
OBJECTIVES: This study focuses on the relevance of size, eloquence, type of venous drainage, the Spetzler-Martin scale as a whole, and other factors, such as rupture of cerebral arteriovenous malformations (AVMs) for the prediction of neurological deficits in the context of microsurgical AVM removal. METHODS: One hundred and fifty patients with AVMs, whose data were retrieved from a prospectively employed computerised data bank were included. Seventeen patients (11.3%) underwent preoperative embolisation. According to the Spetzler-Martin scale they were graded as follows: 22.0% grade I, 32.0% grade II, 29.3% grade III, 14.0% grade IV, and 2.7% grade V. Intracerebral haemorrhage was present in 39.0%. The AVMs were <3 cm in 52.00/0, 3-6 cm in 43.3% and >6 cm in 4.7%; 59.3% of the AVMs were eloquently located and 29.3% had deep venous drainage (DVD). Follow up information was assessed 6 months after surgery in all but one patient, who died. The applied statistical test was chi2. RESULTS: Surgical morbidity was 15.3%. Early new deficits were noted in 39.3%, permanent new deficits in 10.6%, being significant (major) in 7.3%. The occurrence of permanent deficits correlated significantly with size, deep venous drainage, and the Spetzler-Martin scale. There was statistical evidence for a trend in risk of poor surgical outcome across the three categories non-eloquent, "less eloquent" (for example, visual cortex) and "highly eloquent" (brainstem, basal ganglia, or precentral cortex) with the last being associated with the highest risk for permanent neurological compromise. CONCLUSION: "Eloquence" of the Spetzler-Martin scale should be divided into "highly eloquent" and "less eloquent", which is important for risk analysis of the treatment of asymptomatic and deep seated AVMs and for future trials comparing various treatment modalities. In addition, resection of eloquent AVMs v non-eloquent ones is significantly associated with higher surgical morbidity.
Adolescent↗
Expression of 5alpha-reductase in the human temporal lobe of children and adults.
Androgens exert important biological effects on the brain, and 5alpha-reductase plays a crucial role in androgen metabolism. Therefore, we investigated the expression of the two isozymes of 5alpha-reductase in the human temporal lobe to determine the predominant isoform and to elucidate the existence of possible sex differences and differences between children and adults. We studied biopsy materials from the temporal lobe of 34 women, 32 men, and 12 children. Quantification of 5alpha-reductase 1 and 2 messenger ribonucleic acid (mRNA) was achieved by competitive RT-PCR. 5Alpha-reductase activity was determined in tissue homogenates using [1,2-3H]androstenedione as the substrate. Only 5alpha-reductase 1 mRNA was expressed in human temporal lobe tissue; 5alpha-reductase 2 mRNA was not expressed. 5Alpha-reductase 1 mRNA concentrations did not differ significantly in the cerebral cortex of women [25.9+/-7.9 arbitrary units (aU); mean +/-SEM] and men (20.4+/-2.8 aU) or in the cerebral cortex (23.3+/-4.4 aU) and the subcortical white matter of adults (32.6+/-5.6 aU), but they were significantly higher in the cerebral cortex of adults than in that of children (6.4+/-2.3 aU; P < 0.005). The apparent Km of 5alpha-reduction did not show significant differences between the two sexes. In conclusion, 5alpha-reductase 1 mRNA is expressed in the temporal lobe of children and adults, but 5alpha-reductase 2 mRNA is not. 5Alpha-reductase 1 mRNA concentrations did not differ significantly in the sexes, but they were significantly higher in specimens of adults than in those of children.
Adult↗
Automobile exhaust as a means of suicide: an experimental study with a proposed model.
Experiments were conducted to investigate the concentration of carbon monoxide (CO) in a car cabin under suicide attempts with different vehicles and different start situations, and a mathematical model describing the concentration of CO in the cabin was constructed. Three cars were set up to donate the exhaust. The first vehicle didn't have any catalyst, the second one was equipped with a malfunctioning three-way catalyst, and the third car was equipped with a well-functioning three-way catalyst. The three different starting situations were cold, tepid and warm engine start, respectively. Measurements of the CO concentrations were made in both the cabin and in the exhaust pipe. Lethal concentrations were measured in the cabin using all three vehicles as the donor car, including the vehicle with the well-functioning catalyst. The model results in most cases gave a good prediction of the CO concentration in the cabin. Four case studies of cars used for suicides were described. In each case measurements of CO were made in both the cabin and the exhaust under different starting conditions, and the mathematical model was tested on these cases. In most cases the model predictions were good.
Adult↗
[An alternative dorsal fusion technique after transoral dens resection in basilar impression with atlas assimilation].
Current therapy of basilar impression includes transoral dens resection. The essential disadvantage of this procedure is the instability of C1/C2 due to loss of the transverse ligament. We describe two patients in whom this instability was treated by modified screwing. Using MAGERL-screws between C0-C2 an interposition of an iliac graft between the occiput and the arc of C2 was made. Neurological symptoms improved in both patients postoperatively. In both cases stable fusion C0/C2 was achieved with an additional treatment with a HALO-fixateur for eight weeks. In our opinion the combined transoral/dorsal procedure is most suitable and efficient in treatment of basilar impressions. By this approach both, decompression of the myelon as well as stable fusion C0/C2, is achieved.
Adolescent↗
Metabotropic glutamate receptors modulate synaptic transmission in the perforant path: pharmacology and localization of two distinct receptors.
Metabotropic glutamate receptors (mGluRs) have emerged as an interesting family of eight different receptor subtypes that can be divided into three groups according to their pharmacology and sequence similarity. In the present study, the specific mGluR agonists (1S,3R)-1-aminocyclopentane-1,3-dicarboxylic acid ((1S,3R)-ACPD) and L(+)-2-amino-4-phosphonobutyric acid (L-AP4) depressed field excitatory postsynaptic potentials (fEPSPs) in the rat dentate gyrus evoked by perforant path stimulation in a concentration-dependent, rapid and reversible manner (EC50: L-AP4 5.9 +/- 1.6 microM, (1S,3R)-ACPD 80 +/- 34 microM). In a 'paired-pulse' stimulation protocol, the first fEPSP showed a stronger reduction, resulting in 'paired-pulse' facilitation. The effects of L-AP4 but not of (1S,3R)-ACPD could be antagonized by the group III mGluR antagonists (S)-2-amino-2-methyl-4-phosphonobutanoic acid (MAP4) and (RS)-alpha-methyl-4-phosphonophenylglycine (MPPG). Moreover, (1S,3R)-ACPD was still potently depressing fEPSPs after preperfusion of near saturating concentrations of L-AP4. Together, the results suggest that both substances act on different mGluRs. The effects of (1S,3R)-ACPD could not be further differentiated by selective group I or group II mGluR agonists. Although (2S,1'S,2'S)-2-carboxycyclopropylglycine (L-CCG-I) blocked fEPSPs at concentrations >> 1 microM, these effects, as well as L-AP4 effects, were potently antagonized by MAP4. This suggests that mGluR8 might be responsible for the actions of L-AP4 and L-CCG-I. The two different mGluRs showed a distinct distribution when fEPSPs were recorded simultaneously in the outer and middle molecular layer (OML/MML): The L-AP4 sensitive receptor, possibly mGluR8, seems to be located in the OML while (1S,3R)-ACPD showed its main effect in the MML.
Aminobutyrates↗
Potassium currents in acutely isolated human hippocampal dentate granule cells.
1. Properties of voltage- and Ca(2+)-dependent K+ currents were investigated in thirty-four dentate granule cells acutely isolated from the resected hippocampus of eleven patients with therapy-refractory temporal lobe epilepsy (TLE). 2. When intracellular Ca2+ was strongly buffered with 11.5 mM EGTA-1 mM Ca2+ in the recording pipette, K+ currents (IK) with a slow activation and biexponential time-dependent decay could be elicited, which showed a threshold for activation around -30 mV. 3. A contribution of Ca(2+)-dependent K+ currents became apparent with intracellular solution containing 1 mM BAPTA-0.1 mM Ca2+. Superfusion of low-Ca2+ extracellular solution blocked 43% of outward currents in this recording configuration. Outward current components could also be blocked by substituting 5 mM Ba2+ for extracellular Ca2+ (78%), or by application of 100 microM Cd2+ (25%). 4. The Ca(2+)-dependent K+ currents could be pharmacologically subdivided into two components. One component was sensitive to 500 microM tetraethylammmonium (TEA; 41%) and 10 nM charybdotoxin (CTX; 47.2%). The blocking effects of 10 nM CTX and 500 microM TEA were not additive, suggesting that both agents block the same conductance. A second, smaller outward current component was blocked by 50 nM apamin (13%). 5. A transient A-type K+ current could be observed in six neurones and showed a fast monoexponential time-dependent inactivation with a steady-state voltage dependence that was distinct from that of IK. The A-type current was blocked by 4-aminopyridine (4-AP) but not by TEA or low-Ca2+ solution. 6. We conclude that outward currents in human hippocampal dentate granule cells can be separated into at least four types by their kinetic and pharmacological properties. These include at least one voltage-dependent current similar to those observed in mammalian hippocampal neurones, and two Ca(2+)-dependent K+ currents that most probably correspond to SK- and BK-type currents. A classical A-type current could be detected in some patients with Ammon's horn sclerosis (AHS) but not in patients with lesion-associated TLE.
Epilepsy, Temporal Lobe↗
How many residents shall we train--the situation in Germany.
The situation of training neurosurgeons in Germany is reviewed taking into consideration the current figures of finished residencies, the development in the number of training neurosurgical units in Germany, and the personnel and structure of a neurosurgery unit allowed to train. The jurisdiction for specialist training as well as typical problems encountered in training residents are being discussed. In 1992; 130,364 patients, equivalent to 0.94% of all patients in Germany were treated in 4.792 dedicated neurosurgical beds. A total of 644 working neurosurgeons were registered. Between 1986 and 1993, 349 residents qualified as neurosurgeons, an average of 43.5 per year. 57% of neurosurgical units employ non-trainee junior doctors, 13% have no residents, and 62% of departments trained less than one resident per year on average. Only 57% of units finish residency training always on time and only 79% of major units have full training permission. The figures provided in this article do not substantiate the assumption that we have trained too many specialists in the past. The problems which some residency programmes encounter however do suggest that it could be wise not to continue to train specialists at the same rate as in the past.
Cross-Cultural Comparison↗
What consequences should result from failure to meet internal standards?
This paper tries to approach a difficult problem, namely how to deal with a resident who has failed to meet the internal standards of a residency training program. First the problems of the definition of a standard and the associated problems of its reproducibility, documentation, teaching, update, and internal variability inside the same teaching program are dealt with. Consequently the question needs to be answered that constitutes a failure to meet the standard. The results of a survey of residents' attitudes are quoted as are some responses to a survey among the chiefs of teaching programs. Considering the attitudes of residents on how to handle breaches of standard the basic message was that residents want to be told that they do not function. Both parties want the collaboration of senior staff members on this topic. Whereas residents want to re-train, exercise and talk they do not want sanctions. Chiefs, however, want much less re-training, exercising and talking but earlier sanction. The difficult point of dealing with a true failure is discussed in the light of the German legal situation and the actual possibilities of how to handle the case. Before it comes to the point of discontinuing the training of a resident, it needs to be agreed upon what would be a classical situation of failure in which both the chiefs responsible for training and the residents agree that training is better discontinued. The author describes his experience with the real course of events in 7 cases he witnessed in 22 years.
Attitude of Health Personnel↗
Surgical management of brain-stem cavernomas.
We present a series of seven patients who were operated on for symptomatic brain-stem cavernomas. The following approaches were used: medial suboccipital (N = 4), lateral suboccipital (N = 1), subtemporal-transtentorial (N = 1), and frontal transcortical-transventricular-subchorioidal-trans velum interpositum (N = 1). Intraoperative motor (N = 4) and somatosensory (N = 1) evoked potential monitoring revealed temporary changes in 3 patients. Immediately postoperatively, the following additional deficits were observed in 6 patients: oculomotor nerve paresis (N = 2), abducens nerve paresis (N = 3), facial nerve paresis (N = 2), deafness (N = 1), and increased ataxia (N = 3). One patient died due to septic complications not related to surgery. After a mean observation time of 2 years, 2 patients had improved, 3 were unchanged, and 1 patient deteriorated as compared to his preoperative status. In conclusion, surgical treatment of brain-stem cavernomas, although carrying a significant risk of temporary neurological deterioration is recommended in symptomatic patients in whom the cavernoma seems to reach the surface of the brain-stem. Intraoperative functional topographic mapping and monitoring have proven useful tools lowering the surgical risks in these patients.
Adult↗
Diagnosis and treatment of frontobasal skull fractures.
During a five-year-period (January 1990 to December 1994) a total of 67 patients were operated on for frontobasal skull fractures. The indication for surgical treatment was based on the evidence of fractures encroaching paranasal sinuses or the cribriform plate on high-resolution axial or coronal CT scans. The following clinical signs indicating frontobasal trauma were observed: 25 patients (37%) showed rhinoliquorrhea, 14 (21%) had raccoon's eyes, and 2 (3%) had meningitis. Distinct dura laceration was observed intraoperatively in 64 of 67 patients (96%). In our experience, high resolution CT has proven to be a sensitive diagnostic tool for frontobasal skull fractures. With respect to the high coincidence of fractures and dura lacerations, the indication for surgical treatment based on CT findings seems to be justified.
Adolescent↗
Pituitary apoplexy with spontaneous cure of acromegaly and its possible relation to Gd-DTPA-administration.
A case of pituitary apoplexy occurring after Gd-DTPA-administration for contrast enhanced MRI in a patient with an hGH-producing macro-adenoma is presented. Within days the initially increased hGH level fell to the normal range, the oral glucose tolerance test (OGTT) showed a normal suppression of hGH and complete anterior pituitary insufficiency developed. At this time repeated MRI suggested a haemorrhagic infarction of the macro-adenoma. Fourteen months later re-examination confirmed spontaneous cure of the acromegaly, improvement of adenopituitary function and shrinkage of the sellar content. The causal linkage between the pituitary adenoma apoplexy and Gd-DTPA-administration is unclear. It might be due to contrast induced blood pressure and endothelial permeability changes, possibly promoted by pre-existing diabetes mellitus associated vasculopathy.
Acromegaly↗
Molecular genetic evidence for subtypes of oligoastrocytomas.
The histogenesis of oligoastrocytomas remains controversial, with some data arguing similarity of oligoastrocytomas to astrocytic tumors, and other data suggesting closer relationships with oligodendroglial neoplasms. Since the molecular genetic changes in astrocytomas differ from those of oligodendrogliomas, we characterized 120 astrocytic and oligodendroglial tumors, including 38 oligoastrocytomas, for genetic alterations that occur disproportionately between astrocytomas and oligodendrogliomas, i.e. TP53 gene mutations and allelic loss of chromosomes 1p, 17p and 19q. As previously reported, TP53 mutations were common in astrocytic gliomas, occurring in approximately half of WHO grade II and III astrocytomas, but in only 5% of WHO grades II and III oligodendrogliomas. Allelic losses of chromosomes 1p and 19q, however, were common in oligodendrogliomas (41% and 63%), but less frequent in astrocytomas (9% and 35%). Oligoastrocytomas showed TP53 mutations in 12/38 (32%) cases and allelic losses of chromosomes 1p and 19q in 52% and 70%, respectively. Most importantly, TP53 mutations and allelic losses on chromosomes 1p and 19q were inversely correlated in oligoastrocytomas (p < 0.011 and p < 0.019). These data suggest the existence of two genetic subsets of oligoastrocytomas, one genetically related to astrocytomas and the other genetically related to oligodendrogliomas. Histologically, those oligoastrocytomas with TP53 mutations were more often astrocytoma-predominant, while those with chromosome 19q loss were more often oligodendroglioma-predominant.
Adult↗
Microsurgical results for small arteriovenous malformations accessible for radiosurgical or embolization treatment.
OBJECTIVE: A consecutive series of microsurgically treated small arteriovenous malformations (AVMs), up to 3 cm in diameter, which are theoretically ideal candidates for radiosurgery or embolization, is described. We intended to elucidate the safety and efficacy of the microsurgical removal of AVMs, as compared with the results for radiosurgery and embolization reported in the literature. METHODS: Sixty-two patients (32 female and 30 male patients) ranging in age from 7 to 72 years (mean age, 33.3 yr) were included in the series. Of these, 46.8% presented with intracerebral hemorrhage and 24.2% presented with seizures. The 62 patients underwent microsurgical removal of their small (<3 cm) cerebral AVMs by the same surgeon. Twenty-six AVMs (41.9%) were assigned Spetzler-Martin Grade I, 24 (38.7%) were assigned Grade II, and 12 (19.4%) were assigned Grade III. Thirty-three AVMs (53.2%) were located in eloquent brain regions. All patients underwent postoperative control angiography and clinical follow-up after 3 and 6 months. RESULTS: The AVMs were extirpated, as confirmed by angiography, in all except one patient, thereby accounting for a 98.4% success rate per angiographic findings. The rate of immediate new postoperative neurological deficits or worsening of preexisting neurological deficits was 27.4%, and the rate of permanent significant neurological deficits was 3.2% at late follow-up. The rate of permanent significant deficits occurring after the microsurgical removal of small AVMs in eloquent regions was 6.1%. The mortality rate in the reported series was 0%. The surgical morbidity rate was 9.7%. CONCLUSION: Microsurgery for small AVMs is superior to radiosurgery or interventional neuroradiology because of its high rate of efficacy and low rate of permanent morbidity and because immediate cure of the AVMs can be achieved in the vast majority of patients. The place for radiosurgery in the treatment of small AVMs needs to be more sharply defined after careful assessment of relevant cases by an experienced vascular neurosurgeon.
Adolescent↗
Surgical and neurological complications in a series of 708 epilepsy surgery procedures.
OBJECTIVE: There are few modern data on the complications of surgery for epilepsy from the neurosurgeon's point of view. A survey of complications observed in a large current epilepsy surgery series is presented to facilitate the assessment of a risk:benefit ratio, which must be known when planning for epilepsy surgery and counseling patients. METHODS: A series of 429 consecutive patients operated on during 6.5 years in the newly established University of Bonn epilepsy surgery program was, in part, retrospectively, and, in larger part, prospectively analyzed for complications originating from 279 invasive diagnostic procedures and 429 therapeutic procedures. Neuropsychological and psychiatric complications as well as the rate of failure to control seizures are not addressed in this article. RESULTS: Two hundred and seventy-nine temporal operations, 59 frontal operations, 22 other extratemporal operations, 33 callosotomies, 3 multilobectomies, and 33 hemispherectomies were performed. Complications were grouped into general surgical and neurological complications. No mortality resulted from 708 invasive procedures. Two hundred and seventy-nine invasive diagnostic procedures (various combinations of strip, grid, and depth electrode insertions) resulted in 3.6% transient morbidity (2.9% surgical complications, 0.7% neurological complications) and 0.7% permanent morbidity (dysphasia). During 429 therapeutic procedures, 33 surgical complications were encountered. None of these resulted in permanent morbidity, except for the necessity for permanent shunt insertion in three patients. Wound infection was the most frequent surgical complication, but we were able to demonstrate a steady decrease during the 6.5-year observation period. The total rate of neurological complications in 429 therapeutic procedures was 5.4%, with 3.03% causing transient morbidity and 2.33% causing permanent morbidity. CONCLUSION: Our data indicate that epilepsy surgery can be performed with an acceptable rate of resultant morbidity. The indications for epilepsy surgery, the learning curve determined, and the results from other series are discussed in the light of these figures.
Adolescent↗
Surgical treatment of neoplasms associated with medically intractable epilepsy.
OBJECTIVE: Surgical treatment in patients with brain tumors and medically intractable epilepsy is aimed at the removal of the neoplasm and complete seizure control. However, an adequate surgical approach is still controversial. This study was designed to analyze the factors for the optimum surgical treatment of these patients. METHODS: The clinical, electrophysiological, operative, and histopathological data of 146 consecutive patients who underwent surgery between November 1987 and May 1995 for intrinsic brain tumors and pharmacoresistant epilepsy were evaluated. RESULTS: The majority of the tumors were located in the temporal lobe (n = 116) and involved the cortical gray matter. The most frequent tumors were gangliogliomas (n = 65), pilocytic astrocytomas (n = 21), and dysembryoplastic neuroepithelial tumors (n = 19). All but three tumors (98%) were of low histopathological grade (World Health Organization Grades I or II). The biological behavior of the tumors was strikingly indolent, as indicated by a long preoperative history of chronic seizures (mean, 14 yr). In all cases, complete resection of the tumor, including the epileptogenic area (as determined by noninvasive and/or invasive recordings of the zone of seizure onset and persistent interictal activity), was intended. Complications were encountered in 11 cases (8%). However, no patient died and there was no permanent morbidity. Of the 124 patients who had postoperative follow-up examinations more than 6 months after resection, 71% were seizure-free, 11% had no more than two seizures per year, 13% showed a reduction of seizure frequency of at least 75%, and 5% had no appreciable reduction in seizure frequency. CONCLUSION: The data indicate that neoplasms associated with medically intractable epilepsy constitute a distinct clinicopathological group of tumors that arise in young hosts, involve the cortex, and exhibit indolent biological behavior for many years. Complete surgical removal of these tumors, including the epileptogenic area, can achieve excellent seizure control.
Adolescent↗
Multiple subpial transection for control of epileptic seizures: effectiveness and safety.
PURPOSE: To assess the efficacy and safety of multiple subpial transection (MST), a new technique in epilepsy surgery, alone and in combination with resection. METHODS: MST was performed in 22 patients with intractable epilepsy, 10 of whom were treated with a combination of a resection and MST in functionally important cortex, 6 of whom were treated with a combination of a resection and MST performed outside functionally important cortex, and 6 of whom were treated with MST alone. RESULTS: Of the 6 patients who received MST alone, none became seizure free and 4 showed > 50% reduction of all seizure types. In 2 patients, including 1 with Rasmussen's encephalitis, no change in seizure frequency or intensity occurred. Of the 16 patients in whom MST was combined with a resection, 9 (56%) became seizure free. Six of the remaining 7 patients showed > 95% reduction of all seizure types. Disappearance of epileptiform potentials in the postoperative EEG correlated significantly with complete relief from seizures. Subtle, permanent neurological deficits remained in 5 of 14 patients who received MST in functionally important brain areas. CONCLUSIONS: Reduction of the seizure frequency was substantial in 4 of 6 patients who received MST alone, but complete seizure control was not observed. MST surrounding a lesionectomy may be a new surgical approach which would minimize the excised volume and improve seizure control.
Adolescent↗
Safety of intrahippocampal depth electrodes for presurgical evaluation of patients with intractable epilepsy.
PURPOSE: Intracerebral depth electrodes are used in preoperative evaluation of selected patients with intractable epilepsies. In spite of their usefulness, safety of depth electrodes is disputed, and the number of insertions is decreasing. This study examined retrospectively possible deleterious effects such as perioperative complications, induction of epileptogenesis, and neuropsychologic deficits. METHODS: Clinical course and neuroradiologic findings of 115 patients with bilaterally inserted longitudinal intrahippocampal depth electrodes (IDEs) were analyzed. Hippocampal resection specimens were examined histopathologically. To detect newly developed epileptogenic areas, EEG recordings, seizure control, and semiology after standardized resection procedures were compared between patients who received IDEs and those who did not. To demonstrate functional deficits caused by IDE insertion into an unaffected hippocampus of the speech-dominant hemisphere, changes of verbal learning and memory performances before and after right amygdalohippocampectomy were compared between patients evaluated with and without IDEs. RESULTS: Five significant complications without any permanent neurologic deficit were noted, and only one was specifically linked to IDE insertion. The tissue damage associated with the insertion was minimal and sharply circumscribed. No differences of seizure outcome after standardized resections were identified between patients with and without IDEs. In postoperative EEG recordings, there was no evidence of new epileptogenic areas. No verbal memory deficit caused by IDE implantation into the hippocampus of the speech-dominant hemisphere was detectable. CONCLUSIONS: Results indicate that it is safe to implant these IDEs in selected patients.
Adult↗