Susanne Greschus

Mechanical Thrombectomy Compared to Local-Intraarterial Thrombolysis in Carotid T and Middle Cerebral Artery Occlusions

PurposeThe aim of the study was to examine the effects of mechanical thrombectomy using the Solitaire stent in patients with thromboembolic occlusions of the intracranial carotid artery bifurcation (carotid T) or middle cerebral artery (MCA) and to compare the results with a historical cohort treated with local intraarterial thrombolysis using urokinase.MethodsThe time intervals from stroke onset to treatment, recanalization rates, occlusion sites, recanalization times and functional outcomes on the modified Rankin scale at 3 months in 25 patients treated with the Solitaire stent between 2010 and 2011 were evaluated. The data were compared with those of a historical cohort of 62 patients treated with local intraarterial thrombolysis between 1992 and 2001.ResultsA total of 15 out of 25 (60%) patients treated with mechanical thrombectomy and 25 out of 62 (40%) treated with local intraarterial thrombolysis achieved a modified Rankin score of  £ 2 (p = 0.07). Occlusion sites, intervals from stroke onset to treatment and rates of parenchymal hematomas, 3 out of 25 (12%) versus 8 out of 62 (13%), were similar in both cohorts while the recanalization rate was significantly higher, 22 out of 25 (88%) versus 33 of 62 (53%), in the mechanical thrombectomy group (p £ 0.01).ConclusionThe data show that mechanical thrombectomy is superior to local intraarterial thrombolysis with respect to the recanalization rate in patients with thrombeoembolic carotid T or MCA occlusions.

Endovascular Treatment of Epistaxis: Indications, Management, and Outcome

ObjectiveEpistaxis is a common clinical problem, and the majority of bleedings can be managed conservatively. However, due to extensive and sometimes life-threatening bleeding, further treatment, such as superselective embolization, may be required. We report our experience with endovascular treatment of life-threatening epistaxis.MethodsAll patients presenting with excessive epistaxis, which received endovascular treatment at a German tertiary care facility between January 2001 and December 2009, were retrospectively identified. Demographic data, etiology, origin and clinical relevance of bleeding, interventional approach, therapy-associated complications, and outcome were assessed.ResultsA total of 48 patients required 53 embolizations. Depending on the etiology of bleeding, patients were assigned to three groups: 1) idiopathic epistaxis (31/48), 2) traumatic or iatrogenic epistaxis (12/48), and 3) hereditary hemorrhagic telangiectasia (HHT) (5/48). Eleven of 48 patients required blood transfusions, and 9 of these 11 patients (82%) were termed clinically unstable. The sphenopalatine artery was embolized unilaterally in 10 of 53 (18.9%) and bilaterally in 41 of 53 (77.4%) procedures. During the same procedure, additional vessels were embolized in three patients (3/53; 5.7%). In 2 of 53(3.8%) cases, the internal carotid artery (ICA) was occluded. Long-term success rates of embolization were 29 of 31 (93.5%) for group 1 and 11 of 12 (91.7%) for group 2 patients. Embolization of patients with HHT offered at least a temporary relief in three of five (60%) cases. Two major complications (necrosis of nasal tip and transient hemiparesis) occurred after embolization.ConclusionsEndovascular treatment proves to be effective for prolonged and life-threatening epistaxis. It is easily repeatable if the first procedure is not successful and offers a good risk–benefit profile.

Noninvasive imaging after stent-assisted coiling of intracranial aneurysms: comparison of 3-T magnetic resonance imaging and 64-row multidetector computed tomography--a pilot study.

Background and Purpose: Follow-up imaging after stent-assisted coiling of intracranial aneurysms is limited by signal loss in the stented vessel segment using magnetic resonance imaging or by streak artifacts caused by aneurysm coils using multidetector computed tomography. In the search for a noninvasive surveillance in this condition, we propose a technique to minimize streak artifacts in multidetector computed tomography by gated data reconstruction and shifting the reconstruction window. Methods: The effect of the gated data acquisition in 64-row computed tomographic angiography (gCTA) on artifact reduction was evaluated in a preliminary phantom study and compared with nongated CTA, time-of-flight magnetic resonance angiography (TOF-MRA), and digital subtraction angiography (DSA). Scans were also obtained from 5 patients treated with stent-assisted coiling as part of their follow-up protocol. The length of impaired vessel segments (LIVS) in TOF-MRA and gCTA was compared and correlated with the stents length, the number of coils, and the packing density. The assessment of treatment outcome in TOF-MRA and gCTA was compared with DSA as the standard of reference. Results: The phantom study revealed 2 aspects: first, a distinct reduction of streak artifacts caused by coils using gated data acquisition; and second, because artifact orientation could be rotated systematically by shifting the reconstruction window, visualization of treated vessel segments was significantly superior in gCTA. In magnetic resonance imaging, all stented vessel segments were characterized by signal loss in both phantom and patients. The LIVS was 78% shorter in gCTA (4.86 ± 6.93 mm) compared with that in TOF-MRA (21.82 ± 7.47 mm, P < 0.01). In TOF-MRA, the LIVS correlated with the stents length, in gCTA with the number of coils. With regard to assessment of treatment outcome, gCTA and TOF-MRA correlated with DSA in 3 and in none of 5 patients, respectively. Conclusions: Gated CTA is a promising technique to reduce the amount of artifacts induced by stent-assisted intracranial coils. Image quality and assessment of treatment outcome in patients with stent-assisted coiling is superior compared with TOF-MRA.

Posterior Reversible Encephalopathy Syndrome as an Overlooked Complication of Induced Hypertension for Cerebral Vasospasm Systematic Review and Illustrative Case

Background and Purpose— Delayed cerebral ischemia associated with cerebral vasospasm is a common cause of secondary neurological decline after aneurysmal subarachnoid hemorrhage (SAH). Triple-H therapy, induced hypertension, hypervolemia, and hemodilution, is often used to treat cerebral vasospasm. However, hypertensive treatment may carry significant medical morbidity, including cardiopulmonary, renal, and intracranial complications. Posterior reversible encephalopathy syndrome (PRES) is a reversible intracranial complication that has rarely been reported in the setting of induced hypertension. Methods— We present an illustrative case of PRES in a patient with induced hypertension for SAH-related cerebral vasospasm and performed a systematic review. Furthermore, the electronic database MEDLINE was searched for additional data in published studies of PRES after induced hypertension. Results— Overall, 7 case reports presenting 10 patients who developed PRES secondary to induced hypertension were found. Eighty-two percent of the patients were women. In all cases, the clinical symptoms were attributed to cerebral vasospasm before the diagnosis of PRES. The time from onset of induced hypertension to the development of PRES was 7.8±3.8 days. After the diagnosis of PRES and careful taper down of the blood pressure, the neurological symptoms resolved almost completely within a few days in all patients. Conclusions— PRES in the setting of SAH is an overlooked complication of hypertensive therapy for the treatment of vasospasm. However, the diagnosis of this phenomenon is crucial given the necessity to reverse hypertensive therapy, which is contrary to the usual management of patients with vasospasm.

Transarterial endovascular treatment in the management of life-threatening intra- and postoperative haemorrhages after otorhinolaryngological surgery

Management of life-threatening postsurgical bleeding is complex. If conservative or surgical therapy is demanding, an endovascular treatment can be considered. The goal of this study was to evaluate the outcome of endovascular approaches in the diagnosis and therapy of otherwise intractable postoperative haemorrhages with a study design of outcomes research. Charts of all patients with postsurgical bleedings receiving endovascular treatment were reviewed for clinical outcome, complications, and demographic data. 15 patients were identified. They had rhinosurgery (12/15), tonsillectomy (2/15) or transoral tumour debulking (1/15) prior to the endovascular procedure. In more than 70%, the source of bleeding was directly located angiographically and subsequently superselectively embolized. The remaining patients suffered from post-rhinosurgical epistaxis and underwent a bilateral embolization of the sphenopalatine artery. All bleedings were successfully controlled and no procedure-related complication was noted. In conclusion, endovascular treatment of life-threatening postsurgical haemorrhages should be considered if the source of bleeding is unknown or if surgery is difficult and may result in devastating postoperative complications.

Extracranial glioblastoma with synchronous metastases in the lung, pulmonary lymph nodes, vertebrae, cervical muscles and epidural space in a young patient - case report and review of literature

BackgroundExtraneural and extracranial metastases of glioblastoma (GB) are very rarely reported in the literature. They occur in only 0.2% of all GB patients.Case presentationWe present a 40 year old caucasian male with secondary GB and first diagnosis of an astrocytoma world health organisation (WHO) grade II through stereotactic biopsy in 2006. He presented a new hemiparesis and a progress of the known mass lesion in 2008. Subtotal tumor resection was performed and the histological examination verified a GB. After combined radio- and chemotherapy the adjuvant temozolomide therapy was not started because of non-compliance.In 2011 a second local relapse was resected and 4 month later the patient presented a fast progressing tetraparesis. Cervical CT and MRI scan showed a mass lesion infiltrating the fifth and sixth vertebra with infiltration of the spinal canal and large paravertebral tumor masses. Emergency surgery was performed. By additional screening further metastases were detected in the thoracal and lumbal spine and surprisingly also in the lung and pulmonary lymphnodes. Palliative radio- and chemotherapy of the pulmonal lesions was completed, further antitumor therapy was rejected. The patient died 10 months after diagnosis of the extraneural metastases.ConclusionEspecially young “long-term-survivors” seem to have a higher risk of extraneural metastasis from a GB and appropriate staging should be performed in these cases.

Hypomorphic mutations in POLR3A are a frequent cause of sporadic and recessive spastic ataxia

Despite extensive efforts, half of patients with rare movement disorders such as hereditary spastic paraplegias and cerebellar ataxias remain genetically unexplained, implicating novel genes and unrecognized mutations in known genes. Non-coding DNA variants are suspected to account for a substantial part of undiscovered causes of rare diseases. Here we identified mutations located deep in introns of POLR3A to be a frequent cause of hereditary spastic paraplegia and cerebellar ataxia. First, whole-exome sequencing findings in a recessive spastic ataxia family turned our attention to intronic variants in POLR3A, a gene previously associated with hypomyelinating leukodystrophy type 7. Next, we screened a cohort of hereditary spastic paraplegia and cerebellar ataxia cases (n = 618) for mutations in POLR3A and identified compound heterozygous POLR3A mutations in ∼3.1% of index cases. Interestingly, >80% of POLR3A mutation carriers presented the same deep-intronic mutation (c.1909+22G>A), which activates a cryptic splice site in a tissue and stage of development-specific manner and leads to a novel distinct and uniform phenotype. The phenotype is characterized by adolescent-onset progressive spastic ataxia with frequent occurrence of tremor, involvement of the central sensory tracts and dental problems (hypodontia, early onset of severe and aggressive periodontal disease). Instead of the typical hypomyelination magnetic resonance imaging pattern associated with classical POLR3A mutations, cases carrying c.1909+22G>A demonstrated hyperintensities along the superior cerebellar peduncles. These hyperintensities may represent the structural correlate to the cerebellar symptoms observed in these patients. The associated c.1909+22G>A variant was significantly enriched in 1139 cases with spastic ataxia-related phenotypes as compared to unrelated neurological and non-neurological phenotypes and healthy controls (P = 1.3 × 10-4). In this study we demonstrate that (i) autosomal-recessive mutations in POLR3A are a frequent cause of hereditary spastic ataxias, accounting for about 3% of hitherto genetically unclassified autosomal recessive and sporadic cases; and (ii) hypomyelination is frequently absent in POLR3A-related syndromes, especially when intronic mutations are present, and thus can no longer be considered as the unifying feature of POLR3A disease. Furthermore, our results demonstrate that substantial progress in revealing the causes of Mendelian diseases can be made by exploring the non-coding sequences of the human genome.

Surgical Treatment of Spinal Dural Arteriovenous Fistula: Management and Long-Term Outcome in a Single-Center Series.

OBJECTIVE To perform a retrospective chart review of surgically treated patients with spinal dural arteriovenous fistula (SDAVF), a rare disease but the most common vascular malformation of the spine, focusing on clinical characteristics and functional outcome during long-term follow-up. METHODS Between June 1990 and April 2012, 29 patients with SDAVFs were treated surgically in a single institution. Patient characteristics, time from onset of symptoms to treatment, radiologic features, treatment-related complications, and functional outcome were analyzed. Outcome was assessed according to the Aminoff-Logue scale during follow-up. Results of magnetic resonance imaging scans performed during long-term follow-up were correlated with functional outcome. RESULTS There were 3 female (10%) and 26 male (90%) patients with SDAVFs treated surgically. Mean age was 61 years ± 11. Location of the fistula was at the thoracic level in 20 patients (69%), at the lumbar level in 8 patients (28%), and at the sacral level in 1 patient (3%). Mean postoperative Aminoff-Logue scale scores regarding gait and micturition improved after treatment compared with preoperatively (P = 0.02; P = 0.03). After surgical treatment, 22 patients (76%) achieved improvement in neurologic symptoms. In 6 patients (21%), neurologic status was the same as preoperatively. In 1 patient (3%), neurologic status worsened. Medullary signal alteration of diagnostic magnetic resonance imaging scans did not correlate with functional outcome (P = 0.2). Mean follow-up time was 63 months ± 55. All SDAVFs were treated in a single session without recurrence during the long-term follow-up period. CONCLUSIONS Surgical treatment of SDAVFs is safe and effective and leads to an improvement of neurologic symptoms in most patients. Surgical treatment of SDAVFs also provides long-term stability.

The Enterprise Stent for the Treatment of Intracranial Aneurysms

AbstractBackground:Self-expanding microstents are typically placed before a wide-necked aneurysm is filled with coils. Alternatively, the stent may be placed at the end of the procedure, when the coil mass blocks or slows down the flow in the parent artery or a branching vessel.Patients and Methods:Between March 2007 and 2009, 20 aneurysms in 20 patients were treated with a total of 21 Enterprise stents. Nine aneurysms had reopened after coiling, eleven aneurysms were primarily treated, seven after acute subarachnoid hemorrhage. Eight aneurysms were primarily filled with coils and the stent was placed when the flow in the parent or a branching artery was blocked or slowed down. In four of these cases in which secondary stenting was considered an option in advance, the “artery at risk” was initially catheterized with a microcatheter.Results:Stent placement was successful in 19 aneurysms. In a reopened basilar tip aneurysm, Y-like stent placement through a Neuroform stent failed. With secondary stent placement (n = 8), all affected vessels could be safely reopened. None of these patients suffered from transient or permanent neurologic deficits. Using the jailing technique, it was possible to place two parallel stents in the internal carotid artery and posterior cerebral artery (PCA) in a reopened wide-necked PCA aneurysm.Conclusion:Apart from the primary use of the Enterprise stent in wide-necked aneurysm secondary stenting after coiling is a feasible technique to reopen occluded parent or branching arteries. Initial microcatheter placement in the artery which is at risk to get occluded when filling the aneurysm is a valuable option.ZusammenfassungHintergrund:Selbstexpandierende Mikrostents werden typischerweise platziert, bevor weithalsige Aneurysmen mit Platinspiralen gefüllt werden. Alternativ kann der Stent am Ende der Untersuchung platziert werden, wenn der Fluss im Trägergefäß oder in einem größeren abgehenden Gefäß blockiert wird oder abnimmt. Patienten und Methodik:Zwischen März 2007 und 2009 wurden 20 Aneurysmen bei 20 Patienten mit insgesamt 21 Enterprise- Stents versorgt. Neun Aneurysmen waren wiedereröffnet, elf Aneurysmen wurden primär behandelt, sieben davon nach frischer Subarachnoidalblutung. Acht Aneurysmen wurden primär mit Platinspiralen gefüllt, und der Stent wurde platziert, nachdem der Fluss im Trägergefäß oder in einem größeren Ast blockiert wurde oder abnahm. Bei vier dieser Patienten wurde die verschlussgefährdete Arterie primär mit einem Mikrokatheter markiert.Ergebnisse:Der Stent wurde bei 19 Patienten an der gewünschten Position freigesetzt. Bei einem Patienten misslang eine Y-förmige Stentimplantation über einen Neuroform-Stent mit Jailing des Basilariskopf-Aneurysmarests. Nach sekundärer Stentimplantation wurden alle Gefäße wiedereröffnet, kein Patient erlitt ein transientes oder permanentes neurologisches Defizit. Mit Hilfe der Jailing-Technik war es in einem Fall eines weithalsigen Aneurysmarezidivs der A. communicans posterior möglich, Stents parallel von der A. carotis interna in die A. cerebri media und die A. cerebri posterior zu platzieren.Schlussfolgerung:Neben der primären Stentimplantation vor Coiling ist die sekundäre Stentimplantation nach Coiling eine alternative Technik, um verschlossene Gefäße wiederzueröffnen. Zudem können verschlussgefährdete Arterien primär mit einem Mikrokatheter markiert werden.

Non‐paraneoplastic limbic encephalitis and central nervous HHV‐6B reactivation: Causality or coincidence?

Autoantibody‐related encephalopathies represent an important differential diagnosis in adult onset epilepsy. Here, we report the case of a 25‐year‐old patient with new‐onset epilepsy and psychotic syndrome, who underwent biopsy resection for etiological classification. MRI analysis and neuropathological examination showed a T‐lymphocytic dominated encephalitis with involvement of the limbic system. An indirect immunohistochemistry approach identified autoantibodies against glutamic acid decarboxylase (GAD) in cerebral spinal fluid and serum, which were confirmed by affinity purification / mass spectrometry analysis. Further examinations revealed evidence of chromosomally integrated human herpes virus type 6B (HHV‐6B). However, astrocytic expression of HHV‐6 lytic protein was detected by double immunofluorescence analysis. The cerebral expression of HHV‐6 antigen, a clinical improvement under antiviral therapy as well as an initial finding of HHV‐6 IgM antibodies strongly argue for an additional active HHV‐6B infection. Review of the literature reveals singular reports of patients with GAD antibody‐positive limbic encephalitis and central nervous system infections with HHV‐6B. Since herpes simplex virus encephalitis has been recently reported as a trigger of N‐methyl‐D‐aspartate receptor antibody encephalitis, it is tempting to speculate that HHV‐6B infections may trigger a non‐paraneoplastic form of limbic encephalitis in a parallel cascade.