Schilder's disease or myelinoclastic diffuse sclerosis (MDS) is a rare variant of multiple sclerosis. We report a 66-year-old woman with progressive motor weakness and diffuse white matter degeneration on MRI, which satisfies the Poser's restrictive criteria of MDS. As previously reported cases of probable Schilder's disease did not meet the criteria correctly, we consider our patient is the first pathology-proven case of MDS in Korea.
Aged ; Demyelinating Diseases ; Diffuse Cerebral Sclerosis of Schilder ; Female ; Humans ; Multiple Sclerosis

Acute disseminated encephalomyelitis(ADEM) and acute relapsing disseminated encephalomyelitis(ARDEM) are representative demyelination diseases that occur among young children with a fulminant onset similar to encephalitis or meningitis. The diseases often occur after some viral infection of immunization and the etiology of these diseases is considered to be an autoimmune response because of the similarity in pathologic findings to experimental allergic encephalomyelitis. Cerebral computed tomography(CT) findings of demyelination in ADEM or ARDEM show normal to low density areas in the white matter. In cerebral MRI findings, a scattered distinct high intensity lesion considered to be demyelination is observed in 72-weighted imaging even in the early stages. ADEM is usually monophasic, but recurrent episodes may occure. When ADEM is reccurent, the distinction from multiple sclerosis becomes difficult. We report here a case of acute relapsing disseminated encephalomyelitis(ARDEM) in a 9 years old male child who experence ADEM, 3 times.
Autoimmunity ; Child ; Demyelinating Diseases ; Encephalitis ; Encephalomyelitis, Acute Disseminated* ; Encephalomyelitis, Autoimmune, Experimental ; Humans ; Immunization ; Magnetic Resonance Imaging ; Male ; Meningitis ; Multiple Sclerosis

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

Balo's concentric sclerosis is regarded as a rare variant of multiple sclerosis. Traditionally, Balo's concentric sclerosis was a post-mortem diagnosis, but the recent introduction of brain magnetic resonance imaging (MRI) scans may allow noninvasive access without biopsy. Brain MRI findings of Balo's concentric sclerosis is characteristic concentric configuration of alternating bands of white matter of different pathology, with relatively preserved myelination alternating with regions of demyelination in the cerebral white matter. We report a case of Balo's concentric sclerosis with recurrent optic neuritis.
Biopsy ; Brain ; Demyelinating Diseases ; Diagnosis ; Diffuse Cerebral Sclerosis of Schilder* ; Humans ; Magnetic Resonance Imaging ; Multiple Sclerosis ; Myelin Sheath ; Optic Neuritis* ; Pathology

Acute disseminated encephalomyelitis(ADEM) is an uncommon acute inflammatory disease of the CNS which follows viral infections or Vaccination. ADEM is thought to be an immune mediated disease. The classic featues of ADEM include an antecedent event, commonly a viral illness, followed after a latent period by acute onset of multifocal or diffuse CNS signs, the potential for extensive or even complete recovery, and pathologic evidence of perivascular inflammation and demyelination. We experienced a 43-year-old patient with ADEM and reviewed literatures.
Adult ; Demyelinating Diseases ; Encephalomyelitis, Acute Disseminated* ; Humans ; Inflammation ; Vaccination

No abstract available.
Demyelinating Diseases*

Multiple sclerosis and acute disseminated encephalomyelitis are demyelinating diseases of the central nervous system those can present initially as an acute focal demyelinating syndrome. Multiple sclerosis is characterized by a multiphasic disease with stepwise or progressive deterioration in neurologic function, whereas acute disseminated encephalomyelitis is a monophasic disease with good prognosis. So the differentiation of the two diseases in a patient with single clinical episode attributable to central nervous system demyelination is of prognostic importance, but the differentiation is not easy. We report a case of multiple sclerosis with spinal cord involvement initially misdiagnosed as acute disseminated encephalomyelitis in the brain stem.
Brain Stem ; Central Nervous System ; Demyelinating Diseases ; Encephalomyelitis, Acute Disseminated* ; Humans ; Multiple Sclerosis* ; Prognosis ; Spinal Cord

Acute disseminated encephalomyelitis(ADEM) is believed to be an immunologically mediated acute inflammatory demyelinating disease of the central nervous system. Usually, it is a monophasic illness that may follow a viral infection or vaccination with favorable long-term prognosis. We report an uncommon case of ADEM, which may be caused by upper respiratory infection and/or vaccination.
Central Nervous System ; Demyelinating Diseases ; Encephalomyelitis, Acute Disseminated* ; Influenza, Human* ; Prognosis ; Vaccination*

BACKGROUND AND PURPOSE: A few groups have suggested that activated cytokines and nitrosative stress are closely involved in the pathogenesis of different demyelinating disorders induced by the neuroinflammatory destruction of neurons. The purpose of this study was to elucidate the associations of cytokines and S-nitrosothiols (RSNO) with the severity of neurodegeneration during relapse in demyelinating disorders of the central nervous system. METHODS: We measured levels of interleukin-6 (IL-6), erythropoietin, RSNO, and phosphorylated neurofilament heavy chain (pNfh) in cerebrospinal fluid (CSF) samples obtained from patients with different demyelinating disorders: multiple sclerosis (MS, n=52), acute disseminated encephalomyelitis (ADEM, n=9), and neuromyelitis optica spectrum disorders (NMOSD) with aquaporin-4 immunoglobulin G (AQP4-IgG, n=12). We compared these levels with those measured in a control group (n=24). RESULTS: We found that IL-6 in CSF was elevated in NMOSD with AQP4-IgG and ADEM patients as well as in MS patients after the destruction of soluble IL-6. Erythropoietin levels were lower in MS, while RSNO levels were higher in NMOSD with AQP4-IgG and MS patients than in the control group. CSF pNfh levels were elevated in MS and ADEM patients. CONCLUSIONS: These results confirm that IL-6 is activated in different demyelinating disorders, with this elevation being more prominent in the CSF of NMOSD with AQP4-IgG and ADEM patients. Moreover, S-nitrosylation is activated in demyelinating disorders with spinal-cord injury and neurodegeneration in these patients. However, we found no correlation between these biochemical markers, and so we could not confirm whether IL-6-mediated nitric oxide production is involved in spinal-cord lesions.
Biomarkers ; Central Nervous System* ; Cerebrospinal Fluid ; Cytokines ; Demyelinating Diseases* ; Encephalomyelitis, Acute Disseminated ; Erythropoietin ; Humans ; Immunoglobulin G ; Interleukin-6* ; Intermediate Filaments ; Multiple Sclerosis ; Neuromyelitis Optica ; Neurons ; Nitric Oxide ; Recurrence ; S-Nitrosothiols*

Acute disseminated encephalomyelitis (ADEM) is a demyelinating disease of probable autoimmune etiology. The MR images of patients with clinically suspected ADEM were retrospectively reviewed. The clinical symptoms occurred 5 days to 1 month after viral upper respiratory infection (4) and Coxsakie viral infection(1). The symptoms had begun with fever(3), headache(3), sore throat(1), and drowsy mental state (1), which progressed with monophasic course to altered mental change(2), extremity weakness(2), seizure(1) and/or cerebellary symptom(I) MRI findings of ADEM showed patchy(4), nonhemorrhagic(5), asymmetric(5) high signal intensity lesions on T2-weighted images. The number of the lesions was mostly multiple(4). The lesions mainly involved the brain stem(3) and subcortical white matter(3). Follow-up MR images of 13 days to 20 days after high dose steroid therapy showed marked improvement in two of three, which well correlated with clinical manifestations. MR findin of multiple, patchy, nonhemorrhagic and asymmetric lesions in subcortical white matter and brain stem on T2-weighted images seem to be characteristic features of ADEM, but nonspecific. Therefore, clinical correlation is required in evaluating ADEM.
Brain ; Brain Stem ; Demyelinating Diseases ; Encephalomyelitis, Acute Disseminated ; Extremities ; Follow-Up Studies ; Humans ; Magnetic Resonance Imaging* ; Retrospective Studies ; White Matter