Vascular Myelopathy
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J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.30.3.195 on 1 June 1967. Downloaded from J. Neurol. Neurosurg. Psychiat., 1967, 30, 195 Spinal cord arteriosclerosis and progressive vascular myelopathy KURT JELLINGER From the Neurological Institute of University of Vienna, Austria Spinal cord arteriosclerosis is considered to be Hughes and Brownell, 1966). Recently we reported infrequent compared with atheromatosis in other on more than 60 cases, all verified at necropsy, in parts of the body. Whereas Staemmler (1939) could which a complex neurological syndrome, often not observe any atheromatous changes in the spinal referable to a combined lesion ofthe upper and lower cords of 700 unselected cadavers, Nunes Vicente motor neurone, was associated with generalized (1964) noted mild arteriosclerosis of major spinal arteriosclerosis and severe aortic atheroma without vessels in 13 out of 200 consecutive necropsy cases. thrombosis or occlusion of the spinal tributaries Mannen (1963) even reported the incidence of 2-6% (Jellinger and Neumayer, 1966). The pathogenesis of atheromatous plaques in the anterior spinal artery this rarely diagnosed condition is obscure as, till of 300 unselected cases upon which necropsies were now, there have been neither sufficient observations performed in a geriatric hospital. Moderate to severe regarding the incidence of spinal cord atherosclerosis atheroma of cord nor data on in spinal vessels has been observed relevant the changes of spinal vessels Protected by copyright. incidentally but only exceptionally has spinal cord old age, generalized arteriosclerosis, and systemic infarction been caused by documented occlusion of hypertension. spinal arteries (Thill, 1923; Zeitlin and Lichtenstien, In this communication the incidence of arterio- 1936; Antoni, 1941; Garstka, 1953; Hogan and sclerotic changes and vascular fibrosis in the spinal Romanul, 1966; Jellinger, 1966, 1967). Angioscler- cords of 1,037 necropsied cases described in the files osis of the small intramedullary arteries as a local of the Neurological and Pathological Institutes of variant of atheromatosis is also extremely rare Vienna University is reported and correlated with (Arendt and Wunscher, 1954). The relationship the age and the frequency of atherosclerosis of the between thickening of the small intramedullary aorta, the coronary and cerebral arteries, as well as vessels in advanced age, known as 'perisclerosis', the incidence ofcerebrovascular lesions and systemic 'hyalinosis', or 'fibrosis' and arteriosclerosis, how- hypertension. Inflammatory vascular processes ever, has been uniformlydenied (Liithy and Zollinger, (syphilis, panarteritis nodosa) and their consequences 1946; Stochdorph and Meessen, 1957). are not included. Table I sets out the age distribu- On the other hand, spinal cord lesions due to tion of the examined cases. In addition, corrobor- arteriosclerosis have been known since the late ative studies were made of 76 necropsy cases of nineteenth century (Demange, 1884; Campbell, vasocirculatory myelopathy in advanced age, and 1894) but have subsequently received only an in the remaining series of necropsied cases of the http://jnnp.bmj.com/ occasional mention in the literature. Reviewing same ages without vascular disorders of the spinal myelopathy due to vascular diseases, Keschner and cord. Davison (1933) found only two cases referable to MATERIAL AND METHODS arteriosclerosis in 200 instances of cerebral athero- For the examination of the spinal cord vessels serial sclerosis. Recent reports, however, suggest that the sections of at least six to 30 blocks of various segments condition may be far more common than was of the spinal cord stained with haematoxylin and eosin, formerly supposed (Jellinger and Neumayer, 1962; cresyl violet or Nissl's method, Van Gieson's elastica on October 1, 2021 by guest. TABLE I AGE DISTRIBUTION OF THE EXAMINED NECROPSY CASES Age Group Total 0-10 11-20 21-30 31-40 41-50 51-60 61-70 71-80 81-90 200 80 103 103 116 170 180 77 8 1,037 Vascular myelopathy -- - 3 9 33 28 3 76 195 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.30.3.195 on 1 June 1967. Downloaded from .~196 Kurt Jellinger technique, and Heidenhain's or Kluver-Barrera's myelin thickening of the vessel wall with obstruction causing method were available. Selected sections were stained almost complete stenosis of the lumen). Similar criteria with Azan or Masson's trichrome technique, Weigert's were used to grade adventitial fibrosis of the vessels of method for fibrin, P.A.S., toluidin blue, Gomori's silver the spinal leptomeninges. impregnation, Bodian's stain for neurofibrils, and the Complete necropsy records were available for 407 coupled tetrazonium method. Frozen sections were cases aged over 21 from the 757 cases in these age groups. stained with Sudan III, Sudan-black B, and Spielmeyer's They were examined to assess the incidence of aortic myelin stain. atheroma, including aortic thrombosis, and of coronary The classification of the vascular lesions of the spinal and cerebral atheromatosis, grading the conditions cord followed the usual pathological criteria recently according to the four degrees of intensity of the coding summarized for the cerebral vessels by Arendt and guide of the World Federation of Neurology (1959), and Bachmann (1966): intimal fibrosis (collagenization), also in relation to the frequency of cerebrovascular hypertrophy of the internal elastic lamina, adventitial lesions and the necropsy findings ofsystemic hypertension fibrosis, and combined atheromatous changes. The (Table II). intensity of arteriosclerosis of the spinal arteries was graded as follows: 0 normal, 1+ mild involvement RESULTS (slight circular or sectional intimal fibrosis and/or duplication and splitting of the internal elastic fibres Arteriosclerotic changes in the major spinal arteries with or without adventitial fibrosis), 2+ moderate sclerosis (same lesions of greater intensity without were seen in 12-7 % of our total material, i.e., in much narrowing of the lumen), 3+ severe changes 22-2 % of the age group over 41 years and in 27-1 % (plaque-like intimal cushions and hypertrophy of the from those over 61 years respectively. Of these, internal elastic layer with narrowing of the lumen), however, 10-5 % showed only mild intimal fibrosis 4+ typical atheroma (containing cholesterol crystals and/or hypertrophy of the internal elastic lamina with stenosis or thrombosis of the vessel). Regarding without much narrowing of the lumen (Fig. la). fibrotic thickening of the sulcal and intramedullary These lesions had always to be clearly distinguished vessels, five degrees of intensity were considered: from the 'physiological' intimal cushions in spinal Protected by copyright. 0 normal, 1 + mild changes (slight thickening of the arteries (Hassler, 1961). Moderate, non-stenosing vessel wall with condensation of perivascular connective tissue without narrowing of the lumen), 2+ moderate atherosclerosis (Fig. lb) was noted in 1-8 %. Only lesions (thickening of the vessel wall up to twice the two cases showed proliferative lesions with obstruc- normal calibre with slight narrowing of the lumen), tion (Fig. lc) and another two typical atheromatous 3+ considerable (higher degree of lesions with consider- plaques (Fig. ld), indicating an incidence of 04% able narrowing of the lumen), 4+ severe lesions (extreme of severe atherosclerosis in major spinal vessels. As FIG. 1. Arteriosclerosis of major spinal arteries. (a) Mild hypertrophy ofinternal elastic lamella and slight thickening of the intima in anterior spinal artery at the T 11 level. NI 131/63. Van Gieson elastica stain x 44. (b) Moderate intimal fibrosis and thickening ofinternal elastic lamella http://jnnp.bmj.com/ with slight narrowing oflumen in posterolateral spinal artery at the cervical level. NI 42/61 Van Gieson stain x 140. (c) Proliferation ofsubintimal connective tissue with partial obliteration ofposterior spinal artery at midthoracic kvel. NI 154/59. on October 1, 2021 by guest. Haematoxylin and eosin stain x 140. (d) Atheroma ofanterior spinal artery at the T 11 level in progressive myelopathy in a 66-year-old sclerotic woman. NI 49/66. Haematoxylin and eosin stain x 200. J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.30.3.195 on 1 June 1967. Downloaded from Spinal cord arteriosclerosis and progressive vascular myelopathy 197 TABLE II CORRELATIVE DATA ON SPINAL AND GENERALIZED ATHEROSCLEROSIS Author Material Analysis of Material Keschner and Davison (1933) Cerebral sclerosis Spinal sclerosis and myelopathy ............................ 1% 200 cases Staemmler (1939) Unselected necropsies Spinal atherosclerosis ......................................0 700 cases Pial artery atheroma .............. ...................... 1-4 / (502 aged over 41yr. or Intermedullary arteriolosclerosis, rare 283 aged over 61 yr. Severe generalized atheromatosis, systemic hypertension ....... 10% Mannen (1963) Geriatric necropsies Sclerotic plaques (anterior spinas artery) ................... 2-6% 300 cases Vascular myelopathies .................................... 9% Cerebrovascular lesions .................................. 80% Nunes Viscente (1964) Consecutive necropsies Spinal sclerosis (11 cases + 2 cases + +) .... 6-5% 200 cases Cerebral sclerosis ....................................... 37-5 % (122 aged over 41 yr. or Coronary sclerosis....................................... 36-0% 50 aged over 61 yr.) Aortic atheroma .......................................