Edith Reske-Nielsen

Association of trigeminal neuralgia with multiple sclerosis: clinical and pathological features.

The clinical characteristics of facial pain in patients with trigeminal neuralgia (TN) and associated multiple sclerosis (MS) were studied. Among 900 patients with TN 22 (2.4%) had associated MS. Sixteen patients had typical TN, while six had atypical TN. Facial pain commenced at a younger age (P < 0.05) and a significantly higher proportion had bilateral facial pain (P < 0.01) in the group with MS compared to TN patients without MS. In three patients TN was the first manifestation of MS, in the remainder facial pain occurred on an average of 12 years after onset of MS. Seventeen patients had associated signs of brain‐stem involvement. The facial pain was, however, indistinguishable from the pain in patients without brain‐stem deficits. While sclerotic plaques in the central nervous system probably are of etiological significance for development of TN neither clinical nor neuropathological findings suggest that this is due to affection of trigeminal nuclei complex in the brain‐stem.

Fiber types in the striated urethral and anal sphincters

SummarySeven normal human striated urethral and anal sphincters obtained by autopsy were examined using histochemical techniques. In both the urethral sphincter and the subcutaneous (s.c.) and superficial part of the anal sphincter a characteristic pattern with two populations of muscle fibers, abundant connective tissue, and numerous intramuscular nerves are seen. No spindles are observed. The muscle fibers, particularly the predominant type 1 fibers are very small (about 15 μm in diameter). The fiber characteristics of the sphincters indicate that these muscles have a capacity to produce sustained contractions and to react in stress conditions with fast increase in tension.

Juvenile ceroid-lipofuscinosis and calcifications of the CNS

The post‐mortem brains and spinal cords of 20 juvenile ceroid‐lipofuscinosis (JC‐L) cases from 1973 to 1987 were investigated. Clinical course of the disease was characterized by impaired vision from the age of 5–8 years; progressive dementia, seizures, somatic retardation, and early death (16–29 years of age). Microscopy showed classic intracytoplasmic autofluorescent lipopigment in the nerve cells throughout the CNS and viscera. Immunoperoxidase staining for glial fibrillary acidic protein (GFAP) showed marked gliosis with enlarged reactive astrocytes mainly in the superficial layers of the cerebral cortex. Calcifications of the nervous system principally along the outer and inner brain surfaces were demonstrated by X‐ray, macroscopic examination and microscopy. We suggest that the calcifications are secondary to a suspected generalized metabolic error.

Progressive external ophthalmoplegia. Evidence for a generalised mitochondrial disease with a defect in pyruvate metabolism.

Muscle biopsies from four patients with chronic progressive external ophthalmoplegia and pigmentary retinopathy with symptoms and signs from other organs were studied by means of light and electron microscopy. Examination revealed a marked proliferation of abnormal mitochondria with a degeneration of both muscle and nerve tissue. Blood levels of lactate and pyruvate were measured and abnormal values of these metabolites were found in the three patients with the most pronounced ultrastructural changes. On the basis of these findings it is suggested that there is a biochemical defect in pyruvate‐lactate metabolism which could be responsible for the marked proliferation of the abnormal mitochondria.

Flow cytometry in tumours of the brain.

SummaryFlow cytometry was used for studying the distribution of single cell DNA content in biopsy material from 85 patients with benign and malignant lesions of the brain and spinal cord. In inflammatory lesions and in most benign tumours, cells with diploid DNA values were observed, sometimes with additional smaller amounts of tetraploid DNA. Cells from malignant tumours were characterized by marked hyperploid DNA values. Flow cytometry is found a rapid and valuable method adding important information to the biological nature of tumours of the central nervous system.

A Neuropathological and Neuropsychological Study of Turner's Syndrome

A neuropathological study was made in 2 women with Turners syndrome. Neuropsychological investigation in one of them correlated with what has previously been found in Turners syndrome as well as with the localization of the most pronounced neuropathological aberration which was of atherosclerotic nature, most pronounced in the right temporo-parietal area. These findings as well as the findings of acidophile hypoplasia of the pituitary gland are discussed.

Distribution of dietary mercury in a dog. Quantitation and localization of total mercury in organs and central nervous system

An Alsatian dog which had been fed fish contaminated with methyl mercury for 7 years was examined after its death at the age of 12, 4 years after the exposure to methyl mercury had ceased. Two dogs of the same age and breed served as controls. In the exposed dog, mercury was found in all of the organs examined; the highest concentrations were found in the kidneys, and the lowest in the gastrointestinal tract and skeletal muscles. In the central nervous system (CNS) the mercury was fairly uniformly distributed, with 93% in the inorganic state, whereas the skeletal muscles contained approximately 30% inorganic mercury. This demonstrates time-dependent demethylation and suggests a variation in the rate from one type of tissue to another. At the time of death, the mercury level in the dog was still falling. In the control dogs, detectable amounts (0.01 mg kg-1) of mercury were only found in the kidney and liver. The distribution of mercury was determined by a histochemical method (autometallography) for locating mercury in tissue sections. Sections from autometallography of the central nervous system showed large deposits of mercury in all areas of the cerebral hemispheres, the brainstem and the spinal cord, including nerve cells, astrocytes, microglial cells and vessel walls. The granular layer of the cerebellar hemispheres was especially loaded, while only a few granules were present in the Purkinje cells. In the leptomeninges the vessels and the macrophages were heavily encrusted. High amounts of histochemically demonstrable mercury were observed in the liver, thyroid gland and kidney. In the control dogs, all the organs examined were practically devoid of deposits.

Menkes' disease — a new leucodystrophy(?)

SummaryIn 1962 Menkeset al. established a new syndrome among the numerous neurodegenerative disorders of early childhood. Since then over 20 cases have been reported—few in combination with a sufficiently detailed description of the neuropathological substrate. The relevant literature is reviewed and a new case is reported in which extensive and systematic histologic investigations of the central and peripheral nervous system have revealed unique traits which make it reasonable to place this peculiar syndrome among the leucodystrophies.

PATHOLOGICAL CHANGES OF THE CENTRAL NERVOUS SYSTEM IN GIANT-CELL ARTERITIS

Giant-cell arteritis is a generalized vascular disease of unknown aetiology which may affect the vascular system in all parts of the body (Paulley &Hughes 1960). The most commonly affected vessels are the internal and external carotid arteries with their branches, and the disease was first described as arteritis of the superficial temporal artery. When the cerebral vessels are involved, neurological signs may develop (Paulley & Hughes 1960). Frequently, the disease is complicated by ocular signs, when the ophthalmic artery or the central retinal artery are involved (Wagener & Hollenhorst 1967). The pathological finding is a granulomatous lesion in the vessel wall with foreign-body giant cells. The present case is interesting on account of its clinical course which was characterized by symptoms and signs from the superficial temporal artery, visual disturbances, and neurological signs, as well as pathological changes, especially in the brain and spinal cord.

Histological and morphometric observations on the reticular fibers in the arterial beds of patients with ruptured intracranial saccular aneurysms.

Histological and morphometric studies on the collagenous, reticular, and elastic fibers of the tunica media were performed on the middle cerebral and brachial arteries of patients with ruptured intracranial saccular aneurysms and controls. There was no difference between aneurysm patients and controls in the amount of collagenous and elastic fibers. The amount of reticular fibers was, however, reduced about 35% in aneurysm patients within both the middle cerebral artery (P less than 0.01) and the brachial artery (P less than 0.02). The reticular fibers were irregularly distributed and often appeared shorter and coarser than normally. The observations point to a generalized abnormality of the arterial bed in some patients with rupture of saccular aneurysms.