Clinical Neurology and Neurosurgery - Articles in Press

Delayed leukoencephalopathy after alprazolam and methadone overdose: A case report and review of the literature - Corrected Proof

2012-02-20

Highlights: ► A case of delayed leukoencephalopathy after mixed overdose is described. ► MR images and spectroscopy are characteristic of demyelinating lesions. ► DLE is characterised by similar findings regardless of the initial insult. ► The role of errors of myelin metabolism e.g. arylsulfatase A remains unconfirmed.

Very late IV thrombolysis in acute ischemic stroke: A successful case in proximal MCA occlusion - Corrected Proof

Julien Joux, Boris Nazarov, Stéphane Olindo, Didier Smadja — 2012-02-20

Highlights: ► We describe a case of M1-MCA cardioembolic occlusion admitted 13h after the symptoms onset. ► Because MRI showed a franck clinical and FLAIR-DWI mismatch, we performed an IV thrombolysis, with a very good clinical and angiographic result. ► Very late IV thrombolysis is feasable in carefully selected stroke cases.

Asymptomatic posterior reversible encephalopathy revealed by brain MRI in a case of axonal Guillain-Barré syndrome - Corrected Proof

C. Parmentier, Y. Vandermeeren, P. Laloux, E. Mormont — 2012-02-20

Posterior reversible encephalopathy syndrome (PRES) is a clinicoradiologic syndrome characterized by headaches, encephalopathy, visual disturbances and seizures, with a transient vasogenic oedema on brain imaging. The lesions are predominantly but not exclusively located in the posterior circulation system. The most frequent aetiologies include hypertension, cytotoxic or immunosuppressive drugs, sepsis, eclampsia or preeclampsia . Some cases of PRES are described in association with Guillain-Barré syndrome (GBS) . We report the case of a young woman who presented with a GBS, dysautonomia, and a brain MRI suggesting PRES but lacking any encephalic symptom suggestive of that syndrome. We discuss the relationship between, PRES, GBS and dysautonomia.

Recurrent cerebral infarction in Klippel-Trenaunay–Weber syndrome - Corrected Proof

Dimitri Renard, Aurore Larue, Guillaume Taieb, Luc Jeanjean, Pierre Labauge — 2012-02-20

KTWS is characterised by capillary hemangioma, varicose veins, and bone and soft tissue hypertrophy. We present a 53-year-old woman with sporadic congenital Klippel-Trenaunay–Weber syndrome (KTWS) with involving asymmetrically the four limbs (i.e. bone and soft tissue hypertrophy predominant in the right-sided limbs, and varicose veins in both lower limbs), the trunk (large capillary hemagioma on the left-sided trunk, ), and the head. In absence of cardiovascular risk factors, she had a history of transient ischemic attack (aphasia) at the age of 9 years, ischemic stroke at the age of 20 years with right-sided hemiparesis lasting for days without leaving permanent deficit, and another ischemic stroke at the age of 29 years leaving a permanent right-sided hemiplegia. No underlying cause for these cerebral infarctions was identified at that time.

Marked response of gliomatosis cerebri to temozolomide and whole brain radiotherapy - Corrected Proof

Austin K. Mattox, Amy L. Lark, D. Cory Adamson — 2012-02-20

Abstract: Gliomatosis cerebri (GC) represents an unfortunate, rare variant of glioma with a very poor prognosis. Given this lesion's rarity, little information exists on appropriate treatment options. The diffuse, infiltrative nature of GC precludes any surgical resection and limits therapy. Because of the improved survival seen with the use of temozolomide (TMZ) in malignant glioma, a rigorous systematic review of the published literature was performed to ascertain the benefit of TMZ in GC. We identified all GC cases in the literature where there was enough information to ascertain a clear response to a specific chemoradiotherapeutic treatment. In addition to our experience with a recent case, we have identified 61 patients with GC in the published literature who demonstrated a positive radiographic or clinic response after treatment. Statistical analysis of survival was performed by Kaplan-Meier analysis. A positive radiographic and clinical response was seen in patients ranging in age from 4 to 84 years. Overall median survival in patients diagnosed with GC who demonstrated a response after treatment was 25 months, with 1- and 2-year survival rates of 89% and 55%, respectively. The most common treatment regimens for responders included TMZ alone (26.2%), external whole-brain radiotherapy (WBRT) (26.2%), and concomitant TMZ and WBRT (20%). Our patient was treated with concomitant TMZ (150mg/m2/day over 5 days) and WBRT (50Gy) and has remained with a complete radiographic response after 36 months. In conclusion, patients with GC confirmed by surgical biopsy should be aggressively treated with concomitant TMZ and WBRT, as marked responses have been seen, and this appears to offer overall survival benefit.

Reversion of cerebral artery stenoses due to tuberculomas with TNF-α antibodies - Corrected Proof

Stefaan J. Vandecasteele, An S. De Vriese, Geert T. Vanhooren — 2012-02-16

Intracranial tuberculomas are a rare but severe complication of tuberculosis infections. Their pathogenesis is partially immune-mediated, and they can present throughout the course of the disease . Anti-TNF treatment such as infliximab inherits an increased risk for tuberculosis . A case of disseminated tuberculosis under infliximab treatment is presented illustrating the subsequent risk of paroxysmal reactions after cessation of the infliximab and the potential role of infliximab in the treatment of these paroxysmal reactions.

Cilostazol versus aspirin therapy in patients with chronic dizziness after ischemic stroke - Corrected Proof

2012-02-15

Abstract: Background: Chronic dizziness is frequently reported by patients in the chronic stage after ischemic stroke. The aim of this study was to determine the efficacy of cilostazol versus that of aspirin for the chronic dizziness that follows ischemic stroke.Methods: We performed a prospective, randomized, open-label, blinded endpoint trial. One hundred six patients who suffered supratentorial ischemic stroke within the previous 1–6 months and subsequently complained of persistent dizziness without other obvious sequelae were enrolled. Patients were randomly given cilostazol (200mg/day) or aspirin (100mg/day) for 6 months. Rates of improvement in the dizziness were then evaluated. Changes in fixation suppression of the vestibulo-ocular reflex (an indicator of cerebral control over the brainstem reflex related to balance), regional cerebral blood flow (CBF) in the cerebrum, cerebellum, and brainstem; and the Zung Self-Rating Depression Scale (SDS) were also evaluated.Results: Dizziness was significantly improved in the cilostazol group versus the aspirin group (P<0.0001) after the 6-month therapy. The capacity for fixation suppression of the vestibulo-ocular reflex was improved (P<0.0001), and regional CBF in the cerebrum (relative to that in the brainstem [P=0.003] and to that in the cerebello-brainstem [P=0.012]) was increased only in the cilostazol group. There was no statistical difference in the change in SDS scores between the two groups.Conclusion: Cilostazol improves the chronic dizziness that follows ischemic stroke and increases supratentorial CBF and cerebral function for adaptation of the brainstem reflex related to the sense of balance.

A case of cerebrotendinous xanthomatosis presenting with epilepsy as an initial symptom with a novel V413D mutation in the CYP27A1 gene - Corrected Proof

S. Koyama, T. Kawanami, H. Tanji, S. Arawaka, M. Wada, N. Saito, T. Kato — 2012-02-15

Highlights: ► Epilepsy as a presenting feature of cerebrotendinous xanthomatosis. ► A spotty focal priventricular lesion on brain MRI in cerebrotendinous xanthomatosis. ► A novel mutation in the CYP27A1 gene in a case of cerebrotendinous xanthomatosis.

Elevated blood urea nitrogen/creatinine ratio is associated with poor outcome in patients with ischemic stroke - Corrected Proof

Jon W. Schrock, Michael Glasenapp, Kristin Drogell — 2012-02-14

Abstract: Objective: Dehydration may impair cerebral oxygen delivery and worsen clinical outcome in patients with acute ischemic stroke (AIS). We evaluated if elevated blood urea nitrogen to creatinine ratio (BUN/Cr) as a marker of dehydration was associated with poor clinical outcome in emergency department (ED) patients presenting with AIS.Methods: We conducted a prospective cohort study using a stroke registry enrolling all ED patients with AIS from 10/2007 through 6/2009. Poor clinical outcome was defined as death, placement in a nursing home for purposes other than rehabilitation, or hospice within 30 days of ED presentation. A BUN/Cr ratio of ≥15 was considered elevated. (IQR). Logistic regression was performed adjusted for age >64 years, NIHSS >8, diabetes, prior CVA, and coma at presentation reporting odds ratios with 95% confidence intervals.Results: 324 patients had a final diagnosis of AIS. 163 (50%) were female, 19 (6%) died, 44 (14%) received t-PA, and 89 (27%) had a poor clinical outcome. The median NIHSS, BUN and Cr were 4 (IQR 1–9), 14mg/dL (IQR 11–21), and 1.02mg/dL (IQR 0.87–1.27) respectively. The median BUN/Cr was 13.9 (IQR 10.6–18.5). The variables associated with a poor clinical outcome were: high NIHSS OR 6.5 (3.6–11.8), age >64 years OR 2.7 (1.5–5.0), and BUN/Cr ratio of ≥15 OR 2.2 (1.2–4.0).Conclusion: An elevated BUN/Cr ratio in patients with AIS is associated with poor outcome at 30 days. Further study is needed to see if acutely addressing hydration status in ED patients with AIS can alter outcome.

The “carotid CT crescent” sign - Corrected Proof

Jean-Marc Bugnicourt, Pauline Monet-Desblache, Hervé Deramond, Olivier Godefroy — 2012-02-13

Spontaneous carotid artery dissection (CAD), characterized by the presence of a mural hematoma located in the arterial wall, is a major cause of cerebral infarction in young adults . Mural hematoma, defined as a crescent-shaped rim of hyperintense signal surrounding a lumen that is reduced in size, is the most specific sign for CAD. It is easily seen on T1-weighted axial cervical MRI scans by use of a fat-saturation technique, but can be missing within the first days of developing CAD .

Advanced MR imaging techniques and characterization of residual anatomy - Corrected Proof

2012-02-13

Abstract: Advances in technology in recent decades have contributed to rapid developments in non-invasive methods for imaging human anatomy, and advanced imaging methods are now one of the primary tools for clinical diagnosis after neurological trauma or disease. Here we review the current and upcoming capabilities of one of the most rapidly developing methods, magnetic resonance imaging (MRI). The underlying theory is introduced so that the reasons for the strengths, weaknesses, and future expectations of this method, can be explained. Current techniques for imaging anatomical changes, inflammation, and changes in white matter, axonal integrity, blood flow and function, are reviewed. Applications for specific purposes of assessing traumatic injury in the brain or spinal cord, and for multiple-sclerosis are also presented, and are used as examples of how the advanced techniques are being used in practice.

Central nervous system immune reconstitution inflammatory syndrome in AIDS: Experience of a Mexican neurological centre - Corrected Proof

2012-02-13

Abstract: Background: Highly active antiretroviral therapy (HAART) restores the inflammatory immune response in AIDS patients and it may unmask previous subclinical infections or paradoxically exacerbate symptoms of opportunistic infections. Up to 25% of patients receiving HAART develop immune reconstitution inflammatory syndrome (IRIS). We describe six patients with IRIS central nervous system (CNSIRIS) manifestations emphasizing the relevance of CSF cultures and neuroimaging in early diagnosis and management.Methods: Patients with CNSIRIS were identified among hospitalized HIV-infected patients that started HAART from January 2002 through December 2007 at a referral neurological center in Mexico.Results: One-hundred and forty-two HIV-infected patients with neurological signs were hospitalized, 64 of which had received HAART, and six (9.3%) developed CNSIRIS. Five patients were male. Two cases of tuberculosis, two of cryptococcosis, one of brain toxoplasmosis, and one possible PML case were found. IRIS onset occurred within 12 weeks of HAART in five patients. Anti-infective therapy was continued. In one case, HAART was temporarily suspended. In long-term follow-up the clinical condition improved in all patients.Conclusions: CNSIRIS associated to opportunistic infections appeared in 9% of patients receiving HAART. Interestingly, no cases of malignancy or neoplasm IRIS-related were found. Frequent clinical assessment and neuroimaging studies supported diagnosis and treatment. Risk factors were similar to those found in other series.

Successful treatment for giant pituitary adenomas through diverse transcranial approaches in a series of 15 consecutive patients - Corrected Proof

2012-02-13

Abstract: Object: Giant pituitary adenomas (GPAs) remain a therapeutic challenge with high mortality and morbidity. We described our experience in a consecutive series of GPAs with extensive suprasellar extension.Methods: A series of 15 consecutive patients with maximum dimension of more than 4cm was enrolled in present study. These cases were microsurgically treated through diverse transcranial approach in our neurosurgical department from January 2006 to January 2011. Four different transcranial microsurgical approaches were selected based on tumor localization and expansion as well as neurosurgeon's experience.Results: Gross total removal (GTR) was achieved in 10 of all patients (67%), subtotal removal was achieved in 5 of 15 (33%). Nine patients experienced visual improvement postoperatively compared with those of preoperative symptom (82%), no intraoperative or postoperative death was observed in present series. The most striking features of this study indicate that an experienced team can reach 67% with no mortality, no panhypopituitarism and no permanent diabetes insipidus dealing with GPAs. No recurrent tumor was found in the GPAs with GTR, adjuvant radiation therapy had been performed in 5 patients and the continuous shrinkage of the residual adenomas was achieved in 2 out of 5 with radiotherapy.Conclusions: Transcranial approach was still a relatively reliable and safe management for complex GPAs with extensive suprasellar extension.

Reduction of shunt obstructions by using a peel-away sheath technique? A multicenter prospective randomized trial - Corrected Proof

2012-02-10

Abstract: Objective: Shunt obstructions may partly be caused by brain debris, which intrude into the ventricular catheter during ventricle puncture. Avoiding contact between the catheter and brain tissue, by using a peel-away sheath, should reduce the number of shunt failures caused by obstruction. To test this hypothesis, we conducted a randomized, prospective multicenter study.Methods: 201 patients from 6 different neurosurgical centers in Germany receiving a ventriculo-peritoneal shunt were included in this study. Of these, 177 patients completed a 1-year follow-up period. Surgery was randomized in a 1 to 1 fashion, such that out of 177 procedures, 91 were performed using a peel-away sheath and 86 were performed without. The rate of surgical re-interventions and shunt obstructions within a 12-month period was recorded.Results: Within 1 year post-surgery, 17 shunt obstructions (9.6%) leading to shunt revisions were recorded. However, no difference was found between surgeries performed using a peel-away sheath (9.9%) or not (9.3%). The overall shunt infection rate was 2.8% and the shunt revision rate for overdrainage was 3.9%.Conclusions: The theoretical advantages attributed to the use of a peel-away sheath to introduce a ventricular catheter could not be confirmed in this randomized study, suggesting that the proposed role of brain debris in shunt obstructions may be overestimated.

Diffusion tensor imaging as a surrogate marker for outcome after perimesencephalic subarachnoid hemorrhage - Corrected Proof

Tom A. Schweizer, Timour Al-Khindi, R. Loch Macdonald — 2012-02-10

Perimesencephalic subarachnoid hemorrhage (pSAH) accounts for approximately 5% of all spontaneous subarachnoid hemorrhage and is characterized by the accumulation of blood in the midbrain cisterns. Angiography usually reveals no source of hemorrhage. Initially, pSAH was associated with a favorable prognosis: patients with pSAH had a low risk of rebleeding, no decrease in quality of life, and no problems with returning to work or other activities. More recent reports, however, suggest that pSAH may not be as benign as previously believed. Madureira et al. found that 72% of patients with pSAH were impaired in at least 1 cognitive domain and 33% exhibited depressive symptoms over 3years after pSAH. Neuroimaging, however, was not used to explain pSAH survivors’ persistent cognitive deficits. Thus, the neuroanatomical changes responsible for pSAH-associated cognitive impairment remain largely unknown.

Gastro-enteritis in hypokalemic periodic paralysis: A life threatening condition - Corrected Proof

Saskia Ebus, Aad Verrips, Ieke B. Ginjaar, Wim I.M. Verhagen — 2012-02-10

Hypokalemic periodic paralysis (hypoPP) is a rare autosomal dominant neuromuscular disorder. Episodic muscle weakness is associated with episodic low serum potassium levels. The periodic inexcitability of the muscle fibre membrane is induced by ion channel dysfunction of either a calcium or sodium channel, depending on the gene mutation. Non-hereditary hypokalemic periodic paralysis can occur in patients with thyrotoxicosis and may be life threatening .

Medical information on the Internet: Quality assessment of lumbar puncture and neuroaxial block techniques on YouTube - Corrected Proof

Bernhard Rössler, Daniel Lahner, Karl Schebesta, Astrid Chiari, Walter Plöchl — 2012-02-10

Abstract: Background: The Internet has become the largest, most up-to-date source for medical information. Besides enhancing patients’ knowledge, the freely accessible audio-visual files have an impact on medical education. However little is known about their characteristics. In this manuscript the quality of lumbar puncture (LP) and spinal anaesthesia (SA) videos available on YouTube is assessed.Methods: This retrospective analysis was based on a search for LP and SA on YouTube. Videos were evaluated using essential key points (5 in SA, 4 in LP) and 3 safety indicators. Furthermore, violation of sterile working techniques and a rating whether the video must be regarded as dangerously misleading was performed.Results: From 2321 hits matching the keywords, 38 videos were eligible for evaluation. In LP videos, 14% contained information on all, 4.5% on 3 and 4.5% on 2 key points, 59% on 1 and 18% on no key point. Regarding SA, no video contained information on all 5 key points, 56% on 2–4 and 25% on 1 key point, 19% did not contain any essential information. A sterility violation occurred in 11%, and 13% were classified as dangerously misleading.Conclusions: Even though high quality videos are available, the quality of video clips is generally low. The fraction of videos that were not performed in an aseptic manner is low, but these pose a substantial risk to patients. Consequently, more high-quality, institutional medical learning videos must be made available in the light of the increased utilization on the Internet.

Subacute combined degeneration of the spinal cord in a vegan - Corrected Proof

2012-02-10

Highlights: ► We present a case of myelopathy and neuropathy by vitamin B12 deficiency in a vegan. ► MRI and neurophysiological studies showed spinal cord and peripheral nerves damage. ► Neurological disturbances, neurophysiological and spinal cord MRI abnormalities disappeared after cyanocobalamin treatment. ► Vegans should be informed about the potential risk for their health and take daily supplements of vitamin B12 for life. ► Neurologists should consider vitamin B12 deficiency in vegans who develop signs of myelopathy and/or peripheral neuropathy, to make a timely diagnosis and avoid irreversible neurological damages.

Systems neurobiology of restorative neurology and future directions for repair of the damaged motor systems - Corrected Proof

John H. Martin — 2012-02-10

Abstract: Restoring movement control after central nervous system injury requires reconnecting the brain and spinal motoneurons, and doing so with sufficient precision and strength to enable robust voluntary muscle recruitment. Whereas the connection between the upper motoneuron in motor cortex and alpha-motoneurons was thought to be the only important connection for normal motor function in humans, we know that a multiplicity of motor circuits are recruited during normal motor control. Multiplicity of functionally important motor circuits points to the myriad possibilities of intervention that restorative neurology can turn to for repairing motor systems connections to recover movement control after injury. New motor systems repair strategies in animal models and humans are tapping into distributed motor control functions of the spinal cord; neural activity-based approaches, especially for corticospinal tract repair; and circuit-selective activation approaches. I focus on studies harnessing activity-based therapeutic approaches to promote sprouting of spared corticospinal tract axons after injury and redirecting potentially maladaptive plasticity. I discuss that we can see on the near horizon, many different strategies for repairing motor systems connections after injury.

Neurophysiological characterization of the New Anatomy and motor control that results from neurological injury or disease - Corrected Proof

William Barry McKay — 2012-02-10

Abstract: Following injury or disease, the central nervous system (CNS), to varying degrees, loses neurons, synaptic connections and conduction-promoting myelin insulation altering the neural circuitry assembled during development. This “New Anatomy” changes neural processing, bringing spasticity, paresis and paralysis to motor function and altered sensation, numbness and pain to sensory function. Focusing on the effects of CNS damage on the motor subsystems, this review offers a neurophysiological assessment perspective developed within the study of human spinal cord injury and extends it to other CNS disorders. It puts forward the concept that there are essential domains of CNS processing, altered by most neurological disorders, that are temporal, the speed of activation and deactivation, and spatial, the distribution across multiple muscles of motor units selected and activated. Measured through multiple-muscle recordings of selected motor-task performance, these domains can be useful in quantifying the severity of CNS damage and changes achieved through recovery or treatment.

Percutaneous discectomy: Minimally invasive method for treatment of recurrent lumbar disc herniation - Corrected Proof

Haytham Eloqayli, Mamoon Al-omari — 2012-02-10

Abstract: Objectives: Recurrent lumbar disc herniation has been reported in 5–11% of patients. Revision surgery carries a higher risk of complications due to epidural scar formation and difficulty in identifying the bony landmarks. The present study was conducted to apply an innovative procedure which optimally has lower complications, and decrease the need for major surgery for high risk patients.Patient and method: Our study presents six patients of recurrent herniation after past performed procedures such as microdiscectomy, or a laminectomy and discectomy who were readmitted and treated with APLD (automated percutaneous lumbar discectomy) as the first line. The APLD criteria for patient selection in the present study are those with predominantly leg pain that failed conservative treatment for at least 6 weeks and, after lumbar surgery for at least 6 months at the same level.Results: Four of the patients have sciatica recovery signs ranging between excellent to good and, two showed no improvement. None of the screened patients in this study developed any serious complications.Conclusion: Although this is a small series with a short follow up duration, it can be postulated that in the absence of objective evidence of spinal instability, recurrent disc herniation with predominantly leg pain may be treated by APLD as a first line. This can be especially helpful in patients with high risk for anesthesia.

Meningioma-related dural arteriovenous fistula fed via a vascular tumor bed: A case report and literature review - Corrected Proof

Rei Enatsu, Minoru Asahi, Masato Matsumoto, Osamu Hirai — 2012-02-10

Dural arteriovenous fistulas (AVFs) are defined as arteriovenous shunts within dural leaflet. The pathogenesis of dural AVFs is still unknown. Although congenital dural AVFs have been reported, most dural AVFs are considered to be acquired secondary to trauma, thrombophlebitis, surgery and infection .

Effectiveness of home rehabilitation program for ischemic stroke upon disability and quality of life: A randomized controlled trial - Corrected Proof

Pakaratee Chaiyawat, Kongkiat Kulkantrakorn — 2012-02-10

Abstract: Background: To develop and examine the effectiveness of individual 6-month home rehabilitation program in ischemic stroke patients upon disability and quality of life at 2 years.Methods: This is a prospective randomized controlled trial (RCT) in 60 patients with recent ischemic stroke. They were randomly assigned to receive either home rehabilitation program once a month for 6 months with audiovisual materials (intervention group) or usual care (control group). We collected outcome data after discharge from the hospital until 2 years. The Barthel index (BI), the modified Rankin Scale (mRS) and utility index (EQ-5D) were measured for function, disability and quality of life respectively.Results: At 2 years, the BI was significantly improved in the intervention group more than the control group: 97.2±2.8 vs. 76.4±9.4, p<0.001. The good outcome, defined as BI 95–100, or mRS 0 or 1. For BI, there were 29 patients (96.7%) in intervention group vs 12 patients (42.9%) in usual care group (95% CI, 42.0, 85.0, p=0.03). For mRS, there were 28 patients (93.3%) in intervention group vs 9 patients (32.1%) in usual care group (95% CI, 38.2, 87.0, p=0.02). Number needed to treat for good outcome in mRS was 2.0 (95% CI: 1.0, 1.3). The mean (SD) of utility index in intervention group and control group were 0.9±0.02 and 0.7±0.04 respectively (p=0.03). There was no significant interaction in baseline characteristics and treatment outcome.Conclusions: Early home rehabilitation program in the first 6 months period after ischemic stroke leads to more rapid improvement in function, reducing disability and increase quality of life than usual care.

Antiepileptic drug-induced skin reactions: A retrospective study and analysis in 3793 Chinese patients with epilepsy - Corrected Proof

2012-02-10

Abstract: Objective: To evaluate the incidence and clinical characteristics of AED-related skin reactions, along with factors influencing these reactions, in a sample of 3793 Chinese epileptic patients.Materials and methods: Between February 1999 and April 2010, consecutive patients with epilepsy were studied retrospectively. A detailed survey of each patient's medical records concerning all treatment with AEDs was performed.Results: A total of 3793 (2323 male) Chinese epileptic patients taking at least one AED were investigated. Overall, 137/3793 (3.61%) patients experienced a skin reaction following one out of 11 different of AEDs marketed in China. In this study, we found skin reactions from carbamazepine (CBZ) in 3.80% of exposures, from lamotrigine (LTG) in 11.11%, and from oxcarbazepine (OXC) in 8.92%. Skin reactions developed significantly more often in females than in males (4.97% vs. 2.76%), and a logistic regression analysis confirmed female gender as a factor linked to AED-related rashes (OR=1.84, p<0.001). LTG-induced rashes were more frequent in girls under age 13 than in women over the age of 13 (p<0.05).Conclusion: The incidence of skin reactions was somewhat higher for LTG, CBZ, and OXC, whereas valproic acid, levetiracetam, and topiramate were rarely associated with skin reactions. Caution should be exercised when prescribing certain AEDs, particularly CBZ, LTG, and OXC. Females have a higher risk for skin reactions compared to males, though further investigation is needed to discern the underlying mechanisms.

A clinical analysis on microvascular decompression surgery in a series of 3000 cases - Corrected Proof

2012-02-10

Abstract: Objective: Despite the microvascular decompression (MVD) has become a definitive treatment for trigeminal neuralgia (TN) and hemifacial spasm (HFS), not all of the patients have been cured completely so far and this sort of operation is still with risk because of the critical operative area. In order to refine this surgery, we investigated thousands MVDs.Methods: Among 3000 consecutive cases of MVDs have been performed in our department, 2601 were those with typical TN or HFS, who were then enrolled in this investigation. They were retrospectively analyzed with emphasis on the correlation between surgical findings and postoperative outcomes. The differences between TN and HFS cases were compared. The strategy of each surgical process of MVD was addressed.Results: Postoperatively, the pain free or spasm cease occurred immediately in 88.3%. The symptoms improved at some degree in 7.2%. The symptoms unimproved at all in 4.5%. Most of those with poor outcome underwent a redo MVD in the following days. Eventually, their symptoms were then improved in 98.7% of the reoperative patients. The majority reason of the failed surgery was that the neurovascular conflict located beyond REZ or the offending veins were missed for TN, while the exact offending artery (arteriole) was missed for HFS as it located far more medially than expected.Conclusion: A prompt recognition of the conflict site leads to a successful MVD. To facilitate the approach, the craniotomy should be lateral enough to the sigmoid sinus. The whole intracranial nerve root should be examined and veins or arterioles should not be ignored. For TN, all the vessels contacting the nerve should be detached. For HFS, the exposure should be medial enough to the pontomedullary sulcus.

Subacute encephalopathy associated with aquaporin-4 autoantibodies: A report of 2 adult cases - Corrected Proof

Joerg-Patrick Stübgen — 2012-02-10

The traditional concept of neuromyelitis optica (NMO) or Devic's syndrome was of a monophasic disease with near-simultaneous optic neuritis and acute transverse myelitis, without involvement of any other part of the central nervous system (CNS). Revised proposed criteria of NMO incorporated the status of a recently discovered serum autoantibody (NMO-IgG) and the presence of brain abnormalities on magnetic resonance imaging (MRI), thereby broadening the clinical spectrum of the disease . NMO-IgG targets aquaporin-4 (AQP4), an ubiquitous water channel in the CNS with an important role in brain fluid homeostasis and maintenance of the blood–brain barrier (BBB) integrity . The anti-AQP4 antibody has been found to be 73% sensitive and greater than 91% specific for clinically defined NMO , and has not been detected in patients with conventional multiple sclerosis. The pathogenetic role of anti-AQP4 autoantibodies has not been established unequivocally, but antibody specificity and spinal cord immunoreactivity suggest that NMO-IgG serves as a primary effector of disease rather than a non-specific phenomenon . The NMO-IgG autoantibody test is very helpful for confirmation of NMO , though some doubts have been expressed that a single biomarker alone absolutely discriminates disorders in the spectrum of CNS inflammatory disease .

Topiramate and erectile dysfunction: Pathogenic mechanisms beyond sexual hormonal changes! - Corrected Proof

Rocco Salvatore Calabrò — 2012-02-10

I read with great interest the work by Civardi et al., dealing with vasogenic erectile dysfunction (ED) secondary to topiramate (TPM) administration . The authors describe two young patients, in which the main causes of ED, including endocrine and psychological factors, were ruled out suggesting the possibility of vasogenic ED induced by TPM.

Leukoencephalopathy, cerebral calcifications, and cysts: Case report - Corrected Proof

Nadir I. Osman, Matthew T. Lorincz, Kerry L. Hulsing, Stephen S. Gebarski — 2012-02-06

The triad of leukoencephalopathy, cerebral calcifications, and cysts (LCC) represents a very rare, and distinct clinico-radiologic entity of unknown etiology, first described in children by Labrune et al. in 1996. The onset ranges from infancy to adults , with varying clinical presentations including a combination of mild cognitive dysfunction or normal intelligence, seizures, cerebellar, pyramidal and extrapyramidal signs. The disorder is characterized by diffusely progressive basal ganglia, brain stem and subcortical white matter calcifications, cerebellar, and supratentorial parenchymal cysts, as well as diffusely abnormal T2-weighted sequences on MRI . The white matter abnormalities were described as a leukoencephalopathy rather than a demyelinating process (leukodystrophy) . Histopathologic examination reveals angiomatous-like blood vessels, gliosis, and beaded, corkscrew-shaped intracytoplasmic inclusions typically associated with highly gliotic tissue surrounding cysts and vascular malformations (Rosenthal fiber formation) in the white matter . Complications are typically a consequence of mass effect including cerebral edema leading to intracranial hypertension, papilledema, syringomyelia and eventually tonsillar herniation .

Developmental and maladaptive plasticity in neonatal SCI - Corrected Proof

Karen E. Pape — 2012-02-06

Abstract: Babies and young children with early spinal cord injury (SCI) have evidence of an improved level of recovery over an extended time period. Enhanced neuroplasticity is well recognized in neonatal animal models. In the young human, developmental apraxia and learned early habitual movements mask expression of residual or recovered motor function. Techniques providing sensorimotor stimulation with threshold electrical stimulation (TES) and EMG triggered stimulation (ETS) act to increase awareness and useful function. Small cohort size and prolonged developmental maturation argue for the use of single subject research designs in this population.

Unusual manifestations in two cases of necrotizing myopathy associated with SRP-antibodies - Corrected Proof

F. Hanisch, T. Müller, G. Stoltenburg, S. Zierz — 2012-02-06

Abstract: Anti-SRP (signal recognition particle) positive necrotizing myopathy is commonly not associated with neoplasms. We demonstrate two histologically confirmed cases with unusual manifestations of anti-SRP positive necrotizing myopathy.A 65-year-old man presented with rapidly progressing weakness and mild difficulties in swallowing and speaking. Screening for underlying disorders revealed a moderately differentiated renal adenocarcinoma. The muscular symptoms partially improved after tumor nephrectomy and prednisone treatment. However, the patient developed pulmonary metastases and died of the sequelae of pneumonia 11 months after the diagnosis of renal cancer.The second patient developed rapidly complete external ophthalmoplegia, severe bulbar dysarthrophonia and dysphagia, bilateral facial palsy, loss of patellar and ankle jerk reflexes, and severe symmetrical tetraparesis of both proximal and distal muscles. CSF showed mildly increased protein levels, neurography axonal impairment of motor nerves. Screening revealed no evidence for infections, ganglioside antibodies, and carcinoma. MRI was normal. The disease course suggested an overlap syndrome of Miller-Fisher-syndrome, axonal Guillain-Barré-syndrome and Bickerstaff brainstem encephalitis.In conclusion SRP antibodies might be found in necrotizing myopathies associated with autoimmune mediated overlap syndromes and neoplasms. The pathomechanism is not clear. Any otherwise unexplained evidence of necrotizing myopathy should prompt the screening for SRP antibodies.

Central Brown–Sequard syndrome caused by hyperextension: An unexpected complication of cervical pedicle screw fixation - Corrected Proof

Hasan Kamil Sucu, İsmail Ertan Sevin, Murat Yıldırım — 2012-02-03

Brown–Sequard syndrome most often is associated with penetrating trauma to the spine . Simultaneous presences of a Horner's syndrome have been reported and are attributed to the involvement of ipsilateral descending sympathetic fibers within the cervical spinal cord. This report describes a case of atypical Brown–Sequard syndrome, following cervical pedicle screw fixation and laminectomy. In our opinion, this complication was related to hyperextension rather than direct trauma or penetration.

Restorative neurology: Consideration of the new anatomy and physiology of the injured nervous system - Corrected Proof

Keith E. Tansey, William Barry McKay, Byron A. Kakulas — 2012-02-03

Abstract: The adult human nervous system is an incredibly complex set of thousands to tens of thousands of connections between a hundred billion neurons that develops via an intricate spatial-temporal process and is shaped by experience. In addition, any one anatomical arrangement of neural circuits is usually capable of multiple physiological states. Following neurological injury, a new anatomy, and consequently a new spectrum of physiology, emerges within this nervous system with its mix of both injured and uninjured parts. It is this new combination of neural components that determines the extent to which natural functional recovery can occur and the extent to which clinical interventions can further that recovery. Detecting the new anatomy and physiology of the injured human nervous system is difficult but not impossible and some methods can track over time changes in neural structure or, more often, functions that correlate with neurological improvement. The goal of restorative neurology is to make best use of this new anatomy and physiology to facilitate neurological recovery. While we are still learning about how neurorehabilitation interventions generate functional recovery, we can begin to test hypothesis regarding the underlying mechanisms of neural plasticity and attempt to augment those processes.

Unusual CNS presentation of thyroid cancer - Corrected Proof

2012-02-02

Abstract: As advanced therapies allow cancer patients to live longer, disease failure in the central nervous system increases from limited therapeutic penetration. Primary thyroid malignancies rarely metastasize to the brain and have a small number of investigations in literature on the subject. The majority of brain metastases involve the brain parenchyma, reflecting the mass and blood distribution within the brain and central nervous system. Here, we report two cases of the most common differentiated thyroid cancers; follicular thyroid cancer having brain involvement from extra-axial growth and papillary thyroid cancer having brain involvement from a single intraventricular metastasis, presumed as metastasis from the vascular choroid plexus. Both of our cases had widespread systemic involvement. For our follicular thyroid cancer, brain involvement was a result of extra-axial growth from cavarial bone, and our papillary thyroid cancer had brain involvement from a single intraventricular metastasis that was initially resected and nearly a year later developed extensive brain involvement. Unlike the usual gray-white junction metastases seen in the majority of metastatic brain tumors, including thyroid, our cases are uncommon. They reflect differences in tumor biology that allows for spread and growth in the brain. Although there is growing genetic knowledge on tumors that favor brain metastases, little is known about tumors that rarely involve the brain.

Peripheral neuropathy response to erythropoietin in type 2 diabetic patients with mild to moderate renal failure - Corrected Proof

2012-02-02

Abstract: This study assessed the added effect of 6 months of erythropoietin (EPO) administration in patients suffering from diabetic neuropathy with mild to moderate chronic kidney disease (CKD) managed with gabapentin.Twenty diabetic patients with mild to moderate CKD were included; 12 in gabapentin and 8 in EPO+gabapentin group. The subjects underwent nerve conduction studies (NCS) at the initiation of the investigation and after 6-month treatment. NCS were made in deep and superficial peroneal, tibial, and sural nerves.After 6 months, in both the groups, proximal motor latency (PML) nonsignificantly improved in deep peroneal and tibial nerves; conversely, dorsal motor latency (DML) got slightly impaired in these two nerves. A nonsignificant disruption and improvement was observed in deep peroneal and tibial motor nerve conduction velocity (MNCV), respectively, in gabapentin group. Although the F-wave of tibial and deep peroneal nerves remained stable in gabapentin group, a nonsignificant improvement was observed in EPO+gabapentin group. H-reflex of tibial nerve and all the evaluated parameters of sural and superficial peroneal nerves remained constant in all patients.Thus, it can be concluded that 6-month administration of EPO+gabapentin, or gabapentin alone in mild to moderate CKD patients with diabetic neuropathy could not improve nerve performance.

Physical modalities in the treatment of neurological dysfunction - Corrected Proof

Mary P. Galea — 2012-02-02

Abstract: Objective: This chapter presents modalities of physical therapy used in optimizing sensorimotor recovery from nervous system injury.Methods: A brief historical perspective, rationale, indications for application, and evidence of effectiveness of various physical treatment modalities is provided.Results: Many of the facilitatory and inhibitory techniques used in the past are no longer used, as they were based on an understanding of recovery after nervous system injury that is now outdated. There has been a paradigm shift in the management of people with neurological dysfunction. In particular there has been a reduction in focus on the positive features or the upper motor neuron syndrome, such as spasticity, and an increasing emphasis on active, task-related practice of functional tasks.Conclusion: Physical therapy for people with neurological disorders has undergone a paradigm shift as a result of new knowledge about motor control, skill acquisition, and recovery of function after injury. Future research should address new applications of electrical stimulation and whole body vibration as well as the optimal dosage and timing of interventions.

Surgical treatment of unruptured intracranial aneurysms in a low-volume hospital – Outcome and review of literature - Corrected Proof

2012-02-02

Abstract: Background: The aim of this study was to evaluate surgical outcome of unruptured intracranial aneurysms (UIAs) in a low-volume hospital and compare the results with the recent literature.Methods: A retrospective review of all consecutive craniotomies for UIA from July 1999 through June 2009 was performed. Morbidity was defined as modified Rankin Scale (mRS)≥3 and evaluated six weeks after surgery. Cognitive function was evaluated at rehabilitation-to-home discharge. A PubMed database search (2001–2011) seeking retrospective, single-center studies reporting on surgical outcome of UIAs was performed.Results: There were 47 procedures performed in 42 patients to treat 50 UIAs (mean of 5 annual craniotomies). The mean age was 54.7±12.1 years and mean aneurysm size was 7.6±4.0mm. Favorable outcome (mRS 0–2) at six weeks after surgery was achieved in 45 of 47 procedures (95.7%). Aneurysm size≥12mm was statistically significant related to adverse outcome defined as mRS change≥1 (71% vs. 29%; p=0.018). Five patients (10.6%) with favorable neurological outcome (mRS 2) presented with cognitive impairment at rehabilitation-to-home discharge. There was no significant difference in overall morbidity and mortality comparing low- and high-volume hospitals (4.0% vs. 4.8%; p=0.85).Conclusions: Low-volume hospitals may achieve good results for surgical treatment of UIAs. The results indicate that defining numeric operative volume thresholds is not feasible to guide centralization of aneurysm treatment.

Atypical meningiomas – A case series - Corrected Proof

Marko Andric, Shreya Dixit, Arvind Dubey, Peter Jessup, Andrew Hunn — 2012-01-30

Abstract: Meningiomas, in particular the Atypical (grade 2), vary greatly in their behaviour and prognosis. Over a 19year period, we operated on 169 meningiomas (on 86 patients) and of those, 9 cases of atypical meningiomas were found which met the 2007 World Health Organization (WHO) classification. The 9 patients represented 5.3% of all meningiomas. The average presenting age was 51years and average follow-up was 103months with 5 patients passing away between 38 and 219months after diagnosis. The time to first recurrence was 24months with 1 patient suffering 12 recurrences and 2 cases having metastases. Although we had a small number of atypical meningiomas, we believe our paper highlights the unpredictable and difficult nature of these tumours.

Dysthermia as an unexpected onset symptom of systemic lupus erythematosus - Corrected Proof

2012-01-30

Systemic lupus erythematosus (SLE) is a clinically heterogeneous autoimmune disease characterized by the presence of autoantibodies directed against nuclear antigens. SLE is, by definition, a multisystem disease, which can present in a large variety of ways, including cutaneous rash, arthritis, oral ulcers and renal disorders.

Hypocalcemic seizure in adult: Rare cause of lumbar fracture - Corrected Proof

2012-01-27

Bone fractures in epileptic patients may be associated with trauma either induced directly by a seizure or resulting from a fall or other accident resulting from the seizure . Muscular contractions generated during a seizure can lead to a variety of musculoskeletal injuries as fractures and dislocations of the shoulder, femur, and acetabulum . A seizure-induced fracture of the spine is a rare clinical entity with only few reported cases in the medical literature to date . Although rare, it is extremely crucial to recognize this emergent fracture because it predisposes the patient to irreversible neurological injury .

Pineal germinoma with granulomatous reaction, often a pitfall in endoscopic biopsy. Report of two cases and review of the literature - Corrected Proof

K. Schmalisch, G. Pantazis, F.H. Ebner, A. Bornemann, J. Honegger, M. Tatagiba — 2012-01-27

Tumours of the pineal region are rare and account for 0.5–2% of all intracranial lesions. There is a higher incidence in male than in female and they are 10 times more common in children than in adults. Germinoma is the most common tumour in the pineal region appearing up to 50% of all pineal tumours . Intracranial germinomas are malignant neoplasms arising from remnants of primitive germ cells, which have failed to migrate to the genital crest during embryonic stage. Therefore germinomas present the same histology as seminomas and dysgerminomas, their gonadal counterparts. Intracranial germinoma is commonly situated in the midline, occurring mostly in the pineal region. Their origin explains this site of predilection . Most of the patients suffer from intracranial hypertension due to obstructive hydrocephalus and/or symptoms due to pressure or infiltration of adjacent structures. The duration of symptoms ranges from 1 week to 6 years (mean 19 months).

Conservative treatment of a ruptured inflammatory infectious aneurysm caused by neurocysticercosis - Corrected Proof

2012-01-27

Intracranial infectious aneurysms (IIA) have been histologically proven to result from neurocysticercosis vasculitis . The subarachnoid inflammatory exudate of the cysts produces endarteritis, which may then lead to aneurysm formation and rupture. Due to their rarity, cases of IIA usually are diagnosed following a devastating hemorrhage.

Progressive optic neuropathy caused by contact with the carotid artery: Improvement after microvascular decompression - Corrected Proof

2012-01-27

Ectatic cerebral arteries may displace cranial nerves leading to their dysfunction. This is the mechanism felt to underlie most cases of trigeminal neuralgia, hemifacial spasm, and glossopharyngeal neuralgia. Optic neuropathy resulting from vascular compression has also been described . Microvascular decompression has provided visual improvement in patients with optic nerve displacement by an ectatic carotid artery .

Overview of neurophysiology of movement control - Corrected Proof

J.C. Rothwell — 2012-01-27

Abstract: The motoneuronal outputs from cortex and spinal cord have quite different patterns of organisation. The cortex consists of a highly intermixed mosaic of small output zones whereas the motoneurones in the cord are located in clearly defined columns of cells, that all project to the same muscle. I describe the pattern of innervation between cortex and cord, indicate the importance of cortical plasticity in allowing flexible control of spinal circuits, and show how these inputs interact. Finally I discuss some of the new methods of stimulating descending motor pathways in humans.

Clinical, neuroradiological and molecular features of a patient affected by pseudoxhantoma elasticum associated to carotid rete mirabile: Case report - Corrected Proof

2012-01-25

Highlights: ► Carotid rete mirabile (CRM) is a rare condition. ► To report association between CRM and pseudoxanthoma elasticum (PXE). ► Case report. ► CRM may be one of the mechanisms leading to ischemic stroke in PXE patients.

Secondary malignant giant cell tumor of the clivus: Case report - Corrected Proof

2012-01-25

Giant cell tumors (GCTs) of bone are rare primary bone neoplasms, representing approximately 5% of skeletal tumors. The epiphyses of the long bones, particularly the distal femur, proximal tibia, and distal radius, account for 75–90% of GCTs . GCTs rarely occur in the skull, and comprise less than 1% of all reported these tumors of bone GCTs and also preferentially involve the sphenoid and temporal bones . Malignancy arising in a GCT can be expected in less than 2% of GCTs. Malignant transformation of GCT in the skull is exceptionally rare . We report a case of GCT of the clivus, which underwent malignant transformation 10 years after initial treatment. We discuss the diagnosis and therapeutic considerations of GCT of the sphenoid, including the clivus, based on a review of the pertinent literature.

Comparison of two methods for the detection of oligoclonal bands in a large number of clinically isolated syndrome and multiple sclerosis patients - Corrected Proof

Aljoša Andlovic, Maša Babič, Slavko Accetto, Uroš Rot — 2012-01-25

Abstract: Objective: A novel oligoclonal band (OB) assay which consists of isoelectric focusing (IEF) and IgG immunodetection by alkaline phosphatase-labeled anti IgG antibody was reported to be very sensitive. It also accurately predicted conversion to MS in patients with CIS. The aim of our study was to compare sensitivity of a novel and the standard procedure with peroxidase immunodetection in a large number of CIS and MS patients.Methods: OB were determined in serum and CSF samples in 161 patients (104 females), 47 with CIS and 114 with MS with median age 38 years (range 19–68) using both methods.Results: Eighty-three percent of patients had CSF OB with the standard and 89% with the novel method. Median number of OB was 5 (range 0–17) with the peroxidase and 8 (range 0–18) with the alkaline phosphatase method; p=0.001. Twenty-one percent of patients had ≥10 OB with the standard and 37% with the novel method of the detection; p=0.021. Subjective impression of band clarity showed that 20% of patients had sharper and stronger bands when the peroxidase and 65% when the alkaline phosphatase method was used; p<0.0001.Conclusion: The alkaline phosphatase method is more sensitive than the peroxidase method and at the same time cheaper, easy to perform and less time consuming.

Diffusion tensor and dynamic susceptibility contrast MRI in glioblastoma - Corrected Proof

2012-01-24

Abstract: Objective: We prospectively investigated the correlation between diffusion tensor (DTI), dynamic susceptibility contrast (DSC) perfusion MRI metrics and Ki-67 labelling index in glioblastomas.Methods: We studied seventeen patients who were operated on for glioblastoma. DTI and DSC MRI were performed within a week prior to surgical excision. Lesion/normal ratios were calculated for the apparent diffusion coefficient (ADC), fractional anisotropy (FA), relative cerebral blood volume (rCBV), relative cerebral blood flow (rCBF) and relative mean transit time (rMTT) ratio. In the excised tumour specimens Ki-67 antigen expression was evaluated by the MIB-1 immunostaining method.Results: A significant correlation was observed between Ki-67 index and ADC ratio (r=−0.528, p=0.029) and FA ratio (r=0.589, p=0.012). rCBV and rMTT presented a trend towards significant correlation with Ki-67 index (r=0.628, p=0.07 and r=0.644, p=0.06 respectively). There was a trend towards better survival for patients with gross total tumour excision and FA values lower than 0.48 (p=0.1 and p=0.09 respectively). No significant correlation was found between ADC ratio, rCBV, rCBF, rMTT and overall survival.Conclusion: ADC ratio, FA ratio, rCBV and rMTT tumour/normal tissue ratios may represent indicators of glioma proliferation. FA values may hold promise for predicting survival in patients with glioblastoma.

Endovascular treatment of subclavian artery stenosis associated with vertebral artery pseudoaneurysm - Corrected Proof

Shah-Naz Hayat Khan, Paul Henry Young, Andrew Joel Ringer — 2012-01-24

The frequency of radiologically demonstrable subclavian or innominate artery stenosis is approximately up to 17%. Of these, 2.5% have angiographic flow reversal in vertebral artery. Only 5.3% of those with angiographic steal have neurologic symptoms . Treatment is indicated only in this small percentage of symptomatic patients. The symptoms are induced by exercise or exertion using the arm ipsilateral to stenosis. We describe a case with concurrent right vertebral artery (VA) pseudoaneurysm and left subclavian artery (SCA) stenosis, where the pattern of symptoms was suggestive of embolic etiology rather than exercise induced. An unusual presentation of SCA stenosis is highlighted, that resolved with endovascular intervention. The management in the presence of concurrent VA pseudoaneurysm is briefly discussed.

External neurolysis may result in early return of function in some muscle groups following brachial plexus surgery - Corrected Proof

Karin R. Swartz, Michael Boland, Dominic B. Fee — 2012-01-24

Abstract: A retrospective chart review, of those individuals seen and operated on by the Multidisciplinary Brachial Plexus Clinic team at the University of Kentucky Chandler Medical Center, was undertaken to determine those individuals who had early return-of-function following surgery for BPI. Seven patients met our criteria, with four of them having substantial improvement of two or more points gained on the MRC rating scale, in one or more muscle groups within six to eight weeks after surgery. Those patients with return-of-function earlier than expected for axonal regrowth from nerve transfer or grafting, had evidence for continuity but no significant reinnervation before surgery in the muscle groups that improved. We theorize that this early improvement is related to a compression-induced dysfunction which inhibited reinnervation and was relieved by performing external neurolysis.

Spontaneous regression of a thoracic calcified disc herniation in a young female: A case report and literature review - Corrected Proof

2012-01-24

Highlights: ► Thoracic intersomatic disc herniation is a rare entity, comprises only 0.25% of herniated discs. ► Two cases of dorsal hernia spontaneous regression have been reported in the literature. ► We document the first case of calcified dorsal hernia spontaneous regression in an adult.