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Biomedical subjects

J Ulrich

Publications and source records attributed to J Ulrich.

At least 145 records · Page 8Linked to original sources
PubMed1981

Experimental allergic encephalomyelitis. Exsudate and cellular infiltrates in the spinal cord of Lewis rats.

Classical acute allergic encephalomyelitis (EAE) was provoked in Lewis rats with bovine spinal cord (BWM) in complete Freund's adjuvant (CFA). An efficient immunohistologic technique (peroxidase-antiperoxidase (PAP) was used to trace exsudates of fibrinogen and immunoglobulin as well as their coexistence with cellular infiltrates and clinical signs. Exsudation was restricted to the vessels exhibiting cellular infiltrates. The findings do not lend support to the assumption that exsudation of circulating factors is the initial local event in EAE. It also remains open, whether the exsudation of fibrinogen and gamma globulin are responsible for the clinical symptoms.

Animals↗
PubMed1981

Connatal polyneuropathy -- a case with proliferated microfilaments in Schwann cells.

A case of connatal polyneuropathy is described in a boy who died of pneumonia at the age of 2 years, and from whom sural nerve biopsies had been taken when he was 4 and 16 months old. Clinically, his disease was characterized by motor weakness and muscular flaccidity in the presence of normal intellectual development. The evolution of the connatal peripheral nerve lesion could be followed from the age of 4 months to death: The first biopsy evidenced the most serious pathologic changes. The findings were reminiscent of those encountered in a fetal nerve at 18 weeks of gestation. Furthermore, it showed numerous filamentous inclusions in Schwann cells. The second biopsy showed a sparsely myelinated nerve with bands of basement membrane apparently unrelated to cells arranged around the nerve's fibers. A few Schwann cells containing filamentous inclusions were still present. At autopsy, the findings were identical to those of the second biopsy. The possibility that this patient was transitionally exposed to a neurotoxic agent during pregnancy and that the biopsy findings represent a lesion that is still florid in the first and in a residual state in the second biopsy is considered.

Biopsy↗
PubMed1981

Sensory ganglioneuropathy in infantile spinal muscular atrophy. Light and electronmicroscopic findings in two cases.

Light and electronmicroscopic findings in two cases. Neuropediatrics 12: 215-31 (1981). Two cases of infantile spinal muscular atrophy (Werdnig-Hoffmann disease) are described in unrelated children deceased at 11 months (acute clinical onset at 6 months) and 2 years (onset at birth). Severe respiratory difficulties, hypotonia, muscular weakness and depressed tendon reflexes were the main clinical features. Bulbar palsy, bilateral ptosis, pale optic discs and atactic movements of the hands were observed in the child deceased at 11 months. Besides severe loss of anterior horn cells and neurogenic muscle atrophy there was evidence of an extensive sensory involvement in both cases. Shrinkage, vacuolation as well as chromatolytic changes of dorsal root ganglion cells, together with the evidence of a primary axonal damage in sural nerve biopsies were interpreted in terms of ganglioneuropathy of the primary sensory neurons. An invasion of fibrous astrocytes into dorsal roots constituted another striking anomaly in one case as well as a pronounced degeneration of cranial nerves V and VIII in the other case, a finding not hitherto reported in Werdnig-Hoffmann disease.

Brain↗
PubMed1980

Histochemical analysis of senile plaque amyloid and amyloid angiopathy.

Histochemical methods were used to obtain information on the chemical constituents of brain amyloid in senile dementia of the Alzheimer type. The staining properties of brain amyloid (senile plaque and amyloid angiopathy) were compared with those of extraneural amyloidosis and endocrine amyloid. We found no histochemical differences between amyloid in senile plaques and in amyloid angiopathy. The content of aromatic amino acids was higher in amyloid of plaques and in amyloid angiopathy than in endocrine amyloid. Furthermore, we found persistent birefringence and affinity of brain amyloid for Congo red after exposure to potassium permanganate, suggesting that AA amyloid is not a major constituent of cerebral amyloid.

APUD Cells↗
PubMed1980

Adrenomyeloneuropathy. A protracted, pseudosystematic variant of adrenoleukodystrophy.

Histopathological, immunocytochemical, and electron microscopical investigations were carried out in a man with a protracted history of spastic paraparesis, adrenal insufficiency and hypogonadism. Pathological findings were identical with those of the few previously reported cases of adrenomyeloneuropathy (AMN) including cytoplasmic lamellar inclusions consisting of two parallel 2.5 nm leaflets separated by a clear space of variable extent, in the brain, spinal roots, adrenal gland, and interstitial cells of the testis. No inclusions could be found in oligodendrocytes. In brain macrophages they are thought to represent breakdown products of pathological myelin stored in lysosomes, whereas in other localizations they might be an expression of the primary metabolic defect of the cell. Special attention was paid to the pseudosystematic type of fiber tract degeneration in the spinal cord. The dying-back pattern of axonal destruction was interpreted as a possible result of the multisegmental demyelination observed in these tracts. All known hormones could be localized in the pituitary by immunocytochemistry. Corticotrophs and gonadotrophs were numerous. The structural damage of the adrenal cortex and the interstitial cells of the testis is, therefore, considered to result from the inborn error of metabolism on the one hand and from an enhanced stimulation exerted by ACTH and gonadotrophins on the other.

Adrenal Glands↗
PubMed1980

Morphological basis of Adie's syndrome.

A female patient with Adie's syndrome died from unrelated disease at the age of 62. Both ciliary ganglia were seen to be severely depleted of nerve cells and still ongoing degeneration was seen in lumbar spinal ganglia, as well as in the posterior funiculi. This confirms earlier reports that primary pathological changes in Adie's syndrome are localized in the ciliary and spinal ganglia. Furthermore, it indicates that the underlying disease is still progressive after years.

Adie Syndrome↗
PubMed1979

Treatment of fulminant hepatic failure with infusions of Co-factors and mannitol and charcoal-hemoperfusions during Forty-one days.

The clinical course of a 26 year old female patient with acute liver necrosis and coma due to hepatitis B is reported. The disturbances of conciousness had improved. The patient survived 41 days after the beginning of the coma and developed liver cell regeneration and an acute post-hepatitic liver cirrhosis. As a grave complication a septicemia with aspergillus was observed. The patient died because of gastro-intestinal hemorrhage. At autopsy there were no signs of brain edema. The treatment consisted in: daily infusions with coenzyme A, nicotinamid-adenin-dinucleotide, alpha lipoic acid and cocarboxylase to improve the metabolic disorders and the clinical picture; mannitol intravenously to prevent and to treat cerebral edema; 33 charcoal-hemoperfusions to remove toxic substances of acute liver failure. Treatment of the aspergillus infection with 5-fluorocytosine and amphotericine B and infusion of concentrated ascites led to a decompensation of liver functions. From this observation the following conclusions can be drawn: after an acute viral hepatic necrosis, new synthetic functions and improvements of the disturbed intermediary metabolism in regenerated liver-cells can eventually be seen only after twenty-four to thirty days. With systematically applicated mannitol infusions it is possible to treat cerebral edema effectively.

Adult↗
PubMed1979

The value of nerve biopsies.

A short review on the usefullness of nerve biopsies is given. After a short description of the features of axonal degeneration and demyelination the possibility of diagnosing neurolipidoses from nerve biopsies is stressed.

Axons↗
PubMed1979

Cerebellar ganglioglioma in a child.

A cerebellar ganglioglioma was surgically removed from a two-year old boy, who had developed manifestations of increased intracranial pressure and cerebellar symptoms. At surgery, the tumor presented as a firm nodular mass displacing the cerebellar cortex. By light microscopy, its architecture differed distinctly from that of hamartomatous diffuse hypertrophy of the cerebellar cortex (Lhermitte-Duclos' disease). Mature ganglion cells were grouped in clusters and linked by thick bundles of nerve cell processes. Nerve cells and processes were enmeshed in a rich network of fibrillary connective tissue. Electron microscopy disclosed typical neuronal perikarya as well as numerous asymmetric chemical synapses. The bulk of the tumor consisted of tightly grouped, (non-myelinated) nerve cell processes arranged in parallel. One of the most prominent features of the tumor consisted of numerous dilatations of these processes. The largest ones contained microfilaments, while the smaller ones were entirely filled with dense bodies (most probably derived from degenerating mitochondria). Only scattered dense core vesicles were seen, which probably did not represent neurosecretory granules. A second cell type consisted probably of astrocytes. Most neuroepithelial cell processes could not be identified with certainty as being of either neuronal or glial origin. A third cell type consisted of numerous slender cells which were probably mesenchymal. They were surrounded by a network of basement membrane which extended between the surrounding nerve cell processes.

Cerebellar Neoplasms↗
PubMed1979

Histological and ultrastructural changes in idiopathic facial palsy.

Light- and electron-microscopic findings in the facial nerve are reported in a patient who died 8 months after the onset of acute idiopathic facial palsy. There were signs of regeneration after axonal destruction, and inflammatory infiltrates confined to the intracanalicular part of the nerve were found. These findings suggest a viral or immunological etiology, which may become pathogenetic due to the length and narrowness of the Fallopian canal.

Facial Nerve↗
PubMed1978

Adrenoleukodystrophy. Preliminary report of a connatal case. Light- and electron microscopical, immunohistochemical and biochemical findings.

This is the first description of a connatal case of adrenoleukodystrophy. The clinical picture consisted of severe psychomotor retardation, convulsions and hypsarrhythmia, but no obvious signs of adrenal insufficiency. Pathologically, the adrenals were small. The entire cortex was largely replaced by large round cells. Ultrastructurally, some cells in the adrenal cortex contained inclusions with electron-lucent clefts surrounded by a membrane. The anterior pituitary lobe could be demonstrated to have produced ACTH. The central nervous system showed extensive zones of demyelination in the brainstem, the cerebellum and the right-sided capsula interna. In the demyelinated areas there was sudanophilic breakdown and an intense gliosis. Ongoing demyelination could also be demonstrated by the chemical analysis. In the gray matter there waere micropolygyria of the insular cortex and swollen nerve cells in the nucleus arcuatus. Ultrastructure revealed the type of inclusions in the microglia of the same type as in the adrenals, and a different type of inclusions in unidentifiable cells, possibly neurons. These latter inclusions consisted of loosely stacked lamellar material. The findings are interpreted as further evidence of storage taking place in this disease.

Adrenal Cortex↗
PubMed1978

Multiple sclerosis: demyelination and myelination inhibition of organotypic tissue cultures of the spinal cord by sera of patients with multiple sclerosis and other neurological diseases.

Sera from 44 patients with Multiple Sclerosis, of three patients with neurological syndromes compatible with Multiple Sclerosis, of 34 patients suffering from other neurological diseases and of 25 pregnant healthy young women were tested for their demyelinating activity in myelinated tissue cultures. In order to leave the investigators unprejudiced, all sera were coded and intermixed with controls of rabbit EAE serum which had a potent demyelinating capacity. Demyelination was graded (from 0--4), heat lability at 56 degrees C (complement dependency?) was also tested with each serum. Only demyelination of a degree of 2 and more, which was abolished by heating to 56 degrees C, was counted as positive. Six of the 44 sera from MS patients (13.6%), 19 of 37 sera from neurological patients and none of the healthy young women demyelinated. Thus, serum demyelination of tissue cultures seems to be a nonspecific indicator of chronic disease of the nervous system and is of considerable general neurological interest, but does not indicate a demyelinating disease. Myelination inhibition was not observed with any of the human sera tested for it.

Animals↗