Allgemeine morphologische Pathologie des Nervengewebes

Hager, Hermann

doi:10.1007/978-3-642-86325-7_1

Cited by 9 publications

(2 citation statements)

References 684 publications

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“…Peters (1949) could produce vacuolization in the cytoplasm of the ganglion cells of the central nervous system with ultrasound. Acute vacuolar change is said by Hager (1968) to occur in experimental viral infections (poliomyelitis, Teschener epidemic in pigs). Srebro (1966) found large vacuoles in neurones of posterior of the telencephalon in stz Miller (1959) found that larger vacu4 originate in confluence or rupture of dil spaces of the endoplasmic reticulum.…”

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confidence: 99%

Clinical and pathological findings in an unusual infantile motor neurone disease

Kohn

1971

Journal of Neurology, Neurosurgery & Psychiatry

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SUMMARY An unusual motor neurone disease with a characteristic denervation atrophy of muscle is described in an infant. The striking change is a vacuolization of the cytoplasm of the affected ganglion cells in many motor nuclei of spinal cord and brain-stem, and in the claustrum. To our knowledge this disease has not yet been described in man and similar pathological changes have been found only in a certain strain of mice ('wobbler').This paper presents the clinical and pathological findings in an infant with an unusual form of motor neurone disease which appeared to be present at birth. Morphologically, the disease is characterized primarily by chromatolysis and vacuolization of nerve cells of the motor system in spinal cord and brain-stem. The changes seem to be similar to those seen by Duchen and Strich (1968) in a hereditary motor neurone disease in a strain of mice 'wobbler', but to our knowledge such changes have not yet been described in man. CASE REPORTThis was the first child, a boy, of young and healthy parents of Egyptian extraction. There were no known inter-marriages in the family. Pregnancy and delivery were normal. Birth weight was 2,500 g without evidence of prematurity. The mother noted soon after delivery that the right hand of the child was floppy.The boy was admitted to hospital at the age of 7 months in order to investigate the cause for his floppy hand. He was not able to sit up. His weight was 5,600 g. The right hand was flaccid and showed some outward rotation. The left hand was somewhat spastic. A subluxation of the left hip joint was found. The electroencephalogram (EEG) was normal. The electromyogram (EMG) (right extensor and flexor carpi muscles) showed an uncharacteristic abnormality, possibly caused by a neurogenic muscular lesion; a primary myogenic lesion could be ruled out. Radiographs of the pelvis showed that the ossification centre on the left side was small, and the acetabulum flat, findings which are compatible with a dysplasia on the left side. The right hand showed 'retardation' in bone age. Skull and thorax were normal. LABORATORY FINDINGS The plasma contained phosphorus5-1 and 5-8 mg/100ml.;alkaline phosphatase 8 and 6 Bodanski units; calcium 9-5 mg/100 ml.; haemoglobin 8-5 g/100 ml.; leucocytes 9100/c. mm; normal differential count; haematocrit 30%; anisomicrocytosis. Plasma iron was 210 and 160 pg/100 ml.; iron binding capacity 170 and 275; total iron binding capacity 380 and 435; iron saturation 55 and 37%. A Sabin-Feldman test for toxoplasmosis was negative; PPD (Mantoux) negative. Cerebrospinal fluid obtained by lumbar puncture showed: Pandy test negative; protein 49 mg/100 ml.; NaCl 660 mg/ 100 ml.; glucose 65 mg/100 ml.; no cells; culture negative.During his stay in hospital he suffered from bronchopneumonia; his weight dropped to 5,300 g.The clinical diagnoses at discharge included cerebral palsy, arthrogryposis, ? Erb's palsy.The second admission was for an upper respiratory tract infection at the age of 9 months. His weight was 5,600 gin. He neither sat nor st...

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Clinical and pathological findings in an unusual infantile motor neurone disease

Kohn

1971

Journal of Neurology, Neurosurgery & Psychiatry

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“…Both cell types are candidates for an active role in neuronophagia. In an earlier study, microglial proliferation and activation of microglia and astrocytes have been demonstrated in the cortex, subcortical white matter and spinal cord of ALS patients [17,[37][38][39]. However, the possible involvement of astrocytes, resident and nonresident macrophages has not been clarified.…”

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Neuronophagia in the motor cortex in amyotrophic lateral sclerosis

Troost

Claessen

Oord

et al. 1993

Neuropathology Appl Neurobio

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This study was designed to identify which phagocytic cells in the cerebral cortex of amyotrophic lateral sclerosis (ALS) patients are involved in the process of neuronophagia. For this purpose a number of single and double immunocytochemical stains were carried out on five ALS cases which were selected on the basis of the presence of degenerative and phagocytic phenomena in the cerebral cortex. The cortical degenerative process is mainly present in the third and fifth layers and is not restricted to the fifth layer which contains the cell bodies of the Betz cells. The present study indicates that a number of cells are involved in the process of phagocytosis in ALS. Resident macrophages (from microglial or perivascular origin) and astrocytes seem to play an immunologically-mediated role in the disappearance of neurons. Some of the cells involved in the degenerative process, i.e. rounded macrophages and microglia, expressed major histocompatibility class II antigen. The phagocytic cells in neuronophagia were phenotypically identical to perivascular macrophages and not to microglia. Therefore, the process of phagocytosis of neurons appears to be primarily the task of the perivascularly located macrophage.

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Kreislaufstörungen und Gefäßprozesse des Rückenmarks

Schneider

1980

Spezielle Pathologische Anatomie

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Allgemeine morphologische Pathologie des Nervengewebes

Cited by 9 publications

References 684 publications

Clinical and pathological findings in an unusual infantile motor neurone disease

Clinical and pathological findings in an unusual infantile motor neurone disease

Neuronophagia in the motor cortex in amyotrophic lateral sclerosis

Kreislaufstörungen und Gefäßprozesse des Rückenmarks

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