Patients with spontaneous subarachnoid haemorrhage - presentation of a 10-year hospital series.

In this study we present the population-based patient material with spontaneous SAH, treated in our Neurosurgical unit during the 10 years (1997-2006) after the introduction of endovascular treatment of aneurysms (EVT) in our hospital. All patients that had a spontaneous SAH and a potential to survive were admitted. The present study comprises 1471 patients. 72 % had one or more aneurysms. AVM or other vascular pathological findings where found in 2%. Angiography was judged as normal in 21% and was not performed in 5%. 664 of the patients with aneurysms were treated by endovascular means, 333 were operated and 31 were treated with both methods. Thirty-five received no treatment. The main difference in admission parameters between the coiled and clipped aneurysms was the location of the aneurysm, with posterior circulation aneurysm exclusively being coiled and MCA aneurysms mainly clipped. The highest 6-month mortality for aneurysms presented in the untreated group (71.4%), and the lowest (3.2%) in the group treated with both clip and coil. Six-month mortality was 3.5% for the patients with a normal angiography. Of these, no one with Fisher grade 1 and 2/120 patients with Fisher 2 died. The mortality was high (89%) for those patients where angiography was not performed. The present patient group was compared to an earlier published a 12-year patient series from our clinic. The patients with aneurysmal SAH in the present series were older and had a lower 6-month mortality in our material compared to the published data from our clinic from 1981-1992. More patients were admitted in a good clinical condition in the present series, but there was also a higher proportion of patients from the worst clinical groups. The percentage of aneurysms treated had increased from 80% to 97% after EVT was introduced.

Frontotemporal dementia-amyotrophic lateral sclerosis complex is simulated by neurodegeneration with brain iron accumulation.

We describe a case of late onset neurodegeneration with brain iron accumulation (NBIA) presenting as frontotemporal dementia (FTD) with amyotrophic lateral sclerosis (ALS). A male patient presented at age 66 with change of personality: disinhibition, emotional blunting, and socially inappropriate behavior, coupled with dysarthria, dystonia, and corticospinal tract involvement. Magnetic resonance imaging showed general cortical atrophy, iron deposits in the globus pallidus, and the "eye of the tiger" sign. Neuropsychologic performance was globally reduced, especially executive functions. Fluorodeoxyglucose positron emission tomography showed hypometabolism predominantly in frontal and temporal areas. Repeated neurophysiologic examinations showed signs of chronic denervation. The patient was diagnosed with NBIA but fulfilled consensus criteria for FTD and had a clinical picture of ALS, without neurophysiologic confirmation. Our finding introduces NBIA as a possible cause of FTD and as a differential diagnosis of the FTD-ALS complex.