Treatment of intracranial atherosclerotic disease with a balloon-expandable paclitaxel eluting stent: procedural safety, efficacy and mid-term patency.

PURPOSE: Procedural safety and high rates of in-stent recurrent stenotic lesions (ISR) remain a concern in the endovascular treatment of intracranial atherosclerotic disease (ICAD). In the present study technical feasibility, safety and efficacy of the paclitaxel eluting balloon-expandable coronary stent Coroflex(®) Please was assessed in the treatment of ICAD. METHODS: A total of 95 patients (79 male; median age 68 years) with 106 intracranial atherosclerotic stenotic lesions underwent endovascular treatment using Coroflex(®) Please stents (B. Braun, Melsungen, Germany). Location and degree of target stenoses before and after treatment and at follow-up and adverse clinical sequelae of treatment were registered. Post-procedural medication included 100 mg acetylsalicylic acid (ASA) and 75 mg clopidogrel for 1 year. Angiographic follow-up was scheduled for 6, 12, 26 and 52 weeks after the treatment. RESULTS: The lesion locations were as follows: internal carotid artery (ICA) petrous (n = 44, 42%), ICA cavernous (n = 43, 41%), ICA paraclinoid (n = 4, 4%), intradural vertebral artery (VA; n = 11, 10%) and basilar artery (BA; n = 4, 4%). Of the lesions seven could not be treated due to difficult anatomy and stent stiffness (7% technical failure rate). The combined post-interventional neurological morbidity and mortality rate, including stroke, intracerebral hemorrhage (ICH), subarachnoid hemorrhage (SAH) and carotid cavernous fistula (CCF) was n = 4 (3.7%) within and n = 1 (0.9%) at and beyond 30 days, respectively. Angiographic and clinical follow-up examinations were carried out for 78 (78%) of the lesions (mean 16.1 months, maximum 48 months). Asymptomatic recurrent stenosis was seen in 3 out of 78 (3.8%) lesions and there was 1 case of late stent thrombosis (0.9%). CONCLUSIONS: Treatment of ICAD using drug-eluting coronary stents is safe and effective but technical failure due to stent stiffness remains a problem. Application of the more flexible, newest generation thin-strut stents, however, shows promising results.

Brain imaging and neuropsychology in late-onset dementia due to a novel mutation (R93C) of valosin-containing protein.

Inclusion body myopathy with Paget disease of bone and frontotemporal dementia (IBMPFD, MIM 167320) is a recently identified autosomal dominant disorder due to mutations in the valosin-containing protein (VCP) that affects muscle, bone and brain. Brain involvement and neuropsychological findings of IBMPFD have not been described in detail. A patient carried a novel heterozygous base pair change, 47832C>T, in the VCP gene that resulted in substitution of an arginine residue by cysteine at position 93 (R93C). He presented first with myopathy while bone involvement remained subclinical. The patient developed behavioral abnormalities in his 60s and showed frank personality change with fluent empty speech at the age of 74 years. This syndrome was best classified as semantic dementia. Magnetic resonance imaging disclosed slight but progressive cerebral atrophy with prominent callosal and frontal white matter loss. Positron emission tomography demonstrated glucose hypometabolism of the frontal and temporal lobes disproportionate to their structural involvement. This first comprehensive clinical and neuroimaging study in IBMPFD may raise the awareness among clinicians as well as basic scientists for this exemplary genetic model of dementia.

[Complexities of "reversible posterior leukoencephalopathy syndrome"]. / "Reversibles posteriores Leukenzephalopathiesyndrom": Eine unglückliche Begriffsprägung für eine komplexe Erkrankung.

We report a case of reversible posterior leukoencephalopathy syndrome in a 50-year-old patient with severe untreated hypertension. Recent advances in magnetic resonance imaging (especially diffusion-weighted imaging) allow new pathopysiological insight: it was found that the resulting vasogenic edema was restricted neither to the posterior vascular territories nor to white matter. The apparent diffusion coefficient helps to differentiate between reversible vasogenic edema and cytotoxic edema, the latter indicating irreversible neuronal death.