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Biomedical subjects

E Neuen-Jacob

Publications and source records attributed to E Neuen-Jacob.

At least 37 records · Page 2Linked to original sources
PubMed2000

Changes in canine peripheral nerves during experimental callus distraction.

In 24 beagles, lengthening of the right tibia was performed by callus distraction after osteotomy and application of a ring fixator. Distraction was started the fifth day after surgery with a distraction rate of 0.5 mm twice per day and ended after 25 days. A control group of six additional dogs underwent tibial osteotomy and external fixation without distraction. Twelve animals with leg lengthening and three animals without leg lengthening were sacrificed immediately after the distraction period of 25 days (Group A), the remaining 15 dogs were sacrificed after an additional consolidation phase of another 25 days (Group B). From the distracted right leg and from the left control side of each animal, the tibial and peroneal nerves were removed and studied by means of morphometric analysis of semithin sections and electron microscopic study. The authors reported only minor alterations of the myelinated nerve fibers consisting in relatively thin myelinated remyelinating nerve fibers, single axon degeneration, or macrophages. There was no axonal sprouting. In contrast, electron microscopic study disclosed signs of late Wallerian degeneration and axonal regeneration of the nonmyelinated axons. Morphometric analysis of the peroneal nerves revealed a significant increase of fiber density and a reduced mean axon and fiber diameter in the consolidation period (Group B). Similar changes were found in the tibial nerves in Group A and disappeared in the consolidation phase (Group B). These features indicate that callus distraction leads to moderate degenerative changes, followed by repair mechanisms, almost complete recovery, and some nerve fiber growth.

Animals↗
PubMed2000

Multiple intracranial juvenile xanthogranulomas. Case report.

The authors report on an 11-year-old boy in whom proptosis of the eye caused by a benign intraosseous xanthofibroma of the left orbital wall became clinically apparent at the age of 4 years. Two years later he developed bilateral papilledema, at which time computerized tomography and magnetic resonance studies revealed multiple enhancing intracranial lesions. The largest mass was located in the left middle fossa; other lesions were located at the tentorium cerebelli, in both lateral ventricles, near the superior sagittal sinus, and extracranially near the left jugular vein. The mass in the left middle fossa was resected and diagnosed as juvenile xanthogranuloma (JXG). Thirty months later, the patient again became symptomatic, exhibiting behavioral abnormalities and a decrease in mental powers. At that time, the two remaining lesions in both lateral ventricles had grown enough to cause trapping of the temporal horns and raised intracranial pressure. These lesions were successively resected and histopathologically confirmed to be JXGs. However, resection of the second intraventricular lesion was complicated by postoperative bilateral amaurosis, presumably caused by postdecompression optic neuropathy. According to a review of the literature, fewer than 20 patients with JXG involving the central nervous system have been reported. The patient described in this report is the first in whom multiple intracranial JXGs developed in the absence of cutaneous manifestations. Although JXGs are biologically benign lesions, the treatment of patients with multifocal and/or progressive intracranial manifestations is problematic.

Brain Diseases↗
PubMed1999

Long-term course and outcome in AIDS patients with cerebral toxoplasmosis.

OBJECTIVES: We compared clinical long-term course and outcome in all AIDS patients admitted to our outpatient department from January, 1989 to June, 1998 with toxoplasma encephalitis (TE) as first AIDS-defining infection (n= 106) and in 106 patients with Pneumocystis carinii pneumonia (PCP) as first AIDS-defining disease. MATERIAL AND METHODS: The 2 groups were matched with respect to age, sex, risk group, degree of immunodeficiency as measured by CD4 cell counts and duration of HIV-1-positivity. TE was diagnosed radiologically and by response to treatment. RESULTS: Forty-three TE patients surviving the first TE symptoms > 14 months developed dementive symptoms, leucoencephalopathy in imaging procedures and died from dementia. In contrast only 5 patients surviving PCP for an equally long period showed dementive symptoms. CONCLUSION: Cerebral infections like toxoplasma encephalitis (TE) may negatively influence HIV-1-activity so far latent in the brain.

AIDS Dementia Complex↗
PubMed1999

Glioblastoma multiforme at the site of metal splinter injury: a coincidence? Case report.

The authors report the case of a man who had suffered a penetrating metal splinter injury to the left frontal lobe at 18 years of age. Thirty-seven years later the patient developed a left-sided frontal tumor at the precise site of the meningocerebral scar and posttraumatic defect. Histological examination confirmed a glioblastoma multiforme adjacent to the dural scar and metal splinters. In addition, a chronic abscess from which Propionibacterium acnes was isolated was found within the glioma tissue. The temporal and local association of metal splinter injury with chronic abscess, scar formation, and malignant glioma is highly suggestive of a causal relationship between trauma and the development of a malignant brain tumor.

Adolescent↗
PubMed1997

Diagnostic criteria and clinical procedures in HIV-1 associated progressive multifocal leukoencephalopathy.

The diagnosis of definite progressive multifocal leukoencephalopathy (PML) has been a neuropathological domain. We reviewed all Human Immunodeficiency Virus Type 1 (HIV-1) seropositive patients in our institution between 01.01.1989 and 31.12.1994 and identified 20/823 cases with PML by clinical and imaging criteria. Diagnosis was neuropathologically confirmed in 5 cases. Diagnostic criteria included rapid onset (< 2 weeks) of multifocal neurological signs and symptoms, advanced immunosuppression and asymmetric uni- or multifocal white matter lesions without mass effect, contrast enhancement or cortical atrophy in magnetic resonance imaging (MRI). The overall incidence of PML was stable over the observation period (approximately equal to 2.5%). The mean age at onset (41.7 years) was significantly lower compared to HIV-1 seronegative PML patients (peak in the sixth decade of life), male patients prevailed (100%). Mean survival (3.9 months) was extremely short. Human polyoma virus JC (JCV) polymerase chain reaction (PCR) in the cerebrospinal fluid (CSF) demonstrated a considerable rate of possible cerebral co-infection with HIV-1 and JCV as well as subclinical infection with JCV. Therefore demonstration of JCV deoxyribonucleic acid by PCR in the CSF alone is not sufficient for clinical PML diagnosis. We present diagnostic criteria on the basis of epidemiological, neuroradiological and CSF parameters that allow us to make the clinical diagnosis of PML. Although quick and safe, routine stereotactic brain biopsy is not necessary to confirm the diagnosis.

AIDS-Related Opportunistic Infections↗
PubMed1995

Focal brain lesions in patients with AIDS: aetiologies and corresponding radiological patterns in a prospective study.

We report the results of a hospital-based study of 188 consecutive patients seropositive for the human immune deficiency virus type 1 (HIV-1) who presented in a 4-year period (1988-1991) with possible signs or symptoms of first-ever central nervous system disease. Confirmed diagnoses were cerebral toxoplasmosis in 47 patients (25.0%), HIV-1 encephalopathy in 19 (10.1%), progressive multifocal leucoencephalopathy (PML) in 9 (4.8%), cerebral lymphoma in 1 (0.5%), and other conditions in 9 patients (4.8%). Seventy-three subjects (38.8%) showed focal brain lesions on initial computed tomography or magnetic resonance imaging, which were assessed prospectively. Positive predictivity for toxoplasmosis was 100% if multiple lesions occurred in combination with mass effect or contrast enhancement (23 patients), or if at least one space-occupying or enhancing lesion was located in the basal ganglia or the thalamus (26 patients). Solitary lesions with mass effect or contrast enhancement were seen in 26 patients and were caused by cerebral toxoplasmosis in 22 (84.6%). Eight of the 9 PML patients presented with one or more non-enhancing, non-mass lesions, although the predictive value of this pattern was low (47.1% for PML). Thus, in our epidemiological context, certain imaging findings in HIV-1-seropositive patients were highly predictive of cerebral toxoplasmosis. This may differ from findings from other parts of the world where cerebral toxoplasmosis may be less prevalent among HIV-1-infected individuals.

AIDS Dementia Complex↗
PubMed1994

A pathologically distinct new form of HIV associated encephalopathy.

We present the clinical, morphological and neuropathological findings in a 44-year-old male suffering from the acquired immunodeficiency syndrome (AIDS) (CDC stage IV C2) who presented with rapidly progressive right-side hemiparesis and developed hemianopia and aphasia. Scans showed multiple, not contrast-enhancing, not space-occupying echo-intensive lesions in T2-weighted MR-imaging. No hint for an opportunistic infection, necrotizing vasculitis or vascular disease was found. All therapeutic regimens failed and 8 weeks after onset of neurological symptoms the patient died because of cardiorespiratory arrest. Post-mortem examination excluded opportunistic infection, progressive multifocal leukoencephalopathy, lymphoma, vasculitis and ischemia of the brain. In the presence of an unusually high amount of HIV-infected macrophages at immunohistochemical examination, the overall pathological findings were atypical both for HIV encephalitis and HIV leukoencephalopathy. We describe a pathologically distinct new form of HIV associated encephalopathy.

AIDS Dementia Complex↗
PubMed1994

Multisystem triglyceride storage disorder without ichthyosis in two siblings.

A four-year-old boy presented with hepatomegaly, vacuolized granulocytes (Jordans' anomaly) and slightly progressive myopathy as signs of multisystem triglyceride storage disease. The nine-year-old sister of the patient also showed Jordans' anomaly and early fatigability, but no overt weakness. Biochemical analysis revealed normal values for carnitines, carnitine palmityl transferase in serum and striated muscle, and beta-oxidation enzymes in striated muscles. Distribution of non-membrane-bound lipids in granulocytes, fibroblasts, smooth muscle cells and striated muscle was compatible with Chanarin-Dorfman syndrome. In contrast to Chanarin-Dorfman syndrome, our patients lacked congenital ichthyosis.

Child↗
PubMed1993

Electrophysiological motor testing, MRI findings and clinical course in AIDS patients with dementia.

Thirty-three HIV-positive patients with clinical signs of dementia according to the 1991 AAN criteria underwent psychometric, electrophysiological and radiological examination and were compared with a group of normal healthy subjects and a cohort of clinically asymptomatic HIV-1-positive individuals of comparable education and social environment. Compared with the other groups, test performance was severely impaired in the demented patients. Results of motor testing and MRI revealed that subcortical structures were not exclusively affected, but most severely and early, thus characterizing the clinical feature in HIV-1-associated dementia. In demented patients a rapid deterioration was observed, leading to death within about 12 months on average, which is a markedly shorter survival time than described in the literature for non-demented HIV-1-positive individuals.

AIDS Dementia Complex↗
PubMed1993

Pontine infarction in acute posterior multifocal placoid pigment epitheliopathy.

In a patient with acute posterior multifocal placoid pigment epitheliopathy (APMPPE), a pontine infarction occurred about 6 months after the ophthalmological manifestation. We report the first case with histopathologically proven vasculitis shown by muscle biopsy and the first positron emission tomographic documentation in APMPPE. The ophthalmological and cerebral symptoms responded well to steroid treatment. Long-term immunosuppression (e.g. azathioprine 1-2 mg/kg) seems to decrease the risk of recurrent systemic vasculitis.

Acute Disease↗
PubMed1993

Neuropathological studies in the brains of AIDS patients with opportunistic diseases.

The brains of 70 fatal cases with AIDS were studied by means of immunohistochemistry and in-situ hybridization in a consecutive autopsy series (1985-July 1992). In addition, the neuropathological changes were correlated with the neurological and neuroimaging findings. Opportunistic infections included toxoplasmosis (15 cases), cytomegalovirus (CMV)-encephalitis (6), progressive multifocal leucoencephalopathy (2) and fungal infections (3). Malignant lymphomas were found in 7 patients; 6 involved primarily the CNS, one was metastatic. In 14 cases the neuropathological changes were consistent with HIV encephalitis and HIV leucoencephalopathy. Non-specific lesions occurred in 31 cases. The clinical diagnosis in patients with opportunistic diseases (n = 27) diverged in 15 cases (55%) from the underlying pathology. Toxoplasma gondii, CMV and JC viruses were identified by immunohistochemistry and in-situ hybridization on serial paraffin sections. In addition, antibodies against lymphocyte subsets, tissue macrophages, the glial fibrillary acid protein (GFAP) and myelin basic protein were used to characterize the phenotype of cells and to highlight the degree of gliosis and demyelination. Our results show that the distribution and degree of morphological changes might be helpful for the differential diagnosis antemortem. Since neurological complications may represent the first or sole manifestation of AIDS and risk factors for AIDS are often not known, it should be taken into account that CNS manifestations of AIDS may contribute to a sudden and unexpected death or accident. Opportunistic diseases should be considered as a possible differential diagnosis in cases mimicking the clinical picture of apoplexia or dementia. Furthermore, CNS lesions may be detected postmortem in patients who were not known to suffer from Neuro-Aids during life, indicating that CNS involvement is more widespread than assumed.

AIDS Dementia Complex↗
PubMed1992

Somatic mosaicism for a deletion of the dystrophin gene in a carrier of Becker muscular dystrophy.

Duchenne muscular dystrophy (DMD) and the allelic milder form of Becker muscular dystrophy (BMD) are caused by mutations of the dystrophin gene on the short arm of the X chromosome. One third of affected individuals are expected to result from de novo mutations. Genetic counselling of families with sporadic cases is complicated by the potential meiotic origin of the mutation in the mother resulting in germline mosaicism. Here we present direct evidence for combined somatic and germline mosaicism for a deletion of the dystrophin gene, thereby proving the mitotic origin of this deletion and pinpointing a further potential pitfall for genetic counselling. The mother of a BMD son and a BMD carrier daughter, both carrying a deletion of dystrophin cDNA 7 (0.5 kb Hind III fragment) and cDNA 8, was herself clinically healthy and had normal creatine kinase levels. A muscle specimen of the mother showed mild overall pathology as well as focal dystrophin deficiency. In contrast chromosomal in situ suppression (CISS) hybridization of metaphase chromosomes using a cosmid clone of the corresponding cDNA deleted in her son revealed no evidence of somatic mosaicism in their lymphocytes. These results emphasize the value of an approach correlating genetic and immunological data for the definition of a carrier state in BMD or DMD. The possibility of somatic mosaicism should be considered when genetic counselling of a family with a sporadic case of BMD or DMD is performed.

Blotting, Southern↗
PubMed1992

Sudden loss of hearing and vestibular function, muscular weakness, and multiple white matter lesions in preschool children.

Sudden cochlear hearing loss, occurring successively or simultaneously in both ears, was observed in four unrelated preschool children. Vestibular testing could be carried out in three patients and showed complete bilateral loss of function. All patients had a mildly retarded motor development due to nonprogressive muscular weakness. On MR imaging all patients showed multiple periventricular and subcortical white matter lesions. These lesions were not clearly progressive in one patient examined repeatedly over 6 years. Virological, bacteriological, immunological, and metabolic examinations were normal in all patients. Muscle biopsy showed morphologically abnormal mitochondria in two and lipid storage in one patient. No indications have been found for a disturbed functioning of the muscle mitochondria. The identical pattern and course of the disease in these patients suggests a new nosological entity, the aetiology of which can only speculatively be attributed to a vascular process associated with a mitochondriopathy.

Biopsy↗
PubMed1991

Ultrastructural abnormalities of mitochondria and deficiency of myocardial cytochrome c oxidase in a patient with ventricular tachycardia.

A 30-year-old woman presented with life-threatening ventricular tachycardia without overt heart disease. Ultrastructural investigation of endomyocardial biopsy disclosed abnormally structured and often enlarged mitochondria. Morphometry revealed the ratio of volume density of mitochondria to myofibrils to be markedly increased to 0.667 as compared with five controls (mean: 0.46; range: 0.445-0.479). Investigation of mitochondrial respiratory chain enzymes revealed a 90% reduction in activity of cytochrome c oxidase. Our data suggest that mitochondrial cardiomyopathy may induce malignant ventricular arrhythmias.

Adult↗
PubMed1990

Unexpected infant death attributable to cardiac tumor or cardiomyopathy. Immunohistochemical and electron microscopical findings in three cases.

The pathological findings, including immunohistochemical and electron microscopical findings, in three infants who died unexpectedly of cardiac tumor or cardiomyopathy are reported. The first was a 13-month-old boy with tuberous sclerosis and multiple rhabdomyomas of the heart, who presented with a postpartal cardiac murmur and moderate cardiomegaly. The further history was unknown. The rhabdomyoma nodules were composed of spider cells containing small amounts of desmin and myosin as well as isolated myofibrils. Microscopically small glioma nodules contained high amounts of GFAP. The second case, a boy 4 months of age, died of a large benign fibrous histiocytoma of the heart after an uneventful history. Tumor cells contained alpha-1-anti-chymotrypsin and lysozyme. The third case, a girl 2 months of age, died unexpectedly of histiocytoid cardiomyopathy. The affected cells contained fat droplets, glycogen granules, many leptomer myofibrils and small amounts of myosin and desmin.

Astrocytoma↗
PubMed1990

Evidence for cytomegalovirus and human immunodeficiency virus infection of the retina in AIDS.

Characteristic ophthalmopathological features of retinal lesions in a patient with the acquired immunodeficiency syndrome (AIDS) are reported. In situ hybridization, immunohistochemistry and electron microscopy revealed severe unilateral cytomegalovirus (CMV) retinitis. The opposite retina which was not involved by CMV showed nonspecific signs of ischaemia in the nerve fiber layer corresponding to cotton-wool spots. Occasional cells of both retinas were positively stained by a mouse monoclonal antibody to the p24 HIV-1 antigen indicating infection of retinal cells by HIV. It is suggested that HIV may directly or indirectly damage retinal tissue and interact with opportunistic pathogens, thus leading to a variety of ocular abnormalities associated with AIDS.

Acquired Immunodeficiency Syndrome↗