Focal polymicrogyria: planar-surface MRI.
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Adult↗
Biomedical subjects
J von Oertzen
Publications and source records attributed to J von Oertzen.
Lesional mesial temporal lobe epilepsy and limited resections: prognostic factors and outcome.
OBJECTIVES: To evaluate the influence of clinical, investigational, surgical, and histopathological factors on postoperative seizure relief in patients with mesial temporal lobe epilepsy (MTLE) due to lesions other than ammonshornsclerosis (AHS). METHODS: Of 738 patients operated for TLE, 78 patients underwent limited resections for lesional MTLE (1990-2000). Seventy four patients with a follow up of more than one year were included. The preoperative clinical, neuropsychological, electroencephalogram, and neuroimaging characteristics were prospectively collected in a database. The histopathological material was re-examined. RESULTS: The mean follow up was 49 months. Fifty eight patients were classified as seizure free (78.4% Class I), and six as almost seizure free (8.1% Class II), grouped together as satisfactory seizure control (64 patients, 86.5%). Five patients (6.8%) were categorised in Classes III and IV, respectively. These were grouped as unsatisfactory seizure control (10 patients, 13.5%). Surgical procedures were: 32 amygdalohippocampectomies (AH), 17 partial anterior AH, 15 AH plus polar resection, seven AH plus basal resection, and three AH plus extended temporal lesionectomy. There was no mortality and 2.7% mild permanent morbidity. Seizure relief did neither differ significantly with these approaches, nor with different classes of pathological findings (43 developmental tumours, 12 glial tumours, 10 dysplasias, and nine others). Even operation of dysplasias resulted in 80% satisfactory seizure control. Seizure onset during childhood proved to be a negative predictor for seizure relief (p = 0.020). MRI revealed 73 suspected lesions (98.6%), one dysembryoplastic neuroepithelial tumour was missed, in four cases no structural abnormalities could be confirmed with histopathological exam. Additionally, multifactorial regression revealed the factors "seizure onset after 10 years of age", "presence of complex partial seizures", "absence of a neurological deficit", and a "correlating neuropsychological deficit" as predictive for satisfactory seizure control. CONCLUSIONS: "Preoperative tailoring" resulting in limit resections has proven to be safe and to provide a very good chance for satisfactory seizure relief in patients with lesional MTLE.
Adolescent↗
Time-efficient T2 relaxometry of the entire hippocampus is feasible in temporal lobe epilepsy.
OBJECTIVE: To test the clinical usefulness and reliability of a new dual-echo turbo-spin-echo (TSE) sequence for rapid and regional hippocampal T2 relaxometry. METHODS: Hippocampal T2 relaxation time (HRT) was determined by a TSE sequence on three to four consecutive coronal images in 16 control subjects and 12 patients with mesial temporal lobe epilepsy. HRT was related to neuropathology findings in hippocampal specimens including neuronal cell density (ND), results of visual analysis of MR images, clinical outcome after epilepsy surgery, and hippocampal volumetry. RESULTS: Rapid HRT differentiated patients from control subjects; all cases of hippocampal sclerosis (HS; n = 10) were correctly diagnosed. HRT showed a strong correlation with ND in CA1 (p < 0.02) and CA3 (p < 0.05). Diagnoses based on rapid relaxometry concurred fully with results of visual inspection. Mean HRT was prolonged ipsilaterally in all patients with excellent postoperative seizure outcome and bilaterally prolonged or normal in patients with poorer outcome. Rapid HRT was concordant with hippocampal volumetry in 10 of 12 patients. Regional HRT of control subjects revealed significantly higher values in the anterior than posterior hippocampus. In patients with unilateral HS, this gradient was absent. The gradient was also absent contralaterally to HS, although surgical outcome was excellent. CONCLUSIONS: Hippocampal dual-echo TSE-relaxometry can be regarded a reliable technique to detect and quantify HS. With a scan time of 3.31 minutes and immediate off-line analysis lasting a few minutes only, TSE-T2 relaxometry is easy to integrate in the routine diagnostic assessment of hippocampal morphology in large numbers of patients.
Adolescent↗
Language mapping in less than 15 minutes: real-time functional MRI during routine clinical investigation.
Neurosurgical interventions often require the presurgical determination of language dominance or mapping of language areas. Results obtained by fMRI are closely correlated with invasive procedures such as electrical stimulation mapping or the intracarotid amobarbital test. However, language fMRI is not used routinely, because postprocessing is time-consuming. We utilized a real-time analysis software installed directly on the MR console computer and SPM99 as reference postprocessing software. We assessed the reliability of the immediate determination of language dominance based on individual activation maps by comparing the results of the visual analysis of images derived from conventional postprocessing with those produced by the real-time tool. All images were rated independently by six senior neurologists blinded to other data. We validated the robustness of the real-time method statistically by comparing global and regional lateralization indices derived from real-time and postprocessing analysis. Functional MRI was performed with a standard 1.5-T whole-body scanner. Brain activity was contrasted between an alternating semantic judgment and letter matching task. Twelve right-handed, healthy control subjects and 12 consecutive patients with drug-resistant, localization-related epilepsy were investigated. The semantic condition induced almost invariably left hemispheric activations in Broca's area, the premotor cortex, the dorsolateral prefrontal cortex, and the temporoparietal region. Although real-time analysis reduced noise less effectively than SPM99, visual ratings and lateralization indices produced highly concordant results with both methods. In conclusion, real-time fMRI, as used here, allowed reliable language lateralization and mapping in less than 15 min during routine clinical MRI investigation with no need for postprocessing.
Adult↗
Phenotype assignment in symptomatic female carriers of X-linked adrenoleukodystrophy.
However, only very few studies have systematically investigated the extent and distribution of nervous and endocrine system involvement in female carriers of X-ALD. To define the phenotype in symptomatic female carriers of X-ALD we performed a prospective study including eight symptomatic women who were followed for a mean period of 3.4+/-1.8 years (range 1-6) using standardized clinical examination protocols, magnetic resonance imaging and spectroscopy, evoked potential studies including visual, brainstem auditory, somatosensory and magnetic evoked potentials, neurographic recordings and endocrine studies. Spastic paraparesis and decreased vibration sense in the lower extremities were the most frequent clinical findings. Slightly hyperintense symmetric parieto-occipital white matter lesions on magnetic resonance imaging were detectable in two of seven cases, and the N-acetylaspartate/choline ratios on magnetic resonance spectroscopy were decreased in three of seven patients. P40 latencies were abnormal in all patients, and central motor conduction times to the lower extremities in seven of eight patients. Prolonged latencies of brainstem auditory evoked potential waves III-V or interpeak latencies of waves I-III, I-V and III-V were detectable in all patients. The degree of walking impairment was positively correlated with the duration of clinical disease (r=0.58, P < 0.05) and inversely correlated with the N-acetylaspartate/choline ratios (r=0.85; P < 0.05). Neurographic recordings revealed only subtle abnormalities, suggesting that nervous system involvement in symptomatic female carriers of X-ALD is confined mainly to the central nervous system. No evidence of adrenal insufficiency was detected in any of the patients.
Adrenal Insufficiency↗
SATSCOM--Selective amobarbital test intraarterial SPECT coregistered to MRI: description of a method assessing selective perfusion.
For evaluation of potential functional deficits, an intraarterial amobarbital test is performed prior to neurosurgical or neuroradiological interventions. To visualize individual amobarbital perfusion patterns, simultaneous injection of (99m)Tc-HMPAO was performed previously. The present study describes for the first time a method of coregistration of intraarterial SPECT during selective amobarbital test to MRI. Three patients undergoing selective amobarbital test of the posterior cerebral artery were included. SATSCOM (Selective amobarbital test intraarterial SPECT coregistered to MRI) was performed by skull extraction in SPECT and MRI followed by surface matching. In all three patients, SATSCOM revealed accurate matching results. With this functional-anatomical mapping, suppression of higher cortical functions can be correlated to anatomical regions. Furthermore, a more precise mapping of amobarbital effect improves planning invasive interventions, particularly those close to eloquent areas.
Adult↗
Increased laminin A expression in regenerating myofibers in neuromuscular disorders.
Laminin is a basement membrane (BM) glycoprotein composed of three of five subunits, the A, M, B1, B2, and the S chain. Four forms of laminin, A-B1-B2, A-S-B2, M-B1-B2, and M-S-B2, have been identified. Laminin is implicated in various biological processes such as cell adhesion and differentiation. We studied immunohistochemically the expression of the four laminin subunits A, M, B1, B2 as well as of neural cell adhesion molecule (N-CAM, CD56), a marker of regenerating myofibers, in various neuromuscular disorders. In normal muscle, the predominant subunits of myofiber laminin were M, B1, and B2. The A chain was only faintly expressed in myofiber BM. In inflammatory myopathies and dystrophinopathies myofiber laminin A expression was greatly increased. An average of 80% and 63% of laminin A-positive myofibers in inflammatory myopathies and dystrophinopathies, respectively, were additionally CD56 positive. Laminin A and CD56 expression in denervating diseases and mitochondrial myopathies were negligible. Expression of M, B1, and B2 subunits did not seem to be altered in the diseased conditions examined above. The data suggest that laminin A is upregulated in inflammatory myopathies and dystrophinopathies and, most markedly in regenerating myofibers.
Antigens↗