Inherited cholesterol lipidosis: cerebrotendinous xanthomatosis (van Bogaert Scherer Epstein disease). A clinicopathological study.

Cerebrotendinous xanthomathosis (CTX) is a rare autosomal-recessively transmitted disease of the lipid storage system with an array of general and neurological symptoms, based on the pathological storage of cholestanol and cholesterol. The histologic manifestations are foamy cell granulomata and cholesterol crystals within various tissues, associated with a loss of both nerve cells and demyelination inside the CNS. We present a case of CTX with clinical progression as well as the pathomorphologic autopsy findings. The CNS affection in our case will be demonstrated and the pathogenesis be discussed. Medical treatment of CTX is possible but with variable success. In the case shown, the patient profited only marginally from a long-term application of chenodeoxycholic acid.

[Interventional endovascular therapy (stenting) for residual stenosis after lysis of the basilar artery]. / Interventionelle endovaskuläre Therapie (Stentimplantation) bei residuellen Stenosen nach Lyse der A. basilaris.

Despite the introduction of local thrombolytic therapy, there is still a very high mortality rate in basilar thrombosis. We report three cases of successful intra-arterial thrombolysis of acute basilar artery occlusion and consecutive interventional endovascular therapy. In all cases, the acute thrombosis of the basilar artery probably developed from preexisting atherosclerotic stenoses. The clinical outcomes were excellent. We discuss early consecutive stenting as a possible therapeutic option in cases of residual stenosis after successful intra-arterial lysis of basilar artery occlusion.