Search

Introduction: HaNDL syndrome (transient headache and neurological deficits with cerebrospinal fluid lymphocytosis) is characterised by one or more episodes of headache and transient neurological deficits associated with cerebrospinal fluid lymphocytosis. To date, few cases of HaNDL manifesting with confusional symptoms have been described. Likewise, very few patients with HaNDL and confusional symptoms have been evaluated with transcranial Doppler ultrasound (TCD). TCD data from patients with focal involvement reveal changes consistent with vasomotor alterations., Development: We present the case of a 42-year-old man who experienced headache and confusional symptoms and displayed pleocytosis, diffuse slow activity on EEG, increased blood flow velocity in both middle cerebral arteries on TCD, and single-photon emission computed tomography (SPECT) findings suggestive of diffuse involvement, especially in the left hemisphere., Conclusions: To our knowledge, this is the first description of a patient with HaNDL, confusional symptoms, diffuse slow activity on EEG, and increased blood flow velocity in TCD. Our findings suggest a relationship between cerebral vasomotor changes and the pathophysiology of HaNDL. TCD may be a useful tool for early diagnosis of HaNDL., (Copyright © 2017 Sociedad Española de Neurología. Publicado por Elsevier España, S.L.U. All rights reserved.)

Introduction: Studying the amino acids in cerebrospinal fluid (CSF) is essential in the diagnosis of some neurological diseases and is an important aid in the diagnosis of others. No research has been published in the literature to prove the physiological relationship between the values of amino acids in CSF and plasma in the paediatric population., Aim: To define a set of ratios for amino acids in plasma and CSF in the paediatric population that can be used in daily clinical practice., Patients and Methods: The aminograms in plasma and CSF of 105 patients with ages between 0 and 12 months were collected and analysed retrospectively. Aminograms with amino acid values that are considered to be normal according to the reference values of our laboratory were included in the sample. The quantitative analysis of amino acids was performed using high-resolution liquid chromatography and statistical analysis with the software application SPSS 19.0., Results: The mean values, range and standard deviation of the amino acid concentrations in plasma and CSF, together with the CSF/plasma ratios, are reported. Significant correlations were found from 0.6 onwards between different neutral amino acids, above all in those with smaller molecular weights (Thr, Ser, Gly and Ala)., Conclusions: The existence of significant correlations between the different neutral amino acids supports the idea that they share the same transporters in the blood-brain barrier. Standardising the amino acid ratios will make it possible to increase sensitivity in the detection of pathological values in plasma and CSF, to further knowledge of the pathophysiology of neurological diseases and perhaps to describe new aminoacidopathies.

Introduction: The pseudomigraine syndrome with cerebrospinal fluid (CSF) and pleocytosis (PMP) or headache with neurologic deficits and CSF lymphocytosis (HaNDL) is an entity that they have been realized multiple contributions to their etiophysiopathology in the 25 years of their discovery., Development: The PMP is described in 1980 by Swanson, Bartleson and Whisnant, and parallelly for Marti-Masso, and from then on there have been contributions of new cases, ones some atypical for mild headache, prolonged recurrence, symptomatic intracranial hypertension or infections for citomegalovirus that simulates PMP. They have carried out several approaches diagnoses along the years being established at the moment in the year 2004 by the International Classification of Headache Disorders. They have carried out contributions to their knowledge thanks to the realization of electroencephalograms, single photon emission computed tomography brain imaging, transcranial Doppler, evoked potentials, brain magnetic resonance imaging diffusion... giving place to the existence of numerous theories like the infectious-autoimmune, dysfunction of the blood brain barrier, spread cortical depression, trigeminous-vascular activation., Conclusions: The PMP or HaNDL is a benign entity with even unknown etiophysiopathology and where it is important the differential diagnosis with other entities potentially more dangerous.

Introduction: Although first described over 100 years ago, lumbar puncture is still an important tool in the diagnosis of neurological diseases. In this article we review its indications, contraindications, the technique for carrying it out, the analysis of the cerebrospinal fluid and possible complications., Development: The lumbar puncture has diagnostic and therapeutic indications. The chief diagnostic indications include infectious, inflammatory and neoplastic diseases affecting the central nervous system. Complications are infrequent, except for headaches and low back pain, but can be severe. Analysis of the cerebrospinal fluid must include a cell count and determination of the glucose and protein concentrations. The other analytical studies of cerebrospinal fluid must be conducted according to the diagnostic suspicion., Conclusion: The lumbar puncture in expert hands is a safe test. The health professional should be suitably familiar with its contraindications, the regional anatomy and the technique used to perform it.

Introduction: Reiber's quotient diagram or reibergram has a growing apply for characterize the intratecal synthesis of proteins. Firstly reibergrama was used for the major classes of immunoglobulins but later it was used to evaluate other proteins based on the theory about molecular flux/cerebrospinal fluid (CSF) flow rate., Method: C3c is a degradation product of complement factor C3 with 145 KDa and approaches to IgG molecular characteristics according with Fick's diffusion laws. It was assumed IgG constants and graphic for IgG constants and graphic to evaluate the intrathecal synthesis of C3c. Twenty-seven patients and 27 controls were studied. Serum and CSF C3c and albumin were quantified by immunodiffusion., Results: The patients with the C3c proposed reibergram were evaluated. It has been proved its validity under several CSF blood barrier conditions., Conclusion: Reibergram for C3c can be used for the evaluation of the intrathecal synthesis of this protein.

Aims: The aim of this work is to describe the clinical, biochemical and genetic characteristics of neurotransmitter diseases at the paediatric age, together with possible forms of treatment. We also sought to determine the diagnostic methodology of these disorders (collection and analysis of samples)., Development: These diseases essentially consist of a deficit of biogenic amines and alterations in GABA metabolism (gamma-aminobutyric acid). Disorders affecting the neurotransmission of biogenic amines often present in the form of hypokinesia, trunk hypotonia with increased limb tone, oculogyric crises, ptosis, faulty temperature regulation or abnormal movements. Defects in GABA metabolism give rise to epileptic encephalopathies and unspecific mental retardation, sometimes associated to signs of cerebellar dysfunction, convulsions and alterations in neuroimaging studies. Overall incidence of these diseases is low but they are unquestionably under-diagnosed, since they cannot be detected by conventional studies in plasma and urine, and require extraction and directed analysis of cerebrospinal fluid (CSF) for their detection. Additionally, the CSF study must be carried out in specific standardised conditions. Segawa's disease, or dopa-responsive dystonia, responds extremely well to therapy, whereas the other entities respond in varying ways to the different therapeutic alternatives., Conclusions: It is important for the paediatrician to know about these entities as a group of treatable neurometabolic diseases. Moreover, their detection would allow prenatal diagnosis in the vast majority of cases.

Aim: To review the fundamental aspects of the theory of the molecular flow/ cerebrospinal flux described recently and it can explained a group of events in the physiology of the cerebrospinal fluid and the physiopathology of neurological diseases., Development: This theory was based on the postulate that a decrease of the flux rate of the cerebrospinal fluid was accompanied by an increment of the protein concentration in it and in the nervous system tissue. The increment of the protein transport from the blood to the cerebrospinal fluid not require structural changes or an increase of permeability. The reibergram or Reiber's quotient diagram, with the discriminatory hyperbolic function with its theoretical basis and its clinical relevance confirm the acceptance of the present theory. This theory was based on the first and second Fick's diffusion laws The increment of the molecular diffusion is the cause of the non-linear decrease of the cerebrospinal flux rate because of the blood-cerebrospinal fluid barrier dysfunction., Conclusions: This theory explain that an increase of the albumin quotient does not means a morphologic change on the barrier structures. The change in the cerebrospinal flux rate it has been considered the principal modulator of the protein concentration in cerebrospinal fluid in pathological conditions characterized by a blood-cerebrospinal fluid barrier dysfunction.

Introduction: The immunological study of cerebrospinal fluid (CSF) is an essential diagnostic tool for evaluating patients with neurological diseases. The quantitative determination of the albumen and immunoglobulin G (IgG) in blood serum and in CSF by single radial immunodiffusion (SRID), together with the calculation of the IgG index to evaluate the presence of intrathecal synthesis of IgG and of the albumen quotient in order to evaluate the state of functioning of the blood brain barrier are essential elements to be evaluated for diagnosis and research in neurological clinical practice, as well as in the follow up of certain neurological diseases such as multiple sclerosis. Specific antiserums from commercial firms such as Boehring, SIGMA, etc. are used for the quantitative determination of IgG and albumen both in blood serum and in CSF by SRID. The high cost and the difficulty in acquiring these immunodiagnostic kits have had an important effect on the diagnostic and research opportunities throughout the country., Materials and Methods: In this work we present the preliminary findings of the evaluation of the human IgG antiserum obtained from a ram, by Labex laboratories, for the quantitative determination of IgG in CSF by SRID, in order to find out whether this antiserum is efficient in the quantitative determination of IgG in CSF., Results and Conclusions: The studies conducted so far show that this antiserum may be a good candidate for use in immunological studies of CSF. Further work needs to be carried out on its validation in order to resolve the problems involved in immunological studies of CSF that we highlighted above. This would be achieved with an antiserum that is cheaper than those used up to now.

The study of cerebrospinal fluid (CSF) dynamics is central to the diagnosis of adult chronic hydrocephalus (ACH). At present, many neurology and neurosurgery departments use one or more tests to guide diagnosis of this syndrome and to predict patient response to shunting. In specialised centres, the study of CSF dynamics is combined with continuous intracranial pressure (ICP) monitoring. Determination of several variables of CSF dynamics and definitions of qualitative and quantitative characteristics of ICP can be used to establish whether the hydrocephalus is active, compensated or arrested. CSF dynamics and ICP monitoring can also be used to check the correct functioning of the shunt and can be of use in the clinical management of patients with pseudotumor cerebri. Moreover, ICP monitoring is used to guide the treatment of several acute neurological processes. The aim of this review is to describe the fundamentals of CSF dynamics studies and the bases of continuous ICP monitoring. The advantages and disadvantages of several hydrodynamic tests that can be performed by lumbar puncture, as well as the normal and abnormal characteristics of an ICP recording, are discussed.

The presence of oligoclonal bands (OCB) of immunoglobulin G (IgG) is in our days the most useful finding in the study of the CSF for the diagnosis of multiple sclerosis (MS). The most sensitive method for the detection of OCB is the isoelectric focusing followed by immunoblotting. The prevalence of OCB changes in different populations with a rank of results from 60 to 95 97%. We have determined the prevalence of OCB in our population and the sensitivity and the specificity of the technique used in our laboratory. We have included 391 patients in whom we analysed the presence of OCB, subdivided in; Group 0: Diagnosed of MS, group 1: First episode of demyelinating process, group 2: Neurological disorders considered noninflammatory or nonautoimmune (NINA),group 3: Neurological disorders considered inflammatory, infectious or autoimmune (IIA). The presence of OCB was searched in CSF and serum simultaneously using isoelectric focusing and immunoblotting. In order to standardize the technique we achieved and internal and external validation. Internal validation: sensitivity and specificity (using as a control group first the group NINA and after the group IA). External validation: we choose 10 pairs of CSF/serum from patients with different diagnostics and sent to a reference laboratory ( Karolinska Institute Medical School) that was blind of our results and of the diagnostics. The prevalence of OCB in each group has been: group 0 (MS): 87.7%, group 1: 54.8%, group 2 (NINA): 17.5%, group 3(IIA): 52.7%. Sensitivity: 97.7%, specificity using group NINA as control 82.5% and using group IIA 45.7%. Concordance with the reference laboratory in 9/10 determinations. We conclude that in our population the prevalence of OCB, in patients with MS, is lower than in Northern Europe. The OCB appear in may inflammatory, autoimmune diseases, their specificity for the diagnostic of MS is low.

Introduction: Synthesis of IgG is useful data for the diagnosis of multiple sclerosis, and different formulas, both direct and indirect, are used to quantify this. We analyze these formulas with the objective of finding whether certain combinations of them would give better results than the individual formulas on their own., Patients and Methods: We studied the cerebrospinal fluid of 45 patients with neurological disorders and determined the results of the two formulas which are most effective, according to some studies (Reiber's formula and IgG index) together with a direct formula (IgG ratio) and another indirect formula (the 'classical' formula of Tourtellotte)., Results: The IgG index was, in general, the formula which best differentiated between patients with multiple sclerosis and persons with other neurological disorders., Conclusions: We found a tendency which supported the original hypothesis that it is possible to use combinations of formulas to obtain better results than individual formulas. This fact may serve as a basis for further studies in which different combinations are tested, including analytical and clinical data, and this may be of use in the diagnosis of multiple sclerosis.

Introduction: Transthyretin is considered an excellent marker for monitoring nutritional status in serum. In cerebrospinal fluid it is synthesized by chroroid plexus. Cuban epidemic neuropathy is an emergent disease with a hypothetically viral and nutritional origin., Objective: To know the behavior of this transport molecule in serum and cerebrospinal fluid in patients with Cuban epidemic neuropathy., Patients and Methods: Serum and cerebrospinal fluid was quantified in 11 patients with Cuban epidemic neuropathy, eight patients suffering from other neuropathies and 15 patients with Down's syndrome and 10 patients with Alzheimer's disease., Results: Serum transthyretin was diminished in patients with Cuban epidemic neuropathy, other neuropathies and Alzheimer's disease. Down's syndrome patients had significantly higher transthyretin levels in comparison with Cuban epidemic neuropathy and Alzheimer's disease. Cerebrospinal fluid transthyretin was significantly increased in patients with Cuban epidemic neuropathy in comparison with the normal values and with Alzheimer's disease patients whose values were settled below the normal values., Conclusions: The decrement of serum transthyretin in Cuban epidemic neuropathy indicates malnutrition and its higher levels in cerebrospinal fluid also indicate a viral infection. These findings support the nutrio-viral hypothesis of the disease.

Objective: To determinate if fibronectin in CSF can be a useful parameter in the diagnosis of some neurologic illnesses., Material and Methods: We have studied 30 patients, subdivided in four groups, depending on the type of neurologic illness. We have chosen as control a 10-patient group, which came to the Emergency Service and were diagnosed as a suspicious of neurologic illness, but after this it was discarded. In the whole group we practiced a lumbar puncture, with cytology, systematic, biochemistry, cultures, immunoglobulins determination and fibronectin quantification by ELISA., Results: We want to emphasize the increase in fibronectin levels in CSF in both the patients with bacterial meningitis and the multiple sclerosis groups, when it's compared with the control group., Conclusion: For this, the determination of fibronectin levels in CSF might be a useful parameter in the diagnosis of some neurologic illnesses.

Introduction: Complementary tests orientated by the clinical findings and correctly interpreted, are useful in some cases in order to exclude certain pathologies and on other occasions to help to orientate towards or to confirm diagnoses. Material and methods. In this paper we analyze the complementary tests most often used in children evaluated during a five year period, from May 1990 to May 1995, by a neuropaediatrician who recently joined the staff of the Hospital Miquel Servet in Zaragoza (which had no neuropaediatrician before then)., Results and Conclusions: Neuroimaging is the technique which was most often useful in establishing the diagnosis. CT or MR orientated or established the diagnosis in 21% of the total number of children evaluated and in 39% of the children in whom this investigation was carried out. The EEG contributed to the diagnosis of epilepsy in 11% of the children. In 16% of the cases of epilepsy the EEG was normal or with nonspecific changes and diagnosis was made on clinical grounds alone. Biochemical tests enabled some pathologies to be ruled out, but contributed to diagnosis on few occasions, most frequently the CSF (basically in meningitis and encephalitis) and muscle enzyme tests were also useful. Routinely used investigations such as those involving amino acids, ammonia, lactate and pyruvate established the diagnosis in a smaller proportion of cases than those used more selectively. Genetics were not found to be very useful in diagnosis. Reallocation of resources is necessary to permit the population as a whole to benefit from the continuous advances being made in techniques of direct study of molecular genetics.

Background: The aim of the present study was to analyze the clinical characteristics and fluid alterations in neurologic infection by varicella herpes zoster virus in hospitalized patients., Methods: A retrospective study of the cases with neurologic involvement by the varicella herpes zoster virus in patients admitted in the authors' hospital from March 1991 to March 1993 was carried out., Results: Our of the 14 patients studied with neurologic involvement by the varicella herpes zoster virus, 10 were males (71%) with a mean age of 38 years (range: 13-83 years). Only 4 patients (28%) presented a base disease (diabetes mellitus in 2 cases and HIV infection in another 2). In 10 cases (71%) the appearance of cutaneous lesions was prior to neurologic manifestations (between 1 and 30 days before neurologic clinical manifestations). All the patients presented hyperthermia at some time. The most common symptoms were: headache, vomiting, confusion and/or neck stiffness, with meningitis, encephalitis and neurologic foci and mixed pictures. In 4 cases (28%) the cephalorhachidian fluid did not present analytical changes suggestive of viral meningitis. All the patients underwent i.v. acyclovir treatment at a dosis of 10-15 mg/kg/8 h with good evolution, with no deaths being observed. In 3 out of the 6 cases presenting neurologic foci the evolution was slow with sequelae following treatment completion., Conclusions: Neurologic involvement by the varicella herpes zoster virus does not clinically defer from other neutrotropic virus. Fluid alterations were compatible with benign lymphocytary meningitis although some cases of encephalitis showed normal LCR. Taking into account that none of the patients herein reported died and considering the mortality associated with meningitis or encephalitis by varicella herpes zoster referred in the literature in untreated patients, the authors believe that the use of acyclovir is obligatory in these cases.

In order to find out the degree to which rupture of the blood-brain barrier (BBB) could influence the diagnostic effectiveness of five equations used to determine the intrathecal synthesis of IgG, a comparative study was performed with results from 733 patients with different neurological processes either related to an immune response (PNI) or not (PNNI). Rupture of the BBB was determined by a quotient of cerebrospinal fluid (CSF)/serum albumin (COA) higher than that obtained in the control group. When the BBB was retained, there was no clear superiority of one formula over another, but an altered BBB was the conditioning factor in the divergence. The extended Index was the least influenced by the rupture of the BBB, while Tourtellotte's equation was the most affected. In agreement with the clinical information data, the IgG Index was the most sensitive equation, while the extended Index was the most specific.

The clinical features of 19 patients with neurological manifestations unexplained by another disease and positive serology for Borrelia burgdorferi were studied. ECM was present in only 11% of the cases and 32% referred tick bite. The characteristic features for suspicion of NB according to our series was the presence of polyneuritis in 84% of the cases specially in the form of multiple mononeuritis and involvement of the facial nerve (79%) leading to even greater suspicion with the association of V pair involvement. Seizures, sleep disorders, and higher mental dysfunction may be found in association with other more characteristic neurological features. The typical triad of NB (aseptic meningitis, facial paralysis and polyradiculoneuritis) was found in 21% of the patients and in the absence of another disease to justify the same neuroborreliosis (NB) seemed evident. In all the cases components of this triad were found. Headache, arthralgia, fever and, less frequently, arthritis are other symptoms often past with the presence of anti-BB antibodies. Patients with the shortest evolution most frequently presented antecedents of facial paralysis, sensory alterations and Romberg's sign than patients of longer evolution. CSF demonstrated the presence of pleocytosis in 24% of the cases and in only one patient a slight increase in the intrathecal activity of IgG was observed which may be of use in differential diagnosis with MS. MR showed alterations in 61% of the patients and, while not specific, the lesions present subcortical predominance.

Spinal fluid from 53 HIV infected patients has been reviewed. A diagnosis was made exclusively from the spinal fluid exam in 15 cases while in 15 others it contributed to diagnosis together with other exams. The exam did not contribute any positive data in 23 cases. AIDS diagnosis was obtained in 11 patients by the spinal fluid exam. The most frequently occurring disease was infectious meningitis (10 cases) being 3 of them HIV aseptic meningitis. In our experience, a spinal fluid exam in HIV infected patients and fever of unknown origin did contribute useful data for the diagnosis of AIDS in a large number of cases.

The polyacrylamide gel electrophoresis of spinal fluid from 488 patients suffering neurological diseases and from a control group are reviewed. One hundred and eleven cases (22.7%) showed oligoclonal bands (OB). The highest percentage of OB appeared in the group of patients with definite multiple sclerosis (84%), followed by those with probable multiple sclerosis (46%) and inflammatory infectious diseases (43%); the group of patients with a possible multiple sclerosis (7%), vascular malignancies (11%) and other diseases (4%) showed a lower incidence of OBs. No OBs were found in groups of patients with degenerative diseases, dementia or the control group. These results confirm the great usefulness of this test to support the diagnosis of multiple sclerosis, although it also shows that it is not a specific test so it should always be evaluated within a clinical context.