Bernhard Schuknecht - Academia.edu (original) (raw)
Papers by Bernhard Schuknecht
Clinical Imaging, Nov 1, 2008
ABSTRACT This retrospective study aimed to describe the differences between image readings done w... more ABSTRACT This retrospective study aimed to describe the differences between image readings done with combined positron emission tomography/computed tomography (PET/CT) and PET read together with contrast-enhanced CT (ceCT) in patients with squamous cell carcinoma of the head and neck. In 46 patients, no differences were found between the two readings for assessing infiltration of adjacent structures (P=.63), transgression of the midline (P=.67), lymph node involvement (P=.32), and T- and N stage. PET/CT and PET read together with ceCT have comparable diagnostic yield.
Journal of Neurology
Of the 96 serologically confirmed neuroborreliosis cases seen in our clinic between 1983 and 1988... more Of the 96 serologically confirmed neuroborreliosis cases seen in our clinic between 1983 and 1988, 11 patients had mild to moderate and 4 patients had serious cerebral and/or spinal cord symptoms. Nine of these 15 patients with CNS involvement exhibited a primary chronic course of the illness. After high-dose intravenous therapy with penicillin, doxycycline or cefotaxime, given mostly in combination with cortisone, gradual recovery occurred with normalization of CSF findings characteristic of neuroborreliosis, and normalization of significantly elevated Borrelia burgdorferi IgG antibody titres in CSF and serum. Brain MRI and CT showed evidence of or were suggestive of vascular involvement which correlated with clinical symptoms in 11 of the 15 patients with CNS involvement. Brain MRI changes that were similar but much slighter in number and intensity were seen in 5 of 12 neuroborreliosis patients without clinical signs of CNS involvement (lymphocytic meningoradiculitis; Bannwarth's syndrome). The frequencies of the HLA-DR7 (75%), HLA-B44 (50%) and HLA-A29 (33%) antigens in 12 neuroborreliosis patients with clinical symptoms of CNS involvement were significantly different from the frequencies in 12 neuroborreliosis patients without CNS involvement and in 100 control subjects. Diagnostic criteria of active neuroborreliosis are proposed.
Frontiers in Neurology, 2020
Background: Lateral medullary stroke (LMS) results in a characteristic pattern of brainstem signs... more Background: Lateral medullary stroke (LMS) results in a characteristic pattern of brainstem signs including ocular motor and vestibular deficits. Thus, an impaired angular vestibulo-ocular reflex (aVOR) may be found if the vestibular nuclei are affected. Objective: We aimed to characterize the frequency and pattern of vestibular and ocular-motor deficits in patients with LMS. Methods: Patients with MR-confirmed acute/subacute unilateral LMS from a stroke registry were included and a bedside neuro-otological examination was performed. Video-oculography and video-based head-impulse testing (vHIT) was obtained and semicircular canal function was determined. The lesion location/extension as seen on MRI was rated and involvement of the vestibular nuclei was judged. Results: Seventeen patients with LMS (age = 59.4 ± 14.3 years) were included. All patients had positive H.I.N.T.S. vHIT showed mild-to-moderate aVOR impairments in three patients (ipsilesional = 1; ipsilesional and contralesional = 1; contralesional = 1). Spontaneous nystagmus (n = 10/15 patients) was more often beating contralesionally than ipsilesionally (6 vs. 3) and was accompanied by upbeat nystagmus in four patients. Head-shaking nystagmus was noted in seven subjects, ipsilesionally beating in six and down-beating in one. On brain MRI, damage of the most caudal parts of the medial and/or inferior vestibular nucleus was noted in 13 patients. Only those two patients with lesions affecting the rostral medulla oblongata demonstrated an ipsilaterally impaired aVOR. Conclusions: While subtle ocular motor signs pointed to damage of the central-vestibular pathways in all 17 patients, aVOR deficits were infrequent, restricted to those patients with rostral medullary lesions and, if present, mild to moderate only. This can be explained by lesions located too far caudally and too far ventrally to substantially affect the vestibular nuclei.
Rhinology journal, 2017
Background: The distance between the anterior wall of the maxillary sinus and the nasolacrimal du... more Background: The distance between the anterior wall of the maxillary sinus and the nasolacrimal duct shows a large individual variation. Methodology: To evaluate the feasibility of accessing the maxillary sinus through a prelacrimal window access (PLWA), a series of 100 paranasal CT scans from adult patients was analysed retrospectively. The distance between the anterior maxillary wall and the anterior border of the lacrimal duct (= prelacrimal window) were measured in 200 sides. Results: A distance of more the 7 mm was found in 12.5% maxillary sinuses and would enable straight forward PLWA. A distance between <7mm and >3mm was present in 56.5% of sinuses, and would make surgical access more demanding. In 31.5% of maxillary sinuses the distance was ≤3mm and in these patients this approach would be difficult without transecting the nasolacrimal duct. Conclusion: Only in 12.5% of sinuses a prelacrimal endoscopic access is readily feasible, while in 56.5% temporary tear sac dislocation is required and in 31.5% lacrimal sac dislocation is always needed along with a significant amount of bone removal to enable PLWA.
British journal of cancer, 2015
Gastro-oesophageal adenocarcinomas rarely metastasize to the central nervous system (CNS). The ro... more Gastro-oesophageal adenocarcinomas rarely metastasize to the central nervous system (CNS). The role of the human epidermal growth factor receptor 2 (HER2) in patients with these cancers and CNS involvement is presently unknown. A multicentre registry was established to collect data from patients with gastro-oesophageal adenocarcinomas and CNS involvement both retrospectively and prospectively. Inclusion in the study required a predefined clinical data set, a central neuro-radiological or histopathological confirmation of metastatic CNS involvement and central assessment of HER2 by immunohistochemistry (IHC) and in situ hybridisation (ISH). In addition, expression of E-cadherin and DNA mismatch repair (MMR) proteins were assessed by IHC. One hundred patients fulfilled the inclusion criteria. The population's median age was 59 years (interquartile range: 54-68), of which 85 (85%) were male. Twenty-five patients were of Asian and 75 of Caucasian origin. HER2 status was positive in ...
Journal of Neurology, 2015
Laryngo-Rhino-Otologie, 1998
BACKGROUND: Many host factors even in immunocompetent patients may have an influence on developme... more BACKGROUND: Many host factors even in immunocompetent patients may have an influence on development of a fungal diseases within the paranasal sinuses. Fungal sinusitis can occur in an acute form or more often to a chronic type of the disease. These mainly relatively asymptomatic chronic forms and further divided into a chronic noninvasive, chronic allergic, and chronic invasive disease. Endonasal microsurgery has significantly changed the management of chronic fungal sinusitis and allows adequate removal of pathologic tissue even in advanced situations. The aim of this study was to analyze the efficacy of endonasal surgery in chronic fungal sinusitis. MATERIAL AND METHODS: In a retrospective study we assessed a group of 40 patients who had endonasal surgery for chronic fungal sinusitis. Patient records, CT and MRI scans, microbiology and histology as well as the postoperative clinical follow-up including endoscopic photo documentation were evaluated over a period of 5 years. All patients underwent endonasal surgery using endoscopic techniques. The microscopic was of additional help in a few cases with extended disease and multiple dehiscences of the skull base. RESULTS: Twenty-four patients had a chronic noninvasive of fungal sinusitis and 16 patients had a chronic invasive form. All these patients underwent endonasal surgery without external incision. The fungal disease was erradicated in 39 cases, and revision surgery was required in only one case in which involvement of the contralateral side was not initially detected. in two cases scar tissue in the middle meatus was later excised but without evidence of residual fungal disease. Only in 6 cases was antifungal chemotherapy required, where the disease had spread into surrounding tissue or the patient had severe symptoms. CONCLUSIONS: Endonasal microsurgical techniques are today the appropriate approach for managing chronic fungal sinus disease even in severe cases with radiologic evidence of expansion or invasion of surrounding tissue. Additional antifungal chemotherapy is only rarely indicated, specifically when the fungal disease invades surrounding tissue.
American journal of human genetics, 1993
Several allelic mutations at the arylsulfatase A (ASA) locus cause substantial deficiencies of th... more Several allelic mutations at the arylsulfatase A (ASA) locus cause substantial deficiencies of this lysosomal enzyme. Depending on the genetically determined degree of the deficiency, the clinical outcome may be very different--either metachromatic leukodystrophy (MLD), a lethal lysosomal storage disorder affecting the nervous system, or, more frequently, the so-called pseudodeficiency (PD), which has no apparent clinical consequence. Because of compound heterozygosity for MLD and PD, 1/1,000 individuals in the population have low residual enzyme activities, which are intermediate between those of MLD patients and those of PD homozygous normal individuals. In order to assess whether PD/MLD compound heterozygotes bear a health risk, we examined clinically and biochemically 16 individuals with this genotype. Of these subjects, two had neurological symptoms and two showed lesions, without clinical symptoms, in magnetic resonance imaging of the brain. None of these symptoms was progress...
Medizinische Klinik (Munich, Germany : 1983), Jan 15, 1990
Deutsche zahnärztliche Zeitschrift, 1990
In a CT- and NMR-study, condylar remodeling after conservative treatment of condylar dislocation ... more In a CT- and NMR-study, condylar remodeling after conservative treatment of condylar dislocation fractures in growing individuals was evaluated. The regenerated condyles displayed great similarities. Two condylar parts could be differentiated--one with and one without muscular attachment. The medial part represents the former small fragment and the lateral part a bony outgrowth. One of the patients studied demonstrated a bifid condyle. NMR-records were remarkable in that each of the three patients presented a lateral pterygoid muscle with the two heads oriented one above the other rather than side by side. This does not seem to support the observation made in experiments, that the disc remains fixed to the caput in low condylar fractures.
The Laryngoscope, 2002
Objectives To assess the potential of color Doppler sonography to identify cervical mass lesions ... more Objectives To assess the potential of color Doppler sonography to identify cervical mass lesions as paragangliomas.Study Design Prospective evaluation.Methods Fifteen patients with 18 paragangliomas (13 carotid body tumors, 3 vagal and 2 temporal) presenting as a mass in the neck were investigated using color Doppler sonography.Results All paragangliomas presented in B‐mode sonography as solid, well‐defined, and hypoechoic tumors. Color Doppler imaging revealed hypervascularity in 15 (82%) tumors. No flow signal was detected in 3 carotid body tumors at standard velocity encoding (30 cm/s). Only carotid body tumors could be assessed in their full extent. Temporal and vagal paragangliomas were only partially visible. Carotid body tumors showed a splaying of the carotid bifurcation with displacement of the external carotid anteriorly and both the internal carotid and the internal jugular vein posteriorly. Anterior displacement of both carotid arteries and posterior displacement of the ...
Neuroradiology, 2007
Background CT angiography (CTA) is a minimally invasive technique that enables precise delineatio... more Background CT angiography (CTA) is a minimally invasive technique that enables precise delineation of extracranial and intracranial vascular anatomy and pathology based on high intravascular density. With a 64-slice MDCT scanner, improved first-pass vascular visualization can be obtained with HCCM (400 mg I/ml) using 25 ml for combined intra-and extracranial studies and 20 ml for intracranial examinations alone. We reviewed 23 patients with extra-cranial stenoocclusive disease and 12 patients with intra-cranial aneurysms. Two additional patients presented with a mycotic aneurysm and a micro arteriovenous malformation (micro-AVM). Results In 23 patients with 27 significant (≥70%) vascular stenoses, high intraluminal contrast density and optimal projection of the stenosis profile facilitated precise delineation of the residual lumen in all vessels affected. Pseudoocclusion was present in 3 of the 27 vessels (11%) and ulceration in 5 (18.5%). CTA, in contrast to digital subtraction angiography (DSA) and magnetic resonance angiography (MRA), was able to delineate the mural constituents of stenoses, particularly marked calcification (present in 17 of 27 vessels, 62.9%). Eccentric vessel wall narrowing indicated dissection in three vessels (11%) and circular postradiation fibrosis was found in two vessels (7.4%). High-quality CTA obviated the need for DSA for diagnostic purposes in every patient. The decision regarding surgical treatment in nine vessels (33%) was influenced by the location of the stenosis relative to the carotid bifurcation, the length of the stenosis, and the level of the carotid bifurcation. Endovascular treatment in 12 vessels (44.4%) and the appropriate choice and placement of endovascular devices was affected by the anatomic configuration of the supraaortic vessels, and by the intrastenotic dimensions relative to the dimensions of the vessel proximal and distal to the stenosis. The anatomic availability of collaterals, relevant for both treatments, was determined via the anterior communicating artery (ACoA) in 91.3% of patients and the posterior communicating artery (PCoA) in 80.4% of patients. Intracranial CTA displayed the lesion location, configuration, size, and orientation in each of the 12 patients with intracranial aneurysms and in the two patients with a micro-AVM and a mycotic aneurysm. The correct diagnosis was established prior to DSA in each patient. Following CTA, the role of DSA was relegated to endovascular therapy in three aneurysms. For surgical candidates, DSA served to exclude potential multiplicity in ten saccular aneurysms and in one mycotic aneurysm. CTA provided information supplementary to DSA in 11 of the 13 aneurysms (84.6%). Delineation of blebs, calcification, thrombus, or incorporation of branches facilitated risk stratification with respect to rupture and to surgical or endovascular treatment. Conclusion Advantages of CTA, such as virtual independence from the hemodynamic situation, delineation of landmarks and vessel wall calcification, and the ability to quantify vessels and aneurysms, distinguish this technique from other noninvasive vascular imaging techniques and DSA. Acquired with the use of iodinated HCCM, CT angiograms permit excellent recognition of appropriate diagnostic and interventional treatment paths, thereby facilitating improved decision-making regarding endovascular or surgical treatment.
Journal of Neurology, Neurosurgery & Psychiatry, 1989
Journal of Neurology, 2008
The Journal of Laryngology & Otology, 2014
Journal of Cranio-Maxillofacial Surgery, 1997
SUMMAR Y Over a period of 3 years, 18 patients with mandibular osteomyelitis were prospectively i... more SUMMAR Y Over a period of 3 years, 18 patients with mandibular osteomyelitis were prospectively investigated by conventional radiograph, computed tomography (CT) and in 6 cases by magnetic resonance imaging (MRI). The diagnosis was based on histology in 17 patients operated upon.
Frontiers in Neurology, 2012
Clinical Oral Implants Research, 2009
OBJECTIVES: To compare the performance and safety of Inion GTR(TM) Biodegradable Membrane System ... more OBJECTIVES: To compare the performance and safety of Inion GTR(TM) Biodegradable Membrane System and Geistlich resorbable bilayer Bio-Gide((R)) membrane in human bone regeneration. MATERIAL AND METHODS: In a multicenter, split blind, comparative, randomized, prospective, pilot study 15 patients have been randomized at surgery whether to be treated either with Inion GTR(TM) Biodegradable Membrane System on one and Geistlich resorbable bilayer Bio-Gide((R)) membrane on the other side or vice versa after surgical removal of both fully impacted wisdom teeth. During the follow-up visits at week 1, 2 and 6 and at months 3 and 6 the general state, the wound, eventual adverse events and the medication of the patients were assessed. Computed Tomography (CT) scans were performed immediately and 3 months after the surgery, before biopsy collection. Semi-quantitative histological evaluation and histomorphometric analyses were performed according to the ISO 10993-6 standard. New bone formation and membrane integration were evaluated by CT scan measurements. Tissue healing was evaluated clinically and by photographs between the time on teeth extraction and during follow ups. RESULTS: Five patients were smokers, none drank alcohol. Mild adverse events like wound infection, haematoma or late swelling of the gums occurred in three patients. The trephine bur harvest of bone biopsies under local anaesthesia was uneventful. Whereas specimens from the sites treated with the Inion membrane yielded 17.0% (SD 24%), the Bio-Gide membrane sites yielded 13.5% (SD 15%) of bone tissue density. In sites treated with the Inion membrane, 9.5% of old bone density and 7.5% of newly formed bone could be found, whereas the Bio-Gide((R)) membrane sites showed 3.8% of old bone density and 9.8% of newly formed bone. There were no statistically significant differences between the two groups with respect to the two variables. The osteoid rim was more extended with the Bio-Gide((R)) (6.6 mm) than with the Inion membrane (5.1 mm) but the difference between the two treatments did not reach statistical significance. Highly significant reductions in the area of the defect with both membranes were detected with significant increases in CT density at the immediate inferio-buccal adjacent bone and in the surgical defect area with both membranes. However, there was neither significant change in CT density in the immediate inferior-lingual adjacent bone of the two membranes, nor significant difference between the membranes on any of the four measurements (area of defect: P=0.1354; CT density immediate inferiobuccal adjacent bone: P=0.7615; CT density surgical defect area: P=0.1876; CT density immediate inferio-lingual adjacent bone: P=0.4212). CONCLUSION: The overall clinical outcome was satisfying and the majority of the patients showed an uneventful healing phase. Both membranes presented similar capacities regarding their barrier function and were associated with analogous bone regeneration. No statistically valid evidence about the superiority of one particular membrane was obtained. For the patient the only difference is that one product is animal derived and the other synthetic.
Clinical Imaging, Nov 1, 2008
ABSTRACT This retrospective study aimed to describe the differences between image readings done w... more ABSTRACT This retrospective study aimed to describe the differences between image readings done with combined positron emission tomography/computed tomography (PET/CT) and PET read together with contrast-enhanced CT (ceCT) in patients with squamous cell carcinoma of the head and neck. In 46 patients, no differences were found between the two readings for assessing infiltration of adjacent structures (P=.63), transgression of the midline (P=.67), lymph node involvement (P=.32), and T- and N stage. PET/CT and PET read together with ceCT have comparable diagnostic yield.
Journal of Neurology
Of the 96 serologically confirmed neuroborreliosis cases seen in our clinic between 1983 and 1988... more Of the 96 serologically confirmed neuroborreliosis cases seen in our clinic between 1983 and 1988, 11 patients had mild to moderate and 4 patients had serious cerebral and/or spinal cord symptoms. Nine of these 15 patients with CNS involvement exhibited a primary chronic course of the illness. After high-dose intravenous therapy with penicillin, doxycycline or cefotaxime, given mostly in combination with cortisone, gradual recovery occurred with normalization of CSF findings characteristic of neuroborreliosis, and normalization of significantly elevated Borrelia burgdorferi IgG antibody titres in CSF and serum. Brain MRI and CT showed evidence of or were suggestive of vascular involvement which correlated with clinical symptoms in 11 of the 15 patients with CNS involvement. Brain MRI changes that were similar but much slighter in number and intensity were seen in 5 of 12 neuroborreliosis patients without clinical signs of CNS involvement (lymphocytic meningoradiculitis; Bannwarth's syndrome). The frequencies of the HLA-DR7 (75%), HLA-B44 (50%) and HLA-A29 (33%) antigens in 12 neuroborreliosis patients with clinical symptoms of CNS involvement were significantly different from the frequencies in 12 neuroborreliosis patients without CNS involvement and in 100 control subjects. Diagnostic criteria of active neuroborreliosis are proposed.
Frontiers in Neurology, 2020
Background: Lateral medullary stroke (LMS) results in a characteristic pattern of brainstem signs... more Background: Lateral medullary stroke (LMS) results in a characteristic pattern of brainstem signs including ocular motor and vestibular deficits. Thus, an impaired angular vestibulo-ocular reflex (aVOR) may be found if the vestibular nuclei are affected. Objective: We aimed to characterize the frequency and pattern of vestibular and ocular-motor deficits in patients with LMS. Methods: Patients with MR-confirmed acute/subacute unilateral LMS from a stroke registry were included and a bedside neuro-otological examination was performed. Video-oculography and video-based head-impulse testing (vHIT) was obtained and semicircular canal function was determined. The lesion location/extension as seen on MRI was rated and involvement of the vestibular nuclei was judged. Results: Seventeen patients with LMS (age = 59.4 ± 14.3 years) were included. All patients had positive H.I.N.T.S. vHIT showed mild-to-moderate aVOR impairments in three patients (ipsilesional = 1; ipsilesional and contralesional = 1; contralesional = 1). Spontaneous nystagmus (n = 10/15 patients) was more often beating contralesionally than ipsilesionally (6 vs. 3) and was accompanied by upbeat nystagmus in four patients. Head-shaking nystagmus was noted in seven subjects, ipsilesionally beating in six and down-beating in one. On brain MRI, damage of the most caudal parts of the medial and/or inferior vestibular nucleus was noted in 13 patients. Only those two patients with lesions affecting the rostral medulla oblongata demonstrated an ipsilaterally impaired aVOR. Conclusions: While subtle ocular motor signs pointed to damage of the central-vestibular pathways in all 17 patients, aVOR deficits were infrequent, restricted to those patients with rostral medullary lesions and, if present, mild to moderate only. This can be explained by lesions located too far caudally and too far ventrally to substantially affect the vestibular nuclei.
Rhinology journal, 2017
Background: The distance between the anterior wall of the maxillary sinus and the nasolacrimal du... more Background: The distance between the anterior wall of the maxillary sinus and the nasolacrimal duct shows a large individual variation. Methodology: To evaluate the feasibility of accessing the maxillary sinus through a prelacrimal window access (PLWA), a series of 100 paranasal CT scans from adult patients was analysed retrospectively. The distance between the anterior maxillary wall and the anterior border of the lacrimal duct (= prelacrimal window) were measured in 200 sides. Results: A distance of more the 7 mm was found in 12.5% maxillary sinuses and would enable straight forward PLWA. A distance between <7mm and >3mm was present in 56.5% of sinuses, and would make surgical access more demanding. In 31.5% of maxillary sinuses the distance was ≤3mm and in these patients this approach would be difficult without transecting the nasolacrimal duct. Conclusion: Only in 12.5% of sinuses a prelacrimal endoscopic access is readily feasible, while in 56.5% temporary tear sac dislocation is required and in 31.5% lacrimal sac dislocation is always needed along with a significant amount of bone removal to enable PLWA.
British journal of cancer, 2015
Gastro-oesophageal adenocarcinomas rarely metastasize to the central nervous system (CNS). The ro... more Gastro-oesophageal adenocarcinomas rarely metastasize to the central nervous system (CNS). The role of the human epidermal growth factor receptor 2 (HER2) in patients with these cancers and CNS involvement is presently unknown. A multicentre registry was established to collect data from patients with gastro-oesophageal adenocarcinomas and CNS involvement both retrospectively and prospectively. Inclusion in the study required a predefined clinical data set, a central neuro-radiological or histopathological confirmation of metastatic CNS involvement and central assessment of HER2 by immunohistochemistry (IHC) and in situ hybridisation (ISH). In addition, expression of E-cadherin and DNA mismatch repair (MMR) proteins were assessed by IHC. One hundred patients fulfilled the inclusion criteria. The population's median age was 59 years (interquartile range: 54-68), of which 85 (85%) were male. Twenty-five patients were of Asian and 75 of Caucasian origin. HER2 status was positive in ...
Journal of Neurology, 2015
Laryngo-Rhino-Otologie, 1998
BACKGROUND: Many host factors even in immunocompetent patients may have an influence on developme... more BACKGROUND: Many host factors even in immunocompetent patients may have an influence on development of a fungal diseases within the paranasal sinuses. Fungal sinusitis can occur in an acute form or more often to a chronic type of the disease. These mainly relatively asymptomatic chronic forms and further divided into a chronic noninvasive, chronic allergic, and chronic invasive disease. Endonasal microsurgery has significantly changed the management of chronic fungal sinusitis and allows adequate removal of pathologic tissue even in advanced situations. The aim of this study was to analyze the efficacy of endonasal surgery in chronic fungal sinusitis. MATERIAL AND METHODS: In a retrospective study we assessed a group of 40 patients who had endonasal surgery for chronic fungal sinusitis. Patient records, CT and MRI scans, microbiology and histology as well as the postoperative clinical follow-up including endoscopic photo documentation were evaluated over a period of 5 years. All patients underwent endonasal surgery using endoscopic techniques. The microscopic was of additional help in a few cases with extended disease and multiple dehiscences of the skull base. RESULTS: Twenty-four patients had a chronic noninvasive of fungal sinusitis and 16 patients had a chronic invasive form. All these patients underwent endonasal surgery without external incision. The fungal disease was erradicated in 39 cases, and revision surgery was required in only one case in which involvement of the contralateral side was not initially detected. in two cases scar tissue in the middle meatus was later excised but without evidence of residual fungal disease. Only in 6 cases was antifungal chemotherapy required, where the disease had spread into surrounding tissue or the patient had severe symptoms. CONCLUSIONS: Endonasal microsurgical techniques are today the appropriate approach for managing chronic fungal sinus disease even in severe cases with radiologic evidence of expansion or invasion of surrounding tissue. Additional antifungal chemotherapy is only rarely indicated, specifically when the fungal disease invades surrounding tissue.
American journal of human genetics, 1993
Several allelic mutations at the arylsulfatase A (ASA) locus cause substantial deficiencies of th... more Several allelic mutations at the arylsulfatase A (ASA) locus cause substantial deficiencies of this lysosomal enzyme. Depending on the genetically determined degree of the deficiency, the clinical outcome may be very different--either metachromatic leukodystrophy (MLD), a lethal lysosomal storage disorder affecting the nervous system, or, more frequently, the so-called pseudodeficiency (PD), which has no apparent clinical consequence. Because of compound heterozygosity for MLD and PD, 1/1,000 individuals in the population have low residual enzyme activities, which are intermediate between those of MLD patients and those of PD homozygous normal individuals. In order to assess whether PD/MLD compound heterozygotes bear a health risk, we examined clinically and biochemically 16 individuals with this genotype. Of these subjects, two had neurological symptoms and two showed lesions, without clinical symptoms, in magnetic resonance imaging of the brain. None of these symptoms was progress...
Medizinische Klinik (Munich, Germany : 1983), Jan 15, 1990
Deutsche zahnärztliche Zeitschrift, 1990
In a CT- and NMR-study, condylar remodeling after conservative treatment of condylar dislocation ... more In a CT- and NMR-study, condylar remodeling after conservative treatment of condylar dislocation fractures in growing individuals was evaluated. The regenerated condyles displayed great similarities. Two condylar parts could be differentiated--one with and one without muscular attachment. The medial part represents the former small fragment and the lateral part a bony outgrowth. One of the patients studied demonstrated a bifid condyle. NMR-records were remarkable in that each of the three patients presented a lateral pterygoid muscle with the two heads oriented one above the other rather than side by side. This does not seem to support the observation made in experiments, that the disc remains fixed to the caput in low condylar fractures.
The Laryngoscope, 2002
Objectives To assess the potential of color Doppler sonography to identify cervical mass lesions ... more Objectives To assess the potential of color Doppler sonography to identify cervical mass lesions as paragangliomas.Study Design Prospective evaluation.Methods Fifteen patients with 18 paragangliomas (13 carotid body tumors, 3 vagal and 2 temporal) presenting as a mass in the neck were investigated using color Doppler sonography.Results All paragangliomas presented in B‐mode sonography as solid, well‐defined, and hypoechoic tumors. Color Doppler imaging revealed hypervascularity in 15 (82%) tumors. No flow signal was detected in 3 carotid body tumors at standard velocity encoding (30 cm/s). Only carotid body tumors could be assessed in their full extent. Temporal and vagal paragangliomas were only partially visible. Carotid body tumors showed a splaying of the carotid bifurcation with displacement of the external carotid anteriorly and both the internal carotid and the internal jugular vein posteriorly. Anterior displacement of both carotid arteries and posterior displacement of the ...
Neuroradiology, 2007
Background CT angiography (CTA) is a minimally invasive technique that enables precise delineatio... more Background CT angiography (CTA) is a minimally invasive technique that enables precise delineation of extracranial and intracranial vascular anatomy and pathology based on high intravascular density. With a 64-slice MDCT scanner, improved first-pass vascular visualization can be obtained with HCCM (400 mg I/ml) using 25 ml for combined intra-and extracranial studies and 20 ml for intracranial examinations alone. We reviewed 23 patients with extra-cranial stenoocclusive disease and 12 patients with intra-cranial aneurysms. Two additional patients presented with a mycotic aneurysm and a micro arteriovenous malformation (micro-AVM). Results In 23 patients with 27 significant (≥70%) vascular stenoses, high intraluminal contrast density and optimal projection of the stenosis profile facilitated precise delineation of the residual lumen in all vessels affected. Pseudoocclusion was present in 3 of the 27 vessels (11%) and ulceration in 5 (18.5%). CTA, in contrast to digital subtraction angiography (DSA) and magnetic resonance angiography (MRA), was able to delineate the mural constituents of stenoses, particularly marked calcification (present in 17 of 27 vessels, 62.9%). Eccentric vessel wall narrowing indicated dissection in three vessels (11%) and circular postradiation fibrosis was found in two vessels (7.4%). High-quality CTA obviated the need for DSA for diagnostic purposes in every patient. The decision regarding surgical treatment in nine vessels (33%) was influenced by the location of the stenosis relative to the carotid bifurcation, the length of the stenosis, and the level of the carotid bifurcation. Endovascular treatment in 12 vessels (44.4%) and the appropriate choice and placement of endovascular devices was affected by the anatomic configuration of the supraaortic vessels, and by the intrastenotic dimensions relative to the dimensions of the vessel proximal and distal to the stenosis. The anatomic availability of collaterals, relevant for both treatments, was determined via the anterior communicating artery (ACoA) in 91.3% of patients and the posterior communicating artery (PCoA) in 80.4% of patients. Intracranial CTA displayed the lesion location, configuration, size, and orientation in each of the 12 patients with intracranial aneurysms and in the two patients with a micro-AVM and a mycotic aneurysm. The correct diagnosis was established prior to DSA in each patient. Following CTA, the role of DSA was relegated to endovascular therapy in three aneurysms. For surgical candidates, DSA served to exclude potential multiplicity in ten saccular aneurysms and in one mycotic aneurysm. CTA provided information supplementary to DSA in 11 of the 13 aneurysms (84.6%). Delineation of blebs, calcification, thrombus, or incorporation of branches facilitated risk stratification with respect to rupture and to surgical or endovascular treatment. Conclusion Advantages of CTA, such as virtual independence from the hemodynamic situation, delineation of landmarks and vessel wall calcification, and the ability to quantify vessels and aneurysms, distinguish this technique from other noninvasive vascular imaging techniques and DSA. Acquired with the use of iodinated HCCM, CT angiograms permit excellent recognition of appropriate diagnostic and interventional treatment paths, thereby facilitating improved decision-making regarding endovascular or surgical treatment.
Journal of Neurology, Neurosurgery & Psychiatry, 1989
Journal of Neurology, 2008
The Journal of Laryngology & Otology, 2014
Journal of Cranio-Maxillofacial Surgery, 1997
SUMMAR Y Over a period of 3 years, 18 patients with mandibular osteomyelitis were prospectively i... more SUMMAR Y Over a period of 3 years, 18 patients with mandibular osteomyelitis were prospectively investigated by conventional radiograph, computed tomography (CT) and in 6 cases by magnetic resonance imaging (MRI). The diagnosis was based on histology in 17 patients operated upon.
Frontiers in Neurology, 2012
Clinical Oral Implants Research, 2009
OBJECTIVES: To compare the performance and safety of Inion GTR(TM) Biodegradable Membrane System ... more OBJECTIVES: To compare the performance and safety of Inion GTR(TM) Biodegradable Membrane System and Geistlich resorbable bilayer Bio-Gide((R)) membrane in human bone regeneration. MATERIAL AND METHODS: In a multicenter, split blind, comparative, randomized, prospective, pilot study 15 patients have been randomized at surgery whether to be treated either with Inion GTR(TM) Biodegradable Membrane System on one and Geistlich resorbable bilayer Bio-Gide((R)) membrane on the other side or vice versa after surgical removal of both fully impacted wisdom teeth. During the follow-up visits at week 1, 2 and 6 and at months 3 and 6 the general state, the wound, eventual adverse events and the medication of the patients were assessed. Computed Tomography (CT) scans were performed immediately and 3 months after the surgery, before biopsy collection. Semi-quantitative histological evaluation and histomorphometric analyses were performed according to the ISO 10993-6 standard. New bone formation and membrane integration were evaluated by CT scan measurements. Tissue healing was evaluated clinically and by photographs between the time on teeth extraction and during follow ups. RESULTS: Five patients were smokers, none drank alcohol. Mild adverse events like wound infection, haematoma or late swelling of the gums occurred in three patients. The trephine bur harvest of bone biopsies under local anaesthesia was uneventful. Whereas specimens from the sites treated with the Inion membrane yielded 17.0% (SD 24%), the Bio-Gide membrane sites yielded 13.5% (SD 15%) of bone tissue density. In sites treated with the Inion membrane, 9.5% of old bone density and 7.5% of newly formed bone could be found, whereas the Bio-Gide((R)) membrane sites showed 3.8% of old bone density and 9.8% of newly formed bone. There were no statistically significant differences between the two groups with respect to the two variables. The osteoid rim was more extended with the Bio-Gide((R)) (6.6 mm) than with the Inion membrane (5.1 mm) but the difference between the two treatments did not reach statistical significance. Highly significant reductions in the area of the defect with both membranes were detected with significant increases in CT density at the immediate inferio-buccal adjacent bone and in the surgical defect area with both membranes. However, there was neither significant change in CT density in the immediate inferior-lingual adjacent bone of the two membranes, nor significant difference between the membranes on any of the four measurements (area of defect: P=0.1354; CT density immediate inferiobuccal adjacent bone: P=0.7615; CT density surgical defect area: P=0.1876; CT density immediate inferio-lingual adjacent bone: P=0.4212). CONCLUSION: The overall clinical outcome was satisfying and the majority of the patients showed an uneventful healing phase. Both membranes presented similar capacities regarding their barrier function and were associated with analogous bone regeneration. No statistically valid evidence about the superiority of one particular membrane was obtained. For the patient the only difference is that one product is animal derived and the other synthetic.