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Biomedical subjects

J W Prineas

Publications and source records attributed to J W Prineas.

13 recordsLinked to original sources
PubMed1979

Multiple sclerosis: presence of lymphatic capillaries and lymphoid tissue in the brain and spinal cord.

Thin-walled channels resembling lymphatic capillaries and containing lymphocytes were observed in perivascular spaces throughout the central nervous system of patients with various neurological disorders. This suggests that immunological surveillance in the central nervous system may normally involve lymphocyte circulation through the perivascular compartment. In some old multiple sclerosis plaques, perivascular lymphoid tissue was found which was organized in a manner similar to the antibody-producing medullary region of lymph nodes. This may indicate continuous processing of the putative multiple sclerosis antigen in such lesions.

Brain↗
PubMed1979

Remyelination in multiple sclerosis.

Chronic plaques in central nervous system tissue fixed by in situ perfusion for electron microscopy were examined for evidence of remyelination in 2 patients with multiple sclerosis (MS). Fibers with abnormal central myelin sheaths of several types were found at the margins of most of the plaques studied. The most common of these were: (1) the presence of bare stretches of axon between contiguous internodes, (2) the presence of thin paranodes, (3) internodes which changed markedly in thickness along their length due to premature termination of superficial or deep myelin lamellae that ended as hypertrophic lateral loops, and (4) abnormally thin internodes which were of uniform thickness along their length, which were shorter than normal, and which terminated in the form of normal nodal complexes. The finding of internodes of the last type at the edges of many plaques indicates that remyelination by oligodendrocytes can occur in the adult human CNS and that it is common in some cases of MS, although limited in its extent.

Central Nervous System↗
PubMed1978

Hypertrophic Charcot-Marie-Tooth disease. Light and electron microscope studies of the sural nerve.

Sural nerve biopsies from 15 patients with hypertrophic Charcot-Marie-Tooth disease have been studied by light and electron microscopy. There is a considerable variation in size of onion bulbs in patients from different kinships but the appearances were similar in patients from the same kinship. Comparison of sural nerve biopsies from patients within the same kinship suggests that with increasing age there is a progressive reduction in myelinated fibre density, an increased number of fibres undergoing demyelination and an increased frequency of onion bulb formations. Motor conduction velocities were reduced in all patients and were inversely proportional to the number of onion bulb lamellae, and to the proportion of demyelinated fibres found on sural nerve biopsy. Abnormalities of unmyelinated fibres were present in all the nerves studied. There was a relative increase in the density of denervated Schwann cell subunits and collagen pockets. The findings suggest that unmyelinated fibres undergo degeneration in the disease and lend some support to the hypothesis that the primary abnormality may be neuronal.

Adolescent↗
PubMed1978

Macrophages, lymphocytes, and plasma cells in the perivascular compartment in chronic multiple sclerosis.

Perivascular cells in CNS tissue from six multiple sclerosis (MS) patients and a patient with motor neuron disease were examined by light and electron microscopy. Lymph node tissue from one MS patient was also examined. CNS perivascular macrophages in both MA and motor neuron disease were found to closely resemble free macrophages elsewhere in the body except that they often contained unusually large primary lysosomes. Cytoplasmic inclusions consisting of membrane-bound stacks of curved linear profiles, presumed to be a product of myelin degradation, were constantly observed in microglia in MS plaques but were rarely observed in perivascular macrophages in the same area. Unidentified cylindrical bodies were observed within cysternae of rough endoplasmic reticulum in some lymph node cells. Quantitative studies of the perivascular cell population in one MS case revealed, in histologically normal white matter 260 lymphocytes and 178 plasma cells per cubic millimeter of fresh tissue. Typical chronic plaque tissue without obvious inflammatory cell cuffing contained 1772 plasma cells per cubic millimeter of fresh tissue. No plasma cells were observed in the CNS in motor neuron disease. The results of this study suggest that perivascular macrophages in the CNS represent a specialized population of monocyte-derived free macrophages, that these cells differ functionally from microglial cells, and that the digestion of myelin breakdown products in MS requires the participation of both cell types. The results also suggest that in some chronic MS cases there is a large, permanent population of CNS plasma cells that persists, like the elevated cerebrospinal fluid IgG level in this disease, for the life of the patient, that these cells, rather than inflammatory cells in fresh lesions, are the major source of this raised IgG, and that the existence of such a population of cells may indicate the continuing expression of antigens in chronic MS lesions in the absence of fresh lesion formation.

Adult↗
PubMed1977

Immunocytochemical studies for the localisation of measles antigens in multiple sclerosis plaques and measles virus-infected CNS tissue.

Actively demyelinating central nervous system (CNS) lesions from a patient with acute multiple sclerosis (MS) were tested for measles antigens using peroxidase-conjugated antimeasles antibody. No evidence of measles antigens was found. Similarly reacted tissue from 2 patients with chronic MS also revealed no evidence of measles antigens. Identically treated and simultaneously tested measles-infected CNS cultures and human SSPE brain tissue stained strongly for measles antigens. The possible reasons underlying the failure to detect measles antigens in MS are discussed.

Adult↗
PubMed1977

Adrenomyeloneuropathy: a probable variant of adrenoleukodystrophy. II. General pathologic, neuropathologic, and biochemical aspects.

Histopathologic features were studied in postmortem examination of two men with adrenomyeloneuropathy, and biochemical studies were performed on one of these individuals. The histopathologic picture of one case included dying-back features in the nervous system and lamellar cytoplasmic inclusions in the brain, adrenal gland, and testis similar to those in adrenoleukodystrophy. Biochemical studies of the cerebral white matter of this individual revealed increased amounts of long-chain saturated fatty acids in cholesterol esters, an abnormality characteristic of adrenoleukodystrophy. Despite differences in clinical presentation and neuropathology, adrenomyeloneuropathy probably represents a distinct variant of adrenoleukodystrophy.

Addison Disease↗
PubMed1976

Acute idiopathic polyneuritis. A clinical and electrophsiological follow-up study.

Fifty patients with acute idiopathic polyneuritis have been studied clinically and electromyographically, and sural nerve biopsy was performed on 8 patients. Motor and sensory conduction studies were within the normal range in 7 patients (14%), and there was pronounced slowing of motor conduction in 25 patients (50%). There was no apparent correlation between the degree of conduction, and the clinical disability of the patient or the duration of the acute illness. Eighteen patients were re-examined at intervals up to 5 1/2 years after the onset of their illness. Eight patients (44%) were clinically normal at follow-up examination and 4 patients (22%) had a significant disability. There was no relationship between the clinical disability at follow-up examination and the results of initial or final nerve conduction studies. Electromyographic evidence of denervation, however, may indicate that complete clinical recovery will not occur. Segmental demyelination was the primary pathological change found in sural nerve biopsies and there was a significant reduction in the density of myelinated fibres in 2 nerves. It is suggested that a subacute onset of the illness,electromyographic evidence of denervation or gross slowing of conduction, and significant reduction of numbers of myelinated fibres or onion-bulb formation on sural nerve biopsy are factors which may indicate a prolonged course of the illness or incomplete recovery.

Acute Disease↗
PubMed1976

Chronic relapsing polyneuritis.

Clinical, electrophysiological and pathological findings in 23 patients with subacute and relapsing idiopathic demyelinating polyneuropathies are described. In 17 patients with relapsing polyneuropathy, the neurological illness was unaccompanied by any systemic disturbances. The term preferred for the neuropathy in this group of patients is chronic relapsing polyneuritis. The findings in this group suggest that the common form of this syndrome is due to a single disease entity. Chronic relapsing polyneuritis differs from acute idiopathic polyneuritis chiefly in regard to the rate of evolution and the severity of the initial episode of polyneuropathy. If these two polyneuropathies have the same pathogenesis, the factor which determines whether the disease is acute and self-limiting or chronically relapsing is often present at the time of onset of the disease. The relationship of chronic relapsing polyneuritis to relapsing hypertrophic polyneuropathy and progressive hypertrophic polyneuropathy is also discussed and it is concluded that these diseases may constitute a spectrum of pathogenetically related disorders. In chronic relapsing polyneuritis, as in other demyelinating polyneuropathies, a marked segmental reduction in axon diameter accompanies demyelination. This corresponds to a more than 50% reduction in the volume of the affected region of the axon and it is associated with increased packing of axoplasmic organelles and wrinkling of the axolemma. It is suggested that in the normal myelinated nerve fibre, the Schwann cell and myelin sheath maintain fluid locally within the axon.

Acute Disease↗
PubMed1976

Gian axonal neuropathy--a generalized disorder of cytoplasmic microfilament formation.

Light and electron microscopy of a sural nerve biopsy obtained from a patient with giant axonal neuropathy revealed the presence of numerous axonal spheroids which were composed chiefly of neurofilaments. Large discrete masses of cytoplasmic microfilaments were also observed in endoneurial fibroblasts, endothelial cells, Schwann cells, perineurial cells and cultured skin fibroblasts. Neuronal degeneration in giant axonal neuropathy appears to be part of a generalized disorder of cytoplasmic microfilament formation.

Axons↗