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Matti Tapio Seppälä, Markku Alarik Sainio, Matti Jouko Johannes Haltia, Jaakko Jyri Kinnunen, Kirsi Hannele Setälä and Juha Erik Jääskeläinen

Object. The aim of this study was to clarify the clinical outcome of schwannomatosis, a rare condition characterized by multiple nonvestibular schwannomas in the absence of meningiomas, intraspinal ependymomas, and other clinical signs of neurofibromatosis type 2 (NF2).

Methods. Nine patients with schwannomatosis treated at one institution are presented and their clinical course during a median follow-up time of 9.9 years is discussed. The patients were typically middle-aged at the time of their first operation (median 43.5 years), none had a positive family history of schwannomatosis or NF2, and none showed cutaneous or ocular signs of NF2. On histopathological examination the tumors from the patients with schwannomatosis showed a lobular appearance and frequent Verocay bodies, signs indicating NF2, more often than 20 sporadic schwannomas that were investigated as controls. Two patients died of unrelated causes at 3.2 and 9.9 years, respectively, of follow up. Magnetic resonance images of the head and spine were obtained in seven patients at the end of the follow-up period. New spinal schwannomas were detected in one patient and a residual schwannoma in three. No germline mutations of the NF2 gene were found in these seven patients. Two additional patients originally included in the schwannomatosis group who were 8.6 and 11.7 years old at initial surgery had NF2. One was diagnosed at follow-up review and the other developed a fulminant disease that led to death in 4 years.

Conclusions. The clinical course, long-term outcome, and genetic mechanism of schwannomatosis differ from that of NF2.

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Juri Kivelev, Elina Koskela, Kirsi Setälä, Mika Niemelä and Juha Hernesniemi

Object

Cavernomas in the occipital lobe are relatively rare. Because of the proximity to the visual cortex and incoming subcortical tracts, microsurgical removal of occipital cavernomas may be associated with a risk of visual field defects. The goal of the study was to analyze long-term outcome after operative treatment of occipital cavernomas with special emphasis on visual outcome.

Methods

Of the 390 consecutive patients with cavernomas who were treated at Helsinki University Central Hospital between 1980 and 2011, 19 (5%) had occipital cavernomas. Sixteen patients (4%) were surgically treated and are included in this study. The median age was 39 years (range 3–59 years). Seven patients (56%) suffered from hemorrhage preoperatively, 5 (31%) presented with visual field deficits, 11 (69%) suffered from seizures, and 4 (25%) had multiple cavernomas. Surgery was indicated for progressive neurological deterioration. The median follow-up after surgery was 5.25 years (range 0.5–14 years).

Results

All patients underwent thorough neuroophthalmological assessment to determine visual outcome after surgery. Visual fields were classified as normal, mild homonymous visual field loss (not disturbing the patient, driving allowed), moderate homonymous visual field loss (disturbing the patient, driving prohibited), and severe visual field loss (total homonymous hemianopia or total homonymous quadrantanopia). At the last follow-up, 4 patients (25%) had normal visual fields, 6 (38%) had a mild visual field deficit, 1 (6%) complained of moderate visual field impairment, and 5 (31%) had severe homonymous visual field loss. Cavernomas seated deeper than 2 cm from the pial surface carried a 4.4-fold risk of postoperative visual field deficit relative to superficial ones (p = 0.034). Six (55%) of the 11 patients presenting with seizures were seizure-free postoperatively. Eleven (69%) of 16 patients had no disability during the long-term follow-up.

Conclusions

Surgical removal of occipital cavernomas may carry a significant risk of postoperative visual field deficit, and the risk is even higher for deeper lesions. Seizure outcome after removal of these cavernomas appeared to be worse than that after removal in other supratentorial locations. This should be taken into account during preoperative planning.

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Elina Koskela, Johanna Pekkola, Riku Kivisaari, Tero Kivelä, Juha Hernesniemi, Kirsi Setälä and Aki Laakso

Object

Terson's syndrome (TS) is a vitreous hemorrhage in association with subarachnoid hemorrhage (SAH). Its diagnosis is often delayed, which may result in vision loss secondary to treatable conditions. Methods to hasten early diagnosis and consequent ophthalmic referral are desirable. The aims of this study were 1) to assess the specificity and sensitivity of conventional head CT for diagnosing TS in patients with aneurysmal SAH (aSAH); and 2) to determine the incidence of TS and its association with age, sex, aSAH severity, and overall mortality.

Methods

Patients admitted to Helsinki University Central Hospital who underwent surgery or endovascular treatment for a ruptured intracranial aneurysm during 2011 were participants in this prospective study. They underwent serial dilated fundoscopic examinations during a 6-month period. Two radiologists independently reviewed ocular findings suggestive of TS on conventional CT head scans obtained in all patients as a routine diagnostic procedure. Associations between TS and relevant clinical, radiological, and demographic data were analyzed with uni- and multivariate logistic regression.

Results

Of 121 participants, 13 (11%) presented with TS, and another 22 (18%) with intraretinal hemorrhages. For reviewing CT head scans, the overall observed agreement between the 2 raters was 96% (116 of 121 cases), with a substantial κ of 0.69 (95% CI 0.56–0.82). The sensitivity of the CT findings for TS was 42%, and the specificity was 97%. Associations of the World Federation of Neurosurgical Societies (WFNS) and Hunt and Hess grades on admission, the presence of intracerebral hemorrhage, female sex, and aneurysm length with TS were all statistically significant. Logistic regression demonstrated that sex and WFNS grade were independently associated with TS and provided the best fit to the data.

Conclusions

Routinely looking for TS findings in CT head scans may prove valuable in clinical practice. Terson's syndrome is associated with female sex and poor clinical condition on admission.