Brain ; Central Nervous System Diseases ; etiology ; therapy ; Child ; China ; Humans ; Lymphohistiocytosis, Hemophagocytic ; complications ; therapy

Waldenstrom's macroglobulinemia is an uncommon low-grade B-cell lymphoproliferative disorder in which monoclonal immunoglobulin M is produced. Neurological symptoms due to hyperviscosity are frequent manifestations of Waldenstrom's macroglobulinemia. However, central nervous system infiltration by plasmacytoid lymphocytes (Bing-Neel syndrome) has only rarely been reported. We report a case of a 51-yr-old woman suffering from Waldenstrom's macroglobulinemia who complained of persistant headache. Brain magnetic resonance imaging revealed an extra-axial soft tissue mass along the left cavernous sinus, left tentorium, right tentorium, and falx cerebri. A stereotactic biopsy of dural tissue from the falx was performed and showed plasmacytoid lymphocyte infiltration. The patient became symptom- free with irradiation of the whole brain followed by chemotherapy with fludarabine.
Antibodies, Monoclonal/therapeutic use ; Brain/pathology ; Central Nervous System Diseases/etiology/*therapy ; Cranial Irradiation ; Female ; Humans ; Middle Aged ; Syndrome ; Vidarabine/analogs & derivatives/therapeutic use ; Waldenstrom Macroglobulinemia/*complications

Adolescent ; Brain ; diagnostic imaging ; Central Nervous System Diseases ; etiology ; therapy ; Child ; Child, Preschool ; Electroencephalography ; Female ; Humans ; Male ; Purpura, Schoenlein-Henoch ; complications ; Tomography, X-Ray Computed

Amino Acid Metabolism, Inborn Errors ; diagnosis ; genetics ; therapy ; Death, Sudden ; etiology ; Hereditary Central Nervous System Demyelinating Diseases ; diagnosis ; etiology ; Humans ; Hydroxymethylglutaryl-CoA Synthase ; deficiency ; Infant, Newborn ; Male ; Mutation ; Oxo-Acid-Lyases ; genetics ; Spectrometry, Mass, Electrospray Ionization ; Tandem Mass Spectrometry

Objective:To analyze the clinical diagnosis, treatment ，and surgical timing of otogenic intracranial complications. Methods:The clinical data of 11 patients with intracranial complications with ear symptoms as the first manifestation in Department of Otorhinolaryngology Head and Neck Surgery, Qilu Hospital of Shandong University（Qingdao） from December 2014 to June 2022 were collected, including 8 males and 3 females, aged from 4 to 69 years. All patients had complete otoendoscopy, audiology, imaging and etiology examination, and the diagnosis and treatment plan was jointly developed through multidisciplinary consultation according to the critical degree of clinical symptoms and imaging changes. Among the 11 patients, 5 cases were treated with intracranial lesions first in neurosurgery department and middle ear lesions later in otolaryngology, 3 cases of meningitis, were treated with middle ear surgery after intracranial infection control, 1 case was treated with middle ear lesions and intracranial infection simultaneously, and 2 cases were treated with sigmoid sinus and transverse sinus thrombosis conservatively. They were followed up for 1-6 years. Descriptive statistical methods were used for analysis. Results:All the 11 patients had ear varying symptoms, including ear pain, pus discharge and hearing loss, etc, and then fever appeared, headache, disturbance of consciousness, facial paralysis and other intracranial complication. Otoendoscopy showed perforation of the relaxation of the tympanic membrane in 5 cases, major perforation of the tension in 3 cases, neoplasia in the ear canal in 1 case, bulging of the tympanic membrane in 1 case, and turbidity of the tympanic membrane in 1 case. There were 4 cases of conductive hearing loss, 4 cases of mixed hearing loss and 3 cases of total deafness. Imaging examination showed cholesteatoma of the middle ear complicated with temporal lobe brain abscess in 4 cases, cerebellar abscess in 2 cases, cholesteatoma of the middle ear complicated with intracranial infection in 3 cases, and sigmoid sinus thrombophlebitis in 2 cases. In the etiological examination, 2 cases of Streptococcus pneumoniae were cultured in the pus of brain abscess and cerebrospinal fluid, and 1 case was cultured in streptococcus vestibularis, Bacteroides uniformis and Proteus mirabilis respectively. During the follow-up, 1 patient died of cardiovascular disease 3 years after discharge, and the remaining 10 patients survived. There was no recurrence of intracranial and middle ear lesions. Sigmoid sinus and transverse sinus thrombosis were significantly improved. Conclusion:Brain abscess, intracranial infection and thrombophlebitis are the most common otogenic intracranial complications, and cholesteatoma of middle ear is the most common primary disease. Timely diagnosis, multidisciplinary collaboration, accurate grasp of the timing in the treatment of primary focal and complications have improved the cure rate of the disease.
Female ; Humans ; Male ; Brain Abscess/therapy* ; Cholesteatoma ; Deafness/etiology* ; Hearing Loss/etiology* ; Lateral Sinus Thrombosis/therapy* ; Retrospective Studies ; Thrombophlebitis/therapy* ; Child, Preschool ; Child ; Adolescent ; Young Adult ; Adult ; Middle Aged ; Aged ; Cholesteatoma, Middle Ear/therapy* ; Central Nervous System Infections/therapy* ; Sinus Thrombosis, Intracranial/therapy* ; Ear Diseases/therapy*

INTRODUCTIONNeuropsychiatric manifestations can occur in up to two-thirds of patients with systemic lupus erythematosus (SLE). The presentations as well as the underlying immunopathogenic mechanisms can be heterogeneous and therefore have an enormous impact on therapeutic options.

CLINICAL PICTUREWe describe 2 patients who presented similarly with acute onset binocular reversible visual loss. The first patient had anti-phospholipid syndrome and optic neuritis, while the second patient suffered from posterior reversible leukoencephalopathy syndrome.

TREATMENTPatient one was treated with anti-coagulation and immunosuppression while the second patient required the withdrawal of immunosuppression and supportive therapy.

OUTCOMEBoth patients responded favourably and had complete visual recovery.

CONCLUSIONSDifferent management strategies have to be employed for similar presentations having different aetiologies, underscoring the need for constant clinical vigilance.

Adult ; Antiphospholipid Syndrome ; complications ; etiology ; Brain Diseases ; etiology ; immunology ; Epilepsy, Tonic-Clonic ; etiology ; Female ; Humans ; Lupus Erythematosus, Systemic ; complications ; microbiology ; physiopathology ; therapy ; Lupus Vasculitis, Central Nervous System ; diagnosis ; Magnetic Resonance Imaging ; Optic Neuritis ; etiology ; Salmonella Infections ; complications ; Salmonella enteritidis ; Time Factors ; Vision Disorders ; etiology ; immunology

Glomerulonephritis and pulmonary hemorrhage are features of Goodpasture's syndrome. Goodpasture's syndrome accompanied with central nervous system (CNS) vasculitis is extremely rare. Herein, we report a rare case of CNS vasculitis associated with Goodpasture's syndrome in a 34-year-old man, who presented with a seizure and sudden onset of right sided weakness. He also had recurrent hemoptysis of one month's duration. Goodpasture's syndrome is histologically diagnosed by intense linear deposits of IgG along the glomerular basement membrane in both renal and lung tissues.
Adult ; Anti-Glomerular Basement Membrane Disease/complications/*diagnosis/therapy ; Anti-Inflammatory Agents/administration & dosage ; Brain/*pathology ; Contrast Media/administration & dosage ; Diagnosis, Differential ; Fluorescent Antibody Technique ; Hemoptysis/etiology ; Humans ; Image Enhancement/methods ; Immunoglobulin G/immunology ; Kidney/ultrasonography ; Lung/pathology/*radiography ; Magnetic Resonance Imaging ; Male ; Methylprednisolone/administration & dosage ; Muscle Weakness/etiology ; Plasmapheresis ; Rare Diseases ; Seizures/etiology ; Tomography, X-Ray Computed ; Vasculitis, Central Nervous System/*diagnosis/etiology/therapy

Enterovirus 71 frequently involves the central nervous system and may present with a variety of neurologic manifestations. Here, we aimed to describe the clinical features, magnetic resonance imaging (MRI) findings, and cerebrospinal fluid (CSF) profiles of patients presenting with neurologic complications of enterovirus 71 infection. We retrospectively reviewed the records of 31 pediatric patients hospitalized with acute neurologic manifestations accompanied by confirmed enterovirus 71 infection at Ulsan University Hospital between 2010 and 2014. The patients' mean age was 2.9 ± 5.5 years (range, 18 days to 12 years), and 80.6% of patients were less than 4 years old. Based on their clinical features, the patients were classified into 4 clinical groups: brainstem encephalitis (n = 21), meningitis (n = 7), encephalitis (n = 2), and acute flaccid paralysis (n = 1). The common neurologic symptoms included myoclonus (58.1%), lethargy (54.8%), irritability (54.8%), vomiting (48.4%), ataxia (38.7%), and tremor (35.5%). Twenty-five patients underwent an MRI scan; of these, 14 (56.0%) revealed the characteristic increased T2 signal intensity in the posterior region of the brainstem and bilateral cerebellar dentate nuclei. Twenty-six of 30 patients (86.7%) showed CSF pleocytosis. Thirty patients (96.8%) recovered completely without any neurologic deficits; one patient (3.2%) died due to pulmonary hemorrhage and shock. In the present study, brainstem encephalitis was the most common neurologic manifestation of enterovirus 71 infection. The characteristic clinical symptoms such as myoclonus, ataxia, and tremor in conjunction with CSF pleocytosis and brainstem lesions on MR images are pathognomonic for diagnosis of neurologic involvement by enterovirus 71 infection.
Acute Disease ; Brain/diagnostic imaging ; Central Nervous System Diseases/etiology/*pathology ; Child ; Child, Preschool ; Encephalitis/pathology ; Enterovirus A, Human/genetics/*isolation & purification ; Enterovirus Infections/drug therapy/*pathology/virology ; Feces/virology ; Female ; Humans ; Immunoglobulins/administration & dosage ; Infant ; Injections, Intravenous ; Leukocytes/cytology ; Leukocytosis/cerebrospinal fluid/pathology ; Magnetic Resonance Imaging ; Male ; RNA, Viral/genetics/metabolism ; Real-Time Polymerase Chain Reaction ; Republic of Korea ; Retrospective Studies ; Seasons