Chronic Lymphocytic Meningitis Research Articles

Infections à entérovirus de l’adulte traité par rituximab : un nouveau cas de méningite et myofasciite chronique et revue de la littérature

IntroductionChronic enterovirus infections can occur in primary immunodeficiency with hypogammaglobulinemia. They usually associate meningitis and myofasciitis. Such infections have also been described in adults with rituximab-induced hypogammaglobulinemia. Case reportWe report the case of a 33-year-old woman who was given rituximab for immune thrombocytopenia and developed rituximab-induced hypogammaglobulinemia (IgG 4.4g/L). One year after the last rituximab infusion, she developed lower limbs myofasciitis, followed two months later by a chronic lymphocytic meningitis. PCR in the serum and the cerebrospinal fluid at the time of the meningitis and the myofasciitis were positive to the same enterovirus (echovirus 11) while it was negative in the fascia biopsy. Under treatment with intravenous immunoglobulins, all symptoms and laboratory abnormalities improved and enterovirus PCR became negative. ConclusionWe report a case of chronic enterovirus infection associating meningitis and myofasciitis in an adult with rituximab-induced hypogammaglobulinemia. Outcome was favorable under treatment with intravenous immunoglobulins.

HERPES SIMPLEX TYPE 2 ASSOCIATED NEUROLOGICAL SYNDROMES

Three women with neurological syndromes associated with Herpes Simplex type 2 (HSV2), diagnosed by PCR, are presented. Patient 1 had lymphocytic meningitis with no antecedent or concurrent genital symptoms. Patient 2 had chronic lymphocytic meningitis in the context of immunosuppressive medication and Mollaret cells in the cerebrospinal fluid. She has persistent neurological symptoms despite prolonged treatment with intravenous acyclovir, cessation of immunosuppression and clearance of viral nucleic acid from the cerebrospinal fluid. Patient 3 had a 6 year history of a recurrent vesicular eruption in a dermatomal distribution, accompanied by headache, neck stiffness, reduced speech output and confusion. Only Patient 3 gave a history of genital ulceration which had occurred during her initial clinical presentation, with no subsequent genital recurrences. While the role of long-term antiviral therapy is well-established in the prevention of genital recurrences, evidence for its efficacy in recurrent HSV2 meningitis is equivocal. Challenges encountered in managing these patients illustrate: (i) the protean neurological manifestations of HSV2 infection; (ii) the need for better evidence supporting acute and prophylactic antiviral treatment to prevent neurological recurrence; and (iii) the need to develop novel therapeutic approaches to prevent long term morbidity.

Chronic meningitis and central nervous system vasculopathy related to Epstein Barr virus

Chronic active Epstein Barr virus (EBV) infection causes a wide spectrum of manifestation, due to meningeal, parenchymal and vascular involvement. An 11-year-old boy presented with chronic headache, fever and seizures of 18 months duration. His magnetic resonance imaging Brain showed fusiform aneurysmal dilatations of arteries of both the anterior and posterior cerebral circulation. Cerebrospinal fluid (CSF) showed persistent lymphocytic pleocytosis, raised proteins and low sugar with positive polymerase chain reaction for EBV. He later developed pancytopenia due to bone marrow aplasia, with secondary infection and expired. From clinical, imaging and CSF findings, he had chronic lymphocytic meningitis with vasculopathy, which was isolated to the central nervous system. He later had marrow aplasia probably due to X-linked lymphoproliferative disorder related to EBV infection. Vasculopathy, especially diffuse fusiform aneurysmal dilatation associated with chronic EBV infection, is rare, but has been described, similar to our case report.

False-Positive PCR Detection of Tropheryma whipplei in Cerebrospinal Fluid and Biopsy Samples from a Child with Chronic Lymphocytic Meningitis

We report the case of a teenager with chronic lymphocytic meningitis for whom Tropheryma whipplei 16S rRNA PCR results were positive in two cerebrospinal fluid samples and one duodenal biopsy specimen. PCR targeting another specific sequence of Tropheryma whipplei and sequencing of the initially amplified 16S rRNA fragment did not confirm the results.

Tuberculous meningitis in HIV-infected individuals

HIV-infected individuals are at increased risk for all forms of extrapulmonary tuberculosis, including tuberculous meningitis. This risk is increased at more advanced levels of immunosuppression. The time interval between onset of symptoms and presentation to medical care may vary widely, and consequently individuals may present with acute or chronic meningitis. The clinical presentation of tuberculous meningitis in HIV-infected individuals is more likely to include an altered level of consciousness, cranial imaging is more likely to show cerebral infarctions, and the yield of culture of cerebrospinal fluid may also be greater. Given that delayed initiation of therapy is a strong predictor of mortality in cases of tuberculous meningitis, clinicians must consider tuberculosis in the differential diagnosis of the HIV-infected individual with acute or chronic lymphocytic meningitis. Additional treatment considerations for HIV-infected individuals include the timing of initiation of antiretroviral therapy, the potential for drug-drug interactions, and the role of adjunctive corticosteroid therapy.

Isolated central nervous system histoplasmosis in immunocompetent hosts: A series of 11 cases

Histoplasmosis of the central nervous system occurs in a significant percentage of patients with Histoplasma capsulatum infection, but has usually been described in association with immunosuppression and/or disseminated histoplasmosis. We aim to review the clinical and laboratory features of isolated histoplasmosis of the central nervous system in the immunocompetent host by presenting a series of 11 cases with this condition. Most of these patients presented with headache, meningeal irritation signs and mental status changes, comprising a somewhat different picture from that described in immunosuppressed patients. Moreover, almost all patients had signs of ventricular dilatation in neuroimaging studies, and 8 of the 11 patients had a ventriculoperitoneal shunt at the time of diagnosis, suggesting hydrocephalus to be an important feature of this condition and/or the possibility of shunt infection by the fungus. Immunodiffusion analysis of the cerebrospinal fluid appeared to be the most efficient way to reach the diagnosis and should be considered in immunocompetent patients with chronic lymphocytic meningitis, especially in those who have ventricular shunt or live in endemic areas of Histoplasma capsulatum.

Neurosarcoidosis

Chronic involvement of the nervous system is relatively rare in sarcoidosis. We describe 7 cases that fulfil Zajicek's criteria for neurosarcoidosis (NS) and propose some modifications to such criteria. The patients were admitted for various neurological syndromes: 2 cases presented with chronic lymphocytic meningitis, 4 with spinal cord symptoms, one case was initially confused with multiple sclerosis. Serological tests, immunological screening, cerebrospinal fluid (CSF) analysis, bacteriological and viral testing were performed in all patients. Spinal and cerebral MRI, gallium scan, bronchoscopy with biopsy and bronchoalveolar-lavage fluid analysis, high-resolution computed tomography (HRCT) of the chest, biopsy of the lungs, skin, mediastinal lymph-node and meninges, were useful in diagnosing NS. Laboratory tests showed serum inflammatory abnormalities, but were negative for infectious diseases, while CSF showed inflammatory signs in all patients. MRI revealed meningeal enhancement or hypertrophic pachymeningeal lesions in 4 patients, white matter abnormalities and mass lesions in 2 patients, and a spinal mass lesion in 1 patient. Gallium scan, HRCT, bronchoscopy were positive in most cases. Patients were treated with steroid and immunosuppressive therapy, with improvement in six cases. One patient died from infectious complications. A definite diagnosis of NS requires demonstration of non-caseating granulomas affecting nervous tissues. In most cases, histological evidence of systemic disease (probable NS) is sufficient in the presence of compatible alterations in the CNS. In our patients the bronchoalveolarlavage fluid analysis, gallium scan, and chest HRCT were important for diagnosis, while serum ACE was always normal and chest radiographs were not suggestive of sarcoidosis.

Chronic Lymphocytic Meningitis in an Adolescent

A 13-year-old white female complained of a 2-month history of diffuse, often intense headaches and a 1-month history of intermittent nausea, vomiting, and anorexia with intermittent low-grade fevers. An outpatient evaluation included 2 negative rapid screens for group A streptococcus, a negative heterophil antibody test, a negative rapid influenza antigen test, and a normal gall bladder ultrasound. Symptoms persisted despite a 5-day course of azithromycin. She was hospitalized secondary to worsening headaches, nausea, and the onset of diplopia. The patient lived in a rural area in Kentucky and had multiple animal exposures. Family pets included a dog and a fish, and the patient’s home was infested with mice. Just before the onset of her symptoms, the patient had explored a deserted house and found a dead opossum, but she denied touching it. She had recently visited a local stockyard and denied direct contact with the cattle, but did have contact with cow manure; her face had been splashed with manure while she was riding an all-terrain vehicle across a cow pasture. On initial evaluation, the patient had a temperature of 36.4� C, a heart rate of 80 bpm, and blood pressure of116/57 mm Hg. Her neurologic exam was remarkable for bilateral papilledema, left esotropia, left abduction deficit, mild left upper lid ptosis, and mild gait ataxia. Respiratory, cardiovascular, joint, extremity, and lymph node examinations were normal. At admission, the white blood cell (WBC) count was 7.7 3 10 3 /mL with 59% neutrophils, 29% lymphocytes, and 9% monocytes. Hemoglobin was 13.8 gm/dL, and platelets were 371 3 10 3 /mL. Electrolytes and serum transaminases, bilirubin, and albumin were within normal limits. Antibodies to human immunodeficiency virus I and II and Borrelia burgdorferi were negative. A serum rapid plasma reagin (RPR) for syphilis was also negative. Blood and urine cultures were sterile. A tuberculin skin test was negative with positive response to a Candida control. Cerebrospinal fluid (CSF) was clear and colorless with an opening pressure of 310 mm Hg. There were 440 WBCs/mL (7% neutrophils, 80% lymphocytes, 13% monocytes) and 130 red blood cells (RBCs)/mL. CSF protein was 79 mg/dL, and glucose was 38 mg/dL. Negative CSF studies included bacterial and mycobacterial cultures, acid-fast bacilli stain, VDRL, adenovirus polymerase chain reaction, and cryptococcal antigen. Magnetic resonance imaging (MRI) of the brain with and without gadolinium at the time of admission and 5 days later demonstrated no meningeal enhancement or focal lesions. Electroencephalography and echocardiography were also normal. A chest radiograph revealed no parenchymal disease or lymphadenopathy. Cefotaxime was begun empirically, but was discontinued after 4 days. An evaluation for immunodeficiency included normal quantitative immunoglobulins (IgG, 1100 mg/dL; IgM, 172 mg/dL; IgA, not done). Peripheral blood immunotyping revealed normal percentages and absolute numbers of T and B cells with normal lymphocyte response to phytohemagglutinin stimulation.

Connectivite mixte révélée par une méningite lymphocytaire chronique chez un nourrisson

Mixed connective tissue disease (MTCD) is a systemic inflammatory disorder individualised by Sharp et al. in 1972. This entity is rare in children. Case report. – We report an exceptional case of MTCD revealed by lymphocytic meningitis in a two-month-and-a-half-old infant. The disease was diagnosed at the age of nine months when clinical symptomatology was completed by common signs of the illness (Raynaud’s phenomenon, swollen hands), systemic lupus erythematosus-like symptoms (lymphadenopathy, squamous erythema of the limbs, hepato-splenomegaly, pleuritis and ascites) and polymyositis-like findings (muscle weakness with increased serum levels of myogenic enzymes). Laboratory investigations showed an important inflammatory syndrome and the presence of speckled anti-nuclear and anti-U 1RNP antibodies. Specific antibodies of the other connective tissue diseases were also positive (anti-DNA, anti-Sm, anti-SSA and SSB, anti-Scl 70 and JO1) pleading for the mixed feature of the illness. The follow-up after corticosteroid treatment was marked by clinical and biological improvement. But after five months, the patient died following a severe infectious complication. Conclusion. – Chronic lymphocytic meningitis can be part of Sharp’s syndrome even in infants. However, the diagnosis relies on the evidence of characteristic clinical and biological abnormalities of MTCD.

Metastatic adenocarcinoma masquerading as basal pontine tuberculoma

Tuberculous infection of the central nervous system is common in Hong Kong. A 39-year-old woman presented with isolated right sixth nerve palsy which was non-progressive for 10 months. Neuro-imaging revealed a right pontine lesion. Cerebrospinal fluid (CSF) examination showed lymphocytic meningitis with negative bacteriological and cytological studies. Empirical antituberculous drugs with initial corticosteroid resulted in improved CSF parameters. A diagnosis of cerebral tuberculoma complicated by meningitis was made. She subsequently deteriorated clinically and radiologically. Despite a number of clinical features which were atypical of leptomeningeal metastasis, adenosquamous carcinoma was found on biopsy. Her relatively indolent clinical course might be due to the initial corticosteroid treatment. This report illustrates the importance of early tissue diagnosis in uncertain cases of chronic lymphocytic meningitis.

Idiopathic steroid responsive chronic lymphocytic meningitis--clinical features and long-term outcome in 17 patients.

Some patients with chronic lymphocytic meningitis appear to respond to corticosteroid treatment, but investigations fail to identify the cause. The use of corticosteroids in patients with chronic meningitis is controversial and the long-term outcome is unclear. To review the long-term outcome in 17 patients with the syndrome of idiopathic steroid responsive chronic lymphocytic meningitis. Review of patients' records and examination of surviving patients 1.5 to 17.5 years (median 8.8 years) after presentation. The cause of the steroid responsive chronic meningitis was found during follow up in two of the 17 patients: Wegener's granulomatosis and multiple sclerosis in one patient each. A cause was not found in the other 15 patients. Seven of these patients eventually recovered and corticosteroids were withdrawn after six weeks to six years without a recurrence of symptoms. Four patients improved transiently, but died six months to 26 years after starting treatment; in two, steroids were withdrawn a few weeks before death. Four patients had active chronic meningitis and reduction in the dose of corticosteroids was associated with a recurrence of symptoms. Leptomeningeal and brain biopsy in eight patients showed non-specific abnormalities which were not helpful in the diagnosis. A subgroup of patients with idiopathic chronic lymphocytic meningitis responds to corticosteroids. Leptomeningeal biopsy and long-term follow up seldom identify the underlying cause in these patients. Steroid treatment must be undertaken with caution, even after infective causes of chronic meningitis have been excluded.

Durch Zecken übertragene Meningo-Polyneuritis (Garin-Bujadoux, Bannwarth) Erythema-chronicum-migrans-Krankheit des Nervensystems

The clinical spectrum of this remarkable non-syphilitic spirochaetosis (spirochaeta infection) of the nervous system is described in light of 53 further cases with reference to our earlier description of 47 cases. As the etiological diagnosis is not possible in all cases the knowledge of clinical courses is especially important. Males are affected almost twice as often as women. The disease is especially prevalent in the sixth decade. Usually, the syndrome begins in the warm season with exquisite pain and other sensory irritations in combination with marked malaise. Initial signs during the winter as well as painless clinical courses were also noted. About one half of the patients remembered contact with arthropodes or a chronic migrating erythema. In most cases (95%), asymmetrical peripheral signs followed the painful stage. More than two thirds of the patients showed affection of cranial nerves, usually facial palsy. Twenty percent demonstrated paresis of the extremities alone, the combination of involvement of cranial nerves and extremities was noted in one third of the cases. The sensory signs were usually only mild. Occasionally, the neurological impairment was limited to isolated sensory impairment. In contrast to the closely related Lyme disease, central nervous affection as well as cardiac and arthritic involvement is rare: 34 percent demonstrated mild psychopathological impairment; in one case a Babinski-phenomenon was elicited. Knee joint pain and gonarthritis were found in one case each. Even though meningeal signs were usually absent, the CSF showed alterations suggestive of chronic lymphocytic meningitis in all cases. At the same time, local synthesis of all three immune globulins was documented, especially of IgM (85%).

Chronic benign lymphocytic meningitis

After considering the causes of chronic meningitis, we present a group of 7 patients who suffered a relatively benign chronic lymphocytic meningitis without focal neurological signs, and with a normal glucose concentration in the CSF. The illness lasted from 32 weeks to more than 10 years. Extensive investigations failed to reveal any cause. The illness was self-limiting in 6 out of 7 cases, but in 1 patient it is continuing after 10 years. Without speculation about the cause or causes, it appears useful to recognize the existence of such a chronic mengitis, in the hope that study of further cases will be stimulated.