Primary amebic encephalitis (PAM) is a devastating central nervous system infection caused by Naegleria fowleri, a free-living amoeba, which can survive in soil and warm fresh water. Here, a 43-year-old healthy male was exposed to warm freshwater 5 days before the symptom onset. He rapidly developed severe cerebral edema before the diagnosis of PAM and was treated with intravenous conventional amphotericin B while died of terminal cerebral hernia finally. Comparing the patients with PAM who has similar clinical symptoms to those with other common types of meningoencephalitis, this infection is probably curable if treated early and aggressively. PAM should be considered in the differential diagnosis of purulent meningoencephalitis, especially in patients with recent freshwater-related activities during the hot season.
Adult ; Amoeba ; Amphotericin B ; Brain Edema ; Central Nervous System Infections ; Central Nervous System Protozoal Infections ; Diagnosis ; Diagnosis, Differential ; Encephalitis ; Encephalocele ; Fresh Water ; Humans ; Male ; Meningoencephalitis ; Naegleria fowleri ; Seasons ; Soil

Ramsay Hunt syndrome with the complication of encephalitis or meningoencephalitis is rarely reported and uncommon in immunocompetent patients. The radiological manifestations of such cases usually involve the cerebellum and brainstem or exhibit the absence of any abnormality. We report a case of a 78-year-old immunocompetent man hospitalized with Ramsay Hunt syndrome, who later developed meningoencephalitis. The cerebrospinal fluid-study excluded other causes of meningoencephalitis, and the clinical diagnosis indicated varicella zoster virus meningoencephalitis. Magnetic resonance imaging revealed increased signal intensities in the bilateral temporal lobe, midbrain, and pons on T2-weighted imaging, and T2 fluid attenuated inversion recovery and contralateral asymmetric pachymeningeal enhancement. Contrast-enhanced T1-weighted imaging revealed ipsilateral facial nerve enhancement.
Aged ; Brain Stem ; Cerebellum ; Diagnosis ; Encephalitis ; Facial Nerve ; Herpes Zoster Oticus ; Herpesvirus 3, Human ; Humans ; Magnetic Resonance Imaging ; Meningoencephalitis ; Mesencephalon ; Pons ; Temporal Lobe

PURPOSE: To explore clinical features and neurologic complications of pediatric enteroviral meningitis, and to evaluate risk factors according to the presence of cerebrospinal fluid (CSF) pleocytosis and neurologic complications. METHODS: Retrospective review of medical records of patients aged between 1 month and 18 years who were found positive for CSF enterovirus reverse transcription polymerase chain reaction in Dankook University Hospital from March 2009 to February 2015. RESULTS: A total of 151 children was enrolled in the study. Ninety four patients were males and the age at diagnosis was 40.9±47.0 months. Most common season of admission was summer (n=99, 65.6%). Most frequent presenting symptom was fever followed by poor oral intake, decreased activity, and headache. Fifty five (36.4%) patients had no CSF pleocytosis. Complications were observed in 4 (2.6%). Mean blood white blood cell, CSF protein levels were higher, and CSF glucose levels were lower in group with pleocytosis (P < 0.05). There were more seizure development and mental changes, higher peak body temperature, longer fever duration, and longer duration from symptom onset to CSF tapping in group with complications (P < 0.05). All were boys and mean age at admission was younger in group with complications, without statistically significant difference. CONCLUSION: Relatively high proportion of children with enteroviral meningitis showed no pleocytosis. Complications such as meningoencephalitis, refractory status epilepticus, or recurrent seizure rarely occurred, although most had good clinical outcomes.
Body Temperature ; Cerebrospinal Fluid ; Child ; Diagnosis ; Enterovirus ; Fever ; Glucose ; Headache ; Humans ; Leukocytes ; Leukocytosis ; Male ; Medical Records ; Meningitis* ; Meningoencephalitis ; Polymerase Chain Reaction ; Retrospective Studies ; Reverse Transcription ; Risk Factors ; Seasons ; Seizures ; Status Epilepticus

Angiostrongylus cantonensis invades the central nervous system (CNS) of humans to induce eosinophilic meningitis and meningoencephalitis and leads to persistent headache, cognitive dysfunction, and ataxic gait. Infected mice (nonpermissive host), admittedly, suffer more serious pathological injuries than rats (permissive host). However, the pathological basis of these manifestations is incompletely elucidated. In this study, the behavioral test, histological and immunohistochemical techniques, and analysis of apoptotic gene expression, especially caspase-3, were conducted. The movement and motor coordination were investigated at week 2 post infection (PI) and week 3 PI in mice and rats, respectively. The cognitive impairs could be found in mice at week 2 PI but not in rats. The plaque-like lesion, perivascular cuffing of inflammatory cells, and dilated vessels within the cerebral cortex and hippocampus were more serious in mice than in rats at week 3 PI. Transcriptomic analysis showed activated extrinsic apoptotic pathway through increased expression of TNFR1 and caspase-8 in mice CNS. Immunohistochemical and double-labeling for NeuN and caspase-3 indicated the dramatically increased expression of caspase-3 in neuron of the cerebral cortex and hippocampus in mice but not in rats. Furthermore, western-blotting results showed high expression of cleaved caspase-3 proteins in mice but relatively low expression in rats. Thus, extrinsic apoptotic pathway participated in neuronal apoptosis might be the pathological basis of distinct behavioral dysfunctions in rodents with A. cantonensis infection. It provides the evidences of a primary molecular mechanism for the behavioral dysfunction and paves the ways to clinical diagnosis and therapy for A. cantonensis infection.
Angiostrongylus cantonensis* ; Angiostrongylus* ; Animals ; Apoptosis* ; Behavior Rating Scale ; Caspase 3 ; Caspase 8 ; Central Nervous System ; Cerebral Cortex ; Diagnosis ; Eosinophils ; Gait ; Gene Expression ; Headache ; Hippocampus ; Humans ; Meningitis ; Meningoencephalitis ; Mice ; Neurons* ; Rats ; Receptors, Tumor Necrosis Factor, Type I ; Rodentia*

Relapsing polychondritis (RP) is an autoimmune disorder characterized by inflammation in cartilaginous structures including the ears, noses, peripheral joints, and tracheobronchial tree. It rarely involves the central nervous system (CNS) but diagnosis of CNS complication of RP is challenging because it can present with varying clinical features. Herein we report 3 cases of relapsing polychondritis involving CNS with distinct manifestations and clinical courses. The first patient presented with rhombencephalitis resulting in brain edema and death. The second patient had acute cognitive dysfunction due to limbic encephalitis. He was treated with steroid pulse therapy and recovered without sequelae. The third patient suffered aseptic meningitis that presented as dementia, which was refractory to steroid and immune suppressive agents. We also reviewed literature on CNS complications of RP.
Brain Edema ; Central Nervous System* ; Dementia ; Diagnosis ; Ear ; Humans ; Inflammation ; Joints ; Limbic Encephalitis ; Meningitis, Aseptic ; Meningoencephalitis ; Nose ; Polychondritis, Relapsing* ; Trees

BACKGROUND AND PURPOSE: Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is an immune-mediated chorioretinal disease that causes acute visual symptoms with characteristic ophthalmoscopic findings. Neurological complications are rarely reported in the literature. Here we report two new cases of APMPPE that presented with neurological manifestations, one of which was associated with peripheral neuropathy, which has not been described before. METHODS: A retrospective database review of all patients with a diagnosis of APMPPE was performed. Clinical, ophthalmological, and neurological data were analyzed, and only cases of APMPPE with neurological complications were included. A literature review of several databases was also performed, and previous case reports were reviewed and analyzed in detail. RESULTS: In total, 56 cases of APMPPE-associated neurological complications were included in the analyses: 54 from the literature and 2 from our own practice. The most common complication was cerebral vasculitis, which affected 28 patients (50%), followed by headaches in 15 patients (26.8%). The other complications include sixth-cranial-nerve palsy, transient hearing loss, meningoencephalitis, cavernous sinus thrombosis, and viral meningitis. CONCLUSIONS: This report adds to the literature of a novel association of APMPPE with peripheral neuropathy, and comprehensively reviews the neurological manifestations of this disease. A high level of suspicion should be applied when dealing with a case of APMPPE. We recommend applying detailed clinical neurological examinations and magnetic resonance imaging to APMPPE patients, and then early steroid treatment if the examination is positive or even suspicious. Early treatment with steroids and long-term treatment with immunosuppressive azathioprine with interval neurological evaluations will contribute positively to the outcomes and avoid fatal complications, namely strokes.
Adrenal Cortex Hormones ; Azathioprine ; Cavernous Sinus Thrombosis ; Diagnosis ; Headache ; Hearing Loss ; Humans ; Magnetic Resonance Imaging ; Meningitis, Viral ; Meningoencephalitis ; Neurologic Examination ; Neurologic Manifestations* ; Paralysis ; Peripheral Nervous System Diseases ; Retrospective Studies ; Steroids ; Stroke ; Vasculitis, Central Nervous System

BACKGROUND AND PURPOSE: Encephalitis caused by Listeria monocytogenes (L. monocytogenes) is rare but sometimes fatal. Early diagnosis is difficult using routine cerebrospinal fluid (CSF) tests, while next-generation sequencing (NGS) is increasingly being used for the detection and characterization of pathogens. METHODS: This study set up and applied unbiased NGS to detect L. monocytogenes in CSF collected from three cases of clinically suspected listeria meningoencephalitis. RESULTS: Three cases of patients with acute/subacute meningoencephalitis are reported. Magnetic resonance imaging and blood cultures led to a suspected diagnosis of L. monocytogenes, while the CSF cultures were negative. Unbiased NGS of CSF identified and sequenced reads corresponding to L. monocytogenes in all three cases. CONCLUSIONS: This is the first report highlighting the feasibility of applying NGS of CSF as a diagnostic method for central nervous system (CNS) L. monocytogenes infection. Routine application of this technology in clinical microbiology will significantly improve diagnostic methods for CNS infectious diseases.
Central Nervous System ; Cerebrospinal Fluid ; Communicable Diseases ; Diagnosis ; Early Diagnosis ; Encephalitis ; Humans ; Listeria monocytogenes* ; Listeria* ; Magnetic Resonance Imaging ; Meningitis, Listeria ; Meningoencephalitis* ; Methods

Primary diffuse leptomeningeal gliomatosis (PDLG) is a rare condition with a fatal outcome, characterized by diffuse infiltration of the leptomeninges by neoplastic glial cells without evidence of primary tumor in the brain or spinal cord parenchyma. In particular, PDLG histologically diagnosed as gliosarcoma is extremely rare, with only 2 cases reported to date. We report a case of primary diffuse leptomeningeal gliosarcomatosis. A 68-year-old man presented with fever, chilling, headache, and a brief episode of mental deterioration. Initial T1-weighted post-contrast brain magnetic resonance imaging (MRI) showed diffuse leptomeningeal enhancement without a definite intraparenchymal lesion. Based on clinical and imaging findings, antiviral treatment was initiated. Despite the treatment, the patient's neurologic symptoms and mental status progressively deteriorated and follow-up MRI showed rapid progression of the disease. A meningeal biopsy revealed gliosarcoma and was conclusive for the diagnosis of primary diffuse leptomeningeal gliosarcomatosis. We suggest the inclusion of PDLG in the potential differential diagnosis of patients who present with nonspecific neurologic symptoms in the presence of leptomeningeal involvement on MRI.
Aged ; Biopsy ; Brain ; Diagnosis ; Diagnosis, Differential ; Fatal Outcome ; Fever ; Follow-Up Studies ; Gliosarcoma ; Headache ; Humans ; Magnetic Resonance Imaging ; Meningeal Carcinomatosis ; Meningoencephalitis ; Neuroglia ; Neurologic Manifestations ; Spinal Cord

Meningoencephalitis (ME) is a common inflammatory disorder of the central nervous system in dogs. Clinically, ME has both infectious and non-infectious causes. In the present study, a multiplex quantitative real-time polymerase chain reaction (mqPCR) panel was optimized for the detection of eight canine neurologic pathogens (Blastomyces dermatitidis, Cryptococcus spp., Neospora caninum, Borrelia burgdorferi, Bartonella spp., Toxoplasma gondii, Ehrlichia canis, and canine distemper virus [CDV]). The mqPCR panel was subsequently applied to 53 cerebrospinal fluid (CSF) samples collected from dogs with ME. The analytic sensitivity (i.e., limit of detection, expressed as molecules per 1 microL of recombinant vector) was 3.8 for CDV, 3.7 for Ehrlichia canis, 3.7 for Bartonella spp., 3.8 for Borrelia burgdorferi, 3.7 for Blastomyces dermatitidis, 3.7 for Cryptococcus spp., 38 for Neospora caninum, and 3.7 for Toxoplasma gondii. Among the tested CSF samples, seven (15%) were positive for the following pathogens in decreasing order of frequency: Cryptococcus spp. (3/7), Blastomyces dermatitidis (2/7), and Borrelia burgdorferi (2/7). In summary, use of an mqPCR panel with high analytic sensitivity as an initial screen for infectious agents in dogs with ME could facilitate the selection of early treatment strategies and improve outcomes.
Animals ; Bacteria/genetics/*isolation & purification ; Dog Diseases/*diagnosis/microbiology/parasitology ; Dogs ; Meningoencephalitis/diagnosis/microbiology/parasitology/*veterinary ; Multiplex Polymerase Chain Reaction/*veterinary ; Prevalence ; Real-Time Polymerase Chain Reaction/*veterinary ; Republic of Korea/epidemiology

Cryptococcosis is an opportunistic infection that generally occurs in patients with cell-mediated immune dysfunction and involves the central nervous system. Infection is a major cause of morbidity and mortality in systemic lupus erythematosus (SLE) patients because of its innate immune dysfunction along with the administration of steroids and immunosuppressants. However, central nervous system cryptococcosis has rarely been reported in SLE patients. A timely diagnosis is critical because of its significant mortality and morbidity. Most cases of cryptococcal meningitis in SLE patients have been reported in those treated with steroids or immunosuppressants. We report on a SLE patient not on medication, who was diagnosed with cryptococcal meningoencephalitis.
Central Nervous System ; Cryptococcosis ; Diagnosis ; Humans ; Immunosuppressive Agents* ; Lupus Erythematosus, Systemic* ; Meningitis, Cryptococcal ; Meningoencephalitis* ; Mortality ; Opportunistic Infections ; Steroids