Jens Kleffmann

Dosage-sensitive network in polycystic kidney and liver disease: Multiple mutations cause severe hepatic and neurological complications

[1] Athyros VG, Tziomalos K, Gossios TD, Griva T, Anagnostis P, Kargiotis K, et al. GREACE Study Collaborative Group. Safety and efficacy of long-term statin treatment for cardiovascular events in patients with coronary heart disease and abnormal liver tests in the Greek Atorvastatin and Coronary Heart Disease Evaluation (GREACE) Study: a post-hoc analysis. Lancet 2010;376:1916–1922. [2] Babu M, Paul N. Smoking and NASH: a hidden message from the GREACE trial. J Hepatol 2012;56:300–301. [3] Zein CO, McCullough AJ. Reply to: smoking and NASH: a hidden message from the GREACE trial. J Hepatol 2012;56:301.

Comparison of Spontaneous Intracerebral Hemorrhage Treatment in Germany Between 1999 and 2009 Results of a Survey

Background and Purpose— The aim of our study was to examine surgical practice in the therapy of spontaneous intracerebral hemorrhage in Germany over a period of 10 years. Methods— In 1999 and 2009 a questionnaire with 10 different cases of spontaneous intracerebral hemorrhage was sent to all neurosurgical centers in Germany. It included a cranial CT as well as a case description. The question asked if a conservative or surgical procedure was most suitable. When choosing surgery, the participants could decide between large open approach or microsurgery as well as stereotactic aspiration or external ventricular drainage. Results— In 1999, 85 of 121 (70%) and in 2009 111 of 125 (89%) questionnaires could be evaluated. The results of the questionnaires from 1999 and 2009 showed no difference in the decision for or against a surgical procedure, except for a move toward conservative treatment in 1 patient with a massive spontaneous intracerebral hemorrhage. In 2 cases of isolated basal ganglia bleeding, a conservative approach was chosen by approximately 98% of the participants both in 1999 and in 2009. In all other cases of spontaneous intracerebral hemorrhage, in particular when the patient was in poor clinical condition, the decision was very heterogeneous. Conclusions— Despite new studies, there were no significant differences regarding the decision for or against a surgical procedure in 1999 and 2009. Although clearly unfavorable prognostic factors are known, many patients still undergo a surgical procedure. It appears that at least spontaneous intracerebral hemorrhage in the area of the basal ganglia is a unique domain of conservative treatment.

Ruptured cerebral aneurysm and acute bilateral carotid artery dissection in a patient with polycystic kidney disease and polycystic liver disease.

weight was normal but that the size of her abdomen was massively enlarged. A CT scan showed a small hyperintensity in the area of the right Sylvian fissure. Subarachnoidal hemorrhage was suggested and digital subtraction angiography showed three aneurysms of the middle cerebral artery on the right side. Moreover, a dissection of the right internal carotid artery was suspected due to a wedgeshaped high-grade stenosis at the base of the skull. After interdisciplinary discussion, a decision was made to clip the aneurysm. Intraoperative blood clots were found perianeurysmal. Postoperatively, the patient again showed a mild hemiparesis on the left side. Over the following days she developed an organic psychosis. Five days after admission, MRI presented a middle cerebral artery infarction on the right side. Furthermore, fat-suppressed axial T1-weighted images showed a semilunar-shaped hematoma in the arterial wall of both internal carotid arteries with a high-grade stenosis on the right side and only a low-grade stenosis on the left side ( fig. 1 ). A CT scan of the abdomen revealed a massively enlarged polycystic liver reaching down to the pelvis minor, but with only a few cysts of the kidneys ( fig. 1 ). Family history yielded several relatives with polycystic kidney and/or liver disease on both the paternal and maternal sides. Our patient was the only one to have suffered from cerebral vascular disease. Five weeks after admission, the patient could be transferred to a rehabilitation clinic with only a mild cognitive impairment. Liver transplant was performed 20 months after the initial event. Molecular analysis of the patient revealed both a homozygote mutation in the PKD1 gene (ADPKD) as well as in the ADPLD gene (PCLD); we have described this previously [2] . Background Both autosomal dominant polycystic kidney disease (ADPKD) and polycystic liver disease (PCLD) follow an autosomal dominant pattern of inheritance, and cerebral vascular manifestations have been described as occasional part of the disease spectrum [1] .

Early Physiotherapy by Passive Range of Motion Does Not Affect Partial Brain Tissue Oxygenation in Neurocritical Care Patients

Background Studies investigating multimodal cerebral monitoring including partial brain tissue oxygen monitoring (ptiO2) in neuro‐intensive care patients during physiotherapy are completely lacking in the literature. Materials and Methods We performed a post hoc analysis of prospectively collected data of patients on multimodal cerebral monitoring by intracranial pressure (ICP) and cerebral perfusion pressure (CPP) measurement as well as ptiO2. Patients with severe brain diseases were treated with passive range of motion (PROM). We recorded ICP, CPP, and ptiO2 continuously every minute at baseline (15 minutes), during treatment (26 minutes), and 15 minutes after treatment with PROM. Results Overall, 25 treatment units with PROM in 10 patients with combined ICP/CPP and ptiO2 monitoring were evaluated. Median ICP, CPP, and ptiO2 at baseline were 12 ± 6.1 mm Hg, 86 ± 17.1 mm Hg, and 27 ± 14.3 mm Hg, respectively. Values for ICP, CPP, and ptiO2 did not change significantly when comparing mean values before, during, and after therapy. Conclusions Based on ptiO2 measurements, our data provide new information about the feasibility and safety of physiotherapy in patients with severe brain diseases.

Endovascular thrombectomy followed by decompressive surgery in a patient with cerebral venous sinus thrombosis (CVST)

A 45-year-old woman was admitted to our hospital due to headache and hypaesthesia of the left arm (GCS = 15). She developed over the course moderate dysarthria and hemiparesis with sensory and visual neglect on the left side. The initial cranial computer tomography (CCT) was inconspicuous. CT-angiography showed a segmental occlusion of the superior sagittal sinus (SSS) (Fig. 1a). Treatment was started with i.v Heparin (pTT = 70 s). When no improvement was observed on this therapy and patient developed hemiplegia without showing any infarction or bleeding in CCT (Fig. 1b), endovascular thrombectomy (Penumbra 041 catheter ) (ET) of the SSS was done with some residual clot in the distal segment (Fig. 1c, d). The next day patient developed anisocoria (GCS = 3). CCT-scan showed a right frontal intracranial hemorrhage with a consecutive midline shift of about 1 cm (Fig. 1e). ICP was elevated with values above 20 mmHg (intraparenchymatous-pressure probe: Neurovent-P; Raumedic ). Decompressive hemicraniectomy (DH) was carried out (Fig. 1f). Systemic heparinization was continued during ET and DH (pTT = 70 s). Due to the elevated ICP a multimodal therapy with deep sedation, analgesics and osmotic drugs was added. Heparinization was continued until oral anticoagulation was started 2 weeks after the initial event. Three weeks later there was a significant improvement in the clinical status of the patient with residual spastic hemiplegia on the left side and she was transferred to a rehabilitation clinic. Six weeks later the cranial bone flap was reimplanted. The patient was able to use a wheelchair with a hemiparesis on the left side. Five months after the initial event patient was ambulatory with a severe residual paresis of the left arm (Fig. 1g). The modified ranking scale was 3. A protein-S deficiency was detected. Protein-C, antithrombin III, lupus anticoagulant and factor V Leiden mutation tests were unremarkable.

Extensive ischemic brainstem lesions and pneumocephalus after application of hydrogen peroxide (H2O2) during lumbar spinal surgery

BACKGROUND CONTEXT The hemostatic properties of hydrogen peroxide (H2O2) are often used in neurosurgical practice. CASE REPORT We present the case of an 81-year-old woman who underwent lumbar spinal surgery (microsurgical decompression) in an external hospital. H2O2 was used during the procedure. The patient was transferred to our hospital. She remained unconscious postoperatively, with progressive loss of brainstem reflexes. Computed tomography showed intra- and extradurally trapped air ascending from the operated lumbar segment up to frontal lobe. Magnetic resonance imaging demonstrated severe brainstem lesions on T2- and diffusion-weighted series. The patient died 10 days after surgery. Autopsy was not performed. CONCLUSIONS Our case demonstrates a fatal complication with ischemic brainstem lesions and pneumocephalus after the use of hydrogen peroxide. Therefore, H2O2 should only be used in cases without any signs of dural injury.