Acta Neuropathologica

, Volume 83, Issue 4, pp 434–439 | Cite as

Binswanger's disease in the absence of chronic arterial hypertension

A case report with clinical, radiological and immunohistochemical observations on intracerebral blood vessels
  • K. -C. Ma
  • P. O. Lundberg
  • A. Lilja
  • Y. Olsson
Case Reports


The cerebral changes are described in a woman of 54 who suffered from Binswanger's encephalopathy: there were no signs or symptoms of chronic arterial hypertension. The disease presented as dementia of about 3 years duration. Computed tomography of the brain 2. 5 years before her death showed bilateral widespread hypodense lesions in the cerebral white matter. She died of an asthmatic attack. Autopsy disclosed extensive bilateral degeneration of the central white matter, lacunes and gliosis. Severe obliterative arteriolosclerosis occurred in the meningeal vessels and those supplying the affected parts of the brain. Light microscopy showed that the most severe lesions occurred in the arterioles. Immunohistochemistry demonstrated profound extravasation of plasma proteins chiefly albumin, indicating dysfunction of the blood-brain barrier. Thus, the lesions characteristic of Binswanger's encephalopathy may develop in the absence of chronic arterial hypertension. Additional pathogenic factors, possibly genetic predisposition to vascular injury may play a role in the development of this condition.

Key words

Binswanger's disease White matter lesion Arteriolosclerosis Dementia 


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  1. 1.
    Awad IA, Johnson PC, Spetzler RF, Hodak JA (1986) Incidental subcortical lesions identified on magnetic resonance imaging in the elderly. II. Postmortem pathological findings. Stroke 17:1090–1097Google Scholar
  2. 2.
    Babikian V, Ropper AH (1987) Binswanger's disease: a review. Stroke 18:2–12Google Scholar
  3. 3.
    Biemond A (1970) On Binswanger's subcortical arteriosclerotic encephalopathy and the possibility of its clinical recognition. Psychiatr Neurol Neurochir 73:413–417Google Scholar
  4. 4.
    Binswanger O (1894) Die Abgrenzung der allgemeinen progressiven Paralyse. Klin Wochenschr 31:1103–1105, 1137–1139, 1180–1186Google Scholar
  5. 5.
    Caplan LR, Schoene WC (1978) Clinical features of subcortical arteriosclerotic encephalopathy (Binswanger's disease). Neurology 28:1206–1215Google Scholar
  6. 6.
    Chimowitz MI, Awad IA, Furlan AJ (1989) Periventricular lesions on MRI. Curr Concepts Cerebrovasc Dis Stroke 24:7–12Google Scholar
  7. 7.
    Feigin I, Popoff N (1963) Neuropathological changes late in cerebral edema: the relationship of trauma, hypertensive disease and Binswanger's encephalopathy. J Neuropathol Exp Neurol 22:500–511Google Scholar
  8. 8.
    Ferrer I, Bella R, Serrano MT, Marti E, Guionnet N (1990) Arteriolosclerotic leucoencephalopathy in the elderly and its relation to white matter lesions in Binswanger's disease, multi-infarct encephalopathy and Alzheimer's disease. J Neurol Sci 98:37–50Google Scholar
  9. 9.
    Fisher CM (1989) Binswanger's encephalopathy: a review. J Neurol 236:65–79Google Scholar
  10. 10.
    Fukatake T, Hattori T, Kita K, Hirayama K (1985) Familial juvenile encephalopathy (Binswanger type) with alopecia and lumbago-A syndrome. Clin Neurol 25:949–955Google Scholar
  11. 11.
    Goto K, Ishii N, Fukasawa H (1981) Diffuse white matter disease in the geriatric population. Neuroradiology 141:687–695Google Scholar
  12. 12.
    Hachinski VC, Potter P, Merskey H (1987) Leukoaraiosis. Arch Neurol 44:21–23Google Scholar
  13. 13.
    Hsu SM, Raine L, Fanger H (1981) Use of avidin-biotinperoxidase complex (ABC) in immunoperoxidase techniques: a comparison between ABC and unlabelled antibody (PAP) procedures. H Histochem Cytochem 29:577–580Google Scholar
  14. 14.
    Huang KW, Wu L, Luo Y (1985) Binswanger's disease: progressive subcortical encephalopathy or multi-infarct dementia. Can J Neurol Sci 12:88–94Google Scholar
  15. 15.
    Jellinger K, Neumayer E (1964) Progressive subcorticale vasculäre Encephalopathie Binswanger, eine klinische-neuropathologische Studie. Arch Psychiatr Nervenkr 205:523–554Google Scholar
  16. 16.
    Kinkel WR, Jacobs L, Polachini I, Bates V, Heffner RR (1965) Subcortical arteriosclerotic encephalopathy (Binswanger's disease). Computed tomographic, nuclear magnetic resonance and clinical correlations. Arch Neurol 42:951–959Google Scholar
  17. 17.
    Kondo S, Ogasawara N, Ito T, Tsunoda T (1970) Über einen autoptischen Fall von der im ZNS lokalisierten nekrotisierenden Angitis (Periarteritis nodosa?). Adv Neurol Sci 114:274–284 (in Japanese)Google Scholar
  18. 18.
    Liu HM (1988) Extravasation of plasma proteins in brain trauma. Forensic Sci Int 38:285–295Google Scholar
  19. 19.
    Liu HM (1989) Immunohistochemical localization of intracellular plasma proteins in the human central nervous system. Acta Neuropathol 78:16–21Google Scholar
  20. 20.
    Loizou LA, Kendall BE, Marshall J (1981) Subcortical arteriosclerotic encephalopathy: a clinical and radiological investigation. J Neurol Neurosurg Psychiatry 44:294–304.Google Scholar
  21. 21.
    Loizou LA, Jefferson JM, Thomas Smith W (1982) Subcortical arterioslcerotic encephalopathy (Binswanger's type) and cortical infarcts in a young normotensive patient. J Neurol Neurosurg Psychiatry 45:409–417Google Scholar
  22. 22.
    Lotz PR, Ballinger WE Jr, Quisling RG (1986) Subcortical arteriosclerotic encephalopathy: CT spectrum and pathological correlation. Am J Neuradiol 7:817–822Google Scholar
  23. 23.
    Maeda S, Nakayama H, Isaka K, Aihara Y, Nemoto S (1976) Familial unusual encephalopathy of Binswanger's type without hypertension. Folia Psychiat Neurol Jpn 30:164–177Google Scholar
  24. 24.
    Nemoto S (1966) Einige Beiträge zur Encephalitis subcorticalis chronica progressiva (Binswanger). Festschrift zum Zurücktritt von Professor Toshimi Ishibashi. Sendai (in Japanese), pp 51–67Google Scholar
  25. 25.
    Neumann MA (1947) Chronic progressive subcortical encephalopathy. J Gerontol 2:57–64Google Scholar
  26. 26.
    Olszewski J (1962) Subcortical arteriosclerotic encephalopathy: review of the literature on the so-called Binswanger's disease and presentation of two cases. World Neurol 3:359–375Google Scholar
  27. 27.
    Pellisser JF, Poncet M (1989) Binswanger's encephalopathy. Handb Clin Neurol 10:221–233Google Scholar
  28. 28.
    [Reference deleted]Google Scholar
  29. 29.
    Poppe W, Tennstedt A (1963) Ein Beitrag zur Encephalopathia subcorticalis Binswanger. Psychiatr Neurol (Basel) 145:27–35Google Scholar
  30. 30.
    Prencipe M, Marini C (1989) Leuko-Araiosis: definition and clinical correlates—An overview. Eur Neurol 29 [Suppl 2]: 27–29Google Scholar
  31. 31.
    Pullicino P, Eskin T, Ketonenn L (1983) Prevalence of Binswanger's disease. Lancet I:939Google Scholar
  32. 32.
    Revesz T, Hawkins CP, du Boulay EPGH, Barnard RO, McDonald WI (1989) Pathological findings correlated with magentic resonance imaging in subcortical arteriosclerotic encephalopathy (Binswanger's disease). J Neurol Neurosurg Psychiatry 52:1337–1344Google Scholar
  33. 33.
    Salahuddin TS (1991) Consequences of opening the bloodbrain-barrier by carotid infusion of hyperosmolar solutions. Acta Univ Ups 297:1–44Google Scholar
  34. 34.
    Salahuddin TS, Kalimo H, Johansson BB, Olsson Y (1988) Observations on exudation of fibronectin, fibrinogen and albumin in the brain after carotid infusion of hyperosmolar solutions: an immunohistochemical study in the rat indicating long-lasting changes in the brain microenvironment and multifocal nerve cell injuries. Acta Neuropathol 76:1–10Google Scholar
  35. 35.
    Schneider R, Ringelstein EB, Zeumer H, Kiesewetter H, Jung F (1987) The role of plasma hyperviscosity in subcortical arteriosclerotic encephalopathy (Binswanger's disease). J Neurol 234:67–73Google Scholar
  36. 36.
    Sokrab T-E O, Johansson BB, Kalimo H, Olsson Y (1988) A transient hypertensive opening of the blood-brain-barrier can lead to brain damage: extravasation of serum proteins and cellular changes in rats subjected to aortic compression. Acta Neuropathol (Berl) 75:557–565Google Scholar
  37. 37.
    Sourander P, Wålinder J (1977) Hereditary multi-infarct dementia. Acta Neuropathol (Berl) 39:247–254Google Scholar
  38. 38.
    Tanaka M, Ikuta F, Oyake Y (1969) An autopsy case of chronic progressive subcortical encephalopathy without hypertension. Clin Neurol 9:398–405 (in Japanese)Google Scholar
  39. 39.
    Torack RM, Morris JC (1989) Vascular glycosaminoglycans in periventricular leukoencephalopathy. Acta Neuropathol 78:492–496Google Scholar
  40. 40.
    Valentine AR, Moseley IF, Kendall BE (1980) White matter abnormality in cerebral atrophy: clinicoradiological correlations. J Neurol Neurosurg Psychiatry 43:139–142Google Scholar
  41. 41.
    Yamori Y (1989) Predictive and preventive pathology of cardiovascular diseases. Acta Pathol Jpn 39:683–705Google Scholar
  42. 42.
    Zeumer H, Hacke W, Kollmann HL, Ringelstein EB (1982) Subcortical arteriosclerotic encephalopathy (Binswanger's disease). Exp Brain Res 5 [Suppl]:272–276Google Scholar

Copyright information

© Springer-Verlag 1992

Authors and Affiliations

  • K. -C. Ma
    • 1
  • P. O. Lundberg
    • 2
  • A. Lilja
    • 3
  • Y. Olsson
    • 1
  1. 1.Laboratory of Neuropathology, Institute of PathologyUniversity HospitalUppsalaSweden
  2. 2.Department of NeurologyUniversity HospitalUppsalaSweden
  3. 3.Department of Diagnostic RadiologyUniversity HospitalUppsalaSweden

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