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Biomedical subjects

N Streichenberger

Publications and source records attributed to N Streichenberger.

27 records · Page 2Linked to original sources
PubMed1999

Depletion of mitochondrial DNA associated with infantile cholestasis and progressive liver fibrosis.

Few cases of infantile liver disease associated with mitochondrial DNA (mtDNA) depletion have been reported. Most of the patients died before 1 year of age of severe liver failure. We describe a new case, a 28-month-old child, presenting with cholestasis at age 2 months, complicated by progressive portal and lobular liver fibrosis. Growth and psychomotor development are undisturbed. There is no clinical evidence of either myopathy or neurological involvement. Metabolic investigation in plasma revealed an abnormal oxido-reduction status after fasting and after carbohydrate-rich meals. Light microscopy performed on liver biopsies revealed steatosis, abnormal hepatocytes with an "oncocytic" appearance and extensive fibrosis. Electron microscopic investigation showed an increased number of mitochondria with rare or enlarged cristae. Biochemical studies of liver biopsies showed that the respiratory chain activities containing mtDNA-encoded subunits were severely decreased (complexes I, III and IV). In contrast, the complex II activity was normal and the citrate synthase activity was greatly increased. Southern blotting analysis revealed that the ratio of mtDNA to nuclear DNA in liver was only 15% and 20% of the mean control value at ages 12 and 21 months, respectively. For this mtDNA depletion syndrome which is clinically expressed in the liver, a liver transplantation is discussed.

Child, Preschool↗
PubMed1999

Giant cell angiitis of the central nervous system with amyloid angiopathy. A case report and review of the literature.

We report a new case of giant cell angiitis of the central nervous system associated with cerebral amyloid angiopathy (GA/CAA). A 67-year-old woman was hospitalized with a history of headaches and lapses of consciousness. After improvement with corticosteroidtherpay, treatment was stopped. She relapsed and died 33 days after first admission. Pathological examination showed unusual extension of GA/CAA lesions, in the superficial and deep layer of the cerebral cortex, and in the cerebellum. Simultaneous occurrence of GA and CAA is rare. Histopathologic findings and immunological pathogenesis of the process are discussed: 1) arguments over pre-existence of CAA, responsible for GA; 2) primitive inflammatory process inducing amyloid deposits; 3) GA/CAA may represent an association of histological lesions related to 2 different types of disease: i) neurodegenerative disease with specific lesions (such as presence of diffuse senile plaques and neurofibrillary tangles) inducing inflammatory reaction ii) inflammatory disease, with few or no degenerative lesions, responding to immunotherapy.

Aged↗
PubMed1998

Respiratory bronchiolitis in smokers with spontaneous pneumothorax.

Respiratory bronchiolitis (RB) is defined by the accumulation of pigmented macrophages in the lumen and wall of respiratory and membranous bronchioles of smokers. The aim of this study was to determine whether spontaneous pneumothorax was associated with a high prevalence of RB. Seventy-nine consecutive patients who underwent a surgical procedure (thoracotomy or thoracoscopy) for recurrence or persistence of primary spontaneous pneumothorax despite thoracic drainage were studied retrospectively. RB was found in 70 of 79 (88.6%) smokers operated for spontaneous pneumothorax. Associated interstitial pathological abnormalities were present in 53 of 79 cases (67.1%). In nine patients, the pathological lesions were severe and resembled desquamative interstitial pneumonia. Emphysematous lesions were present in about one-third of the patients. Although the possible pathophysiological consequences of respiratory bronchiolitis remain speculative, this study demonstrates the high prevalence of this pathological abnormality in patients with pneumothorax requiring surgical treatment.

Adolescent↗
PubMed1998

[Oto-neuro-surgical approach and accessibility to the cochlear nuclei. Significance in auditory brain stem implant].

The auditory brainstem implant (ABI) is now used to stimulate the cochlear nucleus to obtain auditory perception in patients with type 2 neurofibromatosis. Only electrical stimulation of the cochlear nucleus complex, in the lateral recess of the fourth ventricle, can achieve auditory rehabilitation of these profound bilateral retrocochlear deafness. With 20 standard translabyrinthine approaches, our personal anatomical study propose to describe surgical landmarks of the cochlear nuclear complex and surgical accessibility of the lateral recess of the fourth ventricle. The root of vestibulocochlear nerve, the glossopharyngeal nerve and the choroid plexus of the fourth ventricle might have surgical significance because of their reliability.

Brain Stem↗
PubMed1995

Lymphocytic bronchitis/bronchiolitis in a patient with primary biliary cirrhosis.

A 39 year old woman with severe primary biliary cirrhosis progressively developed exercise dyspnoea due to airflow obstruction. Sjögren's syndrome was not present. Bronchial and pulmonary biopsies demonstrated constrictive lymphocytic bronchitis/bronchiolitis, possibly a component of a generalized autoimmune process in this patient.

Adult↗
PubMedDate not supplied

Polyglucosan bodies and temporal lobe epilepsy: an incidental finding or more?

This study reports on histological findings in the temporal lobe of a 36-year-old woman who underwent a right temporal lobectomy for pharmaco-resistant complex partial seizures. Since surgery, the patient has remained seizure-free. The patient had an established diagnosis of right temporal lobe epilepsy, based on video EEG recordings of seizures, MRI hippocampal atrophy, focal interictal hypometabolism on fluoro-desoxyglucose, hypofixation of Cl1-flumazenil in PET studies, and ictal intracerebral recordings. Biopsies were studied under light- and electron microscopy. Histology showed diffuse distribution of a large number of polyglucosan bodies (PBs) in the whole right temporal lobe white matter. PBs were mostly confined to the perivascular areas and in subpial zones rarely and were observed in the most superficial cortical layers. There was some neuronal loss, especially in opercular zone T , but no other histological lesion was found. Ultrastructurally, PBs were made of filamentous and amorphous material, and were found both in intra-astrocytic processes and in axons. The presence of numerous PBs in the temporal lobe of patients with refractory temporal lobe epilepsy has been reported in 3 patients in the literature. It raises the questions whether this histological abnormality could be related to the epileptogenic process as a cause or as a consequence.

Adult↗