Bypass Surgery for Complex Internal Carotid Artery Aneurysms : 39 Consecutive Patients

OBJECTIVE: Bypass surgery is a special technique used to treat complex internal carotid artery (ICA) aneurysms. The aim of this retrospective study is to provide a comprehensive description of treatment and outcome of complex ICA aneurysms at different ICA segments (cavernous, supraclinoid, ICA bifurcation) treated with bypass procedures. METHODS: We identified 39 consecutive patients with 41 complex ICA aneurysms that were treated with 44 bypass procedures between 1998 and 2016. We divided the aneurysms into 3 anatomic subgroups to review our treatment strategy. All the imaging studies and medical records were reviewed for relevant information. RESULTS: The aneurysm occlusion (n = 34, 83%) or flow modification (n = 5, 12%) was achieved in 39 aneurysms (95%). The long-term bypass patency rate was 68% (n = 30). Minor postoperative ischemia or hemorrhage was commonly seen (n = 20, 51%), but large-scale strokes were rare (n = 1, 3%). Preoperative dysfunction of extraocular muscles (cranial nerves III, IV, and VI) showed low-to-moderate improvement rates (20%-50%). Preoperative vision disturbance (cranial nerve II) improved seldom (22%). At the latest follow-up (mean; 51 months) 29 patients (74%) were independent (modified Rankin Scale CONCLUSIONS: Bypass surgery for complex ICA aneurysms is a feasible treatment method with an acceptable risk profile. Patients should be informed of the uncertainty related to improvement of pretreatment cranial nerve dysfunctions.Peer reviewe

Moyamoya angiopathy: radiological follow-up findings in Finnish patients

Moyamoya angiopathy (MMA) is a chronic progressive disorder, but imaging changes observed over time are not yet characterized in European populations. We analyzed the progression of MMA with magnetic resonance imaging and angiography (MRI and MRA) in our Finnish MMA registry. Stage classification based on MRA findings was used to evaluate the progress of the disease.Peer reviewe

Moyamoya vasculopathy - Patient demographics and characteristics in the Finnish population

Background and purpose Moyamoya vasculopathy, a rare steno-occlusive progressive cerebrovascular disorder, has not been thoroughly studied in Caucasian populations. We established a registry of Finnish patients treated at the Helsinki University Hospital, to collect and report demographic and clinical data. Methods We collected data both retrospectively and prospectively from all the patients with a moyamoya vasculopathy referred to our hospital between January 1987 and December 2014. All patients underwent a neurological outpatient clinic visit. Results We diagnosed 61 patients (50 females, 10 children) with moyamoya vasculopathy. The mean age at the disease-onset was 31.517.9 years. The two most common presenting symptoms were ischemic stroke (n=31) and hemorrhage (n=8). Forty-four percent underwent revascularization surgery, and 70% were prescribed antithrombotic treatment. Conclusions The results support in part the Western phenotype of the disease considering the later presentation and larger female predominance compared to the Asian moyamoya vasculopathy reports. However, the proportion of ischemic strokes and hemorrhagic strokes is closer to Japanese population than German population. The absence of familial cases points to a different genetic profile in the Finnish patients.Peer reviewe

Genetic and Epigenetic Characterization of Growth Hormone-Secreting Pituitary Tumors

Somatic driver mechanisms of pituitary adenoma pathogenesis have remained incompletely characterized; apart from mutations in the stimulatory G alpha protein (G alpha(s) encoded by GNAS) causing activated cAMP synthesis, pathogenic variants are rarely found in growth hormone-secreting pituitary tumors (somatotropinomas). The purpose of the current work was to clarify how genetic and epigenetic alterations contribute to the development of somatotropinomas by conducting an integrated copy number alteration, whole-genome and bisulfite sequencing, and transcriptome analysis of 21 tumors. Somatic mutation burden was low, but somatotropinomas formed two subtypes associated with distinct aneuploidy rates and unique transcription profiles. Tumors with recurrent chromosome aneuploidy (CA) were GNAS mutation negative (Gsp(-)). The chromosome stable (CS) -group contained Gsp(+) somatotropinomas and two totally aneuploidy-free Gsp(-) tumors. Genes related to the mitotic G(1)-S-checkpoint transition were differentially expressed in CA- and CS-tumors, indicating difference in mitotic progression. Also, pituitary tumor transforming gene 1 (PTTG1), a regulator of sister chromatid segregation, showed abundant expression in CA-tumors. Moreover, somatotropinomas displayed distinct Gsp genotypespecific methylation profiles and expression quantitative methylation (eQTM) analysis revealed that inhibitory G alpha (G alpha(i)) signaling is activated in Gsp(+) tumors. These findings suggest that aneuploidy through modulated driver pathways may be a causative mechanism for tumorigenesis in Gsp(-) somatotropinomas, whereas Gsp(+) tumors with constitutively activated cAMP synthesis seem to be characterized by DNA methylation activated G alpha(i) signaling.Peer reviewe

Aivolisäkekasvainten hoito

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Moyamoya angiopathy: long-term follow-up study in a Finnish population

Moyamoya angiopathy (MMA) is a chronic cerebrovascular disorder predominantly starting in childhood or early adulthood and thus affects the whole lifetime. Little is known on MMAs long-term outcomes in European patients. We report long-term follow-up data on Finnish MMA patients.Peer reviewe

Mutation Analysis of Inhibitory Guanine Nucleotide Binding Protein Alpha (GNAI) Loci in Young and Familial Pituitary Adenomas

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