Ali Harati

Distal Posterior Inferior Cerebellar Artery Aneurysms: Clinical Features and Outcome of 80 Patients

OBJECTIVE Aneurysms of the posterior inferior cerebellar artery (PICA) distal to its origin are rare. Beside their rarity, their treatment is challenged by a high proportion of fusiform aneurysms, torturous course of PICA, and often severe bleeding. Our aim is to represent the characteristics of these aneurysms and their treatment, as well as to analyze outcome. METHODS We reviewed retrospectively 80 patients with PICA aneurysms who were treated at the Department of Neurosurgery, Helsinki, Finland. RESULTS The 80 patients had altogether 91 distal PICA aneurysms. Subarachnoid hemorrhage occurred in 74 (93%), and the distal PICA aneurysm was ruptured in 68 (85%). Compared with aneurysms at other locations, distal PICA aneurysms were smaller, more often fusiform, and more often caused an intraventricular hemorrhage as well as rebleeding. Modified surgical techniques (trapping, wrapping, proximal occlusion, resection, coagulation) were used in 10 (32%) fusiform and in 3 (6%) saccular aneurysms. Revascularization was needed in 3 (4%) cases. One aneurysm was primarily embolized. Within a year after aneurysm diagnosis, 18 patients had died. Among survivors, most returned to independent or previous state of living: 52 (91%); only 1 patient was unable to return home. CONCLUSIONS Microsurgery is a feasible treatment for distal PICA aneurysms. Despite the challenge of often severe hemorrhage, wide-necked aneurysms, and some risk for laryngeal palsy, most patients surviving the initial stage return to normal life. Because of the greater number of rebleedings than for aneurysms at other locations, immediate treatment is crucial.

Neuropsychological effects of deep brain stimulation for Parkinson's disease.

Background: Putative changes of cognition after deep brain stimulation (DBS) in patients with Parkinsons disease (PD) are a matter of debate. The aim of this study was to assess cognitive abilities before and following bilateral subthalamic nucleus (STN) DBS and to review the available literature. Methods: Twenty patients underwent bilateral DBS of the STN. Cognitive skills were assessed in a standardized fashion before and at least at 12 months after the surgical intervention. Results: There was a significant decline of both semantic and phonematic verbal fluency and a mild trend for a deterioration of verbal memory after DBS. Mood, general cognitive screening, and visospatial abilities remained unchanged. Conclusion: STN DBS in the treatment of PD has resulted in a significant reduction of motor symptoms and improved independence and quality of life in appropriately selected patients. However, it may have isolatable effects on verbal fluency and related function. Case series in the literature reported similar findings. Potential candidates for DBS should be counseled about the risk of mild cognitive declines.

Seventy Aneurysms of the Posterior Inferior Cerebellar Artery: Anatomical Features and Value of Computed Tomography Angiography in Microneurosurgery

OBJECTIVE Aneurysms at the junction of the vertebral artery (VA) and posterior inferior cerebellar artery (PICA) are relatively rare. Their treatment is challenged by the diverse anatomy of the VA and PICA, close involvement of the PICA in the aneurysm neck, and scant space deep in the posterior fossa next to the cranial nerves, brain stem, and obstructing structures of the skull base. Computed tomography angiography (CTA) visualizes bony structures in addition to the vasculature, and being noninvasive and easily available, it can serve for planning microsurgical treatment. We analyzed the anatomy of PICA aneurysms imaged by CTA to promote planning the treatment. METHODS We examined the CTA images of 70 consecutive patients with a saccular PICA aneurysm who were all treated in the Department of Neurosurgery, Helsinki, Finland, 2001 to 2011. RESULTS Each of the 70 patients had 1 aneurysm at the VA-PICA junction. The proportion of small aneurysms (<7 mm) was high, 67%. The dome-to-neck ratio was <1.2 in 24 (34%) of the aneurysms. In the coronal view, 58 (83%) aneurysms projected cranially, and only 2 (3%) caudally. The aneurysms were located from 1 mm below the foramen magnum to 31 mm above it, most arising at the level of the jugular tubercle. The median distance to the aneurysm from the midline was 6 mm, ranging from 7.5 mm contralateral to the origin of the parent artery to 14 mm ipsilateral. Compared with the right VA, the left VA was larger, it harbored the most aneurysms, and its aneurysms were more often ruptured. CONCLUSIONS Relation of PICA aneurysms to skull base structures is highly variable; the aneurysms can also be extracranial, or lie on the site of the skull contralateral to the origin of the parent artery. These anatomical variations demand meticulous study of the angiography in each individual case, especially before surgical treatment of the aneurysm.

Early microsurgical treatment for spinal hemangioblastomas improves outcome in patients with von Hippel–Lindau disease

Background: Spinal hemangioblastomas (HB) are rare, histologically benign, highly vascularized tumors often associated with von Hippel–Lindau (VHL) disease. The aim of the current study is to demonstrate the benefit of early surgical resection of large spinal HBs in selected asymptomatic patients with VHL. Methods: Seventeen patients underwent microsurgical resection of 20 spinal HBs at the Department of Neurosurgery at Helsinki University Central Hospital (HUCH). Thirteen tumors were in the cervical spine, five in thoracic and one patient had two lumbar lesions. MRI tumor showed an associated syrinx in 16 patients (94%). Tumor volume ranged from 27 to 2730 mm3. Out of 17 patients, 11 (65%) tested positive for VHL in mutation analysis. Five of these patients with tumors ranging from 55 to 720 mm3 were treated prophylactically. Results: Complete tumor resection was performed in 16 patients (94%) who were followed up for a median of 57 months (range 2–165 months). No patient had neurological decline on long-term follow-up. Among the patients with VHL, five patients with preoperative sensorimotor deficits showed improvement of their symptoms but never regained full function. One patient who presented with tetraplegia remained the same. Otherwise, all five patients with prophylactic surgery remained neurologically intact. Conclusion: Although documented growth on serial MRIs and the need for pathological diagnosis have been suggested as indications for surgery in otherwise asymptomatic patients, our series showed that a potentially larger group of asymptomatic patients with spinal HB associated with VHL would benefit from microsurgical resection. Long-term results of the surgical management of spinal HB are generally favorable. Our results suggest staging and early treatment for spinal HB larger than 55 mm3, especially in patients with VHL. Small spinal HBs may be followed up.

Anti‑proliferative activity of epigallocatechin‑3‑gallate and silibinin on soft tissue sarcoma cells

Disseminated soft tissue sarcomas (STS) present a therapeutic dilemma. The first-line cytostatic doxorubicin demonstrates a response rate of 30% and is not suitable for elderly patients with underlying cardiac disease, due to its cardiotoxicity. Well-tolerated alternative treatment options, particularly in palliative situations, are rare. Therefore, the present study assessed the anti-proliferative effects of the natural compounds epigallocatechin-3-gallate (EGCG), silibinin and noscapine on STS cells. A total of eight different human STS cell lines were used in the study: Fibrosarcoma (HT1080), liposarcoma (SW872, T778 and MLS-402), synovial sarcoma (SW982, SYO1 and 1273) and pleomorphic sarcoma (U2197). Cell proliferation and viability were analysed by 5-bromo-2′-deoxyuridine and MTT assays and real-time cell analysis (RTCA). RTCA indicated that noscapine did not exhibit any inhibitory effects. By contrast, EGCG decreased proliferation and viability of all cell lines except for the 1273 synovial sarcoma cell line. Silibinin exhibited anti-proliferative effects on all synovial sarcoma, liposarcoma and fibrosarcoma cell lines. Liposarcoma cell lines responded particularly well to EGCG while synovial sarcoma cell lines were more sensitive to silibinin. In conclusion, the green tea polyphenol EGCG and the natural flavonoid silibinin from milk thistle suppressed the proliferation and viability of liposarcoma, synovial sarcoma and fibrosarcoma cells. These compounds are therefore potential candidates as mild therapeutic options for patients that are not suitable for doxorubicin-based chemotherapy and require palliative treatment. The findings from the present study provide evidence to support in vivo trials assessing the effect of these natural compounds on solid sarcomas.

Clinical features, microsurgical treatment, and outcome of vestibular schwannoma with brainstem compression.

Background: Presenting symptoms, treatment considerations, and outcome are strongly related to the extension of vestibular schwannomas (VS). The aim of the current retrospective study was to analyze the clinical features, microsurgical treatment, and outcome of VS with brainstem compression. Methods: Forty-nine patients presented with VS (Hannover grading scale T4a or T4b) in our department. A subgroup analysis was performed among patients without (T4a) and with (T4b) compression and dislocation of the fourth ventricle. Results: Patients with type T4b VS presented significantly more often with long tract signs/ataxia (P < 0.05), tonsillar herniation (P < 0.001), and preoperative hydrocephalus (P < 0.01). No significant difference was found between the groups regarding hearing loss and facial nerve, trigeminal nerve, and lower cranial nerve function. Gross total resection was achieved in 83% of the cases, near total resection was achieved in 15% of the cases, and subtotal resection was performed in 2% of the cases. One patient died after massive postoperative bleeding caused by a coagulopathy. At last follow-up, 69% of the patients had excellent facial nerve function (Grade I–II) and the remaining 31% a fair outcome. Six patients (12%) required permanent ventriculoperitoneal shunting. Hearing was preserved in two patients. Forty-six patients (94%) were independent without occasional assistance (Karnofsky scale 70–100%). Conclusions: VS with brainstem compression is frequently associated with hydrocephalus, ataxia, long tract signs, multiple cranial nerve disorders, and occasionally, signs of intracranial hypertension. Primary microsurgical resection is an appropriate management option for large VS.

Microsurgical treatment of large and giant tympanojugular paragangliomas.

Background: Tympanojugular paragangliomas (TJPs) are benign, highly vascularized lesions located in the jugular foramen with frequent invasion to the temporal bone, the upper neck, and the posterior fossa cavity. Their natural history, surgical treatment, and outcome have been well addressed in the recent literature; however, there is no consensus regarding the optimal management while minimizing treatment-related morbidity. In this study, we assessed the interdisciplinary microsurgical treatment and outcome of large TJP collected at a single center. Methods: Out of 54 patients with skull base paraganglioma, 14 (25%) presented with large TJP (Fisch grade C and D). Posterior fossa involvement was present in 10 patients (Fisch D). Eleven patients presented with hearing loss, two patients with mild facial nerve palsy, and two patients with lower cranial nerve deficits. Two other patients with previous surgery presented with tumor regrowth. Results: Preoperative embolization was performed in 13 cases. Radical tumor removal was possible in 10 patients. Hearing was preserved in four patients with normal preoperative audiogram. The facial nerve was preserved in all patients. Temporary facial nerve palsy occurred in two patients and resolved in long-term follow-up. In three patients, preexisting facial nerve palsy remained unchanged. Persistent vocal cord palsy was present in three patients and was treated with laryngoplasty. The global recovery based on the Karnofsky performance scale was 100% in 10 patients and 90% in 4 patients. Conclusion: Preoperative embolization and interdisciplinary microsurgical resection are the preferred treatment for selected patients due to high tumor control rates and good long-term results.

Diadochokinetic movements differ between patients with Huntington's disease and controls.

BACKGROUND Assessment of impaired motor behaviour serves as tool for diagnosis and for evaluation of progression of chronic neurodegeneration. METHODS We measured performance of rapid alternating movements, simple and complex motion series with instruments in scored, drug naïve patients with Huntingtons disease and controls. OBJECTIVES To compare device outcomes with the ones of controls and to correlate them with each other and with the disease symptoms. RESULTS Patients showed lower peak velocities and longer intervals for performance of rapid alternating movements than the controls. On the right dominant side, a correlation was found between intervals needed for standardised alternating movement performance and the simple motion task outcomes in the patients. No relations to the rated disease symptoms appeared. CONCLUSION Execution of alternating movements is disturbed in Huntingtons disease. Performance of the simple motion task and realisation of alternating motions shares a need for low cognitive efforts. Both ask for a more automated motion execution. Therefore they were related to each other to a certain extent. Execution of complex movement series asks for higher cognitive load, as it demands aiming and planning of movement series with visual input. This motion pattern does not resemble to the realisation of rapid alternating movements.

New technique for C1-C2 fixation

Background: There are several techniques for treating atlantoaxial instability, including the Magerl transarticular screw fixation and the Harms/Goel C1-C2 screw rod techniques. Here, we present a novel technique utilizing a polyaxial screw rod system and a combination of C1 lateral mass and C1-C2 transarticular screws. Methods: We retrospectively reviewed 14 patients (7 women, 7 men; mean age 62) who underwent surgery for type II odontoid fractures (n = 7), pseudarthrosis after anterior odontoid screw placement (n = 3), Os odontoideum (n = 2), atlantoaxial instability after C3-C5 fusion (n = 1), and craniovertebral rheumatoid arthritis (n = 1). Ten patients underwent posterior C1-C2 fixation, three patients with osteoporosis had C1-C4 fixation, and one patient had C1-Th1 fixation. The mean follow-up time was 22 months. Results: Intraoperatively, there were no complications (e.g., vertebral artery, nerve root, or spinal cord injury). Postoperative imaging showed no screw malpositioning, and no screw loosening, fracture, or bone absorption around the screws. Furthermore, all patients exhibited postoperative improvement in neck pain. Conclusions: C1 lateral mass and C1-C2 transarticular polyaxial screw rod fixation techniques were effective in achieving immediate rigid immobilization of the C1-C2 motion segment.

Disease and Treatment-Related Sequelae in Patients with Complex Jugulotympanic Paraganglioma

Background: Jugulotympanic paraganglioma (JTP) are benign, high-vascularized lesions that frequently invade the jugular foramen, temporal bone, the upper neck, and the posterior fossa cavity, resulting in a wide variety of clinical symptoms. Methods: In this retrospective study, we assess the clinical symptoms and discuss the individual multidisciplinary treatment and outcome of 22 patients with JTP. Results: In 12 patients, a hearing deficit was the presenting symptom, whereas pulsatile tinnitus and otalgia were present in six and four patients respectively. Facial nerve involvement was seen in six patients (three HB Grade 1–2 and three HB Grade 4–6). Four patients presented with lower cranial nerve impairment. Rare symptoms were ataxia caused by brainstem compression and papilledema due to cerebral sinus obstruction. A new or worsening of the preoperative facial nerve or lower cranial nerve function occurred in two and four patients respectively. Conclusion: The treatment strategy and the surgical approach for JTP should be tailored to the tumor extension and the patient’s clinical symptoms.