Skip to main content
SpringerLink
Log in

Binswanger's disease in the absence of chronic arterial hypertension

A case report with clinical, radiological and immunohistochemical observations on intracerebral blood vessels

  • Case Reports
  • Published:
Acta Neuropathologica Aims and scope Submit manuscript

Summary

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.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  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 17:1090–1097

    Google Scholar 

  2. Babikian V, Ropper AH (1987) Binswanger's disease: a review. Stroke 18:2–12

    Google Scholar 

  3. Biemond A (1970) On Binswanger's subcortical arteriosclerotic encephalopathy and the possibility of its clinical recognition. Psychiatr Neurol Neurochir 73:413–417

    Google Scholar 

  4. Binswanger O (1894) Die Abgrenzung der allgemeinen progressiven Paralyse. Klin Wochenschr 31:1103–1105, 1137–1139, 1180–1186

    Google Scholar 

  5. Caplan LR, Schoene WC (1978) Clinical features of subcortical arteriosclerotic encephalopathy (Binswanger's disease). Neurology 28:1206–1215

    Google Scholar 

  6. Chimowitz MI, Awad IA, Furlan AJ (1989) Periventricular lesions on MRI. Curr Concepts Cerebrovasc Dis Stroke 24:7–12

    Google Scholar 

  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 22:500–511

    Google Scholar 

  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 98:37–50

    Google Scholar 

  9. Fisher CM (1989) Binswanger's encephalopathy: a review. J Neurol 236:65–79

    Google Scholar 

  10. Fukatake T, Hattori T, Kita K, Hirayama K (1985) Familial juvenile encephalopathy (Binswanger type) with alopecia and lumbago-A syndrome. Clin Neurol 25:949–955

    Google Scholar 

  11. Goto K, Ishii N, Fukasawa H (1981) Diffuse white matter disease in the geriatric population. Neuroradiology 141:687–695

    Google Scholar 

  12. Hachinski VC, Potter P, Merskey H (1987) Leukoaraiosis. Arch Neurol 44:21–23

    Google Scholar 

  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 29:577–580

    Google Scholar 

  14. Huang KW, Wu L, Luo Y (1985) Binswanger's disease: progressive subcortical encephalopathy or multi-infarct dementia. Can J Neurol Sci 12:88–94

    Google Scholar 

  15. Jellinger K, Neumayer E (1964) Progressive subcorticale vasculäre Encephalopathie Binswanger, eine klinische-neuropathologische Studie. Arch Psychiatr Nervenkr 205:523–554

    Google Scholar 

  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 42:951–959

    Google Scholar 

  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. Liu HM (1988) Extravasation of plasma proteins in brain trauma. Forensic Sci Int 38:285–295

    Google Scholar 

  19. Liu HM (1989) Immunohistochemical localization of intracellular plasma proteins in the human central nervous system. Acta Neuropathol 78:16–21

    Google Scholar 

  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. 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 45:409–417

    Google Scholar 

  22. Lotz PR, Ballinger WE Jr, Quisling RG (1986) Subcortical arteriosclerotic encephalopathy: CT spectrum and pathological correlation. Am J Neuradiol 7:817–822

    Google Scholar 

  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 30:164–177

    Google Scholar 

  24. Nemoto S (1966) Einige Beiträge zur Encephalitis subcorticalis chronica progressiva (Binswanger). Festschrift zum Zurücktritt von Professor Toshimi Ishibashi. Sendai (in Japanese), pp 51–67

  25. Neumann MA (1947) Chronic progressive subcortical encephalopathy. J Gerontol 2:57–64

    Google Scholar 

  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 3:359–375

    Google Scholar 

  27. Pellisser JF, Poncet M (1989) Binswanger's encephalopathy. Handb Clin Neurol 10:221–233

    Google Scholar 

  28. [Reference deleted]

  29. Poppe W, Tennstedt A (1963) Ein Beitrag zur Encephalopathia subcorticalis Binswanger. Psychiatr Neurol (Basel) 145:27–35

    Google Scholar 

  30. Prencipe M, Marini C (1989) Leuko-Araiosis: definition and clinical correlates—An overview. Eur Neurol 29 [Suppl 2]: 27–29

    Google Scholar 

  31. Pullicino P, Eskin T, Ketonenn L (1983) Prevalence of Binswanger's disease. Lancet I:939

    Google Scholar 

  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 52:1337–1344

    Google Scholar 

  33. Salahuddin TS (1991) Consequences of opening the bloodbrain-barrier by carotid infusion of hyperosmolar solutions. Acta Univ Ups 297:1–44

    Google Scholar 

  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 76:1–10

    Google Scholar 

  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 234:67–73

    Google Scholar 

  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) 75:557–565

    Google Scholar 

  37. Sourander P, Wålinder J (1977) Hereditary multi-infarct dementia. Acta Neuropathol (Berl) 39:247–254

    Google Scholar 

  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. Torack RM, Morris JC (1989) Vascular glycosaminoglycans in periventricular leukoencephalopathy. Acta Neuropathol 78:492–496

    Google Scholar 

  40. Valentine AR, Moseley IF, Kendall BE (1980) White matter abnormality in cerebral atrophy: clinicoradiological correlations. J Neurol Neurosurg Psychiatry 43:139–142

    Google Scholar 

  41. Yamori Y (1989) Predictive and preventive pathology of cardiovascular diseases. Acta Pathol Jpn 39:683–705

    Google Scholar 

  42. Zeumer H, Hacke W, Kollmann HL, Ringelstein EB (1982) Subcortical arteriosclerotic encephalopathy (Binswanger's disease). Exp Brain Res 5 [Suppl]:272–276

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Laboratory of Neuropathology, Institute of Pathology, University Hospital, S-75185, Uppsala, Sweden

    K. -C. Ma & Y. Olsson

  2. Department of Neurology, University Hospital, S-75185, Uppsala, Sweden

    P. O. Lundberg

  3. Department of Diagnostic Radiology, University Hospital, S-75185, Uppsala, Sweden

    A. Lilja

Authors
  1. K. -C. Ma
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. P. O. Lundberg
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. A. Lilja
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Y. Olsson
    View author publications

    You can also search for this author in PubMed Google Scholar

Additional information

Supported by grants from the Swedish Medical Research Council, Project No 12X-03020 and 1987 Års Stiftelse för Strokeforskning

Rights and permissions

Reprints and permissions

About this article

Cite this article

Ma, K.C., Lundberg, P.O., Lilja, A. et al. Binswanger's disease in the absence of chronic arterial hypertension. Acta Neuropathol 83, 434–439 (1992). https://doi.org/10.1007/BF00713538

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF00713538

Key words

Search

Navigation