Clinical Neurology and Neurosurgery

Editorial Board

2010-02-01

Supratentorial neurenteric cysts—A fascinating entity of uncertain embryopathogenesis

2010-02-01

Abstract: The histopathological, immunologic, and ultrastructural findings of neurenteric cysts support an endodermal derivation. These developmental cystic lesions are generally located in the posterior mediastinum, abdomen, and pelvis and may also contain some mesodermal and neuroectodermal elements. In contrast, neurenteric cysts of the central nervous system are very infrequent and occur most commonly in the spinal canal. Intraspinal neurenteric cysts are usually encountered in the cervicothoracic region with an intradural, extramedullary location and are commonly associated with congenital defects of the overlying skin and/or vertebral bodies.Intracranial neurenteric cysts are very uncommon and typically located in the posterior fossa.Several hypotheses have been postulated to explain the origin of intracranial neurenteric cysts. However, the embryologic basis of these fascinating lesions remains incompletely understood.Supratentorial neurenteric cysts are distinctly rare often represent a diagnostic challenge on preoperative neuroimaging. In fact, only 22 cases of supratentorial neurenteric cysts have been reported in the literature including our own patient with a laterally based convexity extraaxial cyst presenting with seizures.In this report, we review the clinical, radiographic, and histological findings of supratentorial neurenteric cysts. We discuss the differential diagnoses and surgical considerations in the management of these intriguing lesions. We also provide an extensive review of normal human embryogenesis and discuss putative mechanisms of embryopathogenesis of supratentorial neurenteric cysts.

Does glycated hemoglobin have clinical significance in ischemic stroke patients?

Sung Hyuk Heo, Seung-Hoon Lee, Beom Joon Kim, Bong Su Kang, Byung Woo Yoon — 2010-02-01

Abstract: Objectives: It has been suggested that patients with an elevated hemoglobin A1c (HbA1c) level have an increased risk of cardiovascular disease regardless of the presence of diabetes. However, an association between HbA1c and stroke has not yet been determined. In this study, our purpose was to examine whether HbA1c was independently associated with various types of cerebral vascular lesions in stroke patients.Methods: A consecutive series of acute ischemic stroke patients were included for this analysis from October, 2002, to March, 2006. HbA1c was examined on admission, and MR imaging was performed for analysis of large artery diseases (LADs) and small artery diseases (SADs). Symptomatic or asymptomatic LAD was diagnosed by MR angiography, and SAD was classified as leukoaraiosis, microbleeds, or old lacunar infarctions.Results: A total of 639 stroke patients were analyzed (diabetics, n=247; non-diabetics, n=392). There was no relationship between the level of HbA1c and any type of cerebrovascular lesion in the non-diabetic patients. In contrast, HbA1c showed a significant negative association with symptomatic LAD and leukoaraiosis in the diabetic patients using univariate analysis (p=0.01 and p<0.05, respectively). These associations did not remain significant, however, after adjustment for age and hypertension. This was, in part, because the HbA1c level in our diabetic population decreased gradually with age (p=0.03).Conclusions: Our results indicate that HbA1c is not associated with risk for various types of cerebrovascular lesions in ischemic stroke patients. The negative association between age and HbA1c in diabetic patients should be further investigated.

Diagnostic problems and delay of diagnosis in amyotrophic lateral sclerosis

Markus Kraemer, Melanie Buerger, Peter Berlit — 2010-02-01

Abstract: Objective: Initial symptoms of amyotrophic lateral sclerosis (ALS) mimic several neurological syndromes that may decelerate a correct diagnosis. The aim of our study was to investigate if diagnostic and therapeutic parameters have influence on the time of diagnosis.Methods: We retrospectively reviewed the medical records of 100 consecutive ALS patients focusing on clinical and diagnostic data, the timing of diagnosis and treatments attributed to the onset of symptoms of ALS.Results: Among 100 consecutive patients with ALS, 12% underwent surgery due to symptoms retrospectively attributable to ALS. The comparison of duration from first symptoms to correct diagnosis showed a significant difference between operated and non-operated patients. 35% of all ALS patients had bulbar onset symptoms. The mean time from first symptoms to diagnosis was 9 months in this group. In patients without bulbar onset it was 16.4 months which also represents a significant difference. In 44% of patients other diagnoses were considered and medically treated previous to correct diagnosis, but there was no significant delay of diagnosis.Conclusion: Our study confirms that diagnosis of ALS is still a common clinical problem and shows the need of sensitive and specific diagnostic tests.

A pilot study: Microlesion effects and tremor outcome in the ventrointermediate deep brain stimulation (VIM-DBS)

Oraporn Sitburana, Michael Almaguer, William G. Ondo — 2010-02-01

Abstract: Objectives: To perform a pilot study to investigate the relationship between the microlesion effect (MLE) seen in deep brain stimulation (DBS) of the ventralis intermedius nucleus (VIM) and subsequent tremor response and DBS parameter settings.Patients and methods: Nineteen thalami in 12 patients (11 essential tremor and 1 Parkinson's disease), who underwent unilateral (n=5) and bilateral VIM-DBS (n=7) were assessed at pre- and 24-h post-operation, at their initial DBS activation, and at 6-month follow-up. The severity of tremor was rated (from 0 to 4) for each activity including hand at rest, outstretched, wing beating, finger–nose–finger, dot approximation and spiral drawing (total score ranging from 0 to 24). The difference of total tremor score before and 24-h after electrode implants (MLE) was segregated into 3 groups based on immediate (24h) post-operative tremor improvement: (1) minimal (none or mild, 0–2), (2) moderate (>2–4), and (3) marked (>4). At the initial activation (23.4±3.7 days post-operation), the mean OFF tremor scores were still marginally better in marked than the minimal and moderate MLE groups.Results: At 6 months, 14 of 19 thalami (74%) were eligible for follow-up analysis. The “OFF” stimulation MLE disappeared in all groups. There was no significant difference of mean ON tremor scores among the groups; however, DBS parameter settings, including amplitude and pulse width, trended to be mildly lower in those with a marked MLE.Conclusion: MLE has minimal long term clinical effect except for possibly allowing for lower DBS settings.

Surgery for perirolandic epilepsy: Epileptogenic cortex resection guided by chronic intracranial electroencephalography and electric cortical stimulation mapping

Ni DuanYu, Zhang GuoJun, Qiao Liang, Cai LiXin, Yu Tao, Li YongJie — 2010-02-01

Abstract: Subject: The objective of this study was to assess outcome with regard to seizure status and neurological function in patients undergoing resective surgery involving the perirolandic area.Method: All 15 patients who underwent perirolandic cortical resection between October 2006 and September 2007 at the Comprehensive Epilepsy Centre of Beijing Xuanwu Hospital were included in the study. The locations of functional cortical areas, ictal onset zones and epileptogenic lesions were mapped by chronic intracranial EEG recordings and electric cortical stimulation. Seizure outcome was determined using the modified classification of Engel and colleagues. Motor and sensory deficits were monitored.Results: At last follow-up 5 patients (33%) were in Engel class I, 4 (27%) were in class II, 3 (20%) were in class III, and 3 (20%) were in class IV. Nine patients suffered immediate functional deficits; 8 of these recovered completely within 2 weeks to 3 months of surgery. One had mild persistent loss of finger motor control.Conclusion: After accurate presurgical evaluation using invasive recordings and functional brain mapping, epileptogenic cortical resection can give excellent results and few deficits in patients with perirolandic epilepsy.

Predictive factors for outcome of invasive video-EEG monitoring and subsequent resective surgery in patients with refractory epilepsy

2010-02-01

Abstract: Objective: This is a descriptive study of patients who underwent invasive video-EEG monitoring (IVEM) at Ghent University Hospital. The aim of the study is to identify predictive factors for outcome of IVEM and resective surgery (RS). These factors may optimize the patient flow following the non-invasive presurgical evaluation towards IVEM and RS or other treatments.Patients and methods: Over the past 16 years, 68/710 refractory epilepsy patients included in the presurgical evaluation protocol (M/F 41/27, mean age 33 years) underwent IVEM at Ghent University Hospital. Patient features and follow-up data were collected from the patients’ medical files and the electronic patient database at the neurology and neurosurgery department. Predictive factors for IVEM outcome were identified by comparing features of patients with a positive IVEM outcome (i.e. ictal onset zone identification) and patients with a negative IVEM outcome. Predictive factors for RS outcome were identified by comparing features of patients with Engel class I and patients with Engel class II–IV outcome.Results: In 56/68 patients (82%) IVEM outcome was positive. The occurrence of a seizure-free interval in the patient's history and a non-localizing ictal scalp EEG in patients with a structural abnormality on MRI (p<0.05) were predictive factors for a negative IVEM outcome. 32/68 patients underwent RS. In 22/32 (70%) patients RS resulted in an Engel class I outcome. A structural abnormality on MRI was a predictive factor for a positive RS outcome in patients in whom a focal or regional focus was resected (p<0.05).Conclusion: This study shows that IVEM identifies one or more ictal onset zone(s) in up to 80% of patients. The potential of IVEM to identify the ictal onset zone is unlikely in patients with a seizure-free interval in their medical history and a non-localizing ictal scalp EEG during the non-invasive presurgical evaluation.Half of these patients underwent RS with long-term seizure freedom in 70%. Patients with structural MRI lesions have the highest chance of seizure freedom. These findings may contribute to the optimization of patient management during both the invasive and non-invasive presurgical work-up.

Impact of cognitive impairment on coping strategies in multiple sclerosis

2010-02-01

Abstract: Objectives: To assess the impact of cognitive impairment (CI) on coping strategies in multiple sclerosis (MS). Materials and methods: Sixty-three patients (40 women, 55 relapsing-remitting and 8 secondary progressive, age 42.6±10.1 years, Expanded Disability Status Scale 2.2±1.7) were assessed using the Coping Orientation for Problem Experiences-New Italian version Inventory, the Beck Depression Inventory and the Rao's Brief Repeatable Battery. Results: MS patients were less likely to use positive and problem-focused strategies, whereas avoiding strategies were adopted more frequently. Twenty-three (36.5%) cases were CI. We found no differences in the type of coping between CI and cognitively preserved patients. Scores on the Stroop test (beta=−0.91, p=0.04) and on the Word List Generation (beta=1.15, p=0.04) were associated with poorer coping strategies. Conclusions: Our study suggests that cognitive functioning (in particular on sustained attention and aspects of executive function) must be considered in a comprehensive account of the factors contributing to successful coping in MS patients.

Efferent and afferent evoked potentials in patients with adrenomyeloneuropathy

2010-02-01

Abstract: Objective: This paper investigates efferent and afferent conductions of the central nervous system by various evoked potentials in patients with adrenomyeloneuropathy (AMN).Patients and methods: Ten pure AMN patients without cerebral involvement were studied. Motor evoked potentials (MEPs), somatosensory evoked potentials (SEPs), auditory brainstem response (ABR), and pattern reversal full-field visual evoked potentials (VEPs) were recorded. For MEP recording, single-pulse or double-pulse magnetic brainstem stimulation (BST) was also performed.Results: Abnormal MEP was observed in all ten patients, abnormal SEP in all ten, abnormal ABR in nine, and abnormal VEP in only one. Brainstem latency was measured in three of the seven patients with central motor conduction time (CMCT) prolongation. The cortical–brainstem conduction time was severely prolonged along the normal or mildly delayed brainstem–cervical conduction time in those three patients.Conclusions: The pattern of normal VEP and abnormal MEP, SEP, ABR is a clinically useful electrophysiological feature for the diagnosis. BST techniques are helpful to detect, functionally, intracranial corticospinal tract involvement, probably demyelination, in pure AMN patients.

Unilateral deep brain stimulation of the nucleus accumbens in patients with treatment-resistant obsessive-compulsive disorder: Outcomes after one year

2010-02-01

Abstract: Objective: To investigate the effects of unilateral deep brain stimulation (DBS) in the right nucleus accumbens in patients with obsessive-compulsive disorder (OCD). Predominantly bilateral stimulation of the anterior limb of the internal capsule was utilized.Methods: The study was designed as a double-blind sham-controlled crossover study. Patients received 3 months of deep brain stimulation followed by 3 months of sham stimulation, or vice versa. Subsequently, stimulation was continued unblinded for all patients. The primary outcome measure was the severity level of OCD, measured using the Yale-Brown Obsessive Compulsive Scale (Y-BOCS). Secondary outcome measures were depressive symptoms, anxiety, psychological symptom severity, global functioning, quality of life, and cognitive function.Results: The mean Y-BOCS scores decreased significantly from 32.2 (±4.0) at baseline to 25.4 (±6.7) after 12 months (p=0.012). Five out of ten patients showed a decrease of more than 25%, indicating at least a partial response. One patient showed a decrease in Y-BOCS severity greater than 35%. Similarly, depression, global functioning and quality of life improved within one year. In contrast, anxiety, global symptom severity and cognitive function showed no significant changes. In general, DBS was well-tolerated.Conclusions: DBS of the unilateral right nucleus accumbens showed encouraging results in patients with treatment-resistant OCD. Five out of ten patients reached at least a partial response after the first year.

Neural surfaces coverage with “collagen films and cigarettes”: A revisited and modified method of protection and retraction during microsurgical approaches to craniospinal lesions

2010-02-01

Abstract: Objective: Despite the surgical cotton patties application and meticulous manipulation, mechanical contact between the different microsurgical instruments and neurovascular structures may jeopardize its integrity through laceration or cerebrospinal perfusion. We present a technique based on using collagen films and cigarettes, both to protect and retract such structures, and compared it with the cottonoid technique.Materials and methods: During the last 3 years, collagen in “film and cigarette format” has been used in several microsurgical procedures for the treatment of craniospinal lesions by the first author. The collagen films were broken into pieces and adapted to the exposed neural surface measurements to protect and/or retract during microsurgical dissection. At the same time, handmade collagen “cigarettes” were used as retractors to keep open the neural lips of the transsulcal and transfissural corridors. To investigate the relevance of this technique for minimizing surgical morbidity, a blind third-party observer quantified the tissue preservation by a postoperative magnetic resonance imaging (MRI) protocol, in a short series with randomized cotton patty vs. collagen film protection.Results: Only two of the 20 examined “collagen group” cases exhibited areas of additional abnormal signal, as against 16 cases of the “cotton patty group.” Furthermore, a statistically significant difference between both the groups based on the radiological results was also demonstrated.Conclusion: The results of the present series support the usefulness of the neurovascular coverage and retraction with collagen films and cigarettes, respectively. It seems to be a good alternative to surgical cotton patties and other materials owing to its hemostatic, protection, retraction, and dissection capacity.

Clinical and neurophysiological improvement of SGCE myoclonus–dystonia with GPi deep brain stimulation

2010-02-01

Abstract: Myoclonus–dystonia (M–D) is characterized by early onset myoclonus and dystonia. It is thought to be subcortical in origin. Response to oral medications may be incomplete, such that deep brain stimulation (DBS) surgery to the globus pallidum interna (GPi) or ventral intermediate thalamic nucleus (VIM) may be considered. The optimal site is not known. The physiology and surgical response for a 63-year-old woman who underwent GPi DBS for M–D with onset at age 2 and related to a mutation in the epsilon-sarcoglycan gene (SGCE) is described. She showed excellent clinical and neurophysiological improvement of both myoclonus and dystonia, suggesting that modulation by DBS is effective even after long disease duration and only partial response to oral medications.

Progressive multifocal leukoencephalopathy developed in incomplete Heerfordt syndrome, a rare manifestation of sarcoidosis, without steroid therapy responding to cidofovir

2010-02-01

Abstract: Progressive multifocal leukoencephalopathy (PML) is a severe demyelinating disease of the central nervous system caused by the JC virus; the mortality rate is high and it is usually refractory to treatment. In non-HIV patients, PML occurs as a late consequence of hematologic malignancies or during prolonged immunosuppression for transplantation or autoimmune disease. We describe a 34-year-old PML patient with incomplete Heerfordt syndrome, a rare type of sarcoidosis, who had not received any immunosuppressants, including steroids, at the onset and who was clinically and radiologically responsive to the antiviral drug cidofovir.

The role of an Ommaya reservoir in the management of children with cryptococcal meningitis

2010-02-01

Abstract: Cryptococcal meningitis is the most common life-threatening fungal infection and is associated with high mortality in children. Amphotericin B plus flucytosine and fluconazole is the optimal current therapy. Implantation of an Ommaya reservoir for intraventricular infusion of medication and aspiration of cerebrospinal fluid (CSF) for the treatment of increased intracranial pressure (ICP) has been reported. Intraventricular injection of amphotericin B through an Ommaya reservoir in children with cryptococcal meningitis has not been reported previously. We report two children who had cryptococcal meningitis and associated increased intracranial pressure, and were treated with an Ommaya reservoir. Both patients experienced rapid reversal of symptoms. At the time of discharge both patients had recovered and have remained asymptomatic.

Polymyositis presenting with cardiac manifestations: Report of two cases and review of the literature

Zeki Odabasi, Robert Yapundich, Shin J. Oh — 2010-02-01

Abstract: We report two patients with severe cardiac manifestations at the time of the initial presentation of polymyositis. Both cases are unusual in that they presented with predominant cardiac disturbances, associated with muscle weakness. One patient had a typical clinical syndrome of congestive heart failure, and the second mimicked an acute myocardial infarction in which coronary angiography was normal. From our cases, we can emphasize that aside from characteristic symmetrical proximal muscle weakness, the clinical features of polymyositis may also include cardiac complications.

Memory impairment due to a small unilateral infarction of the fornix

Kojiro Korematsu, Tetsuya Hori, Motohiro Morioka, Jun-ichi Kuratsu — 2010-02-01

Abstract: A 52-year-old man suffered sudden anterograde and retrograde amnesia without any focal neurological deficit. Assessment using Wechsler Memory Scale-Revised test revealed significant memory impairment with a marked decline of delayed recall and preserved attention and concentration. Wechsler Adult Intelligent Scale-Third Edition indicated poor intelligent quotients. MRI revealed a small infarction at the anterior column of the left fornix. His memory loss persisted for more than 3 months with only slight improvement. This rare case indicates that isolated damage to the anterior column of the unilateral fornix is sufficient to cause significant memory disturbance, and that cerebral infarction should be considered in the differential diagnosis of a patient presenting with amnesia as the only symptom.

Endovascular angioplasty and stent placement in venous hypertension related to dural arteriovenous fistulas and venous sinus thrombosis

Poh-Shiow Yeh, Te-Chang Wu, Wen-Sheng Tzeng, Huey-Juan Lin — 2010-02-01

Abstract: Venous hypertension in lateral sinuses resulting from dural arteriovenous fistula (DAVF) and venous sinus thrombosis (VST) can manifest with severe neurological deficits, such as infarction or intracerebral hemorrhage. It has been proved that venous hypertension plays a significant role in the evolution and progression of DAVF and VST. The definite treatment in complicated conditions such as multiple DAVFs or multiple sinus occlusions is still unknown. Traditional transarterial embolization, transvenous embolization or radiosurgery alone has limited effects on these conditions. We reported one case with venous hypertension presenting with severe neurological symptoms. The case had quick clinical recovery after correction of venous hypertension by endovascular angioplasty and stent placement in occluded lateral sinuses. Accordingly, we propose this method can be an ideal treatment option either in single or staged therapy of venous hypertension related to DAVFs and VST.

Intracranial plasmacytoma with apoplectic presentation and spontaneous intracerebral hemorrhage: Case report and review of the literature

2010-02-01

Abstract: Involvement of the nervous system is not uncommon in patients with multiple myeloma, with polyneuropathy and myelopathy predominating. Intracranial involvement producing neurological symptoms, however, is distinctly uncommon. Massive intraparenchymal hemorrhage from a previously unrecognized intracranial plasmacytoma is exceedingly rare. The authors report the case of a 57-year-old male who presented with sudden onset of severe headache, rapid onset of right-sided weakness and deterioration in level of consciousness while at work. Two years earlier the patient had completed treatment for multiple myeloma and was considered to be in remission, with a recent bone marrow biopsy that was negative, and complete normalization of serum protein electrophoresis. Imaging studies revealed a massive intracerebral hemorrhage with the possibility of an underlying lesion, and the patient was taken for emergent hematoma evacuation and tumor resection. The patient made an excellent recovery and was treated with intracranial radiation. Even in patients with multiple myeloma without evidence of systemic disease following successful treatment, the possibility of unrecognized lesions lingers. The onset of new symptoms referable to potential intracranial pathology in this setting should prompt consideration of intracranial plasmacytoma in the differential diagnosis.

Addendum to “Mutations in the NKX2-5 gene in patients with stroke and patent foramen ovale” [Clin. Neurol. Neurosurg. 111 (2009) 574–578]

2010-02-01

Concerning our paper “Mutations in the NKX2-5 gene in patients with stroke and patent foramen ovale” that has been recently published in Clinical Neurology and Neurosurgery, [2009 September;111(7)574–8], we want to shed light on some aspects of the nomenclature of the mutations employed.