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Biomedical subjects

H Urich

Publications and source records attributed to H Urich.

At least 55 records · Page 3Linked to original sources
PubMed1980

Oculocerebral malformations. A reappraisal of Walker's 'lissencephaly'.

We describe an infant with multiple ocular and cerebral malformations. The eye lesions included microphthalmia, detachment and dysplasia of the retina, hypoplasia of the optic nerve, persistence of the primary vitreous, cataracts, obliteration of the anterior chamber, and vascularization of the cornea. In the brain, the salient features were microencephaly, agyria of an unusual type, cerebellar hypoplasia and dysplasia, persistent corpus pontobulbare, obliteration of the subarachnoid space, and a midline arachnoid cyst in the posterior fossa. We suggest that these lesions were the end result of a fetal infection that operated during a protracted period starting not later than the fourth month of intrauterine life.

Abnormalities, Multiple↗
PubMed1979

Cerebellar malformations: some pathogenetic considerations.

1) Destructive processes are responsible for most cases of cerebellar microgyria of the trabecular pattern. Erosion and subsequent fusion of the folia produce the disorganized pattern in which the various cellular elements retain their noraml relationship and are capable of normal maturation. Intrauterine infection is responsible for most cases; the evidence is conclusive in some cases, presumptive in others. 2) Faulty genetic coding, as illustrated by the trisomies, may lead to formation of heterotopias. The primitive cells aggregating around the dentate nucleus should be interpreted as matrix cells and not as cells of the external granular layer. Cortical heterotopias with attempted internal organisation also occur; their origin is obscure. The unusual, possibly unique, transposition of the internal granular and Purkinje cell layers observed in one case may be ascribed to faulty formation of the Bergmann glia by analogy with the weaver mouse. 3) It is impossible at present to disentangle the role of genetic and environmental factors in the pathogenesis of the hysraphic malformations. It is possible, however, that defective fusion of the intraventricular cerebellar primordium plays a part in the development of the Dandy-Walker malformation, of midine cerebellar clefts in some cases of occipital encephalocele, and of extra-axial ependymal cysts of the posterior fossa.

Brain Diseases↗
PubMed1977

Maturing neuroblastoma and ganglioneuroblastoma: a study of four cases with long survival.

A retrospective study of four tumours of the peripheral nervous system originally diagnosed as neuroblastomas or ganglioneuroblastomas has been made. The patients have all survived since the original diagnosis. Evidence of residual tumour was seen only in one patient, and was associated with evidence of increasing histological differentiation over a period of time. Maturation of primitive cell types is known to alter the prognosis of neuroblastic tumours. It is suggested first, that even minimal evidence of maturation indicates a better prognosis. Second, that evidence of maturation may be uneven, small areas occurring in otherwise totally undifferentiated tumours, or, immature areas remaining even in apparently benign fully mature tumours; if these areas show maturation the prognosis remains good and the tumour should not be confused with a composite ganglioneuroblastoma. Third, that maturing tumours may behave in a malignant fashion, invade and metastasise, but but with maturation of the primary tumour and its metastases the ultimate prognosis may be better than the initial behaviour suggests.

Cell Transformation, Neoplastic↗
PubMed1977

The angioblastic meningioma: a reappraisal of the nosological problem. Light-, electron-microscopic, tissue, and organ culture observations.

The validity of the concept of the angioblastic meningioma, now in dispute, was reexamined by reviewing 79 meningeal and angioblastic tumors of the central nervous system and by comparing the fine structural characteristics and in vitro evolution of 2 typical meningiomas and 1 intracranial hemangiopericytoma. While most tumors show the consistent features of either hemangiopericytoma or hemangioblastoma, there exist transitional forms between these tumors and typical meningioma. There is also a greater degree of morphological overlap at the electron microscopic level than has been recognized up till now. In view of these findings the concept of the angioblastic meningioma deserves to be retained as a generic term to include craniospinal hemangiopericytomas and transitional forms between hemangiopericytoma, hemangioblastoma and classic meningioma. It is postulated that all these tumors share a common origin from polyblastic mesenchymal cells originating in or derived from the meninges.

Brain Neoplasms↗
PubMed1977

Sphincter denervation in anorectal incontinence and rectal prolapse.

Biopsies of the external anal sphincter, puborectalis, and levator ani muscles have been examined in 24 women and one man with long-standing anorectal incontinence, 18 of whom also had rectal prolapse, and in two men with rectal prolapse alone. In 16 of the women anorectal incontinence was of unknown cause, but in eight there was a history of difficult labour. Similar biopsies were examined in six control subjects. In all the incontinent patients there was histological evidence of denervation, which was most prominent in the external anal sphincter muscle biopsies, and least prominent in the levator ani muscles. Myopathic features, which were thought to be secondary, were present in the more abnormal biopsies. There were severe histological abnormalities in small nerves supplying the external anal sphincter muscle in the three cases in which material was available for study. We suggest that idiopathic anorectal incontinence may be the result of denervation of the muscles of the anorectal sling, and of the anal sphincter mechanism. This could result from entrapment or stretch injury of the pudendal or perineal nerves occurring as a consequence of rectal descent induced during repeated defaecation straining, or from injuries to these nerves associated with childbirth.

Adult↗
PubMed1977

Observations on vascular neuropathies.

A series of autopsy studies is presented of 13 cases of obliterative vascular disease of the vasa nervorum in a wide range of conditions, including polyarteritis nodosa, Wegener's granulomatosis, lymphomatoid granulomatosis, polymyositis and rheumatoid arthritis. In most cases, including 1 of rheumatoid neuropathy, the lesions were of the necrotising type. This supports the concept of polyarteritis nodosa as a pattern of reaction common to several diseases rather than that of a specific nosological entity. The remaining 4 cases of rheumatoid neuropathy, were associated with a bland endarteritis known as Bywater's arteritis. A comparison of these autopsy findings with clinical data and observations on sural nerve biopsies leads to the conclusion that all severe and most of the mild cases of rheumatoid neuropathy are due to occlusive vascular disease. Segmental demyelination independent of vascular lesions may be responsible for some of the mild cases and is frequently found in patients without clinical neurological manifestations.

Arthritis, Rheumatoid↗
PubMed1976

Melanotic tumours (Blue Naevi) of spinal nerve roots.

Four cases interpreted as intraspinal blue naevi are reported. The patients were adults females with an age range between 22 and 60 yr. In three there was a single tumour arising from the cervical posterior nerve roots and in the fourth there were multiple tumours arising from the posterior nerve roots of the spinal cord and occurring within the cerebello--pontine angle. The histological appearances of the tumours were similar in every way to those of dermal blue naevi. One was of the more common spindle-celled type and three of the cellular variant. The tumours contained melanin-pigment, and spindle cells with dendritic bipolar processes of the type described in dermal blue naevi. One was of the more common spindle-celled type and three of the cellular variant. The tumours contained melanin pigment, and spindle cells with dendritic bipolar processes of the type described in dermal blue naevi. Definite evidence of malignant tranformation was found in two cases and in a third, the appearances were suggestive for early malignant change. Therefore, unlike their dermal equivalents, intraspinal blue naevi appear to have a greater propensity for malignant transformation. In each case a careful clinical examination failed to reveal any evidence of a primary malignant melanoma. In the one case who died and on whom necropsy was performed, the failure to identify a primary cutaneous, mucosal or ocular melanoma substantiated our contention that these tumours were primary.

Adult↗
PubMed1976

Progressive encephalomyelitis with rigidity.

Two cases of encephalomyelitis are described in which the major clinical manifestation was muscular rigidity and stimulus-sensitive muscular spasms. It is suggested, from pathological evidence, that this rigidity was of spinal origin, and that this disorder is a rare but recognizable entity. Comparison is made with previously reported cases of rigidity of spinal origin, including encephalitis lethargica, and with 'subacute myoclonic spinal neuronitis' and the 'stiff man syndrome.'

Adult↗
PubMed1976

The optic pathway in neurosarcoidosis.

A case is reported of a patient, age 40 yr, with progressive hypothalamic and visual failure. Autopsy revealed widespread multisystemic sarcoidosis with involvement of the central nervous system. The intracranial portion of the optic pathway was diffusely infiltrated by sarcoid granulomas. Comparison of this case with previously reported autopsy and biopsy findings leads to the following conclusions: 1) The intraocular, intraorbital, and intracranial parts of the optic nerves may be involved independently; 2) lesions of the nerve head and of the retrobulbar part of the nerve may be unilateral; and 3) involvement of the intracranial portion is usually bilateral and forms part of more widespread sarcoidosis of the central nervous system.

Adult↗
PubMed1976

Trigeminal neuropathy with nasal ulceration: report of two cases and one necropsy.

Two cases are reported of progressive trigeminal neutopathy with nasal ulceration. One patient developed signs of spinal cord involvement 15 years after the onset of trigeminal symptoms and died after a total course of 21 years. Necropsy revealed an unusual trigeminospinal system degeneration with deposition of amyloid-like substances in the affected structures. The other patient is alive eight years after the onset of symptoms, the only indication of a lesion outside the trigeminal nerve being a patch of numbness in one leg.

Adult↗
PubMed1975

The striatonigral degenerations. Putaminal pigments and nosology.

Three new cases of striato-nigral degeneration (SND) are presented with particular reference to the identification of putaminal pigments. These were studied by histochemical methods, electron microscopy and elemental analysis. Three interrelated parenchymal perikaryal pigments were identified in the putaminal lesions of all 3 patients: a "haematin" pigment, neuromelanin and lipofuscin. The presence of neuromelanin in the putamen may be due to accumulation of dopamine in the synaptic terminals of the nigro-striatal pathway and its polymerization into pigment. This suggests that the putaminal atrophy is the primary lesion in SND which is a true supranigral form of parkinsonism. Clinically this manifests itself in predominance of rigidity over other parkinsonian symptoms and in a poor, or absent, response to treatment with L-dopa and anticholinergic drugs.

Corpus Striatum↗
PubMed1975

Infantile polyneuropathy with defective myelination: an autopsy study.

A case is reported of a boy who developed a severe polyneuropathy in early infancy and died of respiratory failure at the age of 18 months. Autopsy revealed almost total lack of myelin sheaths in the cranial, spinal and peripheral nerves. The defect involved the entire peripheral nervous system and was confined to it, central myelination being normal. It is suggested that this case is another example of the condition described by Lyon (1969) and by Kennedy et al. (1971) in which pathological observations were confined to biopsy material. In spite of some similarities between these cases and those of hypertrophic neuropathy reported by Déjerine and Sottas in 1893, they seem to form a distinct sub-group, possibly even a separate entity: infantile polyneuropathy with defective myelination.

Autopsy↗
PubMed1974

Concurrence of multiple sclerosis and glioma.

Three cases are reported of concurrence of malignant gliomas (astrocytomas and glioblastomas) with multiple sclerosis (MS). This situation may lead to considerable diagnostic difficulties, yet an accurate appraisal is of prognostic, if not therapeutic, value. There is suggestive but inconclusive evidence that the two conditions may be causally related, malignant astrocytic tumours arising from plaques of MS. The small number of reported cases makes statistical analysis impossible, but the scanty literature may not reflect the true incidence.

Adult↗