Aphemia is an apraxia of speech characterized by complete articulatory failure in the presence of preserved writing, comprehension and oropharyngeal function and can be the presenting manifestation of acute stroke. The responsible lesion is commonly in the left inferior frontal gyrus or the left motor cortex near the face M1 area. Three patients who developed aphemia due to acute ischemic stroke are described here. All had apraxia of speech due to acute infarct in the left motor cortex near face M1 area. Understanding the underlying speech disorder is crucial in planning the appropriate rehabilitation strategy.

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Primary melanocytic tumors of the central nervous system (CNS) are uncommon lesions. These lesions arise from the melanocytes located within leptomeninges and include diffuse melanocytosis and meningeal melanomatosis (seen in neurocutaneous melanosis), melanocytoma, and malignant melanoma. To study, the clinical course, neuroradiological features, morphology and immunohistochemistry of primary melanocytic tumor of CNS. Demographic, clinical and surgico-pathologic findings of five patients with melanocytic tumors seen between 1996 and 2003 were studied. In this study, five cases of primary melanocytic tumors have been reported: four cases of malignant melanoma and one case of melanocytoma. Three of the 5 cases were intracranial and 2 were spinal. The mean age in the present study was 26 years. Presenting features varied according to the location. Primary melanocytic tumor of CNS are rare. Whenever possible, complete surgical excision is the best treatment.

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Growing skull fracture is a rare complication of skull fracture and remains almost undetected in the first few years of life. Here, we report a series of 11 patients with growing skull fracture treated at our institute over a period of five years and discuss their clinical features, radiological findings, and principles of management. Of the 11 patients, six were females and five males, with the age ranging between 9 months and 12 years (mean, 3 years). Progressive scalp swelling was the most common presenting feature. Other clinical features included generalised tonic clonic seizures, eyelid swelling, and proptosis. Computed tomography scan of the head defined the growing skull fracture in all 11 patients and detected the underlying parenchymal injury. Postoperatively, all patients had a complete resolution of the scalp swelling. Two patients had postoperative seizures and one had cerebrospinal fluid leak. Early recognition and surgical repair is essential to prevent the development of neurological complications and cranial asymmetry.

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Cervical intradural disc herniation (IDH) is a rare condition and only 25 cases of cervical have been reported. We report a 45-year-old male who presented with sudden onset right lower limb weakness after lifting heavy weight. Magnetic resonance imaging of the cervical spine showed C5/6 disc prolapse with intradural extension. The patient underwent C5/6 discectomy through anterior cervical approach. Postoperatively, the patient improved in stiffness but developed cerebrospinal fluid leak and the leak resolved with multiple lumbar punctures.

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Background : Lateral mass screw placement techniques have been broadly described in the literature. Differences in these techniques are related to entry points, lateral angulations and the cephalocaudal axis. Aim : We evaluated 20 patients who underwent lateral mass screw placement between 2007 and 2009. Computed tomography (CT) scans of the cervical vertebrae were analyzed for each patient. Material and Methods : We measured the maximal transition from the midpoint of the lateral mass to a proposed intersection point by a line connecting the corresponding spinous process and outermost rim of the transverse foramen at each level. This determined an optimal entry point during the tip of screw tilted on the same level of spinous process. Results : The results revealed that a screw entry point less than 3 mm medial to the midpoint of the lateral mass could safely avoid violation of the vertebral artery. Conclusions : The current study uses imaging analysis to demonstrate that spinous processes are an intraoperative landmark to aid surgeons in determining safe lateral mass screw trajectories. The limited-scale case results support our prediction from the image analysis. Depending on intraoperative landmarks, lateral mass screws could be safely and comfortably placed with good clinical outcomes.

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Background : Symptomatic neurosyphilis (NS) can have varied syndromic presentations: Meningitis, meningovascular and parenchymatous involvement. Aims : To evaluate the different types of clinical syndrome of NS in a tertiary care hospital. Material and Methods : This was a study of clinical profile of 16 patients with NS, seen in between August 2008 and December 2010. Results : There were 13 male and 3 female patients in the age group of 23-48 years. The clinical syndromes included: Neuropsychiatric syndromes (10), myelopathy (5), and posterior circulation stroke (1). Neuropsychiatric symptoms were dementia, behavioral abnormalities, chronic psychosis, and myelopathy syndromes included acute transverse myelitis (ATM), chronic myelopathy, and syphilitic amyotrophy. Thirteen patients had positive venereal disease research laboratory test (VDRL) and/or high Treponema pallidum hemagglutination titer in blood. Cerebrospinal fluid was positive for VDRL in 14 patients, raised protein was seen in 13 patients and lymphocytic pleocytosis was seen in 10 patients. Human immunodeficiency virus serology was negative in all the patients. Fourteen patients fulfilled the criteria of definitive NS and two of presumptive NS. All the patients except one received injection Procaine penicillin for 14 days. The patient with myelitis received a course of steroid, and one patient with associated hypothyroidism received thyroid supplement in addition to penicillin. On follow-up, dementia of short duration and ischemic stroke improved significantly and clinical status remained the same for ATM; others with mild symptoms improved with residual deficit. Conclusion : Syphilis can affect any part of the neuraxis. A high index of clinical suspicion is required to diagnose NS and institute the treatment early, particularly in patients with promiscuous sexual behavior.

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Background : Patients with acute ischemic stroke who had early neurological improvement had better functional outcome. The purpose of this study was to determine factors associated with early clinical improvement and early worsening in patients with acute ischemic stroke treated with intravenous thrombolysis. Patients and Methods : Patients treated with intravenous recombinant tissue plasminogen activator (rtPA) between August 2008 and November 2010 were the subjects of this study. Early improvement was defined by marked, clinical improvement or complete recovery at 24 h (National Institutes of Health Stroke Scale (NIHSS) 0-4 at 24 h). Early worsening was defined by an increase in NIHSS ≥1 from baseline. The baseline characteristics were compared between patients with and without outcome of interest. Results : Of the 203 patients studied, 19 (9.4%) patients had complete recovery and 68 (33.5%) patients had marked clinical improvement (NIHSS 1-4) at 24 h. Most patients with early clinical improvement (86%) had favorable outcome at three months. Of the 22 (10.8%) patients who had early clinical worsening, only three (14%) patients achieved favorable outcome at three months and six (29%) patients died. Multivariate analysis revealed that older age (≥70 years old) (odd ratio (OR) 0.498, P = 0.049), severe stroke (NIHSS ≥15) (OR 0.154, P < 0.0001) and having intracerebral hemorrhage (ICH) (OR 0.364, P = 0.032) were inversely associated with early improvement. History of transient ischemic attack (TIA) (OR 7.724, P = 0.043) and ICH (OR 4.477, P = 0.008) were related to early worsening. Conclusions : The presence of early clinical improvement or worsening within 24 h after treatment with rtPA had major impact on the outcome at three months.

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Objectives : To document our experiences, technical procedure and outcomes of carotid endarterectomy (CEA) in patients with symptomatic carotid stenosis. Material and Methods : A total of 49 consecutive patients underwent 53 CEAs (three bilateral, one redo) for severe carotid artery stenosis. There were 39 males and 10 females, with mean age of 63 years. All patients underwent neck Doppler, computed tomography/magnetic resonance angiography/digital substraction angiography and a detailed cardiological evaluation. Antiplatelet drugs were continued perioperatively. Surgery was performed under general anesthesia with propofol cerebral protection, mild hypothermia and continuous electroencephalogram monitoring. The procedure was done under microscope and closure was done using 6-0 prolene. Clinical and radiological follow-up was done. Results : Our mean follow-up was 4.69 years. All patients underwent primary suturing except one redo CEA done with venous patch graft. Three patients required intraoperative shunting. One patient died secondary to myocardial infarction peroperatively prior to carotid manipulation. One patient had stroke within 6 h, secondary to operative site intraluminal thrombus and was re-explored. Two patients had transient postoperative hemiparesis and aphasia while two patients had altered sensorium, all self-limiting, with normal imaging. One patient developed temporary twelfth-nerve paresis. One patient had persistent transient ischemic attack on the follow-up. Thus, the perioperative mortality rate was 1.89% and stroke rate was 1.89%. Conclusions : CEA for severe carotid stenosis is a safe procedure with good protection from ischemic events. Detailed preoperative cardiac evaluation and appropriate patient selection is essential.

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Symmetrical hyperintense signals in lateral columns and posterior columns in T2-weighted images is a characteristic finding in subacute combined degeneration (SCD) secondary to vitamin B12 deficiency. Manifestation of SCD on diffusion-weighted imaging (DWI) has not been well characterised till date. We reported a case of SCD with spinal cord hyperintense signals in DWI sequence. We suggest that spinal cord DWI may be a useful magnetic resonance sequence for the diagnosis of SCD.

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Papillary tumors of the pineal region (PTPRs) are rare and are recognized as a distinct entity in the recent 2007 World Health Organization (WHO) classification of tumors of nervous system. Till date, only 55 cases have been reported. We report another three patients of PTPRs with characteristic radiological features. Histologically, all the three patients exhibited remarkable uniform histology of epithelioid morphology with variable presence of single to multilayered papillary architecture. Consistent absence of fibrillary matrix was noted in all the three cases. None of the cases showed aggressive histology. A large multicenter study is essential for upfront characterizing the biological behavior, as frequency of these tumors is very low.

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Angioplasty and stenting in symptomatic intracranial stenosis is technically possible and may reduce the risk of stroke in patients with symptomatic arterial stenosis. We report a patient with P1 segment stenosis of posterior cerebral artery treated successfully with angioplasty and stenting with a favorable outcome. He had 5 years of clinical and imaging follow-up and no in-stent stenosis or new ischemic event was observed.

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Carotid stenosis is seen in 10% of patients with ischemic stroke, and carotid endarterectomy (CEA) and carotid artery stenting (CAS) are the two invasive treatments options available. Pooled analysis of the three largest randomized trials of CEA involving more than 3000 symptomatic patients estimated 30-day stroke and death rate at 7.1% after CEA. Some subgroups among the symptomatic patients appeared to have more benefit from CEA. These include patients aged 75 years or more, patients with ulcerated plaques, and patients with recent transient ischemic attacks within 2 weeks of randomization. Selection of asymptomatic patients for carotid revascularization should be guided by an assessment of comorbid conditions, life expectancy, and other individual factors, and should include a thorough discussion of the risks and benefits of the procedure with an understanding of patient preferences. The recent trials comparing CEA with CAS has not established its superiority over CEA. The carotid revascularization endarterectomy versus stenting (CREST) study showed that CAS is still associated with a higher periprocedural risk of stroke or death than CEA. In patients over 70 years of age, CEA is clearly superior to CAS. The increased risk of nonfatal myocardial infarction in the CREST group subjected to CEA clearly suggests that patients being considered for CEA or CAS require a careful preliminary cardiac evaluation. CAS can be justified for patients whose medical comorbidities or cervical anatomy make them questionable candidates for CEA. The benefit of revascularization by either method versus modern aggressive medical therapy has not been established for patients with asymptomatic carotid stenosis.

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We report an adolescent male with X-linked agammaglobulinemia (XLA) and recurrent episodes of pyogenic meningitis. The workup for proportionate short stature revealed isolated growth hormone deficiency. This patient highlights the delayed presentation of the XLA variant and the need to consider primary immunodeficiency in patients with recurrent serious infections, irrespective of age.

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We report the technique and results of the endovascular treatment of jugular foramen dural arteriovenous fistulas (DAVFs) in 4 (3 men and 1 women, mean age 50.75 years) symptomatic patients. The jugular foramen DAVFs accounted for 5.9% of intracranial DAVFs. Three patients presented with pulsatile tinnitus and 1 patient presented with intracranial hemorrhage. Angiography demonstrated an AV fistula at the jugular foramen, mostly arising from the middle meningeal, ascending pharyngeal and vertebral arteries with direct drainage to the internal jugular vein. All patients underwent transarterial embolization using Onyx-18. Complete shunt obliteration was achieved in 3 patients; and shunt reduction, in 1 patient, who was cured with additional surgery. Our study suggests that in jugular foramen DAVF transarterial embolization with Onyx should be considered when access is available.

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Background : Until now, no method has been available to rapidly differentiate between the motor and sensory nerve fascicles introperatively. Aim : To establish a method to rapidly differentiate between the sensory and motor fascicles in the peripheral nerves. Material and Methods : Annexin V-CdTe-conjugated polymer was used to stain the sciatic and sural nerve fascicles of adult male Wistar rats for 10, 15, 20, and 30 min. Results : Under a light microscope, the sural nerves and the sensory fascicles of the sciatic were visualized as bright red fluorescence with Annexin V-CdTe-conjugated polymer staining. In contrast, no fluorescence on the motor fascicles of the sciatic nerve could be visualized. Fluorescence intensity was not strong enough to show the nerve fascicles with 10 min of staining; however, the intensity was clearly visible after 15 min of staining. No significant difference in the intensity of staining was observed among samples stained for 15, 20, and 30 min. Conclusions : Our study shows that Annexin V-CdTe-conjugated polymer can differentiate the motor and sensory nerve fascicles of the peripheral nerve rapidly and precisely in vitro. This technique represents a new method for the identification of peripheral nerve fascicles.

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Early restoration of flow to perfuse salvageable brain tissue has been shown to significantly reduce the morbidity and mortality of ischemic stroke. Several mechanical devices have shown promising results in patients with large vessel occlusive stroke. Solitaire revascularization device (ev3, Inc., Irvine, California) is a recoverable self-expanding thrombectomy device that can also be used as a temporary endovascular bypass. We report a patient in whom solitaire aneurysm bridging stent was used as a temporary bypass procedure to recanalize carotid T occlusion patient with good functional outcome.

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Background : Experimental studies have yielded a wealth of information related to the pathological mechanism of optic nerve injury. However, there is no suitable animal model to study intracranial optic nerve injury. Aim : To establish an experimental model of acute optic nerve injury. Material and Methods : We established an animal model of acute intracranial optic nerve injury using the classic pterional approach in cats and investigated electrophysiological and ultrastructural changes. We applied immunohistochemical staining to examine the expression of glial fibrillary acid protein, neurofilament protein, myelin basic protein pre- and post-injury. Results : We successfully established an animal model of acute intracranial optic nerve injury. Conclusion :The pathological processes of acute optic nerve injury may involve the following series of steps. Direct mechanical injury of the optic nerve leads to the death of oligodendrocytes in the optic nerve, which consequently results in optic nerve demyelination. Following optic nerve injury, the astrocytes in the injured area die and produce excitatory amino acids, which have an adverse effect on neurons, resulting in the proliferation and activation of astrocytes. The astrocytes can absorb the glutamic acid and transform it into atoxic glutamine. The glutamic acid can then injure retinal ganglion cells, resulting in the reduction of neurofilament proteins in the axons. We believe that the application of the pterional approach to establish optic nerve injury animal models has both a clinical and theoretical basis.

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Despite medical management, symptomatic intracranial atherosclerotic disease has a high risk of stroke and death. This unacceptably high rate has provided the impetus for development and continuous advancements in the field of endovascular revascularization. From early attempts at angioplasty alone to state-of-the art stents including self-expanding and drug-eluting stents specially designed for the cerebral vessels, developments in this field has come a long way. As we stand today, however, there are still mixed views on the use of these endovascular techniques vs aggressive medical management. In this article, we review the mechanisms of stroke in patients with intracranial atherosclerotic disease and review the current status of stenting in this condition. A brief discussion of the important clinical and procedural considerations is also provided along with a mention of the ongoing trials likely to provide valuable information on the future of stenting.

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The gene encoding RhoA guanine nucleotide exchange factor 10(ARHGEF10) has been reported to be a risk factor for atherothrombotic stroke (AS) in Japanese. The single-nucleotide polymorphism (SNP) rs4376531 in intron 16 on ARHGEF10 is associated with AS and may play a role in the disease pathology. In order to explore the nature of this association in greater detail and in a new ethnic group, we carried out a case-control study to determine whether the rs4376531 polymorphism in ARHGEF10 is a risk factor of AS in Han Chinese people. This study was carried out to assay the frequency of genotypes and alleles of SNP rs4376531 in ARHGEF10 in patients with ischemic stroke and healthy controls using the polymerase chain reaction and the restriction fragment length polymorphism (PCR-RFLP) technique. A total of 383 individuals with AS in West China Hospital and 214 unrelated healthy controls were recruited. The frequencies of the G allele and GG genotype of the rs4376531 polymorphism were higher in the patients with AS than in control individuals: frequency of G, 91.0% vs 83.4%, P<0.001; GG, 82.2% vs 67.8%, P<0.001. After adjusting for sex, age, and multiple cardiovascular risk factors, the homozygous GG genotype for this variant was associated with a higher risk of AS, with an adjusted odds ratio of 4.99 (95% CI, 2.55-7.81, P< 0.001). Our findings suggest that the rs4376531 polymorphism in the ARHGEF10 gene is a risk factor for AS in the Han Chinese population.

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Background : Intraprocedural aneurysm rupture is considered to be one of the most formidable complications of the endovascular treatment of cerebral aneurysms and is associated with high mortality. Objective : To report the clinical outcomes of cerebral aneurysms that ruptured during endovascular coiling. Patients and Methods : Over a period of six years, 559 endovascular embolizations were performed in 467 patients, with 507 cerebral aneurysms. Intraprocedural aneurysm rupture occurred in 14 cases (mean aneurysm size, 3.8 mm). Follow-up angiograms, at a minimum of three months post embolization, were available in 11 living patients. Acute and follow-up results were reviewed. Results : The difference in the rates of aneurysm perforation during endovascular coiling between ruptured and unruptured aneurysms was significant (P < 0.05). There were three (21.4%) deaths related to this complication and three (21.4%) patients developed new deficits (modified Rankin Scale scores 1 to 2). Acute results of embolization were: complete occlusion in eight (57.1%), neck remnant in two (14.3%), and incomplete occlusion in four (28.6%) patients. Long-term follow-up results in 11 living patients were: major recanalization in one (9.1%), minor recanalization in one (9.1%), and stable occlusion in nine (81.8%). Conclusion : Intraprocedural aneurysm rupture frequently occurs in small aneurysms and appears to be associated with relatively high rates of mortality.

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Intracranial primary extraskeletal chondrosarcomas are extremely rare. We report two cases of the classical variant which were dural based: one falcine and the other parasagittal are presented. Only 10 cases of this variant have been reported in this location. The pathology and management of these lesions have been reviewed.

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Rosai Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a benign histiocytic proliferative disorder mainly affecting the lymph nodes. Although several cases of extra-nodal involvement have been reported previously, central nervous system involvement, particularly in the absence of nodal disease is extremely rare. We report a case of isolated intracranial RDD occurring in a relatively elder patient, which was shown by histological examination to have a dura-based involvement.

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Sinus histiocytosis with massive lymphadenopathy or Rosai-Dorfman disease is a rare, but well documented entity. We report a lady who presented with progressive quadriparesis, with cervical extradural lesion on magnetic resonance imaging. She underwent decompression of the lesion and histological diagnosis of the lesion was Rosai-Dorfman disease. On one-year follow-up, she had complete improvement of the deficits with no further progression of the lesion. The presentation of this disease as an isolated spinal extradural mass lesion is quite rare, with only six cases reported in literature.

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Background : Vertebral artery ostial stenosis (VAOS) is one of the most frequent causes of posterior circulation stroke. Percutaneous angioplasty and stenting (PTAS) is one of the treatment modalities. Patients and Methods : This is a longitudinal observational study from September 2006 to February 2009, conducted at hospitals affiliated with the Shiraz University of Medical Sciences, Shiraz, southern Iran. The patient cohort included patients with posterior circulation stroke or transient ischemic attack (TIA) and more than 70% VAOS, and patients with asymptomatic VAOS of more than 70% and aplasia or total occlusion of the contralateral VA or subclavian artery. All the patients underwent PTAS with balloon-mounted bare coronary stents. Technical success, procedural complications, composite outcomes of death, stroke or TIA in the vertebrobasilar territory during the first 30 days, stroke or TIA in the vertebrobasilar territory, and restenosis during follow-up, were assessed. Results : During the study period, 81 patients (mean age 68 + 8.9 years, 63 (78%) males, 71 symptomatic and 18 asymptomatic) underwent the procedure. Technical success was achieved in 88 (99%) patients. Procedure-related complications, other than puncture site complications, were seen in two patients (2.2%). The composite outcome of death, stroke or TIA in the vertebrobasilar territory during the first 30 days was 1%. None of the patients had clinical recurrence or restenosis during the follow-up (mean follow-up 14 months). Conclusion : Percutaneous transluminal angioplasty and stenting of the proximal VA was feasible and safe. The risk of restenosis should be analyzed in long-term studies with angiographic follow-up.

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Background : Pre-operative evaluation to localize function within the cerebral cortices is essential before brain surgery. Blood oxygenation level-dependent functional magnetic resonance imaging (fMRI) has been used for this purpose. Aims : To obtain clearer and more understandable functional images. Patients and Methods : Ten patients with brain tumors underwent fMRI including hand-gripping and word generation tasks. The statistical parametric mapping (SPM) approach was used for subsequent analysis to localize the motor or language functions. SPM includes image pre-processing, statistical computation, and significance testing. In order to demonstrate a spatial relationship between the lesions and a functioning area in the individual structural MR images, normalization to the Montreal Neurological Institute coordinates was intentionally not performed. Results : In seven cases out of 10, the patient's motor area was clearly visualized. Language areas were also demonstrated in seven cases. Conclusions : We conclude that application of SPM (version 8) analysis to non-normalized individual data for the purpose of performing pre-operative fMRI is a useful method for investigation of functional localization.

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