Thromboangiitis Obliterans Spatz-Lindenberg – The cerebral form of von Winiwarter-Buerger's disease: a rare but typical form of “multi-infarct“ dementia

Cerebral involvement in thromboangiitis obliterans (von Winiwarter-Buerger disease (WBD)), including pure CNS forms with predominant neurological symptoms, has been described by Spatz and Lindenberg (1935–1940) and named Spatz-Lindenberg disease (SLD). Subsequently, many additional cases were reported by various authors. The type II of SLD, morphologically characterized by sickle-shaped granular atrophy of the cerebral cortex, includes, as a rule, a clinical course of progressive dementia. In addition to multiple small, old and recent, complete and incomplete, ischemic infarcts scattered along the “watershed“ regions of the arterial blood supply, a significant decline in the cortical synaptic density could be demonstrated in a former investigation into cortical regions apart from overt ischemic damage (Zhan et al., 1993, Acta Neuropathol. 86:259–264).

An inflammatory nature of WBD/SLD, that discriminates it from common arteriosclerotic thromboembolism, has been discussed in the literature and favored by some but denied by others. In 1939 Spatz already stressed that the SLD type II phenotype with its characteristic sickle-shaped cortical granular atrophy could even more frequently be mimicked by deep bilateral arteriosclerotic stenosis of the internal carotid artery at its origin.

Here we show two observations of sickle-shaped cerebral granular atrophy with typical small-vessel occlusions, involving both arteries and veins, in two non-smoking progressively demented women aged 43 and 48 (brain weights 760 and 1060 gs, respectively) without significant general atheroscleosis and extracranial sources of thromboembolism. No overt inflammatory signs could be demonstrated either clinically or morphologically. However, blood examination in the 48 year-old patient had revealed antithrombin (AT)-III deficiency. AT-III deficiency has been shown, in general, to predispose to thromboembolism and, if familial, to be a possible causal factor of cerebral infarction in the elderly.

The present cases support former notions that (1) SLD exists as a disease entity separate from arteriosclerotic stenosis and/or thromboembolism, the latter of which does not involve veins, and (2) that anticoagulation factor (e.g., AT-III) deficiency and other factors of the blood coagulation system, if examined in future cases, might provide new clues to the etio-pathogenesis of SLD.