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Biomedical subjects

H J Manz

Publications and source records attributed to H J Manz.

At least 55 records · Page 3Linked to original sources
PubMed1984

Neuropathologic features, including pontine schwannosis, in a four-year survivor of probable Reye's syndrome with secondary Möbius syndrome.

Frequent seizures and loss of motor, language, and intellectual skills necessitated care in an institution for the physically and mentally handicapped for 4 years after diagnosis of presumed Reye's syndrome in a 10-month-old boy. M obius syndrome was diagnosed and bilateral tarsorrhaphy performed for exposure keratitis. Postmortem examination revealed multiple old boundary zone infarcts in the cerebral cortex, small cavitated infarcts in basal ganglia and thalami, diffuse neuronal loss and gliosis, and a focus of old necrosis in the pons; brain stem herniation during the acute phase of Reye's syndrome was responsible for the latter lesion. Irregular congeries of proliferated peripheral nerves, confirmed by ultrastructural study, were ramifying in the gliotic pontine tegmentum. The pathologic substrate for the neurovegetative state in a long-term survivor of probable Reye's syndrome appears to be multifocal cerebral infarction.

Atrophy↗
PubMed1984

Childhood medulloblastoma presenting with hemorrhage.

Cerebellar hemorrhage is rare in childhood, and is usually the result of trauma or a ruptured arteriovenous malformation. We report acute subarachnoid hemorrhage as the presenting manifestation of medulloblastoma and review the atypical presentation and causes of cerebellar hemorrhage in infants and children.

Cerebellar Neoplasms↗
PubMed1983

Dissecting aneurysm of intracranial vertebral artery: case report and review of literature.

A spontaneous dissecting aneurysm of the intracranial portion of the dominant right vertebral artery presented as massive subarachnoid hemorrhage, excruciating headache, and respiratory arrest in a 57-year-old white man with a history of systemic hypertension. He died on the 3rd day. Postmortem examination revealed a dissecting hemorrhage extending for 2.1 cm along the artery; rupture of the intima, media, and adventitia could be demonstrated. The intramural accumulation of blood in the proximal segments appeared to be related to retrograde dissection within a media weakened by cystic degeneration. Accumulation of pools of mucoid ground substance was also demonstrated in other intracranial and extracranial arteries. Hemodynamic stresses due to arterial hypertension and physical exertion may have played a contributory role in the etiopathogenesis of this uncommon form of cerebrovascular accident. A comprehensive literature review permits a comparison of supratentorial and infratentorial dissecting aneurysms; vertebral and basilar artery dissections are presented in tabular form.

Age Factors↗
PubMed1983

Pathobiology of neurosarcoidosis and clinicopathologic correlation.

In a retrospective study of 4,294 consecutive postmortem cases, there were 15 with histologically proved sarcoidosis, including 4 with CNS involvement. Whereas in previously reported autopsy series the prevalence of neurosarcoidosis was 15%, it is 27% in this review. Five cases with incidental, clinically inapparent sarcoidosis died at a mean age of 54.6 years; those six with systemic sarcoidosis, not involving the CNS, died at a mean age of 47.0 years; while those four with a variety of neurologic manifestations in addition to systemic sarcoidosis died at a mean age of 38.2 years. Thus, it appears that at one end of the spectrum of morbidity, sarcoidosis may cause no or only trivial symptoms and permit prolonged survival. At the other extreme, sarcoidosis may affect a younger population more severely. CNS involvement occurs relatively early in the course and its rather rapidly progressive, accounting for the poor prognosis, despite appropriate medical and neurosurgical management. In its turn, neurosarcoidosis may present with a variety of signs and symptoms, depending on the site of involvement along the craniospinal axis. This feature is illustrated by the manifestations of compression myelopathy, hydrocephalus with dementia, hydrocephalus with seizures and ataxia, and anosmia, blindness, seizures, and diabetes insipidus. In addition, one patient developed a Nocardia brain abscess as a complication of the altered immune system in sarcoidosis.

Adult↗
PubMed1983

Cervical myelopathy due to atlantoaxial and subaxial subluxation in rheumatoid arthritis.

Four patients aged 41 to 73 years, who had had rheumatoid arthritis for eight to 25 years, had signs and symptoms of cervical myelopathy and radiculopathy due to either atlantoaxial dislocation with herniation of the odontoid through the foramen magnum, or subluxation of the middle to lower cervical vertebrae. Spastic paraparesis or quadriparesis, severe nuchal immobility and pain, and flaccid paresis of the upper limbs necessitated anterior medullary decompression and posterior cervical fusion. Postmortem examination disclosed old ischemic necrosis, atrophy, and gliosis in the low medulla and cervical cord. Anterior and posterior gray horns and contiguous posterior and lateral funiculi bore the brunt of the damage. Ascending and descending wallerian degeneration and atrophy of the cervical nerve root were evident. In three cases, anterior spinal or radicular arteries demonstrated intimal fibrosis with moderate stenosis; two cases depicted chronic phlebitis or subarachnoid vessels. Previous reports have infrequently provided evidence of a vasculopathy.

Adult↗
PubMed1982

Neuropathology, pathogenesis, and neuropsychiatric sequelae of Reye syndrome.

A clinico-pathologic correlation can be established for those survivors of Reye syndrome in the higher clinical stages who have sustained irreversible and often major neuropsychiatric deficits. The neuropathologic substrate for the clinical manifestations of mental retardation, recurrent seizures, movement disorders, sensori-motor deficits, psychobiologic maladjustment, and mediocre performance in school and on formal psychometric tests consists of multifocal infarction and astrocytosis in cerebral cortex, diencephalon, basal ganglia, and brainstem. While the mortality rate of Reye syndrome in acute stages is still significantly high (25% in our 20 cases, 52% in the tabulated cases in the higher clinical grades), the occurrence of permanent psychomotor deficits is an equally tragic socioeconomic and medical problem. Of our 15 survivors, 3 have suffered major and 1 has sustained minor brain damage. From a review of the literature, over one-third of survivors are consigned to cerebral malfunction, hence their potential for full enjoyment of life and their intellectual, emotional, and economic contributions to their families and society are sadly curtailed.

Adolescent↗
PubMed1981

Pathologic fracture of odontoid process secondary to amyloid deposition.

Immunocyte dyscrasias have increasingly been reported in patients with gammopathy-associated systemic amyloidosis. In "primary" amyloidosis, deposits occur in heart, smooth and skeletal muscle, bones and joints, skin, lungs and peripheral nerves. The formation of amyloid fibrils appears related to the lambda light chains in these monoclonal gammopathies. A 72-year-old white man is reported who developed massive deposits of amyloid in joints and bones, as well as other tissues. Twenty percent of his bone marrow population was comprised of plasma cells in various maturational stages, without forming solitary masses diagnostic of myeloma. Lambda light chains were the sole contributors to a selective increase in IgD, with a concomitant reduction of IgA, IgG, and IgM. The patient sustained a pathologic fracture through a deposit of amyloid in the odontoid. While he suffered severe posterior nuchal and occipital pain, no clinical features of compression myelopathy were evident. Postmortem examination revealed a minor degree of posterior column degeneration and an axonal reaction of anterior horn cells of the C-1 spinal cord segment. This case represents the second to be reported with amyloidosis of the vertebral column, pathologic fracture, and a spinal cord lesion.

Aged↗
PubMed1981

[Pathology of cerebral edema. I. Concept, etiopathogenesis and classification].

Taking into account its complex pathogenesis, we stress the inadequacy of considering brain edema as a single clinical or pathological entity. Vasogenic, cytotoxic and hydrocephalic varieties are considered. Some clinical conditions underlied by the common morphologic feature of "status spongiosus" are discussed. The methods of investigation of brain edema are presented as well as problems concerning its diffusion and resolution.

Blood-Brain Barrier↗
PubMed1981

[Pathology of cerebral edema. II. Experimental models and modifying agents].

Current experimental models of brain edema are described and evaluated for their contribution to the knowledge of basic processes involved in its production as well their contribution to the understanding of different clinical forms. The participation of each main pathogenic mechanism in a given experimental model is analyzed and proves to vary with each particular model and site studied. The importance of various experimental models in the evaluation of different therapeutic procedures directed to control the genesis and evolution of brain edema is stressed.

Animals↗
PubMed1981

Multifocal primary ocular and central nervous system malignant lymphoma.

A patient had bilateral posterior uveitis of two weeks' duration and rapid neurologic deterioration. Obstructive hydrocephalus developed as a result of compression of the fourth ventricle by an enlarged cerebellar vermis. Autopsy revealed multiple foci of infiltration by malignant lymphomas in the eyes, brain, and cerebellum; no evidence of systemic lymphoma was found.

Brain Neoplasms↗
PubMed1980

iron-overload-associated myopathy in patients on maintenance haemodialysis: a histocompatibility-linked disorder.

Unexplained proximal muscle weakness developed in 10 patients on chronic maintenance haemodialysis. Proximal muscle biopsy in 7 patients disclosed iron deposition in muscle fibres and/or macrophages. All 10 patients had severe iron overload (serum-ferritin level > 1000 ng/ml). Since HLA-A3, B7, and B14 antigens are associated with idiopathic haemochromatosis and, therefore, are linked with alleles regulating iron metabolism, HLA type was reviewed in 61 haemodialysis patients, including the 10 myopathic patients. Serum-ferritin levels showed a significant correlation (p<0.001) with the presence of the HLA-antigens. Close relatives of these patients with HLA-antigen-associated iron-overload myopathy did not have raised serum-ferritin levels. Patients on maintenance haemodialysis who have inherited the "haemochromatosis alleles" thus have an increased risk of iron overload and muscle iron deposition.

Adult↗
PubMed1980

Pathology of skeletal muscle: principles of reaction patterns and histochemistry and experience with 195 biopsies.

Technique and interpretation of muscle biopsies require the coordinated team work between referring clinician, surgeon, and pathologist so that optimal management of a patient with neuromuscular disease may be attained. Investigation of muscle diseases has advanced so much with an interdisciplinary approach that not only can accurate diagnosis be offered for the benefit of the patient in certain instances, but also genetic counselling provided and pre-natal diagnosis established. The principal reaction patterns and pathogenetic mechanisms of skeletal muscle as a contractile and metabolically active tissue are described; the diagnostic usefulness of enzyme histochemistry and the basic principles of the motor unit are discussed and illustrated. For the practicing pathologist, adequate tissue preparation of a muscle biopsy specimen requires interest and a willingness to dedicate time, effort and funds. While the paraffin-embedded section is still very valuable, enzyme histochemistry provides certain highly diagnostic information not otherwise obtainable. Likewise, there must be an interest in electron microscopy and appreciation for its value in depicting ultrastructural abnormalities when certain reaction patterns are apparent on light microscopic sections, enzyme histochemical stains, or Epon-embedded "thick" sections. Finally, concurrent sural nerve biopsy also requires optimal processing and interpretation.

Biopsy↗
PubMed1980

New phenotypic variant of adrenoleukodystrophy. Pathologic, ultrastructural, and biochemical study in two brothers.

Adrenoleukodystrophy is not usually considered in the differential diagnosis of the infantile onset of failure to thrive with motor and intellectual retardation. Rather, symptoms have started in childhood and have progressed over some years; not all patients have had overt adrenocortical insufficiency. The two brothers reported here developed symptoms in the neonatal period. In each the nature of the primary cerebral disorder was not recognized, because other etiologic factors clouded the diagnostic studies. In the younger brother, Case 1, a high titer (1:256) for cytomegalovirus (CMV) led to the suspicion that CMV infection accounted for the neurologic and ophthalmologic findings. Progressive neurologic deterioration at the age of 6 years prompted brain biopsy to confirm the diagnosis of progressive CMV encephalitis. In the older brother, Case 2, hemogenic hydrocephalus due to traumatic birth injury was held responsible for the psychomotor retardation and cerebral palsy. At necropsy, the adrenal glands in both cases were severly atrophic. In Case 1, a markedly inflammatory leukodystrophic process affected chiefly the frontal centra semiovalia and internal capsules, with relative sparing of parieto-occipital white matter and subcortical U-fibers. Heavy lymphocyte and monocyte cuffs surrounded many blood vessels in the white matter, and oil-red-O and PAS-positive macrophages were scattered in the zones of myelin disintegration and loss. Focally, the leukodystrophic process was so intense that cavitation necrosis was present, especially in the internal capsules. Further, PAS-positive, striated macrophages were aggregated in large clusters in liver, spleen, and lymph nodes. At the ultrastructural level, linear and gently arced, parallel, coapted or widely separated leaflets measuring 3-4 nm in width were identified in macrophages of the brain biopsy and in autopsy liver and lymph node. Biochemical analysis of fresh, frozen autopsy brain demonstrated cholesterol esters with long-chain fatty acids by thin-layer and gas-liquid chromatography. In Case 2, the leukodystrophic process could be readily identified in the brainstem and cerebellum but was masked in the cerebral hemispheres by the extensive hydrocephalus. The adrenal glands were atrophic and at light microscopy revealed adenomatoid nodules, many ballooned coritcal cells and very rare cells with striated cytoplasm. Masses of PAS-positive macrophages were encountered in liver and lymph nodes. In both cases, only old Wallerian degeneration of the corticospinal tracts was found in the spinal cord.

Adrenal Cortex↗
PubMed1980

Malignant melanoma with melanosis. Ultrastructural and histological studies.

Ultrastructural and histological investigations were performed on a case of generalized melanosis associated with superficial spreading melanoma. The hyperpigmentation of the general body surface, mucous membranes and nail beds was associated with deposition of melanin in macrophages in the dermis, together with some hyperactivity of epidermal melanocytes. Melanin granules were observed lying free in the stroma, suggesting pigment incontinence and phagocytosis by macrophages. Giant melanosomes were noted in melanocytes, keratinocytes and melanophages in the hyperpigmented skin. No evidence was found to suggest dissemination of individual malignant cells throughout the skin. Subcutaneous nodules of malignant melanoma were, however, present, as well as metastases to the iris, liver and to other organs.

Humans↗
PubMed1980

A new CT pattern in adrenoleukodystrophy.

A new CT pattern was observed in 2 patients with adrenoleukodystrophy (ALD). This pattern, which the authors call Type II, is characterized by the absence of posterior periventricular areas of decreased attenuation around the trigone on non-contrast scans: after contrast infusion, however, there is striking enhancement of various white-matter structures (tracts or fiber systems) such as the internal capsules, corpus callosum, corona radiata, forceps major, and cerebral peduncles. This is different from numerous previous descriptions of the CT pattern in ALD. Type II ALD does not appear to have been seen in any other leukoencephalopathy and is probably specific for a phenotypic variant or an evolving stage of ALD.

Child↗