Listeria brainstem encephalitis with myelitis is extremely rare in clinical practice.Since the clinical manifestations are non-specific,MRI is helpful for diagnosis.Positive cerebrospinal fluid culture is considered the gold standard for diagnosis.This article reports a case of an immunocompetent individual with listeria brainstem encephalitis with myelitis,aiming to enhance the awareness of this condition.
Humans ; Brain Stem/pathology* ; Diagnostic Errors ; Encephalitis/complications* ; Encephalomyelitis, Acute Disseminated/diagnosis* ; Listeriosis/complications* ; Myelitis/complications*

Guillain-Barré syndrome (GBS) and acute disseminated encephalomyelitis (ADEM) are demyelinating neurologic disorders with different target organs. Although they share similar pathogenetic mechanism, reports of simultaneous occurrence of the 2 disorders are rare. A 2 year 6 month old girl visited our hospital for fever, cough, and general weakness. Although the muscle power of extremities showed mild weakness and voiding difficulty, initial deep tendon reflex of both knees and ankles was normal. A nerve conduction study to evaluate the weakness revealed the absence of F waves. Cerebrospinal fluid analysis demonstrated pleocytosis with lymphocyte predominance and elevated protein levels. Magnetic resonance imaging showed abnormal T2 hyperintensity in pons, medulla and spinal cord. Serum anti-GD1b antibody was positive. Based on clinical findings, laboratory findings, nerve conduction study, and neuroimaging, the diagnosis of GBS and ADEM was made. This is the first case of GBS accompanied by ADEM in Korea.
Ankle ; Cerebrospinal Fluid ; Cough ; Demyelinating Diseases* ; Diagnosis ; Encephalomyelitis ; Encephalomyelitis, Acute Disseminated ; Extremities ; Female ; Fever ; Guillain-Barre Syndrome ; Humans ; Knee ; Korea ; Leukocytosis ; Lymphocytes ; Magnetic Resonance Imaging ; Nervous System Diseases ; Neural Conduction ; Neuroimaging ; Peripheral Nervous System* ; Pons ; Reflex, Stretch ; Spinal Cord

The International Pediatric Multiple Sclerosis Study Group (IPMSSG) put forward the 2007 version of the diagnostic criteria for multiple sclerosis and other immune-mediated demyelinating diseases of the central nervous system in children in 2007 ("2007 version" for short). In 2012, IPMSSG proposed the new diagnostic criteria with reference to the latest research achievements of 150 members ("2012 version" for short). The 2012 version of the consensus statements covers the diagnostic criteria for acute disseminated encephalomyelitis, clinically isolated syndrome, neuromyelitis optica, and multiple sclerosis in children. As the two IPMSSG members in China, the authors give an interpretation of the 2012 version of the consensus statements with reference to related literature and clinical and scientific experience. The authors focus on how the 2012 version comprehensively and thoroughly elaborates on the clinical features, diagnostic criteria, influencing factors, and new ideas of acute demyelinating diseases of the central nervous system in children. These become more operable in clinical diagnosis and treatment of multiple sclerosis and other immune-mediated demyelinating diseases of the central nervous system in children.
Child ; Consensus ; Demyelinating Diseases ; diagnosis ; Encephalomyelitis, Acute Disseminated ; diagnosis ; Humans ; Multiple Sclerosis ; diagnosis ; Neuromyelitis Optica ; diagnosis

BACKGROUND AND PURPOSE: Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disorder that predominantly affects children. Previous studies have mostly involved children in Western developed countries. METHODS: This study retrospectively reviewed the clinical profiles of ADEM in adult Chinese patients. RESULTS: ADEM occurred during summer and autumn in about two-thirds of the 42 included patients. Prior infection was found in five patients and no preimmunization was recorded. The most frequent clinical presentations were alterations in consciousness (79%) and behavior changes (69%), followed by motor deficits (64%) and fever (50%). About one-quarter (26%) of the patients showed positive results for oligoclonal bands, and about half of them exhibited increases in the IgG index and 24-hour IgG synthesis rate. Magnetic resonance imaging showed white- and gray-matter lesions in 83% and 23% of the patients, respectively. Steroids were the main treatment, and full recovery occurred in 62% of the patients, with residual focal neurological deficits recorded in a few patients. After a mean follow-up period of 3.4 years, two patients exhibited recurrence and one patient exhibited a multiphasic course. One patient was diagnosed with multiple sclerosis (MS). CONCLUSIONS: With the exception of the seasonal distribution pattern and prior vaccine rate, the clinical profiles of ADEM in adult Chinese patients are similar to those in pediatric populations. No specific markers are available for distinguishing ADEM from MS at the initial presentation. Careful clinical evaluations, cerebrospinal fluid measurements, and neuroradiological examinations with long-term follow-up will aid the correct diagnosis of ADEM.
Adult* ; Asian Continental Ancestry Group* ; Cerebrospinal Fluid ; Child ; Consciousness ; Demyelinating Diseases ; Developed Countries ; Diagnosis ; Encephalomyelitis, Acute Disseminated* ; Fever ; Follow-Up Studies ; Humans ; Immunoglobulin G ; Magnetic Resonance Imaging ; Multiple Sclerosis ; Oligoclonal Bands ; Recurrence ; Retrospective Studies ; Seasons ; Steroids

BACKGROUND AND PURPOSE: Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disorder that predominantly affects children. Previous studies have mostly involved children in Western developed countries. METHODS: This study retrospectively reviewed the clinical profiles of ADEM in adult Chinese patients. RESULTS: ADEM occurred during summer and autumn in about two-thirds of the 42 included patients. Prior infection was found in five patients and no preimmunization was recorded. The most frequent clinical presentations were alterations in consciousness (79%) and behavior changes (69%), followed by motor deficits (64%) and fever (50%). About one-quarter (26%) of the patients showed positive results for oligoclonal bands, and about half of them exhibited increases in the IgG index and 24-hour IgG synthesis rate. Magnetic resonance imaging showed white- and gray-matter lesions in 83% and 23% of the patients, respectively. Steroids were the main treatment, and full recovery occurred in 62% of the patients, with residual focal neurological deficits recorded in a few patients. After a mean follow-up period of 3.4 years, two patients exhibited recurrence and one patient exhibited a multiphasic course. One patient was diagnosed with multiple sclerosis (MS). CONCLUSIONS: With the exception of the seasonal distribution pattern and prior vaccine rate, the clinical profiles of ADEM in adult Chinese patients are similar to those in pediatric populations. No specific markers are available for distinguishing ADEM from MS at the initial presentation. Careful clinical evaluations, cerebrospinal fluid measurements, and neuroradiological examinations with long-term follow-up will aid the correct diagnosis of ADEM.
Adult* ; Asian Continental Ancestry Group* ; Cerebrospinal Fluid ; Child ; Consciousness ; Demyelinating Diseases ; Developed Countries ; Diagnosis ; Encephalomyelitis, Acute Disseminated* ; Fever ; Follow-Up Studies ; Humans ; Immunoglobulin G ; Magnetic Resonance Imaging ; Multiple Sclerosis ; Oligoclonal Bands ; Recurrence ; Retrospective Studies ; Seasons ; Steroids

Meningitis-retention syndrome (MRS) is a clinical entity that has recently appeared in the literature. We present the case of a 22-year-old man with fever and headache who, in the course of his hospitalization with a diagnosis of aseptic meningitis, developed acute urinary retention. Fewer than 30 such cases have been described and in several of them, no clear associations with other disorders have been made. In some cases, direct association with viral infection has been proved, and in others, there are indications of an underlying demyelinating condition. To further complicate the issue, various conditions such as Elsberg syndrome and acute disseminated encephalomyelitis, which not only have some similarities but also have some distinct differences, have been placed under the umbrella definition of MRS. In our review, we attempt to address these conditions and better define MRS by establishing diagnostic criteria based on what has thus far been described in the literature.
Autoimmunity ; Diagnosis ; Encephalomyelitis, Acute Disseminated ; Fever ; Headache ; Hospitalization ; Humans ; Meningitis ; Meningitis, Aseptic ; Urinary Retention ; Young Adult

Acute disseminated encephalomyelitis (ADEM) is a demyelinating and inflammatory condition of the central nervous system, occurring predominantly in white matter. ADEM involving the rhombencephalon without affecting the white matter is very rare. Here, we present an unusual case of ADEM involving only the rhombencephalon in a 4-year-old Asian girl. The patient complained of pain in the right lower extremities, general weakness, ataxia, and dysarthria. The initial brain CT showed subtle ill-defined low-density lesions in the pons and medulla. On brain MRI, T2 high signal intensity (T2-HSI) lesions with mild swelling were present in the pons, both middle cerebellar peduncles, and the anterior medulla. The initial diagnosis was viral encephalitis involving the rhombencephalon. Curiously, a cerebrospinal fluid (CSF) study revealed no cellularity, and negative viral marker findings. Three weeks later, follow up brain MRI showed that the extent of the T2-HSI lesions in the brain stem had decreased. After reinvestigation, it was found that she had a prior history of upper respiratory infection. In this case, we report the very rare case of a patient showing isolated involvement of the rhombencephalon in ADEM, mimicking viral rhombencephalitis on CT and MR imaging. ADEM can involve unusual sites such as the rhombencephalon in isolation, without involvement of the white matter or deep gray matter and, therefore, should be considered even when it appears in unusual anatomical areas. Thorough history taking is important for making a correct diagnosis.
Asian Continental Ancestry Group ; Ataxia ; Biomarkers ; Brain ; Brain Stem ; Central Nervous System ; Cerebrospinal Fluid ; Child, Preschool ; Diagnosis ; Dysarthria ; Encephalitis, Viral ; Encephalomyelitis ; Encephalomyelitis, Acute Disseminated* ; Female ; Follow-Up Studies ; Humans ; Lower Extremity ; Magnetic Resonance Imaging ; Pons ; Rhombencephalon

BACKGROUND: Subacute sclerosing panencephalitis (SSPE) is a delayed and fatal manifestation of measles infection. Fulminant SSPE is a rare presentation in which the disease progresses to death over a period of 6 months. The clinical features are atypical and can be misleading. CASE REPORT: We report herein a teenage boy who presented with acute-onset gait ataxia followed by right hemiparesis that evolved over 1 month, with left-hemispheric, delta-range slowing on the electroencephalogram (EEG). Magnetic resonance imaging disclosed multiple white-matter hyperintensities, suggesting a diagnosis of acute disseminated encephalomyelitis. He received intravenous steroids, and within 4 days of hospital admission he developed unilateral slow myoclonic jerks. Repeat EEG revealed Rademecker complexes, pathognomonic of SSPE, and an elevated titer of IgG antimeasles antibodies was detected in his cerebrospinal fluid. The disease progressed rapidly and the patient succumbed within 15 days of hospitalization. The diagnosis of SSPE was confirmed by autopsy. CONCLUSIONS: This case illustrates the difficulty of recognizing fulminant SSPE when it manifests with asymmetric clinical and EEG abnormalities.
Adolescent* ; Antibodies ; Ataxia* ; Autopsy ; Cerebrospinal Fluid ; Diagnosis ; Electroencephalography ; Encephalomyelitis, Acute Disseminated ; Gait Ataxia ; Hospitalization ; Humans ; Immunoglobulin G ; Magnetic Resonance Imaging ; Male ; Measles ; Myoclonus ; Paresis* ; Steroids ; Subacute Sclerosing Panencephalitis*

Adolescent ; Child ; Child, Preschool ; Encephalomyelitis, Acute Disseminated ; diagnosis ; therapy ; Female ; Follow-Up Studies ; Humans ; Infant ; Magnetic Resonance Imaging ; Male

Acute disseminated encephalomyelitis (ADEM) is a monophasic autoimmune demyelinating disease of the central nervous system, which typically follows acute viral or bacterial infection or vaccination. We report a case of ADEM associated with hepatitis C virus (HCV) infection with positive serum and cerebrospinal fluid (CSF) anti-HCV antibody. After steroid treatment, neurologic symptoms were improved. Virus triggers autoimmunity or direct viral invasion plays a part in the genesis of ADEM. This is the first reported case of ADEM with anti-HCV antibody in the CSF.
Encephalomyelitis, Acute Disseminated/*diagnosis/drug therapy/etiology/virology ; Female ; Hepacivirus/pathogenicity ; Hepatitis C/*complications ; Humans ; Methylprednisolone/therapeutic use ; Middle Aged