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1.
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.  相似文献   

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Multiple sclerosis (MS) is a well-known disease characterized by the distribution of plaques in the periventricular and subcortical white matter. Although plaques can also be found in the striatum, pallidum and thalamus, extrapyramidal symptoms are very rare in MS. However, the association of MS and parkinsonism is still a controversial topic as it has not been established whether these two conditions occur coincidentally or causally. In the literature, eleven cases of parkinsonism associated with MS have been described. Here, we report a patient with clinically definite MS and signs of parkinsonism. Our patient had slow progressive bradykinesia, static tremor and bradymimia that were not associated with exacerbation or progression of the MS. This rare and interesting association of multiple sclerosis with parkinsonism is discussed in the light of literature reports.  相似文献   

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Multiple sclerosis (MS) is an autoimmune disease that targets the myelin of the brain, spinal cord, and optic nerves. The inciting trigger that activates this immune response is unknown. Primary central nervous system (CNS) lymphoma is usually a diffuse large B-cell non-Hodgkin's lymphoma that originates in the brain, spinal cord, leptomeninges, or eyes. We report a 33-year-old patient who was diagnosed to have multiple sclerosis initially and a CNS lymphoma was noted 38 months later. Primary CNS lymphoma is a potential complication of chronic immunosuppression. Due to the uses of steroid, immunosuppresants and beta-interferon for multiple sclerosis, our patient developed an immuno-compromised state. These agents might contribute to the occurrence of a primary CNS lymphoma. On the other hand, a demyelinating disease may have preceded the diagnosis of primary CNS lymphoma. A possibility of neoplastic transformation in CNS inflammatory diseases such as multiple sclerosis may occur. The association of coexistent primary CNS lymphoma and multiple sclerosis may be more than coincidental.  相似文献   

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IntroductionFew cases of patients with both Familial Mediterranean Fever (FMF) and Multiple Sclerosis (MS) have been reported, mainly from Turkey. Central nervous system manifestations are rare in FMF.Case reportWe report the case of a 37-year-old right-handed man with FMF diagnosed at 17 the age of years and successfully treated with colchicine. The patient was born in Algeria and lived in France since he was four years old. He had a brother who had multiple sclerosis. When the patient was 23 years old, he experienced diplopia and leg numbness that resolved spontaneously without treatment. Ten years later, new neurological events appeared every six months and were treated with corticoid-steroids. The diagnosis of MS was made. In 2006, he was hospitalized for new explorations in order to search for neuro-Behçet's disease, because of the development of a canker sore. There was no argument for neuro-Behçet's disease.DiscussionNeurological complications of FMF are rare. It is important to rule out a neuro-Behçet disease in a FMF patient with neurological disorders. Previous studies and case reports on the association between FMF and MS have failed to draw a clear conclusion as to whether this is a true association or a simple coincidence. In our patient's clinical situation, we found no argument for changing the treatment of MS and FMF.  相似文献   

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Abstract A patient with atypical multiple sclerosis (MS) with clear concentric structure was studied using high field magnetic resonance imaging (MRI). This case was considered to be Balo's concentric sclerosis. Magnetic resonance imaging showed diffuse multiple concentric demyelinating lesions in the bilateral centrum semiovales, which finally regressed with the necrotic lesions remaining when the patient was discharged. During his clinical course, he showed some higher cerebral dysfunctions, such as memory disturbance, constructual apraxia and acalculia. He was treated with glycerin, prednisone and rehabilitation; all of which were effective in his recovery. Over a 4 month period, the patient recoveredclinically, but some intellectual impairment remained.  相似文献   

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患者,女性,27岁,因头痛头晕并行走不稳2月入院。查体:神志清,双眼视乳头水肿,左手指鼻试验及轮替动作差,Romberg征阳性,同时双下肢感觉减退。CT示左侧小脑半球一4cm×4cm囊性占位,MRI示小脑囊性占位,强化后发现囊内偏外侧一5mm×5mm结节;同时发现C5~T12脊髓空洞,T10平面脊髓空洞内一瘤结节。于2003年12月3日行小脑肿瘤切除术,术中切除小脑瘤结节后,探查发现延髓背侧表面一瘤结节约3mm大小,呈黄红色桑椹样,一并切出。病理检查证实均为血管网织细胞瘤。两周后再次手术切除脊髓内瘤结节,病理证实为血管网织细胞瘤。术后病人症状消失。追问…  相似文献   

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Multiple sclerosis co-existent with myxedema. An autopsy case report   总被引:1,自引:0,他引:1  
A 64-year-old woman developed impaired consciousness and vision, sensorimotor paresis, hypothermia, bradycardia, and edema. Symptoms fluctuated with seasonal exacerbations in winter and terminated in coma with respiratory insufficiency at age 69. High CSF protein content and low serum T4 and TSH levels were noted. Treatment with prednisolone and thyroxin considerably improved her consciousness and edema. The patient suddenly expired of pulmonary embolism. Postmortem examination revealed a marked atrophy of pituitary and thyroid glands, while multiple demyelinating plaques were disclosed in the optic tract and cervical cord. A review of the literature indicates that this is the first report of the co-existence of two such disorders.  相似文献   

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A 2 year old boy was admitted owing to a subacute episode of ataxic gait and hearing deficit. Computerized tomography (CT) was normal and cerebrospinal fluid (CSF) analysis revealed gamma globulins level of 15.4% (normal 7 to 14%). There was spontaneous remission after 7 months. At 5 years of age the boy incurred a second episode with predominantly right appendicular ataxia and tonic gaze deviation to the right side. CT showed a low-density lesion in the white matter adjacent to the right frontal horn. Visual and auditory evoked potentials were abnormal. CSF revealed a mild increase in gamma globulins level of 14.5% with an abnormal T lymphocyte subsets study. The combination of visual, cerebellar, brain stem and paraventricular lesions with clear remissions and exacerbations, supported by CT, CSF and evoked potentials findings suggests the diagnosis of multiple sclerosis even at this early age.  相似文献   

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Neurological Sciences - An increasing number of cases of comorbid hereditary spastic paraplegia (HSP) and multiple sclerosis (MS) have been described. We report a patient with the SPG3A form of HSP...  相似文献   

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We report the coexistence of multiple sclerosis (MS) and an intradural extramedullary spinal cord tumour in a 46-year-old woman with a 2-year history of MS. The patient presented with right hemitrunk and lower extremity paraesthesias, urinary incontinence, and intermittent lower right back and abdominal pain, which did not respond to pulse steroid therapy. A spinal magnetic resonance imaging (MRI) study revealed an intradural extramedullary spinal cord tumour in the lower thoracic spine, later diagnosed as schwannoma. We call attention to this rare association of MS and a spinal cord tumour, and emphasize the need for scrutiny of new and uncommon symptoms during the follow-up of MS patients. Received: 2 April 2002 / Accepted in revised form: 28 May 2002 Correspondence to V. Etus  相似文献   

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An autopsied case of multiple cerebral tuberculomas with 37 lesions, the one with the greatest number of lesions described in the literature, is reported. The patient was a chronic alcoholic and had generalized tuberculosis. The literature on multiple cerebral tuberculomas is reviewed and the neurological clinical picture presented by the patient and the association between generalized tuberculosis and chronic alcoholism are commented upon.  相似文献   

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K Takeda 《Clinical neurology》2000,40(12):1206-1210
The mysterious maneuvers of the neuropsychological examination actually conceal the very intellectual process by which diagnosis is obtained. Five features that are usually sufficient to diagnose aphasia are 1) spontaneous speech, 2) speech comprehension, 3) repetition, 4) reading aloud with comprehension, and 5) writing. In spontaneous speech, fluent aphasics have normal rhythm, melody, and well-articulated sentence, but the speech is filled with paraphasias. The speech of non-fluent aphasics is slow, labored, and poorly articulated. When patients with spatial neglect are asked to perform a variety of tasks in space, they neglect the hemispace contralateral to their lesion. For example, when asked to draw a picture of a flower, they may draw only a half of the flower. The patients may fail to cross out lines distributed over a page. Amnesic patients, despite normal scores on psychometric tests of intelligence and normal performances on immediate memory, show significant impairments in new learning (anterograde amnesia) and remote memory (retrograde amnesia). In this article, The author also explained about two topics briefly of language functions (tactile reading) and hemispatial neglect (reference frames).  相似文献   

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多发性硬化合并葡萄膜炎二例报告   总被引:1,自引:0,他引:1  
目的 探讨合并葡萄膜炎的多发性硬化(MS)的临床及可能的病理机制。方法 对2例合并葡萄膜炎的MS进行临床、电生理、头颅MRI和寡克隆带检查及结合文献分析可能的病理机制。结果 例1男性,56岁,主要表现为肢体麻木1个月,突发双眼视力下降20d。视觉诱导电位示双眼P100潜伏期延长,头颅MRI示双额顶白质内T2WI多发性高信号,寡克隆带阳性。例2女性,35岁,主要表现为反复发作的双眼视力下降伴双下肢麻木无力12个月,视觉诱发电位P100及脑干听觉诱导电位I~V潜伏期延长,头颅MRI示额顶叶白人多发性圆形T2WI高信号,寡克隆带阳性。结合文献复习合并葡萄膜炎的MS临床表面大多轻微,病因不明,可能不是自身抗原髓鞘碱性蛋白,而是星形胶质细胞来源的S100β引起的。结论 合并葡萄膜炎的MS临床表现大多轻微,其发病机制不详,  相似文献   

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We report a child in whom multiple cardiac rhabdomyomas were identified on routine fetal ultrasonography. Molecular genetic studies identified the TSC2 gene missense mutation (E36; 4672 G>A, 1558 E>K TSC2). Both general and neurodevelopment of the patient have been normal. When last examined at age 6 years, he had no skin manifestations of tuberous sclerosis complex. Computed tomography of the brain revealed two periventricular calcifications consistent with the molecular diagnosis. This is the first report of molecularly confirmed tuberous sclerosis complex in a child with multiple cardiac rhabdomyomas and no other clinical manifestations of the disease. We propose that all cases of multiple cardiac rhabdomyomas in an infant be given a tentative diagnosis of tuberous sclerosis complex.  相似文献   

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We report the association a multiple sclerosis (MS), spinal cord tumour and intracranial tumor in a 63 years-old female patient with a 10 years history of relapsing/remitting MS. Symptoms usually remitted in response to costicosteroid therapy. In 1997 the patient presented with paraparesis and paresis of right arm which did not respond to corticotherapy. A spinal RMI revealed in the cervical spinal an intra spinal cord tumour, further diagnosed as ependymoma, and a parietal region meningioma. We call attention to this rare association of central nervous system tumour and MS, enphasizing the need for investigation of new and uncommon symptoms during the evolution of MS.  相似文献   

19.
The authors report a case of a newborn with severe encephalopathy and cerebral calcifications. The newborn was admitted to Neonatal Intensive Care Unit in Chieti at 2 days of age suffering from continuous generalized seizures. He was the child of healthy first-degree cousins. Cerebral ultrasonography showed bilateral ventricular dilatation and an intrathalamic hyperechoic image. Computed tomography and magnetic resonance imaging showed ventricular and corpus callosus hypoplasia, pachygyria, widespread delayed myelination areas, and basal nuclei and periventricular calcifications. All serum and urine analyses showed normal results. In particular, all demyelinizing metabolic diseases were excluded. Based upon these findings, we speculate that this infant may be suffering from fetal encephalopathy with cerebral calcifications.  相似文献   

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A 38 year-old male presented with an acute onset of left hemiplegia. Brain magnetic resonance imaging (MRI) revealed a bright lesion by diffusion-weighted imaging with low apparent diffusion coefficient value in the right subcortical region, a finding compatible with an acute cerebral infarct. An old infarct was also noted in the same imaging. Both enzyme-linked immunosorbent assay and Western blot method were positive for human immunodeficiency virus infection. The white blood cell count was 2930 cells / mm3, and the subpopulation study for lymphocyte revealed a decreased cluster of differentiation 4+ count of 149 cells/mm3. Studies for prothrombotic states showed decreased protein S and increased anticardiolipin antibodies. We concluded that this was a case of acquired immunodeficiency syndrome (AIDS) with acute and old cerebral infarcts. This patient might be the first reported case in Taiwan. AIDS might be related with stroke in young patients, a condition probably under-recognized in Taiwan.  相似文献   

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