Neurological Symptoms In Patients Research Articles

Vasa Vasorum Enhancement on Computerized Tomographic Angiography Correlates With Symptomatic Patients With 50% to 70% Carotid Artery Stenosis

Significant stenosis of the internal carotid artery (ICA) is an established stroke risk factor. Recent evidence suggests that features within the atherosclerotic plaque also have prognostic value. The purpose of this study was to correlate the enhancement of the vasa vasorum (VV) overlying the carotid artery plaque with acute neurological symptoms in patients with 50% to 70% ICA stenosis. We conducted a 4-year retrospective computerized tomographic angiographic review to identify patients with 50% to 70% stenosis of the ICA. Three types of plaques were identified: enhancing VV, calcified, and nonenhancing-noncalcified. Medical records were reviewed for cardiovascular risk factors and neurological status, and imaging was reviewed for signs of a recent stroke. We identified a total of 428 patients with 50% to 70% ICA stenosis: 103 (24.1%) had enhancing VV, 202 (47.2%) calcified, and 123 (28.7%) nonenhancing-noncalcified arteries; 97 were symptomatic and 331 asymptomatic. Thirty-three (34%) symptomatic subjects demonstrated enhancing VV, 42 (20%) had calcified arterial plaques, and 22 (17%) had nonenhancing-noncalcified arterial plaques. Fisher exact tests revealed that the proportion of symptomatic individuals with enhancing VV plaque was double that of the other groups combined (P=0.015; odds ratio, 1.92; 95% confidence interval, 1.17-3.16). Regression analyses confirmed this association as independent from other known cardiovascular risk factors. In patients with 50% to 70% ICA stenosis, VV enhancement recognized on computed tomographic angiography is strongly associated with acute neurological symptoms compared with calcified and nonenhancing-noncalcified arterial plaques. This finding may aid in the identification of patients at increased risk for ischemic stroke within populations with the same degree of stenosis.

Treatable inborn errors of metabolism causing neurological symptoms in adults

BackgroundThe identification of inborn errors of metabolism (IEM) in adults presenting with a wide range of neurological symptoms is a relatively new field in medicine. We sought to identify which treatable IEM have been diagnosed for the first time in adults and generate a protocol for metabolic screening targeting those treatable disorders. MethodsMedline/Pubmed searches of English language literature limited to the adult age group were performed. Diseases identified through this search were then compared to previously published lists of treatable IEM in both adults and children. Results85% of the treatable conditions known to cause global developmental delay or intellectual disability in children had reports where the diagnosis of that IEM was made in one or more adult patients with neurological symptoms. Screening tests in blood, urine, CSF and MRI can detect most of these treatable conditions but the diagnostic accuracy of these screening tests in adults is not clear. ConclusionTreatable IEM need to be considered in the differential diagnosis of neurological symptoms in patients of any age.

Non-Convulsive Status Epilepticus Associated With Glutamic Acid Decarboxylase Antibody

Autoimmune encephalitis associated with glutamic acid decarboxylase antibodies (GAD-Ab) often presents with treatment-resistant partial seizures, as well as other central nervous system symptoms. In contrast to several other well-characterized autoantibodies, GAD-Ab has very rarely been associated with status epilepticus. We report a 63-year-old woman initially admitted with somnolence and psychiatric findings. The EEG findings, of generalized and rhythmical slow spike-wave activity over the posterior regions of both hemispheres, together with the clinical deterioration in responsiveness, led to the diagnosis of non-convulsive status epilepticus. Investigation of a broad panel of autoantibodies, revealed only increased serum GAD-Ab levels. Following methylprednisolone and intravenous immunoglobulin treatments, the patient's neurological symptoms improved, EEG findings disappeared and GAD-Ab levels significantly decreased. GAD-Ab should be added to the list of anti-neuronal antibodies associated with non-convulsive status epilepticus. Disappearance of clinical findings and seroreversion after immunotherapy suggest that GAD-Ab might be involved in seizure pathogenesis.

Cranial irradiation in adults diagnosed with acute myelogenous leukemia presenting with hyperleukocytosis and neurologic dysfunction

This study describes our institution's experience using whole brain radiation therapy (WBRT) to treat patients with acute myelogenous leukemia (AML) presenting with hyperleukocytosis. After approval by the institutional review board, we identified patients with AML and hyperleukocytosis using hospital records. The primary endpoints in the study included alleviation of neurological symptoms (or prevention if prophylactic RT was used), overall survival, development of intracranial hemorrhage (ICH) and ≥ grade 3 toxicities using the Common Terminology Criteria for Adverse Events version 4.0 (CTCAE v4.0). Eighteen patients received WBRT for the treatment of AML hyperleukocytosis. Thirteen patients received treatment in order to control neurological symptoms. Clinical assessment showed that 12 of 13 patients (92%) achieved resolution of neurological symptoms either concurrent with RT or immediately after RT. The mean overall survival for all of the patients who received WBRT was 14.2 months (95% confidence interval, 5.4–23.0). No patient who received RT experienced ≥ grade 3 toxicity. Two (6%) patients developed ICH following therapy. Our institution's experience demonstrates that WBRT may be utilized as part of multimodality therapy in order to alleviate or prevent neurological symptoms in patients with AML presenting with leukostasis.

Steroid management in newly diagnosed glioblastoma

Glucocorticoids ameliorate neurologic symptoms in patients with glioblastoma, but their adverse effects limit long-term use. This study sought to identify factors associated with steroid taper success or failure in the early stages of glioblastoma treatment. We retrospectively reviewed steroid prescribing practices from date of surgery until one month following radiotherapy (RT) completion among 85 patients with newly diagnosed glioblastoma who were treated on a prospective clinical trial with RT and temozolomide. Sufficient information on steroid dosing was available in 72 patients included in the final analysis. The mean age was 54 years, and 65 % were men. Thirty-nine percent had a gross-total resection. Fifteen patients (21 %) tolerated steroid taper without requiring dose increase during the study. Men and patients with Karnofsky performance scale 90-100 were more likely to have a successful steroid taper. The most common symptom of taper failure was headache, but the reason for steroid increase differed among the different time intervals examined: worsening neurologic deficit in the early post-operative period, headache and non-focal symptoms during RT, and headache and seizure post-RT. Of the 50 patients in whom steroid use during RT was known, 36 (72 %) underwent dose reduction and of those, 21 (58 %) required an increase. The successful early taper of steroids in glioblastoma was associated with male gender and better functional status. Steroids are often tapered during RT, but there is frequent taper failure with this approach. A prospective trial with standardized steroid dosing regimens would be needed to verify these findings.

Blood–cerebrospinal fluid barrier dysfunction in patients with neurological symptoms during the 2011 Northern German E. coli serotype O104:H4 outbreak

ARTICLE Sir, we read with great interest the recently published article by Magnus et al. (2012) describing the neurological symptoms in patients with a complicated Shiga toxin–producing Escherichia coli infection. This article is of great relevance owing to the large number of 104 cases with neurological sequelae out of a cohort of 217 patients of this rare disease. These 104 patients were subjected to neurological, neuroradiological, neurophysiological, CSF and neuropathological examinations. CSF analysis was performed in 10 patients due to haemolytic uremic syndrome–associated thrombocytopaenia in many others. The only CSF abnormality reported was a slight protein elevation (600–1200 mg/l) in three patients. During the outbreak of Shiga toxin–producing E. coli in 2011, we also had the opportunity to be involved in the treatment of 52 patients (Weissenborn et al. , 2012). Five female patients with severe neurological symptoms (Table 1) were subjected to CSF analysis. In contrast to the report of Magnus et al. …

A study of sacrum with five pairs of sacral foramina in western India.

Background: The sacrum is a large, triangular fused five sacral vertebrae and forms the postero-superior wall of the bony pelvis. Numerous anatomical variations of the sacrum have been reported including complete bilateral sacralization of the fifth lumbar vertebra or complete bilateral sacralization of first coccygeal vertebra in different race generates sacrum with five pairs of sacral foramina. There has never been reported among Gujarati population in Western India. These variations may be found in the living during radiological investigations for pain and neurological symptoms of patients or may be found during post mortem examination or during dissection of human body and osteology class for undergraduate students. Aims & Objective: The study was designed to know the prevalence of sacrum with five pairs of sacral foramina among Gujarati population in Western India as there is paucity of available literature. Considering the variation, we conduct this study as a prelude to any type of experimental work in biomechanics, for diagnostic and therapeutic purposes in low back pain, sciatica, coccydynia, spinal surgery and for interventional procedures like spinal anaesthesia and lumbar puncture. Material and Methods: Observational study was carried out on 302 dry human sacra from Department of Anatomy, B.J. Medical college Ahmedabad, Government Dental college Ahmedabad, Medical college Vadodara and Government Medical College, Surat in Gujarat. Morphological study was done on the sacrum. The sacra consisting of six vertebral segments were selected. The specimens were then carefully examined and recorded. Results: 302 dry human sacra were studied. Sacra with five pairs of sacral foramina were recorded. We founded 51 specimens of sacrum with five pairs of sacral foramina representing Bilateral complete sacralization of fifth lumbar vertebra (24 sacra) and bilateral complete sacralization of first coccygeal vertebra (27 sacra). Conclusion: The prevalence of a sacrum with five pairs of sacral foramina varies in general population is not reported separately. The present study shows that the incidence of sacrum with five pairs of sacral foramina among Gujarati population in Western India is 16.9%. Prevalence of sacrum with five pairs of sacral foramina is 7.9% and 8.9% due to sacralization of fifth lumbar vertebra and sacralization of first coccygeal vertebra, respectively. The knowledge of this anatomical variation is of paramount importance to spinal surgeons, obstetricians, radiologists, forensic experts, morphologists and clinical anatomists.

Dengue: a new challenge for neurology

Dengue infection is a leading cause of illness and death in tropical and subtropical regions of the world. Forty percent of the world's population currently lives in these areas. The clinical picture resulting from dengue infection can range from relatively minor to catastrophic hemorrhagic fever. Recently, reports have increased of neurological manifestations. Neuropathogenesis seems to be related to direct nervous system viral invasion, autoimmune reaction, metabolic and hemorrhagic disturbance. Neurological manifestations include encephalitis, encephalopathy, meningitis, Guillain-Barré syndrome, myelitis, acute disseminated encephalomyelitis, polyneuropathy, mononeuropathy, and cerebromeningeal hemorrhage. The development of neurological symptoms in patients with positive Immunoglobulin M (IgM) dengue serology suggests a means of diagnosing the neurological complications associated with dengue. Viral antigens, specific IgM antibodies, and the intrathecal synthesis of dengue antibodies have been successfully detected in cerebrospinal fluid. However, despite diagnostic advancements, the treatment of neurological dengue is problematic. The launch of a dengue vaccine is expected to be beneficial.

Dorsal myelopathy secondary to epidural fibrous scar tissue around a spinal cord stimulation electrode

The authors describe a patient with delayed thoracic spinal cord compression due to fibrous scar tissue around an epidural electrode used in spinal cord stimulation (SCS). One year after implantation of the system the stimulation became ineffective, and 1 year later the patient developed progressive paraparesis. There was no evidence of device-related complications on plain radiographs and CT scans, so the system was removed to perform MRI studies. These studies showed a dorsal myelopathy secondary to scar tissue around the electrode. At surgery thick scar tissue was resected, and the patient's neurological symptoms improved. The histological examination confirmed fibrosis, and microbiological studies excluded chronic infection. As far as the authors are aware, this complication has never been reported before at the thoracic level. Scarring around SCS electrodes should be considered as a late complication and as a possible cause of the tolerance phenomenon.

Cerebrospinal fluid HIV escape associated with progressive neurologic dysfunction in patients on antiretroviral therapy with well controlled plasma viral load

To characterize HIV-infected patients with neurosymptomatic cerebrospinal fluid (CSF) 'escape', defined as detectable CSF HIV RNA in the setting of treatment-suppressed plasma levels or CSF RNA more than 1-log higher than plasma RNA. Retrospective case series. Four urban medical centers in the United States and Europe. Virologically controlled HIV-infected patients on antiretroviral therapy (ART) with progressive neurologic abnormalities who were determined to have CSF 'escape'. INTERVENTION Optimization of ART based upon drug susceptibility and presumed central nervous system exposure. Levels of CSF HIV RNA and inflammatory markers, clinical signs and symptoms, and MRI findings. Ten patients presented with new neurologic abnormalities, which included sensory, motor, and cognitive manifestations. Median CSF HIV RNA was 3900 copies/ml (range 134-9056), whereas median plasma HIV RNA was 62 copies/ml (range <50 to 380). Median CD4 T-cell count was 482 cells/μl (range 290-660). All patients had been controlled to less than 500 copies/ml for median 27.5 months (range 2-96) and five of 10 had been suppressed to less than 50 copies/ml for median 19.5 months (range 2-96). Patients had documentation of a stable ART regimen for median 21 months (range 9-60). All had CSF pleocytosis or elevated CSF protein; seven of eight had abnormalities on MRI; and six of seven harbored CSF resistance mutations. Following optimization of ART, eight of nine patients improved clinically. The development of neurologic symptoms in patients on ART with low or undetectable plasma HIV levels may be an indication of CSF 'escape'. This study adds to a growing body of literature regarding this rare condition in well controlled HIV infection.

CDKL5, a novel MYCN-repressed gene, blocks cell cycle and promotes differentiation of neuronal cells

Mutations in the CDKL5 (cyclin-dependent kinase-like 5) gene are associated with a severe epileptic encephalopathy (early infantile epileptic encephalopathy type 2, EIEE2) characterized by early-onset intractable seizures, infantile spasms, severe developmental delay, intellectual disability, and Rett syndrome (RTT)-like features. Despite the clear involvement of CDKL5 mutations in intellectual disability, the function of this protein during brain development and the molecular mechanisms involved in its regulation are still unknown. Using human neuroblastoma cells as a model system we found that an increase in CDKL5 expression caused an arrest of the cell cycle in the G0/G1 phases and induced cellular differentiation. Interestingly, CDKL5 expression was inhibited by MYCN, a transcription factor that promotes cell proliferation during brain development and plays a relevant role in neuroblastoma biology. Through a combination of different and complementary molecular and cellular approaches we could show that MYCN acts as a direct repressor of the CDKL5 promoter. Overall our findings unveil a functional axis between MYCN and CDKL5 governing both neuron proliferation rate and differentiation. The fact that CDKL5 is involved in the control of both neuron proliferation and differentiation may help understand the early appearance of neurological symptoms in patients with mutations in CDKL5.

Fusion PET-CT detection of neurolymphomatosis originating from primary breast lymphoma: A case report and literature review

Neurolymphomatosis (NL) is an extremely rare manifestation. Involvement of the cranial and peripheral nerves is an unusual manifestation of NL which is occasionally difficult to diagnose using conventional imaging modalities. A 45-year-old female in complete remission from primary breast lymphoma presented with multiple neurological symptoms. (18)F-FDG PET/CT revealed multiple nodular hypermetabolic lesions along the cranial and peripheral nerves, which corresponded to the patient's neurological symptoms. These findings suggest that (18)F-FDG PET/CT successfully detects the infiltration of cranial and peripheral nerves by lymphoma.

Pyogenic vertebral osteomyelitis in a breast cancer patient: report of a case

We herein report a rare case of pyogenic vertebral osteomyelitis (PVO) coexisting with breast carcinoma. A 71-year-old female presented with neck pain without fever. Magnetic resonance imaging (MRI) showed suspected metastatic lesions in her neck (C7 and Th1). Fluorine-18 fluorodeoxyglucose positron emission tomography (FDG-PET) showed increased FDG uptake in the neck spines and in the left breast. A core needle biopsy of the left breast revealed the presence of invasive ductal carcinoma. Our first tentative diagnosis of the patient was left breast carcinoma with bone metastases, and first-line endocrine therapy was started. However, surgical intervention for the spines had to be considered, because her neurological symptoms progressed. A repeated MRI scan showed a narrowing of the disc space and fluid accumulation around the vertebrae. This suggested the presence of PVO rather than metastases. Surgery confirmed the presence of PVO in C7 and Th1, and a culture of the abscess yielded Escherichia coli. The patient's neurological symptoms dramatically improved after surgery. Breast conserving surgery was performed 3 months after the surgery for PVO. The patient is well and has no clinical evidence of disease 18 months after the breast conserving surgery. PVO is rare, but should be included in the differential diagnosis in patients presenting with early breast carcinoma.

Similarity of Shiga Toxin–producingEscherichia coliO104:H4 Strains from Italy and Germany

Similarity of Shiga Toxin–producing<i>Escherichia coli</i>O104:H4 Strains from Italy and Germany

Treatment of severe neurological deficits with IgG depletion through immunoadsorption in patients with Escherichia coli O104:H4-associated haemolytic uraemic syndrome: a prospective trial

In May 2011, an outbreak of Shiga toxin-producing enterohaemorrhagic E coli O104:H4 in northern Germany led to a high proportion of patients developing post-enteritis haemolytic uraemic syndrome and thrombotic microangiopathy that were unresponsive to therapeutic plasma exchange or complement-blocking antibody (eculizumab). Some patients needed ventilatory support due to severe neurological complications, which arose 1 week after onset of enteritis, suggesting an antibody-mediated mechanism. Therefore, we aimed to assess immunoadsorption as rescue therapy. In our prospective non-controlled trial, we enrolled patients with severe neurological symptoms and confirmed recent E coli O104:H4 infection without other acute bacterial infection or raised procalcitonin concentrations. We did IgG immunoadsorption processing of 12 L plasma volumes on 2 consecutive days, followed by IgG replacement (0·5 g/kg intravenous IgG). We calculated a composite neurological symptom score (lowest score was best) every day and assessed changes before and after immunoadsorption. We enrolled 12 patients who initially presented with enteritis and subsequent renal failure; 10 (83%) of 12 patients needed renal replacement therapy by a median of 8·0 days (range 5-12). Neurological complications (delirium, stimulus sensitive myoclonus, aphasia, and epileptic seizures in 50% of patients) occurred at a median of 8·0 days (range 5-15) and mandated mechanical ventilation in nine patients. Composite neurological symptom scores increased in the 3 days before immunoadsorption to 3·0 (SD 1·1, p=0·038), and improved to 1·0 (1·2, p=0·0006) 3 days after immunoadsorption. In non-intubated patients, improvement was apparent during immunoadsorption (eg, disappearance of aphasia). Five patients who were intubated were weaned within 48 h, two within 4 days, and two patients needed continued ventilation for respiratory problems. All 12 patients survived and ten had complete neurological and renal function recovery. Antibodies are probably involved in the pathogenesis of severe neurological symptoms in patients with E coli O104:H4-induced haemolytic uraemic syndrome. Immunoadsorption can safely be used to rapidly ameliorate these severe neurological complications. Greifswald University and Hannover Medical School.

Embolization of Spinal Tumors: Vascular Anatomy, Indications, and Technique

Involvement of the spine by primary and secondary tumors can be associated with mechanical instability, pain, and neurologic complications, all of which can adversely affect a patient's quality of life. When surgical excision is planned, preoperative embolization of spinal tumors reduces intraoperative blood loss, making surgery safer and easier. Embolization of spinal tumors can also be used to palliate pain and improve neurologic symptoms in patients with unresectable tumors. A detailed knowledge of the spinal vascular anatomy is essential before performing spinal tumor embolization. Indications, contraindications, embolization technique, and potential complications must be fully understood to ensure a safe and effective procedure. Although the technique used may vary among operators and institutions, familiarity with embolization goals and strategies can ensure sufficient tumor devascularization.

Dissection of the Internal Carotid Artery After SCUBA-Diving

Dissections of the internal carotid arteries are a well known complication of cervical trauma. Neurologic symptoms in patients after SCUBA-diving are often associated with gas embolism or decompression illness. This report presents a rare case of carotid artery dissection immediately after SCUBA-diving in a 37-year-old woman, with left-side facial paresthesias and left-cervical pain as the first symptoms after SCUBA-diving without rapid ascent to the water-surface. A review of the literature focuses on symptoms, morphologic aspects, and therapeutic options in reported cases of cervical artery dissection after SCUBA-diving.

Lumen Morphology in Mild-to-Moderate Internal Carotid Artery Stenosis Correlates with Neurological Symptoms

Several prospective studies have shown that carotid endarterectomy can reduce the risk for subsequent ischemic stroke in patients with 70-99% stenosis of the internal carotid artery (ICA). However, its benefits are still controversial in less than 70% stenosis of the ICA. There is increasing evidence that carotid lumen irregularities may correlate with neurological symptoms. Recent development of computed tomography angiography (CTA) can provide adequate information on the carotid plaque morphology. In this study, therefore, we aimed to clarify whether carotid lumen morphology estimated by CTA correlates with neurological symptoms in patients with 30-69% ICA stenosis. This study included 67 carotid stenotic lesions with 30-69% ICA stenosis in 52 consecutive patients. These 67 lesions were examined by CTA from the viewpoints of the degree of stenosis, the prevalence of ulceration, and lumen morphology. Multivariate analysis was performed to detect significant predictors for the occurrence of ipsilateral ischemic events. Multivariate analysis showed that the irregular shape of the carotid lumen was the most powerful variable to predict symptomatic lesion in 30-69% ICA stenosis. These findings suggest that the morphology of carotid plaque may be associated with the occurrence of ipsilateral ischemic events in 30-69% ICA stenosis.

Venous Cerebral Infarction in a Patient With Peripheral Hemodialysis Shunt and Occlusion of the Left Brachiocephalic Vein

Intracranial venous congestion is a rare condition in hemodialysis patients with central venous occlusion. We report a patient with cerebral venous infarction resulting from high reflex flow into the cranium induced by an arteriovenous hemodialysis shunt in the arm and occlusion of the brachiocephalic vein. This case illustrates that abnormal extracranial venous circulation should be considered when cerebral venous congestion is assumed to produce neurologic symptoms in patients with an arteriovenous shunt.

Mercury exposure in a low-income community in South Africa

To establish whether a specific community in a gold mining area, with potentially associated small-scale gold mining activities, was exposed to mercury. The community was situated in Mpumalanga, where some potential sources of mercury emissions may have an impact. Adults >or=18 years were considered eligible. Biological monitoring, supported by questionnaires, was applied. Thirty respondents completed the questionnaire which covered demographics, energy use, food and water consumption, neurological symptoms, and confounders such as alcohol consumption and brain injuries. Mercury levels were determined in 28 urine and 20 blood samples of these respondents. Three (15%) of the blood samples exceeded the guideline (<10 microg/l) for individuals who are not occupationally exposed, while 14 (50%) of the urine samples exceeded the guideline for mercury in urine (<5.0 microg/g creatinine) for those not exposed occupationally. The cause of these elevated levels is unknown, as only 20% of respondents indicated that they used coal as an energy carrier. Furthermore, nobody from the community was reportedly formally employed in a goldmine. Nineteen (63%) respondents consumed locally caught fish, while 20 (67%) drank water from a river. Some individuals in this study may be occupationally exposed to mercury through small-scale gold mining activities. As primary health facilities will be the first point of entry for individuals experiencing symptoms of mercury poisoning, South African primary health care workers need to take cognisance of mercury exposure as a possible cause of neurological symptoms in patients.