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1.
Acta Neurol Belg ; 108(3): 99-102, 2008 Sep.
Article in English | MEDLINE | ID: mdl-19115673

ABSTRACT

We report two new cases of mitoxantrone-related leukemia occurring in two patients with multiple sclerosis (MS), 14 and 18 months after the last infusion of the drug. One patient was successfully treated. We were able to collect 29 other cases in the literature. Most of them were single reports but some were described within cohorts of mitoxantrone-treated MS patients. The incidence rate was 0.65% from all cohorts totalizing 2299 patients. Acute promyelocytic leukemia with the translocation t(15;17) was over-represented in the MS population in comparison with cancer patients also treated with mitoxanrone. The occurrence of leukemia was dose-independent and appeared with a mean delay of 20 months after the end of the treatment.


Subject(s)
Leukemia, Myeloid, Acute/chemically induced , Leukemia, Promyelocytic, Acute/chemically induced , Mitoxantrone/adverse effects , Multiple Sclerosis/drug therapy , Adult , Analgesics/adverse effects , Analgesics/therapeutic use , Female , Humans , Leukemia, Myeloid, Acute/drug therapy , Leukemia, Myeloid, Acute/genetics , Leukemia, Promyelocytic, Acute/drug therapy , Leukemia, Promyelocytic, Acute/genetics , Male , Middle Aged , Mitoxantrone/therapeutic use , Oncogene Proteins, Fusion/genetics , Translocation, Genetic
2.
J Neurol Neurosurg Psychiatry ; 77(8): 938-42, 2006 Aug.
Article in English | MEDLINE | ID: mdl-16844949

ABSTRACT

OBJECTIVE: To study cerebrospinal fluid (CSF) and serum samples from 34 consecutive patients suspected of having varicella-zoster virus (VZV) infection of the central nervous system (CNS). POPULATION AND METHODS: The patients were divided into three groups. The first group consisted of 27 patients with a rash in one to three dermatomes and clinical suspicion of meningitis and radiculitis; among them, three subgroups were distinguished according to the affected dermatome: ophthalmicus (n = 9), oticus (n = 11) and cervico-thoraco-lumbar zoster (n = 7). Four cases of zoster sine herpete (ZSH) were included in the second group: these patients presented with either radiculitis (n = 2) or meningoencephalitis (n = 2), without cutaneous eruption. The third group consisted of three patients with a generalised rash and encephalitis. A polymerase chain reaction (PCR) for VZV DNA and antigen-driven immunoblots for oligoclonal anti-VZV antibodies were carried out on all CSF samples. RESULTS: PCR of the CSF was positive in 44% of the patients from the first group, mainly within the first 7 days after eruption. In addition, intrathecal synthesis of anti-VZV antibodies was detected in 37% of patients, always after an interval of 7 days (p<0.0001). Among the four patients with ZSH, a positive VZV PCR was detected in three patients and CSF-specific oligoclonal anti-VZV antibodies in two. PCR was also positive in the CSF of two of the three patients with generalised rash and encephalitis; local production of anti-VZV antibodies was seen in a second CSF sample in one patient, and was also present in the third patient. CONCLUSION: Amplification of VZV DNA by PCR in the CSF and antigen-driven immunoblots have important diagnostic value in suspected VZV infection, although their presence depends on the timing of the CSF sampling. VZV is thought to be a causative agent in unexplained cases of meningitis associated with radiculitis or focal CNS symptoms, even in the absence of skin manifestations. In such patients, rapid diagnosis by this combined approach permits early antiviral treatment.


Subject(s)
Central Nervous System Viral Diseases/genetics , Central Nervous System Viral Diseases/immunology , Herpes Zoster/genetics , Herpes Zoster/immunology , Herpesvirus 3, Human/genetics , Herpesvirus 3, Human/immunology , Adolescent , Adult , Aged , Aged, 80 and over , Antigens, Viral/analysis , Central Nervous System Viral Diseases/cerebrospinal fluid , Central Nervous System Viral Diseases/diagnosis , Cerebrospinal Fluid/virology , DNA, Viral/analysis , Female , Herpes Zoster/cerebrospinal fluid , Herpes Zoster/diagnosis , Humans , Immunoblotting , Male , Middle Aged , Oligoclonal Bands , Polymerase Chain Reaction
3.
Eur J Neurol ; 13(5): 499-504, 2006 May.
Article in English | MEDLINE | ID: mdl-16722976

ABSTRACT

L-2-Hydroxyglutaric (L-2-HG) aciduria is a rare inherited metabolic disease usually observed in children. Patients present a very slowly progressive deterioration with cerebellar ataxia, mild or severe mental retardation, and various other clinical signs including extrapyramidal and pyramidal symptoms, and seizures. The disease is characterized by increased levels of L-2-HG in body fluids such as urine and cerebrospinal fluid. We report on two sisters from consanguineous parents, in whom L-2-HG aciduria was diagnosed at an adult age. Although magnetic resonance imaging and spectroscopic findings were severely abnormal in both, they experienced a different clinical course. The older sister presented with severe mental retardation, recurrent epileptic seizures, and progressive deterioration in her ability to walk and to talk; she is now confined to a wheelchair with severe speech deficit. In contrast, the younger sister only had a few epileptic seizures in childhood and moderate mental retardation, is still able to walk, and performs manual work, and has a social life in a specialized institution for moderately mentally handicapped persons. For the two patients, a complete deletion of exon 9 was demonstrated in a gene located on chromosome 14q22.1, which most probably encodes for L-2-hydroxyglutarate dehydrogenase. The pathological findings observed in this metabolic disorder could therefore be related to a toxic effect of L-2-hydroxyglutarate on the central nervous system, although the presence of other toxic metabolites cannot be excluded.


Subject(s)
Brain/pathology , Epilepsy/urine , Glutarates/urine , Intellectual Disability/urine , Metabolism, Inborn Errors/urine , Mutation , Adult , Age of Onset , Electromyography , Epilepsy/genetics , Female , Humans , Intellectual Disability/genetics , Magnetic Resonance Imaging , Metabolism, Inborn Errors/diagnosis , Metabolism, Inborn Errors/genetics , Siblings
4.
J Neurol Neurosurg Psychiatry ; 75(2): 308-10, 2004 Feb.
Article in English | MEDLINE | ID: mdl-14742614

ABSTRACT

BACKGROUND: Oligoclonal free kappa bands are present as frequently as oligoclonal IgG bands in the cerebrospinal fluid (CSF) from patients with definite multiple sclerosis (MS) and can even occur in the absence of oligoclonal IgG. As such, they too are markers of an ongoing intrathecal immune process. OBJECTIVES: To determine how frequently oligoclonal free kappa bands are detectable in the CSF from patients with clinical signs and symptoms suggestive of MS in the absence of CSF restricted oligoclonal IgG. METHODS: An immunoaffinity mediated immunoblotting technique specific for free kappa chains was used, after isoelectric focusing of paired CSF and serum samples from 33 patients with clinical signs and symptoms suggestive of MS but without CSF oligoclonal IgG. CSF data were correlated with MRI results in the context of the new diagnostic criteria from McDonald et al. RESULTS: Eighteen CSF samples contained oligoclonal free kappa bands (54%), mainly from patients with motor dysfunction (83%) and optic neuritis (64%). All patients with a positive MRI according to Barkhof's criteria (n = 6) had free kappa bands in their CSF. CONCLUSIONS: (1) Oligoclonal free kappa bands in the CSF are related to the dissemination of MS lesions; (2) such bands should be looked for in oligoclonal IgG negative CSF, and (3) the presence of free kappa bands in the CSF may be a substitute for oligoclonal IgG in the McDonald's criteria for diagnosis of MS.


Subject(s)
Brain/immunology , Immunoglobulin kappa-Chains/immunology , Multiple Sclerosis/cerebrospinal fluid , Multiple Sclerosis/immunology , Oligoclonal Bands/immunology , Adolescent , Adult , Brain/pathology , Female , Humans , Magnetic Resonance Imaging , Male , Middle Aged , Multiple Sclerosis/diagnosis
5.
Brain Res Bull ; 61(3): 299-308, 2003 Aug 15.
Article in English | MEDLINE | ID: mdl-12909300

ABSTRACT

Polymerase chain reaction assays are a powerful tool for detecting the presence of infectious genomes in the cerebrospinal fluid. Positive results always mean a current or pending infection of the central nervous system. Subacute (>7 days) or chronic infections induce an intrathecal humoral immune response and the appearance of oligoclonal IgG antibodies directed against the causal infectious agent. This local synthesis may be observed even in cases of severe systemic immunodeficiency. The use of polymerase chain reactions in combination with the detection of a specific intrathecal immune response should represent the most reliable strategy for the diagnosis of viral and chronic infections of the central nervous system. The authors describe their experience, using this approach, in herpetic encephalitis, acute and recurrent herpetic meningitis, varicella zoster-induced neurological diseases, cytomegalovirus encephalitis, progressive multifocal leukoencephalitis and tuberculous meningitis.


Subject(s)
Central Nervous System Infections/cerebrospinal fluid , Central Nervous System Infections/diagnosis , Immunoblotting , Polymerase Chain Reaction , Aged , Aged, 80 and over , Central Nervous System Infections/immunology , Cytomegalovirus/genetics , Cytomegalovirus/isolation & purification , Cytomegalovirus Infections/cerebrospinal fluid , Cytomegalovirus Infections/diagnosis , DNA Primers , Diagnosis, Differential , Encephalitis, Herpes Simplex/cerebrospinal fluid , Encephalitis, Herpes Simplex/diagnosis , Encephalitis, Varicella Zoster/cerebrospinal fluid , Encephalitis, Varicella Zoster/diagnosis , Female , Herpesvirus 3, Human/genetics , Herpesvirus 3, Human/isolation & purification , Humans , Immunoblotting/methods , Immunoglobulin G/cerebrospinal fluid , Infant, Newborn , JC Virus/genetics , JC Virus/isolation & purification , Leukoencephalopathy, Progressive Multifocal/cerebrospinal fluid , Leukoencephalopathy, Progressive Multifocal/diagnosis , Mycobacterium tuberculosis/genetics , Mycobacterium tuberculosis/isolation & purification , Polymerase Chain Reaction/methods , Sensitivity and Specificity , Simplexvirus/genetics , Simplexvirus/isolation & purification , Tuberculosis, Meningeal/cerebrospinal fluid , Tuberculosis, Meningeal/diagnosis
6.
Clin Chem Lab Med ; 39(4): 333-40, 2001 Apr.
Article in English | MEDLINE | ID: mdl-11388658

ABSTRACT

In normal conditions, albumin and immunoglobulin (Ig)G in the cerebrospinal fluid (CSF) originate from the blood, and there is no antibody production within the central nervous system. Up to 20% of CSF proteins are intrathecally synthesized, but the major fraction is blood-derived. The CSF/serum albumin quotient (QAlb) is the best marker of the blood-CSF barrier function. The corresponding immunoglobulin quotients (QIGG, QIGA, QIGM) are not linearly related to QAlb and their correlations are defined by an hyperbolic equation. This equation is used to discriminate between a blood-derived and a locally produced fraction of immunoglobulins in case of an intrathecal humoral immune response. The detection of CSF-specific oligoclonal IgG is more sensitive than the quantitative comparison between QIGG and QAlb. A further step is the determination of antibody indices and the detection of specific oligoclonal antibodies by antigen-driven immunoblots. CSF analysis remains a cornerstone for the diagnosis of various neurological disorders, including multiple sclerosis and infectious diseases of the central nervous system.


Subject(s)
Cerebrospinal Fluid/chemistry , Cerebrospinal Fluid/metabolism , Chemistry, Clinical/methods , Immunoglobulins/blood , Immunoglobulins/cerebrospinal fluid , Central Nervous System/metabolism , Humans , Immunoblotting , Immunoglobulin G/metabolism , Models, Statistical , Polymerase Chain Reaction , Sensitivity and Specificity
8.
Eur J Neurol ; 7(6): 703-6, 2000 Nov.
Article in English | MEDLINE | ID: mdl-11136359

ABSTRACT

Toxocara canis causes the visceral larva migrans syndrome in which central nervous involvement is rare. We report the case of a 40-year-old woman presenting with a subacute weakness of the right leg and dysaesthesiae in the right Th8-Th10 dermatomas. Spinal magnetic resonance imaging examination showed abnormal hyperintensity within the spinal cord. Cerebrospinal fluid analysis revealed eosinophilic pleocytosis. Antibody titres to Toxocara canis were higher in the cerebrospinal fluid than in the serum. Treatment using mebendazole led to a complete clinical recovery, normalization of cerebrospinal fluid parameters and improvement in spinal magnetic resonance imaging abnormalities.


Subject(s)
Leukocytosis/etiology , Myelitis/etiology , Toxocara canis , Toxocariasis/diagnosis , Adult , Animals , Eosinophils , Female , Humans , Hyperesthesia , Hypesthesia , Magnetic Resonance Imaging , Spinal Cord/pathology , Toxocariasis/complications , Toxocariasis/physiopathology
10.
Acta Neurol Belg ; 98(4): 322-6, 1998 Dec.
Article in English | MEDLINE | ID: mdl-9922819

ABSTRACT

Anti-GQ1b antibodies were assayed by an enzyme-linked immunosorbent assay in sera from patients with non-neurological disorders (N = 20), and with various neurological disorders (N = 59), including nine cases of Miller Fisher syndrome, 16 cases of Guillain-Barré syndrome and one case of acute post-infectious ophthalmoparesis. Such antibodies were found in most cases (8 out of 9) of Miller Fisher syndrome, and at very high titres, in one case of Guillain-Barré syndrome characterised by an initial ophthalmoparesis, and in the case of isolated post-infectious ophthalmoparesis. The latter was characterised by a long-lasting occurrence of these antibodies. Anti-GQ1b antibodies are specific for an immune-mediated neuropathy of the cranial, especially oculomotor, nerves.


Subject(s)
Autoantibodies/immunology , Autoantigens/immunology , Autoimmune Diseases/immunology , Gangliosides/immunology , Miller Fisher Syndrome/immunology , Polyradiculoneuropathy/immunology , Autoantibodies/blood , Autoimmune Diseases/etiology , Campylobacter Infections/complications , Campylobacter Infections/immunology , Campylobacter jejuni , Enzyme-Linked Immunosorbent Assay , Female , Humans , Immunoglobulin G/blood , Immunoglobulin G/immunology , Immunoglobulin M/blood , Immunoglobulin M/immunology , Male , Middle Aged , Miller Fisher Syndrome/etiology , Nervous System Diseases/immunology , Ophthalmoplegia/etiology , Ophthalmoplegia/immunology , Polyradiculoneuropathy/etiology , Virus Diseases/complications , Virus Diseases/immunology
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