Cerebral Spinal Fluid Examination Research Articles

A case of EBV encephalomyelitis with positive anti-GFAP-IgG antibody with recurrent fever and dysuresia as the main symptoms: Case report and retrospective analysis

Due to neuronal destruction caused by Epstein-Barr virus (EBV) infection, exposure to neuronal surface antigens may lead to an imbalance in immune tolerance, which in turn triggers an autoimmune response. In addition, due to the involvement of nonspecific B-cell activation or molecular mimicry, EBV and Glial Fibrillary Acidic Protein (GFAP) receptors may have common epitopes. Viral infection triggers activation of B-cell and cross-reaction with viral antibodies, resulting in autoimmune encephalomyelitis. The clinical presentation may be complex or even atypical. A small number of patients may develop autoimmune reactions. Most patients with EBV encephalomyelitis have a good prognosis, with the disease generally having a short course, few complications, and a good prognosis. In most patients, after treatment, their neurological function basically recovers within a few weeks or months. The patient had fever and headache. His 3 tests for cerebral spinal fluid (CSF) are consistent with the features of viral encephalomyelitis. Pathogenic examination of CSF confirmed EBV, and imaging suggested brain and spinal cord involvement. After antiviral treatment, the patient's symptoms relieved. The diagnosis of EBV encephalomyelitis was considered. However, the patient's temperature continued to increase. He was transferred to a superior hospital and was given GFAP-Ab in CSF, which was strongly positive. The patient was given immunoglobulin and antiviral therapy. This supports the diagnosis of GFAP-IgG antibody positive with EBV encephalomyelitis. After treatment with antiviral drugs and immunoglobulins, the patient's symptoms improved and he was able to function. EBV encephalomyelitis is a rare clinical disease. Therefore, more attention should be paid to the early diagnosis and treatment of similar patients to avoid misdiagnosis. CSF tests, genetic tests, and imaging tests can confirm the diagnosis.

Relevance of Nerve Biopsy in the Diagnosis of Chronic Inflammatory Demyelinating Polyneuropathy—A Systematic Review

Chronic Inflammatory Demyelinating Polyneuropathy is an immune-mediated pathology of the peripheral nerves and nerve roots that leads to weakness and sensory symptoms. Given its clinical heterogeneity, often times diagnosis is challenging. Even though nerve conduction studies and clinical features are the main criteria used for diagnosis, supplementary investigations, such as nerve biopsies, cerebral spinal fluid examination and magnetic resonance studies, may be used in order to confirm the diagnosis. Given the fact that the hallmark in CIDP physiopathology is the demyelination process, nerve biopsies are used to demonstrate and assess the magnitude of the phenomenon. The question and the main interest of this review is whether histopathological findings are relevant for the diagnosis and can be useful in disease assessment.

Decompressive craniectomy as a rescue treatment in Herpes simplex encephalitis-related cerebral edema: a case report and literature review

We present a case of herpes simplex encephalitis requiring decompressive craniectomy for high intracranial pressure, and a review of decompressive craniectomy in herpes simplex encephalitis. Case description and review: a 32-year-old man was brought to emergency department for headache and fever. The cerebral spinal fluid examination found lymphocytic meningitis; treatment by acyclovir was initiated 4 days after admission. At day 5, patient developed clinical signs of high intracranial pressure and CT-scan showed cerebral herniation. After medical management fail, a decompressive craniectomy was performed on the same day. The procedure resulted in a good clinical recovery with minor neurological sequelae. A literature review, presented here, found few cases of decompressive craniectomy in herpes simplex encephalitis. Due to a lack of robust clinical data, no guideline are yet available. In summary, a surgical approach, such as a decompressive craniectomy, in herpes simplex encephalitis with high intracranial pressure should be discussed early, in association with the medical treatment. Prospective data are needed to better define timing of surgery and decision-making criteria.

Successful Treatment of Acanthamoeba Meningoencephalitis in an Immunocompetent Child.

Acanthamoeba meningoencephalitis is a very rare entity with high mortality rate. Its diagnosis is usually delated, and the optimal approach for its treatment is uncertain. In presented case, early diagnosis was made with direct examination of cerebral spinal fluid, and immediate initiation of accurate treatment with amphotericin B and fluconazole survived patient.

Plasma‐free amino acid (PFAA) profile is a potential blood‐based biomarker for detection of mild cognitive impairment (MCI)

AbstractBackgroundPrimary prevention of cognitive decline is important while development of disease modifying drugs for early Alzheimer's Disease (AD) is progressing rapidly. As a screening tool for early detection of cognitive decline, blood‐based biomarkers prior to invasive testing such as cerebral spinal fluid examination and positron emission tomography scanning could be beneficial. In our small‐scale pilot study, plasma‐free amino acid (PFAA) profiles of AD patients and mild cognitive impairment (MCI) due to AD were shown to be significantly different from those of cognitively healthy elderly. In order to examine the clinical significance of PFAAs as a novel biomarker, we started an MCI cohort study that enrolls hundreds of MCI subjects to determine practical biomarkers for MCI screening among generally healthy subjects, and to distinguish stable MCI and AD‐converted MCI. The results of interim baseline analysis regarding MCI screening will be presented here.MethodMCI subjects from 6 medical sites in Japan (MCI, n=117) and age‐gender‐matched community dwellers in 3 different cities in Japan (healthy control, n=117) were included in the current analysis. At baseline visit, plasma samples were collected under fasting condition. The concentrations of PFAAs and plasma amines were analyzed with LC/MS or LC/MS/MS and serum biochemistry tests were done. Observation of the clinical status of MCI subjects for 3 years after the blood collection is ongoing.ResultSeveral PFAAs including essential amino acids of MCI subjects were significantly lower than those of healthy control. There were no significant differences in plasma amine concentrations between MCI and control group. In addition, several serum biochemistry parameters including serum albumin of MCI subjects were significantly lower. These differences were independent of age and gender.ConclusionBoth PFAAs including essential amino acids and serum biochemistry including albumin were shown to be potential biomarkers for MCI screening. The validation of this results will be conducted in another subset in this MCI cohort study. For further investigation, we will conduct multivariable analysis and create the robust algorithm for MCI screening. Also, observation of the clinical status of MCI subjects will continue to distinguish stable MCI and AD‐converted MCI.

Management of Childhood Headache in the Emergency Department. Review of the Literature.

Headache is the third cause of visits to pediatric emergency departments (ED). According to a systematic review, headaches in children evaluated in the ED are primarily due to benign conditions that tend to be self-limiting or resolve with appropriate pharmacological treatment. The more frequent causes of non-traumatic headache in the ED include primitive headaches (21.8–66.3%) and benign secondary headaches (35.4–63.2%), whereas potentially life-threatening (LT) secondary headaches are less frequent (2–15.3%). Worrying conditions include brain tumors, central nervous system infections, dysfunction of ventriculo-peritoneal shunts, hydrocephalus, idiopathic intracranial hypertension, and intracranial hemorrhage. In the emergency setting, the main goal is to intercept potentially LT conditions that require immediate medical attention. The initial assessment begins with an in-depth, appropriate history followed by a complete, oriented physical and neurological examination. The literature describes the following red flags requiring further investigation (for example neuroimaging) for recognition of LT conditions: abnormal neurological examination; atypical presentation of headaches: subjective vertigo, intractable vomiting or headaches that wake the child from sleep; recent and progressive severe headache (<6 months); age of the child <6 years; no family history for migraine or primary headache; occipital headache; change of headache; new headache in an immunocompromised child; first or worst headache; symptoms and signs of systemic disease; headaches associated with changes in mental status or focal neurological disorders. In evaluating a child or adolescent who is being treated for headache, physicians should consider using appropriate diagnostic tests. Diagnostic tests are varied, and include routine laboratory analysis, cerebral spinal fluid examination, electroencephalography, and computerized tomography or magnetic resonance neuroimaging. The management of headache in the ED depends on the patient's general conditions and the presumable cause of the headache. There are few randomized, controlled trials on pharmacological treatment of headache in the pediatric population. Only ibuprofen and sumatriptan are significantly more effective than placebo in determining headache relief.

Acute cerebellitis caused by herpes simplex virus

Case presentation A 29 year-old woman presented to the emergency with gait imbalance and dysarthria. At admission, neurologic examination revealed normal cognition, ataxia, dysarthria, dysmetria on both sides of the body, bilateral vertical nystagmus and loss of the lateral eye movement. Blood examination was notable for a increase in WBC count and demonstrated erythrocyte sedimentation rate of 18 mm/h. Examination of cerebral spinal fluid (CSF) revealed a protein concentration of 166 mg/ dL, a glucose concentration of 56 mg/dL, and pleocytosis. Serum glucose concentration was 126 mg/dL. The patient had no history of immunosuppression or another comorbidity and anti-HIV test was negative. Neurological evaluation included a head computed tomography (CT) scan which revealed normal findings. An MRI of the brain revealed bilateral increased signal intensity in the cerebellum on fluid-attenuated inversion recovery images (FLAIR)/T2, without contrast enhancement, suggesting an inflammatory process confined to the cerebellum (Figure 1 and 2). Furthermore, the cerebellar cortex appeared swollen, a finding consistent with diffuse cerebellitis. There were no alterations in the brainstem. Initially, the possibility of bacterial rhomboencephalitis caused by Listeria monocytoges was considered, since it is the most commom cause of rhomboencephalitis. After a few days with antibiotic therapy (ceftriaxone and ampicillin), polymerase chain reaction (PCR) test of the CSF was positive for Herpes Simplex Virus 1/2 (HSV). Bacterial culture of CSF samples showed no growth, and the results of Gram staining of CSF were negative. Anti-Listeria antibody was also negative and ampicillin discontinued. CSF PCR analysis for other herpesviruses (varicella-zoster virus, Epstein-Barr virus, cytomegalovirus, and human herpesvirus types 6–8) and enteroviruses were also negative. Upon treatment with acyclovir (50 mg/kg/day) during 21 days, symptoms improved. One month later after the first MRI, a significantly reduce of imaging abnormalities was detected (Figure 3).

Autoimmune inflammatory meningoencephalitis in a patient negative for glial fibrillary acidic protein-specific immunoglobulin G

BackgroundIn this study, we describe clinical findings in a patient with autoimmune inflammatory meningoencephalitis who was negative for antibodies against glial fibrillary acidic protein (GFAP-IgG). MethodsSerum and cerebral spinal fluid (CSF) samples were collected from the patient as part of a study of 520 patients with neurological syndromes. Antibodies against GFAP and other proteins associated with neurological disorders were measured by rat brain- and cell-based indirect immunofluorescence assays. ResultsA 42-year-old female was diagnosed with autoimmune inflammatory meningoencephalitis. She experienced a subacute and relapsing course with decreased vision, fever, headache, ataxia, hemiplegia, and disturbance of consciousness. Brain magnetic resonance imaging showed extensive lesions in the white matter along the ventricle, brainstem, right internal capsule, and meninges. The patient responded well to steroid treatment. Examination of CSF revealed a normal white blood cell count and protein level. Serum and CSF were negative for GFAP-specific antibodies and all other autoantibodies tested. Immunohistochemical staining of a brain biopsy collected during relapse revealed chronic inflammation and severe edema. Extensive and strong staining of CD163+ macrophages were evident throughout the lesions; however, CD3+ cells were rare and CD138+ and CD20+ cells were absent. ConclusionWe describe a case of subacute corticosteroid-responsive nonvasculitic autoimmune inflammatory meningoencephalitis in the absence of GFAP-IgG. The pathological features were distinct from those of patients with GFAP-IgG-positive meningoencephalitis, suggesting that nonvasculitic autoimmune inflammatory meningoencephalitis is a heterogeneous neurological syndrome.

Chronic dysfunction of blood-brain barrier in patients with post-encephalitic/encephalopathic epilepsy

PurposeThis study aimed to elucidate the characteristics and effects of chronic blood-brain barrier (BBB) dysfunction in patients with post-encephalitic/encephalopathic epilepsy (PEE), using brain images and the cerebral spinal fluid (CSF)/serum albumin ratio (albumin quotient, QAlb) as a marker of BBB function. MethodsWe examined the albumin levels in CSF and serum samples from 312 patients with refractory epilepsy in our center between 2004 and 2015. Sixty samples from patients with PEE and 97 samples from age- and sex-matched disease controls (DC) were evaluated. We classified PEE patients into a widespread lesion group and a focal lesion group by severity on brain magnetic resonance images in the chronic phase after acute encephalitis/encephalopathy. ResultsMedian QAlb was higher in PEE than in DC [median (range) ×103: PEE 3.6 (1.0–10.3) versus DC 2.7 (1.0–6.7), p = 0.007]. In a linear regression analysis of the relationship between QAlb and patient’s age at CSF examination or duration of epilepsy, the slope of the regression line was greater in PEE than in DC. Furthermore, in patients under ten years of age, linear regression analysis of QAlb versus seizure frequency showed a weak but positive correlation. Among PEE patients, seizure frequency was higher in the widespread lesion group than in the focal lesion group [300 (4–3000) versus 30 (1–1500) seizures/month, p < 0.001]. ConclusionOur study suggests that patients with PEE have more severe BBB dysfunction, and that the BBB dysfunction is associated with refractory epilepsy.

Diffusion-weighted imaging MRI in Creutzfeldt-Jakob disease

A 67-year-old female with hypertension, autoimmune hypothyroidism and history of epilepsy, presented with a two-month history of progressive cognitive compromission (deficit of short time memory, hallucinations, confusion). Brain MRI showed in diffusion-weighted-imaging (DWI) and in fluid-attenuated-inversion-recovery (FLAIR) sequences an alterated signal in parieto-fronto-temporal cortices (Fig. 1). A 55-year-old male with hypertension, rheumatoid arthritis, presented with a seven-day history of progressive gait imbalance until use of wheelchair. Brain MRI showed in diffusion-weighted-imaging (DWI) and in fluid-attenuated-inversion-recovery (FLAIR) sequences an alterated signal in occipito-parieto-temporal cortices (Fig. 2). A 69-year-old female with dyslipidemia, presented with a three-month history of progressive behavioral disorder, gait imbalance until use of wheelchair, paresis of left limbs. Brain MRI showed in diffusion-weighted-imaging (DWI) and in fluid-attenuated-inversion-recovery (FLAIR) sequences an alterated signal in right parieto-frontal cortices and in putamen (Fig. 3). A 71-year-old male, smoker, with arrhythmic-hypertensive heart disease, presented with a ten-day history of dizziness with progressive balance instability, cerebellar syndrome, with cognitive impairment. Brain MRI showed in diffusion-weighted-imaging (DWI) and in fluid-attenuated-inversion-recovery (FLAIR) sequences an alterated signal in left occipito-parietal cortices, bilateral frontal cortex and in left striatum (Fig. 4). The four patients underwent also to brain MRI, EEG, cerebral spinal fluid (CSF) examination. a.Wernicke’s encephalopathyb.Viral encephalitisc.Creutzfeldt-Jakob diseased.Hypoxic ischemic encephalopathy Creutzfeldt-Jakob disease. We considered 4 cases of suspicious Creutzfeldt-Jakob disease (C.J.) The onset symptoms have often not been characteristic, becoming progressively more specific. C.J. is prion encephalopathy, characterized by progressive dementia with cognitive, behavioral and/or motor dysfunction; it is a rare disease, usually fatal within 12 months after onset; there are several forms of C.J: the most common form is sporadic (SCJD); other forms are familial or genetic. Diagnosis of the disease is based on the clinical, usual EEG pattern, detection of protein 14.3.3 in CSF, and MRI; the definitive diagnosis requires brain autopsy. EEG of four patients showed a typical pattern with periodic or pseudoperiodic sharp-waves complexes (PSWCs). CSF examination documented the presence of 14.3.3 protein in all patients. In the first phase of pathology, our patients had a cerebral cortex involvement, and only in one there was an involvement of basal ganglia. Brain MRI is the gold standard in C.J. diagnosis thanks to its greater sensitivity in T2-FLAIR and DWI than in “classic” long TR sequences. MRI typically showed an T2-weigted and FLAIR alterated signal in basal ganglia; the possible involvement of the cerebral cortex requires the use of DWI sequences. DWI with Apparent Diffusion Coefficient (ADC) mapping showed an increased sensitivity, because of better contrast in cortex study. We declare that there are no conflicts of interest. We confirm that we have read the Journal’s position on issues involved in ethical publication and affirm that this report is consistent with those guidelines.

Leptomeningeal metastasis of pulmonary large-cell neuroendocrine carcinoma: A case report and review of the literature.

Pulmonary large-cell neuroendocrine carcinoma (LCNEC) is a rare and malignant form of lung cancer with a poor prognosis for patients. The common sites of metastases are the liver, adrenal glands, bone and brain. LCNEC rarely metastasizes to the small intestine, ovaries, tonsils, mandible, vulva or spine. To the best of our knowledge, there have been no reports of leptomeningeal metastasis of LCNEC to date. The present case report describes an unusual case of leptomeningeal metastasis from pulmonary LCNEC alongside a review of the literature. Biopsies of pulmonary lesions and cervical lymph nodes confirmed the diagnosis of LCNEC in a 39-year-old male patient. At 2 months after chemotherapy, the patient began to experience hoarseness, epileptic seizures and blurred vision. Furthermore, the patient presented with radiating pain and numbness in his lower left limb. Imaging findings and cytological examination of cerebral spinal fluid supported the diagnosis of leptomeningeal metastasis. The patient's neurological symptoms were markedly alleviated following receipt of radiation and intrathecal chemotherapy. The patient survived for 4.9 months after diagnosis with leptomeningeal metastasis. To the best of our knowledge, the present case report is the first to describe leptomeningeal metastasis from pulmonary LCNEC confirmed by neuroimaging and cerebral spinal fluid cytology. It suggests that leptomeningeal metastasis does occur in this rare disease, and aggressive treatment may result in improved symptoms and possibly survival times.

P15.02 The accuracy of MRI for diagnosing intracranial lymphoma

Abstract Introduction: Intracranial lymphoma is a rare but serious condition. Diagnostic workup includes at least a physical exam, cerebral spinal fluid examination and MRI, though the gold standard is stereotactic biopsy. Stereotactic biopsy is a procedure with possible complications, which include infection, bleeding and diagnostic delay. This inspired our ­multidisciplinary team to study the necessity of biopsy when a lesion is radiologically (very) suspicious for lymphoma. MRI features of lymphoma have been well-described. We investigated the accuracy of MRI for diagnosing intracranial lymphoma. Additionally, we aimed to explore diagnostic delay caused by the biopsy. Materials and methods: We conducted a retrospective single center database study, obtaining MRI reports and biopsy results of patients with brain lesions visiting our outpatient clinic in the past 5 years. We selected all patients with an MRI-report where lymphoma was mentioned as the only possible diagnosis or where lymphoma was mentioned in the differential diagnosis. Primary outcomes were sensitivity and positive predictive value (PPV) of MRI. Secondary outcomes were differences in sensitivity and PPV between subgroups and time between MRI, biopsy and treatment. Results: Positive MRIs had a sensitivity of 87.5%, 95% CI [64.0, 96.5] and PPV of 48.3%, 95% CI [31.4, 65.6]. All MRIs where lymphoma was the only possible diagnosis, had a biopsy proven lymphoma. Mean time in days between MRI and biopsy was 13.60 days, SD10.1, and between MRI and treatment 26.41 days, SD12.7. DISCUSSION: In this study, all MRIs that have lymphoma as their only possible diagnosis, had biopsy proven lymphoma. This suggests that these patients might not need a stereotactic biopsy to prove diagnosis. Further research is necessary to determine whether the same results come up in larger sample sizes and other study designs. In the meantime, our multidisciplinary team designed a diagnostic fast track for these patients, shortening the diagnostic delay caused by the need to biopsy.

Clinical Immunophenotype at Disease Onset in Previously Healthy Patients With Cryptococcal Meningitis.

Cryptococcal meningitis (CM) is a global disease with significant morbidity and mortality. Although low peripheral blood cluster of differentiation 4 (CD4)+ cell counts are found to be related to a high burden of cryptococcus in HIV-infected patients, little is known about possible immune defects in previously healthy patients (PHPs). We performed a retrospective study of 41 CM patients treated from January 2005 to December 2014 who did not have HIV-infection. There were 33 PHPs and 8 not previously healthy patients (non-PHPs). We analyzed clinical test data pertaining to peripheral blood T cells, antibodies, inflammation markers, and cerebral spinal fluid (CSF) completed during the disease onset phase and 5 years following diagnosis. PHPs had significantly higher counts of cluster of differentiation 3 (CD3)+, cluster of differentiation 4 (CD4)+, and cluster of differentiation 45 (CD45)+ cells, and lower percentages of CD8+ cells than non-PHPs (P < 0.05). Measurements of inflammatory markers and immunoglobulin in blood were comparable except for lower immunoglobulin A (IgA) levels in non-PHPs (P = 0.0410). Examination of CSF revealed lower white blood cell (WBC) counts in non-PHPs. Five-year mortality in PHPs was higher than in non-PHPs (22.0% vs 12.5%) but this was not statistically significant (P > 0.05). Multivariate analysis revealed that higher immunoglobulin G (IgG) levels in serum during disease onset may be an independent predictor of mortality (P = 0.015). In conclusion, PHPs demonstrate an immunophenotype that is distinct from that of non-PHPs, leading to an improved understanding of the immunology of cryptococcal meningitis.

Defining a new biomarker for the autoimmune component of Multiple Sclerosis: Th40 cells

Multiple Sclerosis (MS) is a chronic inflammatory, neurodegenerative disease. Diagnosis is very difficult requiring defined symptoms and multiple CNS imaging. A complicating issue is that almost all symptoms are not disease specific for MS. Autoimmunity is evident, yet the only immune-related diagnostic tool is cerebral–spinal fluid examination for oligoclonal bands. This study addresses the impact of Th40 cells, a pathogenic effector subset of helper T cells, in MS. MS patients including relapsing/remitting MS, secondary progressive MS and primary progressive MS were examined for Th40 cell levels in peripheral blood and, similar to our findings in autoimmune type 1 diabetes, the levels were significantly (p<0.0001) elevated compared to controls including healthy non-autoimmune subjects and another non-autoimmune chronic disease. Classically identified Tregs were at levels equivalent to non-autoimmune controls but the Th40/Treg ratio still predicted autoimmunity. The cohort displayed a wide range of HLA haplotypes including the GWAS identified predictive HLA-DRB1*1501 (DR2). However half the subjects did not carry DR2 and regardless of HLA haplotype, Th40 cells were expanded during disease. In RRMS Th40 cells demonstrated a limited TCR clonality. Mechanistically, Th40 cells demonstrated a wide array of response to CNS associated self-antigens that was dependent upon HLA haplotype. Th40 cells were predominantly memory phenotype producing IL-17 and IFNγ with a significant portion producing both inflammatory cytokines simultaneously suggesting an intermediary between Th1 and Th17 phenotypes.

Vaccine strain varicella-zoster virus–induced central nervous system vasculopathy as the presenting feature of DOCK8 deficiency

Vaccine strain varicella-zoster virus–induced central nervous system vasculopathy as the presenting feature of DOCK8 deficiency

Pathology of Central Nervous System Posttransplant Lymphoproliferative Disorders: Lessons from Pediatric Autopsies

Posttransplant lymphoproliferative disorders (PTLD) involving the central nervous system (CNS) in children are uncommon and can prove diagnostically challenging. The clinical and imaging characteristics of CNS PTLD can overlap with those of infection, hemorrhage, and primary CNS tumors. Some cases of CNS PTLD remain clinically unsuspected and are diagnosed postmortem. We report 6 instances of CNS PTLD in children, 2 of which were limited to the CNS and were unsuspected before autopsy. In our autopsy series, PTLD was found outside the CNS in 4 out of 6 cases. Since CNS PTLD has a poor prognosis and the presentation can be subtle, unsuspected, and high grade, it is important to maintain a high index of suspicion and to perform imaging and brain biopsy whenever clinically appropriate. In the presence of leptomeningeal involvement, the diagnosis could be made by cerebral spinal fluid examination.

14-3-3s are potential biomarkers for HIV-related neurodegeneration

Over the last decade, it has become evident that 14-3-3 proteins are essential for primary cell functions. These proteins are abundant throughout the body, including the central nervous system and interact with other proteins in both cell cycle and apoptotic pathways. Examination of cerebral spinal fluid in humans suggests that 14-3-3s including 14-3-3ε (YWHAE) are up-regulated in several neurological diseases, and loss or duplication of the YWHAE gene leads to Miller-Dieker syndrome. The goal of this review is to examine the utility of 14-3-3s as a marker of human immune deficiency virus (HIV)-dependent neurodegeneration and also as a tool to track disease progression. To that end, we describe mechanisms implicating 14-3-3s in neurological diseases and summarize evidence of its interactions with HIV accessory and co-receptor proteins.

Obesity as the initial manifestation of central nervous system relapse of acute lymphoblastic leukemia: Case report and literature review

A 6-year-old boy with acute lymphoblastic leukemia in remission experienced hyperphagia, obesity, and emotional disorders. Cytomorphologic examination of cerebral spinal fluid (CSF) and cranial MRI did not help in differentiating between central nervous system leukemia (CNSL) and other CNS diseases including tuberculosis in this boy. Flow cytometric CSF analysis on repeated lumber puncture detected lymphoblasts, while microscopic CSF examination did not definitively show relapse disease. The diagnosis of CNSL was thus made and confirmed by the response to leukemia treatment. Obesity can be the first manifestation of CNSL and the diagnosis can be challenging. A combination of CSF cytomorphology, CSF flow cytometry, and cranial MRI can be useful in the diagnosis of the disease. Two mechanisms of CNSL-related obesity are discussed based on the literature review.

Aphasic or amnesic status epilepticus detected on PET but not EEG

To describe five patients with ictal aphasia and one patient with ictal amnesia, who had focal positron emission tomography (PET) hypermetabolism but no clear ictal activity on electroencephalography (EEG). (18)F-Fluorodeoxyglucose (FDG)-PET scans with concomitant EEG were obtained in five patients with suspected ictal aphasia or ictal amnesia without ictal activity on EEG. We reviewed medical history, EEG, imaging data, and treatment outcome. Brain magnetic resonance imaging (MRI) showed no structural abnormalities in any of the patients. EEG showed left temporal irregular delta activity in three patients, with aphasia and nonspecific abnormalities in two other patients, all without clear ictal pattern. All patients demonstrated focal hypermetabolism on PET scan. The hypermetabolism was in the left frontotemporal region in patients with ictal aphasia and in the bilateral hippocampal region in the patient with amnesia. Three patients who received intravenous benzodiazepines during their episodes had transient clinical improvement. With antiepileptic drug (AED) treatment, symptoms gradually resolved in all patients. Concomitant resolution of PET hypermetabolism was documented in three patients who had follow up scans. One patient with ictal aphasia later developed recurrent episodes, each with recurrent PET hypermetabolism. This patient and one other patient required immune-modulating therapy in addition to AEDs. FDG-PET imaging should be considered as a diagnostic tool in patients with suspected ictal aphasia or amnesia, who fail to show clear evidence of ictal activity on EEG.

Neurocysticercose cérébrale au stade de dégénérescence : diagnostic différentiel des métastases cérébrales

Introduction Neurocysticercosis is the most common parasitic disease of the central nervous system. It has a worldwide distribution. Case report We report the case of a 70-year-old woman from Guadeloupe presenting gait abnormalities, impaired ideation, right hemiparesis in a context of weight loss, and fatigue. Blood analyses were normal with neither inflammatory syndrome nor blood hypereosinophilia. Brain computed tomography and magnetic resonance imaging showed hydrocephaly in relation with Sylvius’ aqueduc stenosis, and diffuse contrast-enhancing lesions suggesting metastases. Because of clinical symptoms, the patient underwent ventriculostomy; the cerebral spinal fluid examination was normal. Then a frontal biopsy was performed. Histological examination was compatible with neurocysticercosis and confirmed by serology. The patient was successfully treated with albendazole and steroids. Conclusion Neurocysticercosis must be considered as a differential diagnosis of cerebral metastasis, especially in patients from endemic countries.