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Objective: The objective of the study was to study the neurological manifestations of influenza-A in children. Materials and Methods: This retrospective study was conducted in a tertiary care hospital in South India during the seasonal epidemic of flu in 2019. All children aged 1 month–12 years with evidence of neurological manifestations and positive nasopharyngeal swab for influenza A were evaluated for the clinical features, treatment, and outcome. Results: Sixteen patients had neurological manifestations and 44% of them were

BACKGROUND Data on the neurodevelopment of children who experienced central nervous system (CNS) infections with enteroviruses (EV) or parechoviruses (hPeV) is scarce and mostly limited to follow up of short-term outcomes. METHODS Parents of children who presented between 2014 and 2019, underwent a lumbar puncture and whose cerebrospinal fluid was polymerase chain reaction positive for EV or hPeV, were asked to complete a care-giver-administered neurodevelopmental assessment tool (The Ages and Stages Instrument [ASQ3]). Clinical data of the infective episode were collected from patient notes. RESULTS Of 101 children, 43 (10 hPeV+, 33 EV+) submitted ASQ3 results. Median age at assessment was 38.9 months (interquartile range, 15.4-54.8), the follow-up interval 3 years (median 37 months; interquartile range, 13.9-53.1). Age, inflammatory markers, and cerebrospinal fluid pleocytosis during the infective event were not associated with ASQ3 scores. In 23 children (17 EV+, 6 hPeV+), no neurodevelopmental concerns were reported. Two more had preexisting developmental delay and were excluded. Of the remaining, 18/41 (43.9%) reported ASQ3 scores indicating need for monitoring or professional review in at least 1 category, not differing by pathogen (EV 14/31, 45.2%; hPeV 4/10, 40%; P = 0.71). Seven children will require formal review, scoring ≥2 SD below the mean in at least 1 category (6/31 EV+, 1/10 hPeV+, P = 0.7), 3 scored ≥2 SD below the mean in more than 1 area. CONCLUSIONS Parent-administered developmental assessment of children with a history of early picornavirus infection of the CNS identified a subgroup that requires formal neurodevelopmental review. Wider application of community-based developmental screening will complement our understanding of the impact of CNS infections in early childhood.

It is common practice to perform a lumbar puncture in infants presenting with fever and a bulging fontanelle in order to rule out bacterial meningitis. However, most of these infants have benign, self-limiting diseases. The objective was to determine whether there is an association between bulging fontanelle and bacterial meningitis in febrile infants. This retrospective cohort study included febrile children with a bulging fontanelle who underwent lumbar puncture at Meir Medical Center from 2005 through 2015. A total of 764 children ages 2-18 months underwent lumbar puncture during the study period. Among them, 304 had a bulging fontanelle and fever on evaluation and cerebrospinal fluid pleocytosis was found in 115 (37.8%), including 1 case of bacterial meningitis (0.3%). None of the infants described on admission as appearing well on presentation was found to have bacterial meningitis. Of the 764 children who underwent lumbar puncture, 10 infants were diagnosed with bacterial meningitis, and only one (10%) presented with a bulging fontanelle.Conclusion: The finding of a bulging fontanelle has very low sensitivity and specificity for bacterial meningitis. Most causes of a bulging fontanelle in febrile infants are self-limiting diseases. The routine approach of performing a lumbar puncture in febrile infants with a bulging fontanelle should be reconsidered. What is Known: • It is common to perform a lumbar puncture in febrile infants with a bulging fontanelle, to rule out bacterial meningitis. • However, there are only few researches regarding the relationship between bulging fontanelle and bacterial meningitis. What is New: • The finding of a bulging fontanelle has very low sensitivity and specificity for bacterial meningitis • The need for routine lumbar puncture in these cases should be reconsidered.

Acute transverse myelitis is a rare and disabling disorder. Data on the imaging features in children are sparse. The aim of this study was to describe the clinical and magnetic resonance imaging findings characteristic of pediatric idiopathic acute transverse myelitis and to identify those with prognostic value. The database of a tertiary pediatric medical center was retrospectively reviewed for patients aged less than 18 years who were diagnosed in 2002-2017 with acute transverse myelitis that was not associated with recurrence of a demyelinating autoimmune event. Data were collected on clinical, laboratory, and imaging findings and outcome. A total of 23 children (11 male, 12 female) met the study criteria. Mean age at disease onset was 10 years, and mean duration of follow-up was 6 years 10 months. Spinal cord and brain magnetic resonance imaging scans were performed on admission or shortly thereafter. The most common finding was cross-sectional involvement, in 16 patients (70%). The mean number of involved spinal segments was 8. The most frequently involved region was the thoracic spine, in 17 patients (74%). Clinical factors predicting good prognosis were cerebrospinal fluid pleocytosis, absence of tetraparesis, and prolonged time to nadir. In conclusion, most children with acute transverse myelitis appear to have a good outcome. Prompt diagnosis and treatment are important. Further research is needed in a larger sample to evaluate the predictive value of imaging features.

BACKGROUND: The aims of this study were: investigate the frequency of HIV-1 RNA levels discordance between the cerebrospinal fluid (CSF) and plasma and of CSF viral escape (CVE) in patients HIV-1 subtype C on antiretroviral therapy; evaluate the CSF white blood cell (WBC) performance characteristics in predicting CSF discordance in HIV+ group; and the frequency of cognitive impairment in individuals with CSF HIV discordance or escape. METHODS: HIV-1 RNA levels were assessed in plasma and CSF samples from 68 HIV+ participants without opportunistic infection. RESULTS: CSF discordance was found in 7.4% and CVE in 10%, with comparable frequencies between HIV-1B and C. Twenty samples (29%) showed increased CSF WBC counts. This group had higher CSF and plasma HIV-1 RNA levels than the group with normal WBC counts (p < 0.0001 and 0.006, respectively). The odds of CSF discordance were 18 times higher for a person with CSF WBC count of >5cells/mm(3) than the group with normal CSF WBC count. CSF WBC counts (cut-off of 15 cells/mm(3)) showed high-performance characteristics as a predictive biomarker of CSF discordance (AUC the ROC curve 0.98). The frequency of cognitive impairment for CSF escape or discordance, was 83%, and 80%. The odds of cognitive impairment in these groups were 19 and 15 times higher, than those for a HIV(−) person. CONCLUSION: Viral discordance or escape in the CNS occurs at a comparable frequency for HIV-1C and HIV-1B. The CSF WBC count was effective as a predictive biomarker of CSF and plasma discordance.

Guillain-Barré syndrome (GBS) is a rare complication of chickenpox. All cases of post-varicella GBS published in the literature have been associated with the classical albuminocytological dissociation. We report the case of a 48-year-old male with flaccid areflexic quadriparesis and bilateral seventh, ninth, tenth, and twelfth cranial nerve palsies 10 days after chickenpox. Cerebrospinal fluid (CSF) analysis done in the second week showed marked lymphocytic pleocytosis. Electroneurographic studies were suggestive of acute inflammatory demyelinating polyradiculopathy. He had near-total neurological recovery with intravenous immunoglobulin. Our case demonstrates that GBS can occur after primary varicella infection, and marked CSF pleocytosis can be an additional feature.

Background:Status epilepticus (SE) has traditionally been thought to cause cerebrospinal fluid (CSF) pleocytosis. However, attributing CSF pleocytosis solely to SE without addressing the underlying etiology may lead to poor outcomes. Leukocyte recruitment to CSF has been shown to peak around 24 hours after prolonged seizures in animal studies, suggesting that CSF pleocytosis within the first 24 hours of SE onset may be due to underlying causes. The goal of this study is to assess if SE is associated with CSF pleocytosis, independent of other causes within the first 24 hours of onset.Methods:We completed a historical cohort study of adult patients with SE admitted to the intensive care unit of Vancouver General Hospital between March 2010 and May 2019.Results:Of the 441 patients admitted with SE during the study period, 107 met our inclusion criteria leading to 111 lumbar punctures (LPs), with 4 patients receiving two LPs. CSF pleocytosis was seen in 12 of 72 patients who underwent an LP within the first 24 hours of SE onset. In all 12 patients, a secondary etiology for the pleocytosis was observed aside from SE. Of the six CSF samples collected after 24 hours of onset that demonstrated pleocytosis, four had no cause for pleocytosis other than SE.Conclusions:In all 12 patients with CSF pleocytosis in the first 24 hours of onset of SE, an underlying etiology was identified. Therefore, any pleocytosis noticed within the first 24 hours of onset of refractory SE should not be attributed solely to SE.

Rapidly progressive dementia mimicking Creutzfeldt-Jakob disease (CJD) is a relatively rare presentation but a rewarding one to become familiar with, as the potential diagnoses range from the universally fatal to the completely reversible. Patients require urgent decisions about assessment and investigation and have quickly evolving needs for treatments and support, through symptom management and end-of-life care in most cases. We have based this pragmatic review on the experiences of a specialist prion referral centre in the UK, which, unsurprisingly, is strongly biased towards seeing patients with CJD. Cases eventually proven not to have prion disease might be described as 'CJD-mimics'; being referred from UK neurologists, these are the most challenging cases. CJD in its classical presentation is very rarely mimicked; however, it is highly heterogeneous, and atypical forms can mimic virtually all common neurodegenerative syndromes. Warning features of a mimic include generalised seizures, hyponatraemia, fever, a facial movement disorder, a normal neurological examination and a modestly rapid presentation. Contrast-enhancing lesions or MRI signal hyperintensity outside the striatum, thalamus or cortex and a cerebrospinal fluid pleocytosis are key investigation pointers to a CJD mimic.

Background Definitive diagnosis of meningitis is made by analysis of cerebrospinal fluid (CSF) culture or polymerase chain reaction (PCR) obtained from a lumbar puncture (LP), which may take days. A timelier diagnostic clue of meningitis is pleocytosis on CSF analysis. However, meningitis may occur in the absence of pleocytosis on CSF. Areas of Uncertainty: A diagnosis of meningitis seems less likely without pleocytosis on CSF, leading clinicians to prematurely exclude this. Further, there is little available literature on the subject. Methods Ovid/Medline and Google Scholar search was conducted for cases of CSF culture-confirmed meningitis with lack of pleocytosis. Inclusion criterion was reported cases of CSF culture-positive or PCR positive meningitis in the absence of pleocytosis on LP. Exclusion criteria were pleocytosis on CSF, cases in which CSF cultures/PCR were not performed, and articles that did not include CSF laboratory values. Results A total of 124 cases from 51 articles were included. Causative organisms were primarily bacterial (99 cases). Outcome was reported in 86 cases, 27 of which died and 59 survived. Mortality in viral, fungal and bacterial organisms was 0, 56 and 31%, respectively. The overall percentage of positive initial CSF PCR/culture for viral, fungal and bacterial organisms was 100, 89 and 82%, respectively. Blood cultures were performed in 79 of the 124 cases, 56 (71%) of which ultimately cultured the causative organism. In addition to bacteremia, concomitant sources of infection occurred in 17 cases. Conclusions Meningitis in the absence of pleocytosis on CSF is rare. If this occurs, causative organism is likely bacterial. We recommend ordering blood cultures as an adjunct, and, if clinically relevant, concomitant sources of infection should be sought. If meningitis is suspected, empiric antibiotics/antifungals should be administered regardless of initial WBC count on lumbar puncture. Electronic supplementary material The online version of this article (10.1186/s12879-019-4204-z) contains supplementary material, which is available to authorized users.

Background The clinical manifestations of the transient headache and neurologic deficits with cerebrospinal fluid lymphocytosis (HaNDL) syndrome are variable, and involuntary movements have not been reported. The etiology and treatment of the syndrome are not entirely clarified. Case A 25-year-old female presented with recurrent transient headache, involuntary movements (left upper extremity chorea) and paralysis. Lumbar punctures showed intracranial hypertension and cerebrospinal fluid pleocytosis. Symptoms and intracranial hypertension were relieved after administration of steroids. Discussion/conclusion Chorea may also present in the HaNDL syndrome. Steroids might relieve the symptoms of the HaNDL syndrome, which indicates the possible treatment strategy for HaNDL and supports the infectious or postinfectious autoimmune etiology hypothesis.

Neurotuberculosis usually responds well to standard antitubercular therapy. Some; patients have prolonged course A 11 year old boy diagnosed TBM, an immunocompetent patient, had an unusual course of illness in the form of prolonged fever, persistent hyponatremia and CSF; pleocytosis despite adequate treatment. Clinical course in the management of TBM can be; protracted with complications despite adequate therapy.

Background: Sepsis-like illness is a main cause for hospital admission in young infants. Our aim was to investigate incidence, epidemiology and clinical characteristics of enterovirus (EV) and human parechovirus (HPeV) infections in young infants with sepsis-like illness. Methods: This is a prospective observational cohort study in which infants younger than 90 days of age, presenting with sepsis-like symptoms in a secondary care children's hospital, underwent a full sepsis work-up. Clinical signs and infectious indices were recorded. EV or HPeV RNA was detected by polymerase chain reaction in plasma and/or cerebrospinal fluid (CSF). Results: Infants were diagnosed with EV, HPeV, fever of unknown origin or severe infection. EV and HPeV were detected in 132 of 353 (37%) and 52 of 353 (15%) of cases, respectively. EV and HPeV have distinct seasonability. Some differences in clinical signs and symptoms occurred between children with EV and HPeV infection but were of limited clinical value. CSF pleocytosis occurred in 44% of EV positive infants, and only in 13% of those with HPeV infection. Conclusions: EV and HPeV infections are major causes of sepsis-like illness in infants < 90 days of age. Neither clinical characteristics nor laboratory indices were predictive for EV/HPeV infection. CSF pleocytosis occurs, but not in all patients. Testing for EV and HPeV in all young infants with sepsis-like illness is strongly advised.

BACKGROUND: Lumbar puncture with quantification of leukocytes and differential count of cellular subsets in the cerebrospinal fluid is a standard procedure in cases of suspected neuroinfectious conditions. However, a number of non-infectious causes may result in a low leukocyte number (0-1000 cells/ml). We wanted to assess the diagnostic diversity of unselected adult patients with pleocytosis in the cerebrospinal fluid.METHODS: The study is based on data from cerebrospinal fluid (CSF) analyses of all adult patients (15 years or older) admitted to a large university hospital in Denmark during a two-year period (2008-2009). Data from the local patient administrative system supplied with data from patient charts were combined with laboratory data.RESULTS: A total of 5390 cerebrospinal fluid samples from 3290 patients were included. Pleocytosis >5 leucocytes/μl was found in samples from 262 patients of which 106 (40.5%) were caused by infection of the central nervous system (CNS), 20 (7.6%) by infection outside CNS, 79 (30.2%) due to non-infectious neurological diseases, 23 (8.8%) by malignancy, and 34 (13.0%) caused by other conditions. Significantly higher mean CSF leukocytes was found in patients suffering from CNS infection (mean 1135 cells/μl, p-value CONCLUSIONS: CNS infection, non-infectious neurological disease, malignancy, and infection outside CNS can cause pleocytosis of the cerebrospinal fluid. Leukocyte counts above 100/μl is mainly caused by CNS infection, whereas the number of differential diagnoses is higher if the CSF leukocyte counts is below 50/μl. These conditions are most commonly caused by non-infectious neurological diseases including seizures.

Cerebrospinal fluid pleocytosis has rarely been described as a possible transient finding following epileptic activity. We report a 68-year-old woman who presented with acute confusion and Cerebrospinal fluid pleocytosis. No evidence of an inflammatory, infectious or neoplastic cause explaining the pleocytosis was found. We presume that the pleocytosis in this patient was a result of epileptic convulsions. The mechanism for this finding is not fully understood.

The term rhombencephalitis refers to inflammatory diseases affecting the hindbrain (brainstem and cerebellum). Rhombencephalitis has a wide variety of etiologies, including infections, autoimmune diseases, and paraneoplastic syndromes. Infection with bacteria of the genus Listeria is the most common cause of rhombencephalitis. Primary rhombencephalitis caused by infection with Listeria spp. occurs in healthy young adults. It usually has a biphasic time course with a flu-like syndrome, followed by brainstem dysfunction; 75% of patients have cerebrospinal fluid pleocytosis, and nearly 100% have an abnormal brain magnetic resonance imaging scan. However, other possible causes of rhombencephalitis must be borne in mind. In addition to the clinical aspects, the patterns seen in magnetic resonance imaging can be helpful in defining the possible cause. Some of the reported causes of rhombencephalitis are potentially severe and life threatening; therefore, an accurate initial diagnostic approach is important to establishing a proper early treatment regimen. This pictorial essay reviews the various causes of rhombencephalitis and the corresponding magnetic resonance imaging findings, by describing illustrative confirmed cases.

To distinguish between febrile infants with cerebrospinal fluid pleocytosis who are finally diagnosed with Kawasaki disease and those with enterovirus meningitis poses a diagnostic challenge. We compared clinical and laboratory features at admission between two groups of infants, aged 30-90 days, to identify markers of Kawasaki disease that initially presented as cerebrospinal fluid pleocytosis. During a 2-year period, 100 patients exhibiting cerebrospinal fluid pleocytosis were studied, including six (6.0%) with Kawasaki disease and 30 (30.0%) with enterovirus meningitis. A longer duration of fever before admission (P < 0.01), higher absolute neutrophil count (P < 0.01), increased C-reactive protein level (P < 0.01), pyuria (P = 0.02), and less prominent cerebrospinal fluid pleocytosis (P = 0.01) were identified as initial features of infants finally diagnosed with Kawasaki disease. No significant differences were evident in white blood cell count; platelet count; levels of hemoglobin, alanine aminotransaminase, aspartate aminotransferase, albumin, and sodium; cerebrospinal fluid chemistry; or presence of a rash. Our observations may offer early indicators of Kawasaki disease for timely diagnoses in febrile infants with cerebrospinal fluid pleocytosis.

Aim: Haemophagocytic lymphohistiocytosis (HLH) is a life-threatening disturbance of immunoregulation. HLH comprises primary and acquired forms with different disease severity. A large proportion of deaths occur early into treatment. We investigated association with early death for laboratory and clinical parameters before the start of and 2 weeks into therapy. Methods: A total of 232 children from Scandinavia, Germany or Italy, fulfilling diagnostic criteria and/or with familial disease and/or HLH-causing mutations, receiving HLH treatment 1994–2008 were included. The relation between clinical findings and early pre-transplant death was examined using the Cox proportional hazards model, with a 4-month right-truncation of the outcome. Patients were censored at last follow-up or transplant. Statistically significant predictors were adjusted for sex, age and each other. Results: The following features were significantly associated with adverse outcome: hyperbilirubinaemia (>50 μmol/L; adjusted hazard ratio (aHR) 3.2; 95% confidence interval 1.3–8.1, p = 0.011), hyperferritinaemia (>2000 μg/L; aHR 3.2; 1.2–8.6, p = 0.019), cerebrospinal fluid pleocytosis (>100 × 106/L; aHR 5.1; 1.4–18.5, p = 0.012) at diagnosis, and thrombocytopenia ( 2000 μg/L; aHR 10.6; 1.2–96.4, p = 0.037) 2 weeks into therapy. Non-improvement of fever, anaemia and/or thrombocytopenia also had adverse impact. Conclusion: There seem to be easily available clinical predictors of early mortality in HLH patients, which may help guide treatment decisions.

OBJECTIVE. The purpose of this study was to review the experience with herpes simplex encephalitis at the Hospital for Sick Children over the past 12 years. METHODS. All patients who were admitted to our institution with acute encephalitis between January 1994 and December 2005 were enrolled prospectively in an encephalitis registry. Children from the registry with herpes simplex encephalitis were included in this study; we detailed the clinical presentations, laboratory findings, electroencephalographic findings, diagnostic imaging findings, treatments, and outcomes for all cases. RESULTS. Of 322 cases of acute encephalitis, 5% were caused by herpes simplex virus. Initially negative herpes simplex virus cerebrospinal fluid polymerase chain reaction results were found in 2 cases (13%), but results became positive in repeat cerebrospinal fluid analyses. Classic clinical presentations were seen in 75% of cases, cerebrospinal fluid pleocytosis was found in 94%, elevated cerebrospinal fluid protein levels were found in 50%, electroencephalographic changes were observed in 94%, and diagnostic imaging abnormalities were noted in 88%. All patients were treated with intravenous acyclovir. Neurologic sequelae occurred in 63% of cases, including seizures in 44% and developmental delays in 25%. There were no deaths in this study group. CONCLUSIONS. Herpes simplex encephalitis continues to be associated with poor long-term neurologic outcomes despite appropriate therapy. Cerebrospinal fluid polymerase chain reaction results may be negative early in the course of herpes simplex encephalitis; therefore, repeat cerebrospinal fluid analysis should be considered if herpes simplex encephalitis is suspected. Atypical forms of herpes simplex virus central nervous system disease may occur in children.

Between late May and July 2012, 105 children (62 boys) originating from 2 cities of Thrace were examined because of fever, headache and abdominal pain. Thirty-three of them were hospitalized. They had normal hemograms, and mild to moderate cerebrospinal fluid pleocytosis. Echovirus 30 was isolated from fecal and cerebrospinal fluid samples. Among confirmed cases of echoviral illness, the meningitis attack rate was 51.9%.

Research definitions of encephalitis vary widely. When surveyed on the criteria used in clinical diagnosis, 88 pediatric specialists demonstrated diverse responses, with pediatric neurologists and pediatric infectious disease specialists differing significantly in their consideration of cerebrospinal fluid pleocytosis and abnormal neuroimaging. Results emphasize the need for a uniform definition.

Summary Objectives Ommaya reservoirs (OmR) are used in the treatment of cancer yet risk factors and outcome of infection are not well characterized. We therefore examined our experience with this device. Methods Using administrative databases, we identified all patients with OmR in situ between 1993 and 2008 at Memorial Sloan-Kettering Cancer Center. Charts were reviewed for laboratory, demographic, and clinical information. Results During the study period, 616 patients with OmRs received care at MSKCC comprising 462,467 Ommaya-days. 34 patients with OmR infection were identified (5.5% of patients, 0.74 infections per 10,000 Ommaya-days). 32% of infections occurred within 30 days of OmR placement. Most (74%) OmR infections occurring after 30 days post-placement were associated with OmR access in the preceding 30 days. Recovered organisms included coagulase-negative staphylococci (56%) and Propionibacterium acnes (24%). 70% of patients had fever and/or headache and 69% had cerebrospinal fluid pleocytosis. 50% of patients had the reservoir removed during treatment of the infection. Conclusions OmR infection occurs in one of every 20 persons with the device. A third of the infections appear related to OmR placement while the remainder may occur at any time and usually are associated with recent reservoir access. Treatment often includes device removal.

The aim of this paper is to reassess the significance of postmortem cerebrospinal fluid pleocytosis. Published articles of CSF changes after death were reviewed, and reanalysis, in the light of modern views on the significance of bacterial postmortem isolates, was undertaken. There is theoretical and experimental evidence that the blood brain barrier to the movement of protein and cells is preserved in the first few hours after death. The number of mononuclear cells in the cerebrospinal fluid does rise in the first 24 hours after death, and this is most probably due to detachment of leptomeningeal lining cells. But the marked increase in lymphocyte counts seen in some cases of sudden infant death syndrome (SIDS) and in other deaths in the paediatric age range could well be a marker of inflammation.

We report a young adult man who presented with chronic raised intracranial tension features and unusually progressive bilateral visual and hearing impairment of 18 months duration. MR imaging showed extensive dural involvement and contiguous orbital and spinal disease. Cerebrospinal fluid demonstrated persistent high lymphocytic pleocytosis. Dural biopsy obtained from posterior cervical approach with C1 arch excision and meningeal biopsy revealed features of classical of Rosai-Dorfman disease. Histiocytes were strongly positive for CD-68 and S-100 proteins. The illness relentlessly progressed with patient developing total deafness and near total blindness at last follow-up.

The combination of fever, clouding of conscious, seizures and a ubacutely evolving focal neurological deficit together with mild to oderate cerebrospinal fluid pleocytosis and abnormalities within he temporal lobes on MR-imaging is highly suggestive for viral ncephalitis, especially herpes virus encephalitis (HSE) and repreents a medical emergency. Distinguishing viral encephalitis from on-infectious encephalitis of autoimmune origin is of therapeuic relevance, but this can still be very difficult in clinical practice. euro-Behc et’s is an inflammatory disease of unknown origin and an present with subacute onset of new neurological symptoms. e report on a case of neuro-Behc et’s mimicking the clinical nd paraclinical profile of HSE, a constellation that has not been eported previously in the literature. We discuss early CSF and MRmaging findings (diffusion-weighted imaging) that could help to ifferentiate these two conditions.

Mollaret meningitis is a syndrome characterized by recurrent bouts of meningitis that occur over a period of several years in an affected patient. Also known as recurrent lymphocytic meningitis, this entity involves repeated episodes of headache, stiff neck, fever, and cerebrospinal fluid pleocytosis. Herpes simplex virus type 2 is the most frequently implicated causative agent, and treatment involves the use of antiviral medications. We describe a case of Mollaret meningitis in a 47-year-old man who presented to the emergency department with his eighth episode of meningitis during a period of 20 years. Cerebrospinal fluid polymerase chain reaction testing for herpes simplex virus type 2 was positive, and further testing excluded other common viral, bacterial, and inflammatory causes of meningeal irritation. The patient's family history was significant for a brother who also had multiple episodes of aseptic meningitis during a period of several years. This represents the first published report of a possible familial association involving Mollaret meningitis. It is likely that Mollaret meningitis is underrecognized among emergency physicians, and improved recognition of this entity may limit unwarranted antibiotic use and shorten or eliminate unnecessary hospital admission.

Several different sets of criteria have been proposed to establish the diagnosis of Vogt-Koyanagi-Harada disease (VKH). Various investigators have used the criteria proposed by Sugiura, those proposed by by the American Uveitis Society as well as the revised diagnostic criteria proposed by the First VKH International Workshop group. These three sets of criteria share several clinical features that are considered to be essential for establishing the diagnosis of VKH, including bilateral uveitis, meningismus, and other extraocular changes. The detection of cerebrospinal fluid pleocytosis is considered to be an absolute in the criteria proposed by Sugiura but is not required for the diagnosis of VKH by the revised diagnostic criteria. We applied the latter diagnostic criteria to 28 well-documented patients with early phase VKH and to 88 patients examined during the late phase of VKH. All of these early and late phase patients fulfilled the criteria of the revised diagnostic criteria proposed by the workshop group, indicating 100% concurrence. However, none of the above proposed criteria were prospectively validated to show the positive and negative predictive value of the proposed criteria. Such a prospective study should be undertaken to address the validity of any one or all of the above sets of VKH diagnostic criteria.

Mild cerebrospinal fluid pleocytosis in a Parkinson’s disease patient with deep brain stimulation.

In a retrospective review of all cases with a diagnosis of idiopathic intracranial hypertension in two academic departments of neurology over a nine-year period, the authors identified six patients with a clinical course typical of idiopathic intracranial hypertension (IIH) except for the finding of cerebrospinal fluid pleocytosis. There were five women and one man with a mean age at presentation of 25.7 years (range, 25-32 yr). All were obese but had no other associated medical conditions or identifiable risk factors for IIH. In five patients, all or most cerebrospinal fluid cells were lymphocytes. Cerebrospinal fluid pleocytosis persisted for several months in all patients. Patients underwent a thorough laboratory and neuroimaging evaluation that did not reveal a primary cause. Medical treatment directed solely at lowering intracranial pressure was effective in five patients; one patient required lumboperitoneal shunting. Ophthalmic manifestations of increased intracranial pressure stabilized or remitted after treatment was withdrawn with a mean follow-up period of 33 months (range, 14-55 mo). Some patients may present with idiopathic chronic meningitis and elevated intracranial pressure that responds to treatment used for IIH.

비정형 가와사키병은 가와사키병의 진단기준을 충족하지 않는 경우를 말하며, 주로 6개월 미만의 영아에게서 발현하는 경우가 많다. 비정형 가와사키병의 임상소견은 뇌수막염과 같은 감염질환과 비슷할때가 있어서, 이러한 경우 임상적으로 비정형 가와사키병을 감염질환과 감별하기 어려울 때가 많다. 또한 가와사키병과 연관되어 보고된 신경계 이상은 무균수막염, 경막하삼출, 안면신경마비, 뇌경색증, 뇌병증, 뇌자기공명영상의 가역적 뇌량팽대 변화 등이 있다. 본 저자들은 뇌척수액세포증가증과 경막외삼출액이 동반된 비정형 가와사키병으로 진단된 5개월 여아에 대해 보고하는 바이다. 환자의 심장초음파검사에서 관상동맥이 늘어나 있었고, 추적관찰에서 경막외 삼출액과 관상동맥 합병증이 모두 회복되었으며 생후 12개월에 발달이정표는 정상이었다.

The syndrome of transient Headache and Neurological Deficits with cerebrospinal fluid Lymphocytosis (HaNDL) now referred to in the IHS ICHD–II code 7.8, is only infrequently accompanied by a confusional state and severe agitation. We report the case of a 34–year–old man who suffered from three episodes of headache with transient focal neurological deficits that were consistent with HaNDL but that were accompanied by an intense, confusional agitated state that required admission, in the first episode, in a psychiatric unit.

We show that postmortem cerebrospinal fluid (CSF) pleocytosis is a common event. Postmortem cerebrospinal or ventricular fluid was obtained from children and adults. The cells were counted and morphologically characterized using several histochemical markers. Infants exhibit a brisk postmortem CSF pleocytosis. Sudden infant death cases have relatively high CSF counts. Typeable cells are mononuclear and consist of approximately 60%-70% lymphocytes and 20%-40% macrophages. When postmortem duration is greater than 12 h, the cells become vacuolated and cannot be identified. The etiology of these findings requires further study.

Presentation of Case First admission. A 46-year-old woman was admitted to the hospital because of cognitive impairment and cerebrospinal fluid pleocytosis. She was well until four months earlier, w...

A giant fusiform aneurysm of the basilar artery was present in a 10-year-old boy with a 2-year history of intermittent headaches and the acute onset of neurologic signs. Elevated sedimentation rate, cerebrospinal fluid pleocytosis, and an apparent response to corticosteroid therapy suggest a chronic inflammatory vascular process. The uniqueness of this aneurysm in a child and the possible underlying arteriopathies are discussed.

Coxsackie B3 was cultured from 2 children who presented within two weeks of each other from the Cape Cod, Massachusetts, area showing opsoclonus and myoclonus. The organism was recovered from the cerebrospinal fluid of 1 patient and from the stools of both. Both children had cerebrospinal fluid pleocytosis and gradual, spontaneous resolution of their symptoms.

Two patients with acute posterior multifocal placoid pigment epithelialopathy also had cerebrospinal fluid pleocytosis and elevated protein levels. A 24-year-old white women had visual acuity of counting fingers in each eye. The cerebrospinal fluid contained 56 lymphocytes per cubic millimeter and a protein level of 48 mg/100 ml. When the retinopathy resolved, visual acuity returned to R.E.: 6/60 (20/200), AND L.E.: 6/9 (20/30). A 22-year-old white man had visual acuity of counting fingers bilaterally. The initial lumbar puncture showed 70 lymphocytes and a protein level of 76 mg/100 ml. A second cerebrospinal tab showed 64 lymphocytes and a protein level of 86 mg/100 ml. Final visual acuity was R.E.: 6/15 (20/50), and L.E.: 6/6 (20/20). Neither patient received treatment for this disorder. The findings of cerebrospinal fluid cells and elevated protein levels in acute posterior multifocal placoid pigment epithelialopathy probably represent a mild viral meningitis as part of a systemic viral infection that also involves the retinal pigment epithelium or choriocapillaris.