Clinical Neurology and Neurosurgery

Editorial Board

2010-04-01

Management of meningiomas

George A. Alexiou, Pinelopi Gogou, Sofia Markoula, Athanasios P. Kyritsis — 2010-04-01

Abstract: The primary treatment of meningiomas is surgery which can be curative if the tumor is completely removed. For parasagittal, lateral sphenoid wing and olfactory groove meningiomas, gross-total resection should be the goal. Tuberculum and diaphragma sella meningiomas can be resected through the subfrontal or the pterional approaches. In meningiomas of the sphenoid wing with osseous involvement or involvement of the cavernous sinus subtotal resection can be achieved via several surgical approaches. Similarly, subtotal resection rather than gross-total resection of meningiomas of the petroclival, parasellar, and posterior fossa regions can preserve neurological function. Prior to surgery, embolization may reduce intraoperative bleeding and prevent postoperative complications. Stereotactic radiosurgery can be used as an alternative treatment to surgery either as a first-line treatment or at recurrence. Various conventional radiotherapy techniques can be employed for residual tumor post surgery or at recurrence. Chemotherapy has modest activity and is reserved for selected cases.

Short-course whole-brain radiotherapy (WBRT) for brain metastases due to small-cell lung cancer (SCLC)

2010-04-01

Abstract: Objective: Many patients with brain metastases due to SCLC have a poor survival prognosis. The most common treatment is whole-brain radiotherapy (WBRT). This retrospective study compares short-course WBRT with 5×4Gy in 1 week to standard WBRT with 10×3Gy in 2 weeks.Methods: Forty-four SCLC patients receiving WBRT with 5×4Gy were compared to 102 patients receiving 10×3Gy for survival (OS) and local (intracerebral) control (LC). Seven further potential prognostic factors were investigated: age, gender, Karnofsky Performance Score (KPS), number of brain metastases, extracerebral metastases, interval from tumor diagnosis to WBRT, RPA (Recursive Partitioning Analysis) class.Results: After 5×4Gy, 12-month OS was 15%, versus 22% after 10×3Gy (p=0.69). On multivariate analysis, improved OS was associated with age ≤60 years (p=0.013), KPS ≥70 (p<0.001), <4 brain metastases (p=0.011), and RPA class 1 (p<0.001). 12-month LC was 34% after 5×4Gy versus 25% after 10×3Gy (p=0.32). On multivariate analysis, improved LC was associated with KPS ≥70 (p<0.001), <4 brain metastases (p=0.027), and RPA class 1 (p<0.001).Conclusion: In patients with brain metastases due to SCLC, short-course WBRT with 5×4Gy provided similar outcomes as 10×3Gy and appears preferable, particularly for patients with poor estimated survival.

Neurological complications of miliary tuberculosis

2010-04-01

Abstract: Introduction: The symptomatic central nervous system involvement is often seen in patients with miliary tuberculosis.Materials and methods: In this study, we evaluated 60 consecutive miliary tuberculosis patients, who presented with some neurological manifestations. Evaluation included neurological examination, a battery of blood tests, HIV serology, sputum examination, cerebrospinal fluid (CSF) examination along with imaging of the brain and spinal cord. The patients were followed up after completion of 6 months of antituberculous treatment.Results: Patients ranged between 14 and 53 years in age. Three patients tested HIV positive. Forty-eight (80%) patients had tuberculous meningitis. In 12 (20%) patients, the CSF examination was normal. In 27 patients with tuberculous meningitis, neuroimaging revealed intracerebral tuberculoma. Fourteen patients showed multiple tuberculomas, while 7 had a solitary tuberculoma. In six patients, the tuberculomas were small and numerous. In two patients, neuroimaging revealed a spinal tuberculoma. For three patients with tuberculous brain masses, the CSF was normal. Nine (15%) patients presented with myelopathy. Three patients exhibited Pott's paraplegia. Three patients had transverse myelitis (with normal neuroimaging). In three patients, the spinal MRI revealed an intramedullary tuberculoma. On follow-up, 15 (25%) patients expired. Thirty-one (52%) patients showed significant improvement. Eight patients (13%) showed nil or partial recovery. Six of the patients with no improvement developed vision loss. Six (10%) patients were lost to follow up.Conclusion: A variety of neurological complications were noted in military tuberculosis patients, tuberculous meningitis and cerebral tuberculomas being the most frequent complications. However, a majority of patients improved following antituberculous treatment.

Could Helicobacter pylori play an important role in axonal type of Guillain-Barré Syndrome pathogenesis?

2010-04-01

Abstract: In this case-control study, ELISA and Western blot with whole bacterial protein lysate were performed on serum and cerebrospinal fluid (CSF) of 15 controls and 15 patients. According to Griffin subtypes, 10 of our patients were in acute inflammatory demyelinating polyradiculoneuropathy (AIDP) group, 3 in acute motor axonal neuropathy (AMAN) group, and 2 in acute motor sensory axonal neuropathy (AMSAN) subtype. 86.6% of patients were positive for Helicobacter pylori (H. pylori) IgG. Serum anti-H. pylori IgG of patients and controls were significantly different. CSF anti-H. pylori IgG was significantly higher in patients than controls. In patients, the titer of anti-H. pylori IgG in serum was significantly higher than CSF, which may indicate extra-neural antibody synthesis. CSF IgG titer was higher in patients having axonal pattern. Western blot was positive in CSF of 13 patients and negative in all controls. There was a correlation between the number of antibody types against H. pylori particles and the titer of anti-H. pylori IgG in CSF and serum. Also, antibody against cytotoxin associated protein (CagA) was associated with primary axonal pattern.The association between the presence of anti-CagA and primary axonal pattern, is in favor of the relation between axonal neuropathy and H. pylori infection.

Epidemiological and clinical features of Moyamoya disease in Nanjing, China

2010-04-01

Abstract: Objective: The epidemiology of Moyamoya disease in mainland China has not been documented. Therefore, the present study was designed to examine the epidemiological and clinical features of Moyamoya disease in Nanjing, a provincial capital in China.Methods: Patient records from multiple hospitals in Nanjing from January 2000 to December 2007 were collected. The clinical features of Moyamoya disease were retrospectively analyzed.Results: A total of 202 patients were identified. There were 94 males and 108 females, with ages ranging from 2 to 78 years. There was a dual age peak, one in the group of patients 5–9 years of age and another in the group of patients 35–39 years of age. The initial symptoms included cerebral ischemia (81 patients, 40%), cerebral hemorrhage (113 patients, 55.9%) and asymptomatic disease (8 patients, 3.9%). An increasing incidence rate of Moyamoya disease was observed during the period of 2000–2007, with an average detection rate of 0.43cases/100,000 persons/year (prevalence 3.92/100,000 persons). The incidence of ischemia associated with the disease was 0.16cases/100,000 people-years and the incidence of hemorrhage was 0.22cases/100,000 people-years.Conclusion: This first study on the epidemiological and clinical features of Moyamoya disease in mainland China indicated an increasing incidence of Moyamoya disease with bimodal incidence distribution appearing more frequently in adults.

TrueFISP imaging of the pineal gland: More cysts and more abnormalities

Ingo Nolte, Marc A. Brockmann, Lars Gerigk, Christoph Groden, Johann Scharf — 2010-04-01

Abstract: Introduction: Although pineal cysts are found with a frequency of over one third in autopsy series, prevalences reported in standard magnetic resonance imaging (MRI) studies only range between 0.14% and 4.9%. With the advances in scanner technology and more sensitive high-resolution 3D-sequences, pineal cysts with atypical appearance are more frequently encountered as an incidental finding. In order to help the radiologist and the clinician to correctly interpret these incidental findings and to avoid follow-up MRI or even surgical intervention, we analysed the frequency of typical and atypical pineal cysts using standard MRI-sequences and a high-resolution 3D-trueFISP-sequence (true-Fast-Imaging-with-Steady-State-Precession).Methods: In 111 patients undergoing MRI we analysed the prevalence of pineal cysts in relation to gender and age, as well as the frequency of atypical cysts defined by thickened rim, trabeculations, or asymmetric form using three standard MRI-sequences (T1-SE (T1 weighted spin echo), T2-TSE (T2 weighted turbo spin echo), FLAIR (fluid attenuated inversion recovery)) and compared the diagnostic certainty of these standard sequences with the sensitivity of a high-resolution trueFISP MRI sequence.Results: Using trueFISP pineal cysts were detected more frequently than in the standard sequences (35.1% vs. 9.0% (T1-SE), 4.5% (T2-TSE) and 9.0% (FLAIR)). Diagnostic uncertainty was least frequent in trueFISP. In trueFISP, 41.0% of the detected cysts showed one or more features of atypical cysts (standard sequences: 21.4%). Highest prevalence of cysts was detected in the group of 20–30-year-old patients and decreased with increasing age.Conclusion: High-resolution 3D-sequences like trueFISP increase the detection rate of pineal cysts to levels reported in autoptic series while decreasing the diagnostic uncertainty. Atypically configurated pineal cysts are frequently detected as an incidental finding.

Isolated degeneration of the posterior column as a distinct entity—A clinical and electrophysiologic follow-up study

O. Kastrup, D. Timman, H.C. Diener — 2010-04-01

Abstract: Objective: The aim of the study was to better describe the long term clinical course and electrophysiologic and radiologic findings in isolated degeneration of the posterior column.Methods: Four patients with the presenting symptoms of a progressive tabetic ataxia were followed up clinically and electrophysiologically over up to 15 years between 1997 and 2008. They received standardized neurological examinations, electrophysiologic testing with SEP, MEP, NCV, EMG, autonomic testing and cardiac evaluation, head and spine MRI, laboratory evaluation including CSF analysis.Results: Progressive gait ataxia due to pallhypasthesia and loss of position sense with areflexia remained the only symptoms. Pes cavus deformity was a notable clinical feature in all cases. There was no involvement of other systems and all patients remained fully ambulatory. There was no cardiac involvement. Electrophysiology was characterized by absent cortical tibial SEP with normal lumbar complexes and normal nerve conduction studies and transcortical magnetic stimulation as well as sympathetic skin response. MRI of the cord was normal. Laboratory analysis and CSF were unrevealing.Conclusion: Isolated degeneration of the posterior column is a rare condition with a clinically benign course without progression involving other systems and characteristic electrophysiologic findings (isolated loss of cortical tibial-SEP with normal lumbar leads). Pes cavus deformity seems to be an unusual but typical clinical feature. The etiology is most likely a sporadic degenerative disease of the cord.

Effects of subthalamic nucleus deep brain stimulation on sweating function in Parkinson's disease

2010-04-01

Abstract: Purpose: To assess the impact of subthalamic nucleus (STN) deep brain stimulation (DBS) on the sweating function in patients with advanced Parkinson's disease (PD).Methods: Nineteen patients with idiopathic PD (mean age±SD, 61.58±9.47) were examined immediately before and 6 months after DBS. Each examination session included registration of autonomic symptoms by means of a semi-structural questionnaire and recording of sympathetic skin response (SSR) from both palms and one sole. The neurophysiological measurements were compared to those of 19 matched for sex and age healthy controls.Results: Six months post-DBS motor improvement was amounted to 65.9% and the daily levodopa equivalent dose was decreased by 36.4%. Post-operatively, dyshidrosis manifestations were reduced by 66.7% (pre-DBS sudomotor dysfunction in 47.4% of patients and sudomotor fluctuation in 57.1% of the above patients). There were no significant differences in-between pre- and post-DBS results of SSR study. However, the number of patients with at least one abnormal SSR pre-operative was reduced from 6 to 3 post-operative. No correlation was found between this neurophysiological finding and the change of clinical symptoms of hyperhidrosis or the DBS motor improvement.Conclusions: These results, although based on a small sample, suggest that STN DBS, in addition to the effect to the mobility, might also favorably regulate sweat in idiopathic PD.

Differential diagnosis of intracranial ring enhancing cystic mass lesions—Role of diffusion-weighted imaging (DWI) and diffusion-tensor imaging (DTI)

2010-04-01

Abstract: Objective: To evaluate the contribution of DWI and DTI to the differential diagnosis of cerebral ring enhancing lesions by describing DWI and ADC (apparent diffusion coefficient) findings and measuring the two DTI parameters mean diffusivity (MD) and fractional anisotropy (FA).Materials and methods: A total of 17 patients presenting with 26 rim enhancing cysts were investigated with DWI and DTI. Parameter maps of the DTI metrics MD and FA were calculated and quantified using regions of interest (ROIs).Results: Five patients suffered from abscesses with a total of 10 cysts, 9 from glioblastomas with 10 cysts, and 3 from metastases with 6 cysts. All abscess cavities showed hyperintense DWI signal intensity compared to normal appearing white matter (NAWM), low ADC, low MD and high FA. Eight out of 10 glioblastoma cysts and all 6 metastatic cysts revealed hypointensity on DWI, high ADC, high MD and low FA (p<0.0001 compared to abscess group). DWI findings of 2/10 glioblastoma cysts overlapped with those of abscesses showing hyperintensity on DWI, low ADC and low MD and hence mimicked abscesses. FA of these 2 glioblastoma cysts was significantly lower than in abscess cavities (p=0.032).Conclusion: The findings of reduced diffusion compared to NAWM and increased FA within a ring enhancing cyst strongly indicate a cerebral abscess. In contrast, the majority of neoplastic cysts revealed high diffusion and low FA. Reduced diffusion is also found in a very small number of tumour cysts, but in these low FA refers to a non-infectious origin and thus helps distinguishing from infectious abscess.

Motor and cognitive slowing in multiple sclerosis: An attentional deficit?

2010-04-01

Abstract: Background: Action slowing is frequently observed in multiple sclerosis (MS) patients. Several factors may account for response slowing: motor, perceptual, cognitive deficits, global mental slowing. Our objective was to examine mechanisms accounting for action slowing in MS patients.Methods: Twenty MS patients, free of visual impairment and of upper limbs sensory–motor deficit underwent previously validated reaction time (RT) tests using visual stimuli. Three tasks were used: (1) motor tapping, (2) simple reaction time (SRT) in a simple and dual task condition, and (3) choice RT (CRT) with varying response probabilities. Results were compared to those of 20 healthy matched subjects.Results: MS patients had: (1) lower motor tapping frequency (p=0.02); (2) SRT lengthening (p=0.001) related to a lower proportion of fast responses (p=0.001) indicating attentional deficit whereas perceptuomotor index was spared (p=0.5), without higher sensitivity to dual task (p=0.9); and (3) CRT lengthening (p=0.001) with spared decision time (p=0.7).Conclusions: This study showed that action slowing of MS patient is mainly related to (1) attentional deficit resulting in inability to maintain high level of rapid actions, and (2) subtle motor slowing even in patients without motor deficit on clinical examination, whereas (3) divided attention and decisional process are preserved.

Basilar artery occlusive disease in stroke survivors in a multiethnic population

2010-04-01

Abstract: Objectives: To describe clinical, radiological findings, and outcome in a multiethnic population of stroke survivors with basilar artery occlusive disease (BAOC).Methods: Forty patients with infarcts in the basilar artery (BA) territory, alive 30 days after the ictus, participated in the study. BA stenosis (>50%) or occlusion was shown by magnetic resonance or digital subtraction angiography in all patients. Demographical, clinical and radiological characteristics were described. Modified Rankin Scale (MRS) scores at 30 days and 6 months after the ischemic event were evaluated. Association between demographical, clinical, radiological features and outcome were analyzed with Chi-square and Fisher's exact tests. MRS scores at 30 days and 6 months were compared with the Wilcoxon test.Results: Sixty percent of the patients were men, and 33% were Afro-Brazilian. Mean age was 55.8±12.9 years. Most (90%) had multiple vascular risk factors. Stroke was preceded by TIA in 48% of the patients, and 80% had a history of arterial hypertension. The most common neurological symptom was vertigo/dizziness (60%) and the sign, hemiparesis (60%). Most of the infarcts were located in the pons (85%) and the BA middle third was the most frequently affected segment (33%). BA occlusion occurred in 58% of the patients. More severe vascular occlusive lesions were present in Whites (p=0.002) and in patients with involvement of the middle third of the BA (p=0.021). Large-artery atherosclerosis was the most common stroke etiology (88%) and was more frequent in older patients (p<0.001). Most patients were treated with anticoagulation. MRS scores improved significantly at 6 months (p<0.001); at this time, 78% of the patients had MRS scores between 0 and 2.Conclusions: We observed different results compared with other series: greater proportion of Afro-descendents, higher frequency of atherosclerosis and BA occlusion. Rates of preceding TIAs and good outcome at 6 months were similar to previously published data. These results represent a step forward towards understanding BAOC in a multiethnic context.

Low field intraoperative MRI-guided surgery of gliomas: A single center experience

2010-04-01

Abstract: Introduction: The aim of this article is to report on our experience in using a low field intraoperative MRI (iMRI) system in glioma surgery and to summarize the hitherto use and benefits of iMRI in glioma surgery.Patients and methods: Between July 2004 and May 2009, a total of 103 patients harboring gliomas underwent tumor resection with the use of a mobile low field iMRI in our institution. Surgeries were performed as standard micro-neurosurgical procedures using regular instrumentarium. All patients underwent early postoperative high field MRI to determine the extent of resection. Adjuvant treatment was conducted according to histopathological grading and standard of care.Results: All tumors could be reliably visualized on intraoperative imaging. Intraoperative imaging revealed residual tumor tissue in 51 patients (49.5%), leading to further tumor resection in 31 patients (30.1%). Extended resection did not translate into a higher rate of neurological deficits. When analyzing survival of patients with glioblastoma, patients undergoing complete tumor resection did significantly better than patients with residual tumor (50% survival rate at 57.8 weeks vs. 33.8 weeks, log rank test p=0.003), while younger age did not influence survival (p=0.12).Conclusion: Low field iMRI is a helpful tool in modern neurosurgery and facilitates brain tumor resection to a maximum safe extent. Its use translates into a better prognosis for these patients with devastating tumors. Future studies covering the use of iMRI will need to be conducted in a prospective, randomized fashion to prove the true benefit of iMRI in glioma surgery.

Rare E196K mutation in the PRNP gene of a patient exhibiting behavioral abnormalities

2010-04-01

Abstract: Genetic transmissible spongiform encephalopathies (TSEs) account for approximately 10–15% of overall human prion diseases worldwide, but genotype–phenotype correlations remain incomplete. Here we report the case of an 80-year-old man who developed rapidly progressive behavioral abnormalities and myoclonus following a stroke. Repeated electroencephalography (EEG) revealed a general slowing of the basic activity, as well as several episodes of triphasic waves, with neither periodic activity nor recorded seizure. 14.3.3 protein was detected in cerebral cerebrospinal fluid, and direct sequencing of the PRNP gene showed an E196K mutation associated with homozygosity for methionine at codon 129. The patient was diagnosed with probable genetic prion disease with a Creutzfeldt-Jakob disease-like phenotype. The PRNP E196K mutation has only rarely been described in the literature, and generally patients exhibited an atypical initial phenotype, mainly involving abnormal behavioral features. Further observations are needed to confirm this particular clinical pattern associated with the mutation.

Candida parapsilosis meningitis associated with shunt infection in an adult male

2010-04-01

Abstract: Candida parapsilosis is a very rare cause of meningitis. Though several cases have now been reported in neonates and children, only one has been described in an adult. We report on a 55-year-old male that was admitted due to altered mental status. He had recent sinus drainage and polypectomy, craniotomy with drainage of brain abscess, and ventriculo-peritoneal shunt placement. On admission, imaging studies showed no evidence of shunt dysfunction but did reveal extensive white matter decreased attenuation. Microscopic examination of the first 10 daily cerebrospinal fluid (CSF) cultures revealed yeast. Flucytosine and liposomal amphotericin B were started and externalization of shunt was performed on day 3. On day 8, CSF culture from admission grew C. parapsilosis; fluconazole was added. On day 10, daily CSF still showed yeast and cultures consistently grew C. parapsilosis. Shunt was removed and bilateral ventriculostomy drains were inserted. CSF after procedure as well as at follow-up examinations throughout his 3-month hospitalization were negative for yeast. Extended treatment with flucytosine and fluconazole was initiated. At 8-month follow-up, successful treatment of C. parapsilosis infection without recurrence was confirmed. This case underscores the need for suspicion of C. parapsilosis as a cause of meningitis after invasive surgeries in adults.

Intraventricular bleeding and transient hydrocephalus in a patient with hemorrhagic filum terminale ependymoma undergoing anticoagulation therapy with phenprocoumon

Aikaterini Patrona, Albrecht Harders, Kirsten Schmieder, Martin Scholz — 2010-04-01

Abstract: The authors discuss a rare case of hemorrhage in a filum terminale ependymoma presenting with acute paraparesis and transient hydrocephalic dementia in association with long-term phenprocoumon anticoagulation.The CT scan of the brain revealed the presence of blood in both occipital horns and communicating hydrocephalus. The symptoms gradually resolved after tumor removal and there was no need for shunting.This is a complex clinical presentation of a spinal cord tumor associated hemorrhage, which further illustrates the possibility of retrograde passage of blood from the lumbosacral region to the ventricles.

Cognitive forms of multiple sclerosis: Report of a dementia case

2010-04-01

Abstract: Cognitive impairment is now well documented in patients with multiple sclerosis (MS), and series of MS patients with predominant cognitive problems have been published recently. We report the observation of a female patient with severe cognitive presentation at the onset of MS, with dramatically demented evolution, and show MRI examination results. We discuss the published reports of primary cognitive types of MS.

Primary CNS hemangiopericytoma presenting as an intraparenchymal mass—Case report and review of literature

Prakash M. Shetty, Aliasgar V. Moiyadi, Epari Sridhar — 2010-04-01

Abstract: Hemangiopericytomas (HPC) are rare, aggressive tumours that mostly involve the musculoskeletal system. They account for less than 1% of intracranial tumours. Intracranially, they are predominantly meningeal based and are thought to arise from the spindle cells (pericytes) in the vicinity of the blood vessels. We present a case of a 69-year-old male with a hemangiopericytoma in the left perisylvian region which was subcortically located. This is an uncommon location. We discuss the case and review the literature.