Cause Of Neurological Symptoms Research Articles

The anaplerotic flux and ammonia detoxification in hepatic encephalopathy

Metabolic alterations in the brain underly many of the mechanisms leading to acute and chronic Hepatic Encephalopathy (HE). Controversy exists about the role of glutamine accumulation as a causal factor in HE. Glutamine formation contributes to detoxify ammonia, whereby anaplerotic mechanisms in the astrocytes have to be sufficient to replenish Krebs cycle intermediates. The application of ex vivo high-resolution nuclear magnetic resonance (NMR) spectroscopy permits direct measurements of metabolites and different metabolic pathways. Ex vivo (13)C-NMR studies in experimental animal models of acute and chronic HE have provided new insights. In an experimental rat model of ALF, (13)C isotopomer analysis of glucose metabolism showed that alterations of glucose flux through astrocytic pyruvate carboxylase might be linked to the pathogenesis of ALF as a limited anaplerotic flux in the brain, but not in the muscle, correlates with the development of brain edema. Moreover, (13)C-NMR data from a rat model of mild HE demonstrated relative differences in the pathway of glucose through pyruvate carboxylase in thalamus compared to frontal cortex, which might explain the vulnerability of this brain region compared to thalamus. These findings further support that glutamine accumulation might be not the primary cause of neurological symptoms in HE, and show that anaplerotic mechanisms could be essential for ammonia detoxification in HE.

MDCT Detection of Fibromuscular Dysplasia of the Internal Carotid Artery

The purpose of this article is to describe two cases in which fibromuscular dysplasia of the internal carotid artery was diagnosed with CT angiography. CT angiography can depict the characteristic findings of fibromuscular dysplasia. If patients with cerebrovascular symptoms undergo screening with CT angiography of the supraaortic vessels, more cases of fibromuscular dysplasia will be recognized as a cause of neurologic symptoms.

Ornithine Transcarbamylase Deficiency Presenting as Encephalopathy During Adulthood Following Bariatric Surgery

Neurological complications following bariatric surgery are rare. Whereas nutritional deficiencies are the most common cause of neurological symptoms, the unmasking of previously subclinical metabolic disorders can also lead to significant morbidity. To characterize the clinical presentation, serum biochemical fluctuations, and functional enzymatic analysis of a case of functional ornithine transcarbamylase deficiency unmasked by a dietary change following bariatric surgery. Case report. Tertiary referral center, hospital (inpatient) setting. A 29-year-old woman who presented with intermittent encephalopathy associated with recurrent hyperammonemia. Clinical, biochemical, and mutational studies. The pattern of intermittent hyperammonemia and encephalopathy following oral and parenteral nutrition suggested a urea cycle abnormality. Functional enzymatic assay results showed markedly reduced ornithine transcarbamylase activity in the absence of known coding mutations. Previously asymptomatic ornithine transcarbamylase deficiency should be suspected in adult patients who develop recurrent hyperammonemia and encephalopathy following bariatric surgery.

Small-Cell Carcinoma of the Esophagus Associated with a Paraneoplastic Neurological Syndrome: A Case Report Documenting a Complete Response

We describe a case of small-cell carcinoma of the esophagus associated with a paraneoplastic neurological syndrome. Sensorimotor neuropathy had developed 3 years earlier, and neurological symptoms had slowly worsened. Small-cell carcinoma of the esophagus was incidentally diagnosed while investigating the cause of the neurological symptoms. A paraneoplastic neurological syndrome was diagnosed on the basis of cancer and exclusion of other known causes of neurological symptoms. The patient was given combination chemoradiotherapy. There was a complete response to three courses of chemoradiotherapy, with no evidence of disease recurrence 6 years after the diagnosis. There was no progression of paraneoplastic neurological symptoms after the complete response.

Histopathologic effects of discitis on neural tissues: an experimental study.

To determine the cause of neurologic symptoms and signs seen in discitis, the neural histopathologic effects of discitis were investigated in an experimental study carried out on rats. Groups of seven rats each had their intervertebral discs inoculated with either Staphylococcus aureus, Klebsiella pneumoniae, Pseudomonas aeruginosa, or a control solution. Histopathologic examinations of the spinal cord and nerve roots were performed after 3 weeks. On histopathologic examination, vacuolar myelopathy in the spinal cord and vacuolar neuropathy within the nerve roots near the junction with the spinal cord were found. The severity and form of vacuolar myelopathy varied according to the bacteria used for inoculation. The myelopathy and neuropathy seen in this rat model of bacterial discitis might be the result of an immunologic mechanism and could be responsible for the neurologic signs and symptoms of discitis in patients.

Hypertrophic pachymeningitis: case report

Hypertrophic pachymeningits is an unusual cause of neurological symptoms and is often secondary to infections, carcinomatosis or inflammatory diseases. It may also be idiopathic. We report a case of pachymeningitis which was manifested primarily by psychosis and visual loss with optic atrophy and destruction of nasal septum. The patient, a 45 year old woman was submitted to extensive investigation without evidence of any underlying disease. A meningeal biopsy was performed and showed a mostly unspecific inflammatory process with extensive fibrosis of the dura and few early stage granulomas. These findings suggest either neurosarcoidosis or idiopathic hypertrophic pachymeningitis.

Retrospective identification of three undiagnosed cases of measles encephalitis.

It has long been recognised that neuroinvasion may occur as a rare complication of measles. According to cases reported in the medical literature [1], measles virus (MV) can persist in the central nervous system producing chronic neurological disease without systemic viremia [2, 3]. In most industrialised countries, the introduction of live attenuated measles vaccines has resulted in the virtual disappearance of measles as a common childhood disease and strongly reduced the numbers of patients with MV-related neurological disease. However, measles did not completely disappear: occasional clinical cases are still observed due to importation of MV from endemic areas [4], and larger outbreaks continue to occur amongst clusters of unvaccinated individuals [5]. It is difficult to assess how many people are exposed to wild-type MV as a result of these cases, but the virus is only rarely considered as a possible cause of neurological symptoms. The aim of the present study was to identify undiagnosed cases of MV-related encephalitis. Combined evaluation of clinical data, results of diagnostic serological tests and reverse transcriptase polymerase chain reactions carried out on cerebrospinal fluid and bronchoalveolar lavage specimens may shed light on the aetiology of these cases of encephalitis. A retrospective analysis was performed on 53 patients admitted to our hospital during 1998 with encephalitis of unknown origin. Their mean age was 48 years (range, 19–74 years). Clinical features included fever, headache, facial weakness, altered mental status, seizures, impaired hearing, motor disturbances, limb paralysis and respiratory distress. Patients were tested for several viral infections that may be associated with encephalitis including herpes simplex viruses 1 and 2, cytomegalovirus, Epstein-Barr virus, JC virus (the causative agent of progressive multifocal leukoencephalopathy), tick-borne encephalitis virus, dengue virus, HIV-1 and HIV-2 and Mycoplasma pneumoniae. All patients were negative for all of these agents. In the present study, serum and cerebrospinal fluid samples were tested for the presence of MV-specific IgM and IgG antibodies using a commercially available IgM capture enzyme immunoassay (Meddens Diagnostics, The Netherlands) and an enzyme immunoassay developed in-house based on whole virus, respectively. Two patients, 29 and 48 years of age, were found to be MV IgM-positive in both serum and cerebrospinal fluid, and in one 73-year-old patient borderline levels of MV-specific IgM serum antibodies were detected. This last patient had chronic lymphatic leukaemia and was undergoing immunosuppressive treatment. During the previous 2 years he had suffered recurrent opportunistic infections, with fever and pneumonia, zoster, vasculitis, and hepatitis. Serum samples collected 1 year before his encephalitis was diagnosed were MV IgM-negative and contained low levels of MV-specific IgG antibodies. An MV-specific reverse transcriptase polymerase chain reaction [6] was carried out on a bronchoalveolar lavage sample to diagnose the cause of the patient’s pneumonia that had developed during his encephalitis and was found positive. Sequence analysis of the amplicon demonstrated that the virus belonged to genotype C2 (GenBank, http://www.ncbi.nlm.nih.gov/Genbank/; accession number AF519493), which was a common European genotype during the last decade of the previous century. Blast analysis identified a virus isolated in 1992 in Tilburg, The Netherlands, as the only identical sequence in the GenBank database, but almost identical viruses were involved in a measles outbreak in Luxembourg in 1996 [7]. The results of the present study strongly suggest that undiagnosed encephalitis may be associated with MV infection: in 2 of 53 undiagnosed encephalitis cases, MVspecific IgM antibodies were found in serum and cerebrospinal fluid, strongly indicating that these patients were suffering from an MV infection. In a third patient,

Neurological Complications of Paget's Disease

The neurologic complications of Paget's disease are infrequent but can be severe and are often treatable. Paget's disease should be considered as a posible cause of neurologic symptoms, including those with previously undiagnosed disease. Routine neurological assessments should be performed at regular intervals in patients with known Paget's disease of the spine or cranium. The outcome of treatment, particularly in cases of spinal cord compression or hydrocephalus, are particularly rewarding. Treatment should be based on each individual patient's presentation and consists of symptomatic treatment, antiresorptive therapy, or surgery. The latter can frequently be avoided in patients with slow onset spinal cord compression in whom the acute and long-term response to bisphosphonates is impressive.

特発性脊髄ヘルニアの１例

Spinal cord herniation is a rare cause of neurological symptoms. We report a case of a 72-year-old woman presented with myelopathy of the lower right extremity and sensory disturbance of the lower left extremety. Three years earlies the subject experienced weakness in the right leg and numbness in the left leg. The patient did not have a history of injury or spine surgery. The symptoms worsened until examination by the authors three years, later by MRI, myelography, and CT scan. MRI scan well revealed spinal cord anterior deviation at the Th5 level. In operation, dura membrane defect, duplicated dura, and spinal cord herniation were observed. Laminectomy and dural fixation with artificial dura were performed. After operation, the patient's complaints gradually decreased.

Cerebrovascular manifestations in 321 cases of hereditary hemorrhagic telangiectasia.

Patients with hereditary hemorrhagic telangiectasia (HHT) are at risk for developing cerebral vascular malformations and pulmonary arteriovenous fistulae. We assessed the risk of neurological dysfunction from these malformations and fistulae. Three hundred twenty-one consecutive patients with HHT seen at a single institution over a 20-year period were studied. Any evidence of prior neurological symptoms or presence of an intracranial vascular malformation was recorded. All cases of possible cerebral arteriovenous malformation were confirmed by conventional arteriography. Twelve patients (3.7%) had a history of cerebral vascular malformations. Ten patients had arteriovenous malformations, 1 had a dural arteriovenous fistula, and 1 had a cavernous malformation. Seven patients (2.1%) presented with intracranial hemorrhage, 2 presented with seizures alone, and 3 were discovered incidentally. The average age at the time of symptomatic intracranial hemorrhage was 25.4 years. All patients with a history of intracranial hemorrhage were classified as Rankin grade I or II at a mean follow-up interval of 6.0 years. A history of cerebral infarction or transient ischemic attack was found in 29.6% of patients with HHT and a pulmonary arteriovenous fistula. The risk of intracranial hemorrhage is low among people with HHT. Furthermore, a majority of these patients have a good functional outcome after hemorrhage. The data do not suggest a compelling indication for routine screening of patients with HHT for asymptomatic cerebral vascular malformations. By comparison, pulmonary arteriovenous fistulae are a much more frequent cause of neurological symptoms in this population.

Tratamiento oncológico de las metástasis otoneuroftalmológicas y sus complicaciones

Cerebral metastases and the sequelae of their treatment are the major cause of neurological symptoms in patients with cancer. In this article we review the oto-neuro-ophthalmological complications of the treatment of metastases with radiotherapy and/or chemotherapy. When speaking of the iatrogenic diseases caused by radiotherapy treatment of metastases, it is important to emphasize that the major complications of this form of treatment are seen in the long term, in general, months or years later. When dealing with incurable diseases, such as most metastatic cancers, the benefit/risk balance of each therapeutic option has to be taken into account. Thus we have a population of patients with symptoms secondary to metastatic involvement, and with an overall life expectancy which may be measured in months. The oto-neuro-ophthalmological toxicity of the chemotherapy may present as an infrequent and unexpected complication or as a usual, expected secondary effect of the drug used. A large variety of drugs are used for the systemic control of cancer (cystostatic drugs, hormones and modifiers of the biological response) which, in one way or another, may cause neurological signs. The increasingly frequent use of high dose chemotherapy and of the combined use of chemotherapy and radiotherapy mean that these types of toxicity have become common clinical syndromes in current oncological practices.

Sinus venous thrombosis: an unusual cause for neurologic symptoms after bone marrow transplantation under immunosuppression.

Hematopoietic and organ transplantations are increasing worldwide with more patients receiving immunosuppressive therapy. Neurological problems may complicate the posttransplant period. Possible causes include the conditioning regimen (e.g., seizures with busulfan), central nervous system infections (viral, bacterial, and fungal), or factors secondary to the immunosuppressive therapy and side effects of drug treatment (e.g., cyclosporine and tacrolimus). Sinus venous thrombosis, the occlusion of a cerebral venous vessel or a sinus, is an unusual cause of neurologic symptoms in patients after transplantation, and this has not been reported in the literature previously. Three patients presenting with various neurological symptoms after allogeneic bone marrow transplantation underwent computed tomography scans and magnetic resonance imaging as a primary diagnostic procedure. In all patients, sinus venous thrombosis was found as the cause for seizures; it was the cause of disturbance of consciousness in two patients and headaches in two patients. All symptoms resolved without any neurologic deficiency after anticoagulation therapy with heparin followed by dicumarol. We conclude that sinus venous thrombosis should be considered as a cause of neurological symptoms in patients after transplantation under immunosuppressive therapy. Diagnosis is rapidly confirmed by noninvasive magnetic resonance imaging angiography. Therapeutic heparinization is the treatment of choice.

Intrathecal Hyperbaric 0.5% Tetracaine as a Possible Cause of Transient Neurologic Toxicity

Intrathecal Hyperbaric 0.5% Tetracaine as a Possible Cause of Transient Neurologic Toxicity

Cardiac Arrhythmias: Syncope and Stroke

Cardiac arrhythmias are an important cause of neurologic symptoms. Bradyarrhythmias and tachyarrhythmias, by disrupting blood flow to the brain and causing cerebral hypoxia, can lead to loss of consciousness (and ultimately to cerebral necrosis or death). Thus cardiac arrhythmias must be considered as a potential cause in any patient presenting with syncope. For syncopal patients in whom the substrate for reentrant ventricular arrhythmias is found (that is, patients with ventricular dysfunction), management must be extremely aggressive because their near-term risk for sudden death is high, and conservative therapy is ineffective. Embolic stroke is a common result of atrial fibrillation. Several randomized trials now indicate that anticoagulation with low-dose warfarin is necessary, whenever possible, in patients with atrial fibrillation even in the absence of underlying valvular heart disease. Ongoing studies are evaluating the efficacy of aspirin in preventing embolic events.

Spinal Cord Compression in Metastatic Cervical Cancer

Spinal cord compression secondary to metastatic cervical cancer may not be considered as a possible cause of neurologic symptoms by primary care physicians who do not often treat these patients. Delays in diagnosis and treatment may result in irreversible but potentially preventable neurologic changes. This report describes 5 cases of spinal cord compression in patients with metastatic cervical carcinoma, 2 of whom were previously undiagnosed with cervical cancer. These 2 patients represent 1.6% (2 of 121) of all new cervical cancer cases diagnosed during this time period. Two of 5 patients (40%) with spinal cord compression showed improvement following therapy by regaining the ability to walk, while none of the remaining patients had further acute deterioration of neurologic function. The mean survival of patients presenting with spinal cord compression from cervical cancer in this series was 4 months (maximum 6 months). This series illustrates the relative frequency with which spinal cord compression is seen in patients with a new diagnosis of invasive cancer. This diagnosis should be considered when evaluating neurologic complaints in known cervical cancer patients or any woman with apparent pelvic pathology. Rapid diagnosis and treatment of these lesions, while not likely to improve overall survival significantly, can improve function and alleviate symptoms.

Neurologic Consequences of Traumatic Asphyxia

Patients with traumatic asphyxia treated at a single institution during a 10-year period were studied to determine the incidence and sequelae of neurologic impairment associated with this entity. Traumatic asphyxia was identified in 14 patients from 4 to 73 years old. Each had sustained thoracic crush injuries from objects weighing more than 1,000 pounds. The mechanism of injury was crush by farm implement in six patients, entrapment beneath a vehicle in five, compression by a large hay bale in one, crush by a farm animal in one, and a ditch cave-in in one. Craniocervical cyanosis and subconjunctival hemorrhage were apparent in all patients. Associated chest wall and intrathoracic injuries were present in 11 (79%) patients. Neurologic abnormalities included loss of consciousness in eight patients, prolonged confusion in five, seizures in two, and pronounced visual disturbances in two. There were no deaths in this series and no long-term neurologic sequelae were evident. However, careful serial neurologic assessment should be performed in these patients and other causes of neurologic symptoms excluded.

Scanning electron microscopic study of spinal arachnoid plaques

AbstractCalcified or ossified plaques within the spinal arachnoid membranes are often reported at autopsy and are occasionally incriminated as the cause of neurological symptoms in patients undergoing surgery. Histological studies of plaques have been done, but to our knowledge no studies using the scanning electron microscope (SEM) have been reported. In this study, portions of the spinal cord with plaques were removed from adult human cadavers in the dissecting laboratory and processed for SEM and energy‐dispersive x‐ray analysis (EDAX). The surfaces of the plaques appeared to be fibrous in some areas, studded with irregularly shaped projections or nodules in others, and smooth in yet other areas. Cells were seen in lacunae‐like depressions on the surfaces of some plaques and EDAX revealed the presence of calcium on and near the cells. In some areas calcium and phosphorus were both detected, but not in the ratios seen in bone. No calcium was seen on smooth or fibrous areas. It is suggested that the plaques sampled in this study were composed largely of fibrous tissue encrusted in some areas with calcium salts. There was no evidence that any of the plaques contained bone.

Surgical Treatment for Chiari Malformations associated with Congenital Anomalies

Chiari malformations are often associated with congenital anomalies. The authors evaluated several surgical procedures for Chiari malformations associated with basilar impression and/or syringomyelia. Twenty patients with Chiari malformations with and without congenital anomalies were treated by various surgical procedures. Associated anomalies were atlantoaxial dislocation in one, basilar impression in eight, and syringomyelia in 11. There were 11 males and nine females, ranging in age from 6 to 63 years. Nine patients were treated by foramen magnum decompression (suboccipital craniectomy and upper cervical laminectomy), one by foramen magnum decompression combined with terminal syringostomy, one by anterior decompression via the transoral approach, and nine by syringosubarachnoid shunting. The period of follow-up ranged from 5 months to 21 years (mean, 7 years). Seventeen of the 20 patients showed neurological improvement, one was unchanged, and two deteriorated. One of the patients who deteriorated had undergone syringosubarachnoid shunting, and the shunt malfunctioned because of arachnoiditis. A patient with basilar impression died of respiratory failure after suboccipital craniectomy and upper cervical laminectomy. On the basis of their results, the authors recommend posterior decompression for patients with Chiari malformations alone. If a foramen magnum lesion is responsible for the neurological manifestations and the clivoaxial angle is narrow in Chiari malformations associated with basilar impression and/or syringomyelia, anterior decompression with fusion should be performed. If the clivoaxial angle is within normal limits, the authors perform posterior decompression. Syringosubarachnoid shunting appears effective in patients in whom syringomyelia is the main cause of neurological symptoms.

Frequency, severity, and physiologic importance of carotid siphon lesions

Atherosclerotic stenosis of the intracranial portion of the internal carotid artery (SIPHON) may be the cause of neurologic symptoms. The frequency and severity of these lesions were studied in 226 vessels subjected to conventional two-plane carotid angiography, and the impact of sequential or tandem lesions on cerebral flow was assessed in the 167 arteries from this group that also underwent Gee oculoplethysmography (OPG-Gee), a commonly accepted indicator of the physiologic importance of internal carotid stenosis. Atherosclerotic involvement of the SIPHON was present in 46% ( 103 226 ) of the vessels studied, including six total occlusions, 11 severe stenoses (severe, 80% to 99% area reduction [AR]), 27 moderate stenoses (moderate, 50% to 79% AR), 40 mild stenoses (mild, 10% to 49% AR), and 19 instances of mural disease (MUR). This was compared with a 73% rate (166 of 226) of proximal internal carotid artery (PICA) stenosis in the neck, including six to 43 severe, 29 moderate, 50 mild, and 38 MUR. There was little correlation (r = 0.33) between the degree of angiographic stenosis in the PICA and the SIPHON. All of the totally occluded arteries had a positive OPG-Gee, whereas a positive study was noted in only 6% (7 of 109) of the arteries with less than moderate stenosis in the PICA. Twenty-one vessels had moderate stenosis of the PICA. Eight of these arteries had an associated moderate or severe lesion in the SIPHON, and seven of eight (88%) had a positive OPG-Gee. The remaining 13 of this subset had had a normal or mildly diseased SIPHON, and 12 of 13 (92%) had a normal OPG-Gee. This adverse effect of a SIPHON stenosis on the OPG of a moderate stenosis in the PICA is significant (p < 0.001) and demonstrates the additive effect of tandem lesions. It is concluded that many vessels have a SIPHON lesion, but that it is usually not of high degree and bears little correlation to the magnitude of a PICA stenosis. In addition, tandem or sequential lesions appear to have an additive effect on the physiology of cerebral blood flow.

Frequency, severity, and physiologic importance of carotid siphon lesions.

Atherosclerotic stenosis of the intracranial portion of the internal carotid artery (SIPHON) may be the cause of neurologic symptoms. The frequency and severity of these lesions were studied in 226 vessels subjected to conventional two-plane carotid angiography, and the impact of sequential or tandem lesions on cerebral flow was assessed in the 167 arteries from this group that also underwent Gee oculoplethysmography (OPG-Gee), a commonly accepted indicator of the physiologic importance of internal carotid stenosis. Atherosclerotic involvement of the SIPHON was present in 46% (103/226) of the vessels studied, including six total occlusions, 11 severe stenoses (severe, 80% to 99% area reduction [AR]), 27 moderate stenoses (moderate, 50% to 79% AR), 40 mild stenoses (mild, 10% to 49% AR), and 19 instances of mural disease (MUR). This was compared with a 73% rate (166 of 226) of proximal internal carotid artery (PICA) stenosis in the neck, including six to 43 severe, 29 moderate, 50 mild, and 38 MUR. There was little correlation (r = 0.33) between the degree of angiographic stenosis in the PICA and the SIPHON. All of the totally occluded arteries had a positive OPG-Gee, whereas a positive study was noted in only 6% (7 of 109) of the arteries with less than moderate stenosis in the PICA. Twenty-one vessels had moderate stenosis of the PICA. Eight of these arteries had an associated moderate or severe lesion in the SIPHON, and seven of eight (88%) had a positive OPG-Gee. The remaining 13 of this subset had had a normal or mildly diseased SIPHON, and 12 of 13 (92%) had a normal OPG-Gee.(ABSTRACT TRUNCATED AT 250 WORDS)