Acute disseminated encephalomyelitis in a 3-month-old infant

Acute disseminated encephalomyelitis (ADEM) is a demyelinating disease showing multifocal central nervous system lesions due to an autoimmune disorder. We reported a 3-month-old girl with ADEM. One week after having a cold, she presented with somnolence, poor feeding and vomiting. When she was admitted three days after the onset, she could neither fix or follow objects with her eyes nor respond to sound. Her muscle tone was decreased. Cerebrospinal fluid examination revealed pleocytosis, elevated protein concentration and positive myelin basic protein. No oligoclonal band was detected. Diffuse monomorphic slow wave activity was noted on the electroencephalogram. Only wave I was present bilaterally on the auditory brainstem response. T2 weighted images of magnetic resonance imaging revealed multiple areas of high signal in the right posterior limb of the internal capsule, white matter of the cerebellum and brainstem. She was diagnosed as having ADEM, and underwent high dose gamma-globulin therapy. Corticosteroids were not given because of her high blood pressure. The clinical symptoms improved continuously before and after the administration. Two years after the onset, she showed normal growth and development without reoccurrence. The age at onset of childhood ADEM is usually 3 or 4 years. ADEM before one year of age is very rare. The demyelinating lesions of this case corresponded to the regions which normally become myelinated by 3 months. Although ADEM is usually treated with corticosteroids, high dose gamma 1-globulin therapy can be considered if patients are very young or have a high risk for corticosteroid, or respond poorly to corticosteroids.     

A case of myasthenia gravis occurring in the period of remission of chronic inflammatory demyelinating polyradiculoneuropathy]

A 31-year-old woman noticed progressive muscular weakness in the limbs and paresthesia in the fingers in February 1989. Paresthesia worsened and improved 4 times during 2 months. Intravenous edrophonium chloride failed to improve her muscular weakness. She had high antiacetylcholine receptor antibody titer in serum. We made a diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) because of slow nerve conduction velocity (NCV), increased CSF protein, and the clinical course. Treatment with prednisolone improved muscular weakness and the slow NCV. Two years later she acutely had dyspnea, dysphagia, and muscular weakness after upper respiratory infection. Intravenous edrophonium chloride dramatically improved her symptoms. The diagnosis was made as myasthenia gravis (MG). After thymectomy her weakness was getting better without any medications. There may exist an autoimmune mechanism common, at least in part, to both CIDP and MG in our patient.     

Fulminant central nervous system demyelination associated with interferon-alpha therapy and hepatitis C virus infection

Hepatitis C virus (HCV) infection is common in the general population and may coincide with disease in the central and peripheral nervous system. Interferon-alpha (IFN-alpha) is used as treatment for HCV infection. The therapeutic benefit is assumed to result from activation of natural killer cells and CD8+ T cells. Despite its beneficial effects, it has been associated with a number of autoimmune disorders, such as chronic inflammatory demyelinating polyneuropathy and multiple sclerosis. Several clinical reports including magnetic resonance imaging exist, but neuropathological confirmation of MS associated with IFN-alpha therapy and HCV infection is lacking. We report a case of a female patient with chronic HCV infection who developed ;acute MS'-like demyelinating disease after IFN-alpha administration, with extensive lesions throughout brain and thoracic spinal cord. The patient died after a disease duration of 6 months. Brain autopsy revealed Baló-like demyelinating plaques with positive HCV sequences within florid lesions. The development of fulminant demyelinating disease after administration of IFN-alpha suggests that autoimmune mechanisms such as T cell mediated tissue damage might be initiated or aggravated by IFN-alpha therapy. Additionally, the presence of HCV RNA within the demyelinated lesion indicates a possible role in triggering or propagating disease.     

A case of brachial amyotrophic diplegia accompanied with Sj?gren's syndrome presenting good response to immunotherapies in the early course of the disease]

A 74-year-old woman suffered from progressive muscle atrophy and weakness of her arms since she was seventy two years old. Before referral to our department, she was diagnosed as having cervical spondylotic myeloradiculopathy and received spinal fusion. Though spinal decompression was successful, muscle weakness of her upper limbs were progressive even after the surgery. On admission, neurological examinations revealed marked atrophy and weakness of her bilateral upper limbs with absent deep tendon reflexes showing man-in-the-barrel syndrome. Her lower extremities had normal muscle strength, but fasciculations were seen in her all four limbs. Electrophysiologically, motor nerve conduction velocity was almost normal but the amplitude was remarkably decreased, conduction block was not detected, and electromyography showed neurogenic patterns on her all extremities. Spinal progressive musclar atrophy (SPMA) accompanied with Sj?gren's syndrome was the likely diagnosis. Because 50 kDa anti-neuronal antibodies were found in her serum, we assumed that anterior horn cells were impaired by an autoimmune mechanism. Thus we treated her with corticosteroid pulse therapy, plasma exchange (PE) and intravenous immunoglobulin infusion therapy (IVIG). Although steroid pulse therapy only had a minimal effect, PE and IVIG promoted a remarkable improvement on her weakness, and the effect lasted for about three months. This is the first case of SPMA with Sj?gren's syndrome which showed good response to PE and IVIG in the early course of the disease. We considered that some SPMA-like motor neuron syndrome accompanied with autoimmune features may require immunomodulating therapies.     

The early risk of multiple sclerosis following isolated acute syndromes of the brainstem and spinal cord

A combined clinical and magnetic resonance imaging follow-up study was undertaken to determine the risk of early progression to multiple sclerosis in patients who present with clinically isolated lesions of the brainstem or spinal cord. Progression to multiple sclerosis was seen in 13 patients (57%) who had a brainstem syndrome and in 14 patients (42%) who had a spinal cord syndrome after mean intervals of 15 and 16 months, respectively. The risk of progression was increased by the presence of oligoclonal bands in the cerebrospinal fluid of patients with either syndrome and by the presence of disseminated brain lesions, as detected by magnetic resonance imaging, in those with a spinal cord syndrome. Follow-up revealed evidence of multiphasic disease in 24 (69%) of 35 patients who had disseminated lesions on magnetic resonance imaging scans at presentation, suggesting that multiple sclerosis is the usual cause of such lesions.     

Aquaporin-4 autoantibody: a neurogenic cause of anorexia and weight loss

Neuromyelitis optica (NMO) is a severe inflammatory demyelinating disease often associated with a highly specific autoantibody, aquaporin-4 antibody. Although the classic syndrome involves the optic nerves and spinal cord, aquaporin-4 antibody has been important in defining the true spectrum of NMO, which now includes brain lesions in areas of high aquaporin-4 expression. Brainstem involvement, specifically area postrema involvement in the medulla, has been associated with intractable vomiting in some patients with NMO. We describe a 14-year-old female with positive aquaporin-4 antibody whose clinical course was dominated by severe anorexia with associated weight loss (from 68-41kg; body mass index 25.2-15.6). Magnetic resonance imaging showed lesions in the medulla, pons, and thalami. Although she had asymptomatic radiological longitudinally extensive transverse myelitis, she never had symptoms or signs referable to the spinal cord or the optic nerves. We propose that anorexia and weight loss should be considered part of the NMO spectrum, probably related to area postrema involvement.     

A case of multiple sclerosis with incongruous homonymous hemianopia due to lateral geniculate body lesion]

We report a rare case of a 31-year-old woman who was diagnosed as multiple sclerosis with homonymous hemianopia due to a demyelinative lesion in the lateral geniculate body. Paresthesia appeared in the distal parts of both legs from September, 2002. She complained of loss of visual field in both eyes 2 months later. Since a left asymmetric wedge-shaped homonymous hemianopia was detected by Octopus automatic perimetry, she was referred to our hospital on December 2, 2002. Neurological examination showed subjective paresthesia in the distal parts of both legs in addition to Lhermitte's sign. Brain MRI showed demyelinated lesions in the lateral geniculate body on the right and periventricular areas of the lateral ventricles. Spinal MRI revealed a demyelinated lesion in the upper cervical cord. She was diagnosed as multiple sclerosis and underwent a steroid pulse therapy. Homonymous hemianopia resolved a few days later.     

Fatal neurologic involvement in pediatric Wegener's granulomatosis

Wegener's granulomatosis is a potentially life-threatening vasculitis with widely variable presentation. Only three pediatric cases with severe central nervous system involvement are reported in the literature. Early fatal outcome as described here is exceptional. This report describes a 13-year-old female with typical skin lesions, proteinuria, and renal failure initially misdiagnosed as a Schoenlein-Henoch purpura. A kidney biopsy revealed severe extracapillary proliferation in 70% of the analyzed glomeruli but no granuloma. In spite of methylprednisolone pulse therapy and oral high-dose prednisone, end-stage renal failure was reached 4 months later, necessitating peritoneal dialysis. Three months later she presented with pulmonary hemorrhage and positive antineutrophil cytoplasmic antibodies suggesting Wegener's granulomatosis. This episode was controlled by methylprednisolone pulses. Seven months later she presented generalized seizures and coma, suggesting central nervous system involvement confirmed by magnetic resonance imaging. Methylprednisolone pulses and intravenous immunoglobulins led to neurologic improvement. Oral methotrexate was then introduced for long-term disease control. Another severe relapse of central nervous system vasculitis did not respond to any applied therapies and led to death 16 months after initial symptoms. This case emphasizes the need for activity scores to identify patients at risk for progressive systemic vasculitis requiring early and long-term aggressive immunosuppressive therapy.     

Myelin oligodendrocyte glycoprotein antibody-associated disease in a patient with symptoms of aseptic meningitis who achieved spontaneous remission: A case report and review of the literature

BackgroundA few case reports have described patients with myelin oligodendrocyte glycoprotein antibody (MOG-Ab)-associated demyelinating syndrome who presented with symptoms of aseptic meningitis. All such patients required immunotherapy. We report a patient with MOG-Ab-associated disorder (MOGAD) who presented with symptoms of aseptic meningitis and improved without treatment.CaseA 13-year-old girl presented with fever, headache, decreased appetite, and neck stiffness. Cerebrospinal fluid (CSF) analysis revealed pleocytosis and magnetic resonance imaging (MRI) showed leptomeningeal enhancement. The patient was diagnosed with aseptic meningitis at admission. However, there were no signs of recovery 4 days after admission (i.e., 8 days after disease onset). Therefore, we performed extensive investigations to identify the cause of the underlying infection and inflammation. On day 14 after admission, the serum MOG-Ab test performed at admission came back positive (1:128) and she was diagnosed with MOGAD. She was discharged on day 18 after admission, because her symptoms, CSF pleocytosis, and MRI findings had improved. About 6 weeks after discharge, MRI revealed hyperintensity without gadolinium enhancement. However, her serum MOG-Ab test was negative. We did follow-ups for 11 months but found no new neurological symptoms.Discussion and ConclusionTo the best of our knowledge, this is the first ever report of a pediatric patient with MOGAD experiencing spontaneous remission with no demyelinating symptoms during an extended follow-up period.     

自身免疫性胶质纤维酸性蛋白星形胶质细胞病

自身免疫性胶质纤维酸性蛋白星形胶质细胞病是一种可治的中枢神经系统自身免疫性炎性疾病,以脑膜、脑、脊髓和视神经等受累为主要表现,磁共振成像可见脑室旁线样放射状强化和(或)脊髓长节段受累伴中央强化病灶,脑活体组织检查提示小血管周围炎症伴小胶质细胞活化,对类固醇激素治疗敏感.胶质纤维酸性蛋白抗体被认为是本病的特异性生物标志物.     

Cerebral amyloid angiopathy-related inflammation – A case report presenting diagnostic difficulties

We describe an 86-year-old woman with a history of hypertension who presented sudden disturbances of consciousness and left hemiparesis. Brain magnetic resonance imaging (MRI) revealed diffused hyperintensive changes on T2-weighted images localized subcortically in the white matter of both cerebral hemispheres, corresponding to acute vasogenic edema, causing moderate mass effect. Posterior reversible encephalopathy syndrome was initially diagnosed. After implementation of anti-edema intravenous steroid treatment and hypotensive therapy the symptoms began to retire, till the total regression. The successive hospitalizations took place two and eight months later due to the occurrence of seizures, motor deficits and the development of mild cognitive impairment. Brain MRI revealed progression of the white matter changes and diffused subcortical microhemorrhages. Each time pulse steroid therapy was implemented and the symptoms improved significantly after several days. Chronic oral steroid treatment resulted in the stabilization of neurological status. The long-term observation of clinical symptoms, remission after immunosuppressive therapy and white matter changes with subcortical microhemorrhages in brain MRI leaded to the diagnosis of cerebral amyloid angiopathy-related inflammation.     

Reversible lesions in the brain parenchyma in Wilson’s disease conifrmed by magnetic resonance imaging：earlier administration of chelating therapy can reduce the damage to the brain

The aim of this study was to evaluate the resolution of brain lesions in patients with Wilson’s disease during the long-term chelating therapy using magnetic resonance imaging and a possible signiifcance of the time latency between the initial symptoms of the disease and the introduction of this therapy. Initial magnetic resonance examination was performed in 37 patients with proven neurological form of Wilson’s disease with cerebellar, parkinsonian and dystonic presentation. Magnetic resonance reexamination was done 5.7 ± 1.3 years later in 14 patients. Patients were divided into： group A, where chelating therapy was initiated 〈 24 months from the ifrst symp-toms and group B, where the therapy started≥ 24 months after the initial symptoms. Symmetry of the lesions was seen in 100% of patients. There was a signiifcant difference between groups A and B regarding complete resolution of brain stem and putaminal lesions （P= 0.005 andP=0.024, respectively）. If the correct diagnosis and adequate treatment are not established less than 24 months after onset of the symptoms, irreversible lesions in the brain parenchyma could be ex-pected. Signal abnormalities on magnetic resonance imaging might therefore, at least in the early stages, represent reversible myelinolisis or cytotoxic edema associated with copper toxicity.     

Multiple sclerosis with consciousness disturbance: a case report

We report here a nine-year-old girl with multiple sclerosis having consciousness disturbance at admission. Neurological examination revealed drowsiness, unstable emotion, decreased visual acuity, disturbance of convergence, and clumsy coordination movements. Her cerebrospinal fluid IgG and myelin basic protein were increased. Electroencephalogram showed intermittent, high voltage slow waves predominant in the frontal lobes. Magnetic resonance imaging (MRI) found multiple demyelinating plaques in the brainstem, thalamus, periventricular white matter. The brainstem reticular formation was involved. Since she had had bilateral acute optic neuritis and papillitis two years before the admission, the diagnosis of multiple sclerosis was made. Methylprednisolone pulse therapy improved her neurological symptoms and MRI findings. Multiple sclerosis in children, unlike that in adults, may present with symptoms mimicking an encephalopathy. Our case suggested that consciousness disturbance in childhood multiple sclerosis results from lesions in the brainstem activating reticular formation including the thalamus.     

Optochiasmal arachnoiditis following cotton wrapping of anterior communicating artery aneurysm treated by surgical removal of granuloma.

We report the case of a 50 year old female who presented with visual disturbance due to optochiasmal arachnoiditis and foreign body granuloma 9 months after cotton wrapping for ruptured anterior communicating artery (AcomA) aneurysm. Magnetic resonance imaging (MRI) revealed enhanced mass lesion around AcomA complex and hyperintense signal on optic chiasm and right optic tract by fluid-attenuated inversion recovery image. Despite the repeated steroid pulse therapy, she deteriorated and MRI showed expansion of the granulomatous lesion over 5 months. Surgical removal of foreign body granuloma resulted in marked improvement of visual disturbance as well as of the MRI findings. We conclude that the use of cotton sheet close to the optic nerve should be avoided, and that surgical removal of the granuloma would be the optimal choice especially for the patient in whom steroid therapy fails to improve clinical symptoms.     

Deterioration on magnetic resonance imaging despite good clinical recovery after viral encephalitis

We present a 2-year-old patient who showed progressive widespread white-matter abnormalities on magnetic resonance imaging 1 month after viral encephalitis, despite her good clinical recovery. These lesions on magnetic resonance imaging had not responded to therapies commonly used to treat secondary immune-mediated demyelinating lesions, as observed in acute disseminated encephalomyelitis. Acute viral encephalitis caused cortical blindness, but no other clinical signs developed during her clinical course. The progressive white-matter lesion in our case was different from the exacerbation of viral encephalitis or secondary immune-mediated encephalitis. A year later, magnetic resonance imaging-demonstrated brain atrophy with cystic changes in the bilateral occipital area, with remarkably reduced white-matter lesions. We hypothesize that the progressive white-matter changes resulted from the process of atrophy, degeneration, and cystic formation after viral encephalitis, rather than from the immune-mediated demyelinating process.     

An autopsied case of Sjogren's syndrome with massive necrotic and demyelinating lesions of the cerebellar white matter

A 69-year-old woman developed subacute cerebellar ataxia and tremors in all four limbs in April 1996. Laboratory examination showed elevated antibodies against Ro and La. Head magnetic resonance imaging showed T(2) high-intensity lesions in the cerebellar white matter bilaterally and later in the pons. In April 2000, she died of multiple organ failure with incidental colon cancer. The autopsy showed atrophic parotid glands with an accumulation of lymphocytes around the ducts, confirming the diagnosis of Sjogren's syndrome histopathologically. The neuropathological examination revealed severe necrotic lesions in the cerebellar white matter bilaterally with several foci of perivenous demyelination in the periphery of the lesions and similar demyelinated areas in the pons. Immunohistochemistry with anti-JC virus antibody demonstrated no positive inclusions. A single focus of granulomatous arteritis was observed in one subarachnoid artery. The combination of Sjogren's syndrome, granulomatous angitis, and foci of perivenous demyelination suggests that an autoimmune mechanism played an important role in causing the necrotic lesions in the cerebellar white matter in this case.     

Adult post-infectious thalamic encephalitis: acute onset and benign course

We report on two young patients with an encephalitic syndrome and bilateral thalamic lesions following a presumably viral or mycoplasma respiratory tract infection with the main clinical symptoms of organic psychosis in the first and a prolonged amnestic syndrome and ataxia in the second case. Four months later the patients had recovered clinically and the thalamic lesions had resolved on magnetic resonance imaging in one case and almost completely in the other. We interpret the patients' illness as rare cases of a post-infectious acute thalamic encephalitis in adults. The cases and their relationship to possible post-infectious autoimmune inflammatory or toxic pathophysiological mechanisms are discussed and a review of the literature is provided.     

A case of childhood stiff-person syndrome with striatal lesions: A possible entity distinct from the classical adult form

Parainfectious or autoimmune striatal lesions have been repeatedly described in children. We report a 7-year-old girl with painful muscle spasms, leading to the diagnosis of childhood stiff-person syndrome (SPS). Striatal lesions were demonstrated by diffusion-weighted magnetic resonance imaging (MRI) and single-photon emission computed tomography but not by conventional MRI. Autoantibodies against glutamic acid decarboxylase (GAD) were absent. Steroid pulse therapy and high-dose intravenous immunoglobulin resolved all the symptoms with slight sequelae. Childhood SPS may be characterized by absent anti-GAD antibodies and a transient benign clinical course, and it may have a pathomechanism distinct from that in adult SPS.     

Clinical course of spontaneous spinal epidural haematoma mimicking Guillain-Barré syndrome in a child: a case report and literature review

We describe a 9-year-old female with thoracic epidural haematoma. The clinical course simulated Guillain-Barré syndrome (GBS) so intravenous immunoglobulin therapy was started at the paediatric clinic. Magnetic resonance imaging (MRI) 3 days after admission showed thoracic epidural haematoma between T2 and T8. An emergency laminectomy was performed and the patient's neurological symptoms began to improve immediately after surgery and she made a full recovery during the 2 weeks of follow-up. Time is a very important factor in achieving reversibility of symptoms of compressive cord lesions, such as spinal epidural haematoma, and MRI is mandatory for patients with progressive paraplegia, even though the signs and symptoms might suggest GBS.