Eva Bültmann

Neurologic manifestations of E coli infection–induced hemolytic-uremic syndrome in adults

Objective: To describe the neurologic and neuroradiologic complications of Shiga toxin producing Escherichia coli infection (STEC)–associated hemolytic-uremic syndrome (HUS) in adults. Methods: All 52 adult patients with STEC O104:H4 infection cared for at Hannover Medical School during the outbreak in Germany through May–July 2011 are considered in this observational study. Forty-three of the 52 patients underwent a standard neurologic diagnostic procedure including clinical examination, Mini-Mental State Examination, and Glasgow Coma Scale Score. Thirty-six patients underwent EEG, and 26 had cerebral MRI, 9 of them repeatedly. Case records of 9 patients who had not been seen by a neurologist were analyzed retrospectively. Results: Forty-eight of the 52 patients had HUS. All but 1 of these showed neurologic symptoms. Focal neurologic signs like double vision, difficulties in finding words, or hyperreflexia were present in 23, additional deficits in orientation, attention, memory, or constructive abilities in 9, and marked impairment of consciousness in 15. MRI showed brainstem, midbrain, thalamus, corpus callosum, and white matter lesions in half of the patients, predominantly in diffusion-weighted images. The extent of MRI lesions did not correlate with clinical symptoms. General slowing but no focal alteration was found in half of the patients examined by EEG. Conclusion: Our findings suggest a toxic-metabolic pathology behind the neurologic impairment instead of multiple infarction due to microthrombosis. Future studies should aim to clarify if early antibiotic therapy or bowel cleansing might help to decrease the rate of neurologic complications in STEC-HUS.

Craniometric changes in patients with acromegaly from a surgical perspective

OBJECT The objective of this study was to evaluate and analyze morphometric and volumetric changes of the skull due to acromegaly in areas relevant for neurosurgical practice, focusing on the surgical implications. METHODS On preoperatively acquired CT scans, cephalometric and volumetric measurements were performed on 45 patients with acromegaly (Group A) and 45 control patients (Group B). The authors determined thickness of the cranial vault, inner and outer diameters of the skull, and the diameter of sphenoidal and maxillary sinus, as well as frontal and maxillary sinus volumetry. The morphometric and volumetric CT data of the patients with acromegaly were compared with the data of a control group and correlated with clinical parameters. RESULTS Cranial vault thickness differed significantly (p < 0.0001) between the 2 groups. A correlation of the vault thickness with preoperative human growth hormone, insulin-like growth factor-I levels, and duration of clinical history in acromegaly could not be established. The outer anterior-posterior skull diameter of Group A (18.47 ± 0.94 cm) differed significantly (p = 0.0146) from Group B (17.98 ± 0.93 cm) and correlated significantly with preoperative human growth hormone (r = 0.3277; p = 0.0299) and insulin-like growth factor-–I serum levels (r = 0.3756; p = 0.0120). Measurements of the anterior-posterior diameter of the sphenoidal sinus differed significantly (p = 0.0074) between patients with acromegaly and controls. Volumetric analysis of the frontal sinus resulted in a statistically significant difference (p = 0.0382) between patients with acromegaly (14.89 ± 10.85 cm3) and controls (10.06 ± 6.93 cm3). CONCLUSIONS Significant craniometric changes and volumetric remodelling of the paranasal sinus occur in acromegaly. The bone alterations are of surgical importance for using the transsphenoidal approach. Detailed preoperative diagnostic examination and planning as well as selection of appropriate instruments are mandatory for safe and successful pituitary adenoma removal in patients with acromegaly.

Improved imaging of Cochlear nerve hypoplasia using a 3-tesla variable flip-angle turbo spin-echo sequence and a 7-cm surface coil

Magnetic resonance imaging of the temporal bone has an important role in decision making with regard to cochlea implantation, especially in children with cochlear nerve deficiency. The purpose of this study was to evaluate the usefulness of the combination of an advanced high‐resolution T2‐weighted sequence with a surface coil in a 3‐Tesla magnetic resonance imaging scanner in cases of suspected cochlear nerve aplasia.

Quantitative MRI shows cerebral microstructural damage in hemolytic–uremic syndrome patients with severe neurological symptoms but no changes in conventional MRI

IntroductionSevere neurological symptoms in Shiga toxin-producing Escherichia coli infection associated hemolytic–uremic syndrome (STEC–HUS) are often accompanied by none or only mild alterations of cerebral magnetic resonance imaging (MRI). This study aims to analyze if quantitative MRI is able to reveal cerebral pathological alterations invisible for conventional MRI.MethodsIn nine patients with STEC–HUS associated severe neurological symptoms but inconspicuous cerebral MRI findings maps of the parameters T2 relaxation time, relative proton density (PD), apparent diffusion coefficient (ADC), and fractional anisotropy (FA) were generated. Quantitative values of these parameters were measured at the basal ganglia, thalamus, and white matter of the frontal and parietal lobe and compared to those of nine age- and sex-matched controls.ResultsSignificant T2 prolongation (p < 0.01) was found in the basal ganglia of all patients compared to controls. PD and ADC were not significantly altered. A significant reduction of FA in patients was seen at caput nuclei caudati (p < 0.01).ConclusionProlonged T2 relaxation time indicates cerebral microstructural damages in these patients despite their inconspicuous MRI findings. T2 relaxometry could be used as a complementary tool for the assessment of metabolic–toxic brain syndromes.

Neurological Manifestations of Mycoplasma pneumoniae Infection in Hospitalized Children and Their Long-Term Follow-Up.

Objective In this retrospective study, we aimed to assess frequency, types, and long-term outcome of neurological disease during acute Mycoplasma pneumoniae (M. pneumoniae) infection in pediatric patients. Materials and Methods Medical records of patients hospitalized with acute M. pneumoniae infection were reviewed. Possible risk factors were analyzed by uni- and multivariate regression. Patients with neurological symptoms were followed up by expanded disability status score (EDSS) and the cognitive problems in children and adolescents (KOPKJ) scale. Results Out of 89 patients, 22 suffered from neurological symptoms and signs. Neurological disorders were diagnosed in 11 patients: (meningo-) encephalitis (n = 6), aseptic meningitis (n = 3), transverse myelitis (n = 1), and vestibular neuritis (n = 1), 11 patients had nonspecific neurological symptoms and signs. Multivariate logistic regression identified lower respiratory tract symptoms as a negative predictor (odds ratio [OR] = 0.1, p < 0.001), a preexisting immune deficit was associated with a trend for a decreased risk (OR = 0.12, p = 0.058). Long-term follow-up after a median of 5.1 years (range, 0.6-13 years) showed ongoing neurological deficits in the EDSS in 8/18, and in the KOPKJ in 7/17. Conclusion Neurological symptoms occurred in 25% of hospitalized pediatric patients with M. pneumoniae infection. Outcome was often favorable, but significant sequels were reported by 45%.

Measuring in vivo cerebral maturation using age-related T2 relaxation times at 3 T

OBJECTIVE To examine age-related changes in T2 relaxation times during infancy and childhood in order to assess T2 values obtained from routine MRI as a biomarker. METHODS From our pool of clinical pediatric MRI examinations at 3T all patients with normal conventional MRI scans were retrospectively selected. Depending on their clinical findings the identified 99 patients (0-199months) were divided into 43 healthy controls and 56 diseased children with various clinical abnormalities (developmental delay, epilepsy, prematurity, and deafness). T2 maps based on routinely performed triple echo turbo spin echo sequences were created. T2 values were measured in 22 brain regions to determine age-related changes. We also investigated whether such changes differ between healthy and diseased children. RESULTS Age significantly reduced T2 relaxation times across all regions (p<0.05), but health status had no impact. With increasing age, T2 values decreased continuously, with declines faster over the first 10months and slower thereafter. Early rapid and later slow decline was similar in healthy and diseased groups. CONCLUSIONS Using T2 maps based on clinical MRI data we could determine age-related T2 relaxation times in 22 brain regions during infancy and childhood. Our data have relevance for future investigator independent T2 relaxation time measurements in determining whether T2 values are within the normal range or should be considered as potentially pathologic.

Stenting the Eustachian tube to treat chronic otitis media - a feasibility study in sheep

BackgroundUntreated chronic otitis media severely impairs quality of life in affected individuals. Local destruction of the middle ear and subsequent loss of hearing are common sequelae, and currently available treatments provide limited relief. Therefore, the objectives of this study were to evaluate the feasibility of the insertion of a coronary stent from the nasopharynx into the Eustachian tube in-vivo in sheep and to make an initial assessment of its positional stability, tolerance by the animal, and possible tissue reactions.MethodsBilateral implantation of bare metal cobalt-chrome coronary stents of two sizes was performed endoscopically in three healthy blackface sheep using a nasopharyngeal approach. The postoperative observation period was three months.ResultsStent implantation into the Eustachian tube was feasible with no intra- or post-operative complications. Health status of the sheep was unaffected. All stents preserved their cylindrical shape. All shorter stents remained in position and ventilated the middle ear even when partially filled with secretion or tissue. One of the long stents became dislocated toward the nasopharynx. Both of the others remained fixed at the isthmus but appeared to be blocked by tissue or secretion. Tissue overgrowth on top of the struts of all stents resulted in closure of the tissue-lumen interface.ConclusionStenting of the Eustachian tube was successfully transferred from cadaver studies to an in-vivo application without complications. The stent was well tolerated, the middle ears were ventilated, and clearance of the auditory tube appeared possible. For fixation, it seems to be sufficient to place it only in the cartilaginous part of the Eustachian tube.

Quantitative microstructural cerebral changes in neurofibromatosis type 1

OBJECTIVES To evaluate microstructural cerebral changes in children with neurofibromatosis type 1 (NF1) based on T2 relaxation time measurements at 3Tesla. METHODS From our dataset of pediatric MRI examinations at 3T 19 pediatric NF1 patients (1.9-14.3 years of age, 9 girls, 10 boys) were retrospectively selected and compared with the previously published group of 44 healthy children (0-16 years of age). MRI examination included a triple echo TSE sequence as basis for T2 maps. T2 relaxation times were measured in 37 brain regions. RESULTS Compared with healthy controls, T2 relaxation times had the tendency to be increased by 1.01% (GM) to 11.85% (dentate nucleus) for NF1 patients. Only in posterior limb of the internal capsule and parietooccipital white matter values were reduced. No differences were observed between both hemispheres. Overall, no strong evidence supporting a difference between NF1 patients with and without optic glioma or with normal and impaired neuropsychological development was observed. CONCLUSIONS Using T2 relaxation times it was possible to describe measurable microstructural differences in multiple brain regions between NF1 patients and healthy children regardless of whether signal abnormalities were visible on conventional images.

Diagnostische Wertigkeit der absolut quantifizierten, lokalisierten MR-Protonenspektroskopie beim dysplastischen zerebellären Gangliozytom (Lhermitte-Duclos)

ZusammenfassungHintergrund:Die Lhermitte-Duclos’sche Erkrankung (LDE) manifestiert sich als seltene, benigne Raumforderung des Kleinhirns im Erwachsenenalter. Histologisch wird sie teils als Dysplasie, teils auch als WHO-Grad-I-Neoplasie eingestuft. Aufgrund des zumeist langsamen, aber dennoch vorhandenen Wachstums erfolgt insbesondere bei entsprechender klinischer Symptomatik in der Regel eine möglichst vollständige Resektion, da auch Rezidive beschrieben sind. Eine Sonderform stellt das Cowden-Syndrom dar, welches autosomal-dominant vererbt wird und als neurokutanes Syndrom neben der LDE mit Hamartomen der Haut, mit Schilddrüsen-, Kolon-, Mamma- und Ovarialzysten und -tumoren wie auch mit Neurofibromen und Meningeomen vergesellschaftet ist.Fallbericht:Vorgestellt wird eine 33-jährige Frau mit Cowden-Syndrom, bei der die seltene Vergesellschaftung einer LDE mit einer Syringomyelie vorliegt.Diagnostische Verfahren:Anhand dieses Beispiels werden spezifische Zeichen der Standard-MR-Bildgebung, die auf die LDE hinweisen, diskutiert. Des weiteren wird die besondere Wertigkeit der absolut quantifizierten, lokalisierten MR-Protonenspektroskopie (MRS) vorgestellt, weil hierbei ein sehr spezifisches spektroskopisches Muster mit Erniedrigung der Hauptmetaboliten N-Acetyl-aspartat(-glutamat) (NAA/NAA(G)), Kreatin/Phosphokreatin (Cr(PCr)), myo-Inositol (mI) und Cholin (Cho), wie es sonst bislang nur in Radionekrosen beschrieben ist, gefunden werden konnte.AbstractBackground:Lhermitte-Duclos disease (gangliocytoma of the cerebellum) is a rare, benign, space-occupying lesion of the adult cerebellum. Histologically, there is debate as to whether this abnormality is a dysplasia or a WHO grade I tumor. Due to the slowly progressive and potentially recurrent character of these lesions in most of the patients complete resection is favorable. Cowden’s syndrome is a rare autosomal dominant variant of Lhermitte-Duclos disease that usually presents in combination with multiple mucocutaneous lesions associated with high frequencies of breast, thyroid and gastrointestinal malignancies. Other cerebral abnormalities, especially meningeomas and neurofibromas, have also been described in these patients.Case Report:A 33-year-old woman presenting with this rare disease in combination with a syringomyelia is reported.Diagnostic Procedures:The typical findings in MRI and MR spectroscopy (MRS) are discussed. Especially, the specific results of the absolutely quantified, localized MRS are pointed out, which demonstrated a reduction of the dominant resonances N-acetyl-aspartate, creatine, myo-inositol and choline, a pattern which up to now has only been found in radionecrosis.