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1.
Mov Disord ; 29(2): 238-44, 2014 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-24259271

RESUMO

The aim of this study was to investigate corticobasal syndrome with respect to underlying pathologies, the ability of current clinical criteria to detect early stages of disease, and symptoms and signs predicting background pathologies. We retrospectively analyzed the clinicopathological findings from patients with corticobasal syndrome. We also analyzed whether those findings fulfilled the diagnostic criteria for corticobasal degeneration (CBD). Finally, we investigated characteristic clinical features that are specific to each background pathology. Of 10 consecutive autopsied patients who had corticobasal syndrome (mean age ± standard deviation, 67.9 ± 9.3 years; male:female ratio, 6:4), three had corticobasal degeneration pathology, three had progressive supranuclear palsy, three had Alzheimer's disease, and one had atypical four-repeat tauopathy. Nine patients fulfilled Mayo criteria, and all 10 patients fulfilled modified Cambridge criteria at the later stage, but only two patients fulfilled either clinical criteria within 2 years of disease onset. Five patients fulfilled the clinical criteria for possible CBD (p-CBD), and one patient fulfilled the clinical research criteria for probable sporadic CBD (cr-CBD) at the later stage. Only two patients fulfilled the criteria for either p-CBD or cr-CBD within 2 years of disease onset. Although we could not find any predictive characteristic clinical features that were specific to CBD pathology, only patients with progressive supranuclear palsy developed apraxia of eyelid opening and cerebellar ataxia. Myoclonus and memory impairment, especially if they appear at an early stage of the disease, may predict Alzheimer's disease pathology. Sensitivity of the available clinical criteria for corticobasal syndrome was poor within 2 years of disease onset.


Assuntos
Gânglios da Base/patologia , Encefalopatias/complicações , Encefalopatias/diagnóstico , Córtex Cerebral/patologia , Idoso , Doença de Alzheimer/etiologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Paralisia Supranuclear Progressiva/etiologia , Tauopatias/etiologia
2.
Rinsho Shinkeigaku ; 2024 Aug 24.
Artigo em Japonês | MEDLINE | ID: mdl-39183048

RESUMO

A 76-year-old woman with a 2-year history of Parkinson's disease presented with dropped head, which had developed rapidly after she had been prescribed donepezil hydrochloride (DNP) at 3 |mg/day. After one month of medication, the extent of the head drop reached 90°. Examination revealed hypertrophy of the left sternocleidomastoid muscle, but no weakness of the extensor muscles in the cervical region. Surface electromyography demonstrated co-|contraction of the sternocleidomastoid and splenius capitus muscles during head flexion and extension. DNP was withdrawn, resulting in immediate amelioration of the head drop, and complete resolution was achieved after two months. Although head drop is often seen in patients with Parkinson's disease, few previous reports have documented DNP as a causative factor. If patients with Parkinson's disease develop head drop, it is important to investigate any history of DNP medication.

3.
Rinsho Shinkeigaku ; 64(6): 417-421, 2024 Jun 27.
Artigo em Japonês | MEDLINE | ID: mdl-38797685

RESUMO

A 23-year-old man was admitted to our hospital with a one-year history of muscle weakness and atrophy. He had noticed contractures of the fingers of both hands from the age of 18. Examination revealed a skin rash including heliotrope rash and Gottron's sign, joint contractures in the extremities, dysphagia, extensive muscle weakness and marked muscle atrophy. The serum creatine kinase level was 272 |IU/l and muscle biopsy showed typical perifascicular atrophy but little lymphocyte invasion. There was no interstitial pneumonia or malignancy, but muscle tendons showed elevated CT values suggesting calcification or fibrosis. Anti-nuclear matrix protein 2 (NXP-2) antibody-positive dermatomyositis was diagnosed on the basis of the serum antibody level. Methylprednisolone pulse therapy ameliorated the skin rash and bulbar palsy, but muscle weakness, atrophy and joint contractures were resistant to the treatment. There have been no previous reports of young adults with anti-NXP-2 antibody-positive dermatomyositis in whom joint contracture became evident as early as 4 years beforehand, which is a important feature for differential diagnosis of dermatomyositis.


Assuntos
Autoanticorpos , Biomarcadores , Contratura , Dermatomiosite , Pulsoterapia , Humanos , Masculino , Adulto Jovem , Adenosina Trifosfatases , Autoanticorpos/sangue , Biomarcadores/sangue , Contratura/etiologia , Contratura/diagnóstico , Dermatomiosite/complicações , Dermatomiosite/imunologia , Dermatomiosite/diagnóstico , Dermatomiosite/tratamento farmacológico , Diagnóstico Diferencial , Proteínas de Ligação a DNA , Metilprednisolona/administração & dosagem , Proteínas Nucleares/imunologia , Proteínas de Ligação a RNA/imunologia , Fatores de Transcrição
4.
Rinsho Shinkeigaku ; 64(6): 413-416, 2024 Jun 27.
Artigo em Japonês | MEDLINE | ID: mdl-38797686

RESUMO

A 54-year-old man with a university degree was admitted to our hospital because of a two-year history of progressive dementia. He had familial sensorineural hearing loss and had been treated for epilepsy since his 30s. On admission, he showed severe dementia and parkinsonism without fever or skin rash. Systemic inflammation was evident, and the CSF cell count and IL-6 level were elevated to 53/µl and 307 |pg/ml, respectively. Brain MRI demonstrated diffuse brain atrophy. More detailed anamnesis revealed a history of rheumatoid arthritis in childhood and aseptic meningitis in his 20s. Genetic examination for autoinflammatory diseases demonstrated compound heterozygotic mutations in the NLRP3 gene, causing cryopyrin-associated periodic fever syndrome (CAPS). This case was atypical CAPS presenting as early-onset progressive dementia, without recurrent fever or urticaria-like eruption which are usually seen in this disease.


Assuntos
Síndromes Periódicas Associadas à Criopirina , Demência , Mutação , Proteína 3 que Contém Domínio de Pirina da Família NLR , Humanos , Síndromes Periódicas Associadas à Criopirina/diagnóstico , Síndromes Periódicas Associadas à Criopirina/complicações , Masculino , Proteína 3 que Contém Domínio de Pirina da Família NLR/genética , Pessoa de Meia-Idade , Demência/etiologia , Demência/diagnóstico , Exantema/etiologia , Febre/etiologia , Imageamento por Ressonância Magnética , Encéfalo/diagnóstico por imagem , Encéfalo/patologia , Diagnóstico Diferencial , Heterozigoto , Biomarcadores/líquido cefalorraquidiano , Biomarcadores/sangue , Progressão da Doença
5.
Neurobiol Dis ; 46(2): 336-50, 2012 May.
Artigo em Inglês | MEDLINE | ID: mdl-22342974

RESUMO

Dentatorubral-pallidoluysian atrophy (DRPLA) is an autosomal dominant progressive neurodegenerative disorder with intellectual deterioration and various motor deficits including ataxia, choreoathetosis, and myoclonus, caused by an abnormal expansion of CAG repeats in the DRPLA gene. Longer expanded CAG repeats contribute to an earlier age of onset, faster progression, and more severe neurological symptoms in DRPLA patients. In this study, we have established DRPLA transgenic mouse lines (sublines) harboring a single copy of the full-length mutant human DRPLA gene carrying various lengths of expanded CAG repeats (Q76, Q96, Q113, and Q129), which have clearly shown motor deficits and memory disturbance whose severity increases with the length of expanded CAG repeats and age, and successfully replicated the CAG repeat length- and age-dependent features of DRPLA patients. Neuronal intranuclear accumulation of the mutant DRPLA protein has been suggested to cause transcriptional dysregulation, leading to alteration in gene expression and neuronal dysfunction. In this study, we have conducted a comprehensive analysis of gene expression profiles in the cerebrum and cerebellum of transgenic mouse lines at 4, 8, and 12 weeks using multiple microarray platforms, and demonstrated that both the number and expression levels of the altered genes are highly dependent on CAG repeat length and age in both brain regions. Specific groups of genes and their function categories were identified by further agglomerative cluster analysis and gene functional annotation analysis. Calcium signaling and neuropeptide signaling, among others, were implicated in the pathophysiology of DRPLA. Our study provides unprecedented CAG-repeat-length-dependent mouse models of DRPLA, which are highly valuable not only for elucidating the CAG-repeat-length-dependent pathophysiology of DRPLA but also for developing therapeutic strategies for DRPLA.


Assuntos
Atividade Motora/genética , Mutação/genética , Proteínas do Tecido Nervoso/genética , Transcriptoma , Expansão das Repetições de Trinucleotídeos/genética , Fatores Etários , Animais , Humanos , Aprendizagem em Labirinto/fisiologia , Camundongos , Camundongos Endogâmicos C57BL , Camundongos Transgênicos , Ligação Proteica/fisiologia
6.
Rinsho Shinkeigaku ; 62(5): 386-390, 2022 May 31.
Artigo em Japonês | MEDLINE | ID: mdl-35474288

RESUMO

A 61-year-old man who had suffered two episodes of generalized convulsion in a two-year period was admitted to our hospital because of progressive gait disturbance during the previous five months. Neurological examination revealed cognitive impairment, spasticity of the lower limbs, truncal ataxia, and dysautonomia including orthostatic hypotension, dysuria and hypohydrosis. Brain fluid-attenuated inversion recovery (FLAIR) MRI detected high-signal-intensity lesions in the periventricular white matter and centrum semiovale, with punctate gadolinium (Gd) enhancement. Spinal MRI detected swollen cervical long cord lesions extending from C2 to C6. Although methylprednisolone pulse treatment initially ameliorated the symptoms and MRI abnormal findings, clinical symptoms and MRI abnormalities including new cervical lateral column lesions reminiscent of those in autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy (GFAP-A) relapsed two months later. At this point, anti-GFAPα antibody was detected in the cerebrospinal fluid. Although the clinical course of GFAP-A has been well reported, the present case showed a chronic refractory course.


Assuntos
Medula Cervical , Astrócitos , Gadolínio , Proteína Glial Fibrilar Ácida , Humanos , Masculino , Pessoa de Meia-Idade , Medula Espinal/diagnóstico por imagem
7.
Rinsho Shinkeigaku ; 62(9): 736-739, 2022 Sep 28.
Artigo em Japonês | MEDLINE | ID: mdl-36031381

RESUMO

A 65-year-old man was admitted to our hospital with a 6-year history of painful muscle stiffness in his trunk and lower limbs, preventing him from walking. Stiff-person syndrome (SPS) was diagnosed because the patient had symptoms of painful muscle spasms elicited by tactile stimulation without joint contracture. Although SPS- related autoantibodies in the serum, including anti-glycine R, anti-amphiphysin, anti-glutamic acid decarboxylase (GAD), anti-dipeptidyl peptidase-like protein (DPPX) and anti-γ-aminobutyric acid-A (GABAA) R, were negative, the ACTH and cortisol levels were low. On the basis of additional loading tests for anterior pituitary function and ACTH, isolated ACTH deficiency (IAD) was diagnosed. Hormonal replacement therapy with hydrocortisone at 15 mg/day ameliorated the condition quickly, and the patient became asymptomatic after three months. Flexion contractures have been reported as musculoskeletal symptoms of IAD, but are not usually evident in patients with SPS. The present case illustrates that the painful muscle spasms elicited by tactile stimulation without joint contracture characteristic of SPS can also be symptoms of IAD.


Assuntos
Contratura , Rigidez Muscular Espasmódica , Hormônio Adrenocorticotrópico/deficiência , Idoso , Aminobutiratos , Autoanticorpos , Doenças do Sistema Endócrino , Doenças Genéticas Inatas , Glutamato Descarboxilase , Humanos , Hidrocortisona , Hipoglicemia , Masculino , Peptídeo Hidrolases , Espasmo/diagnóstico , Espasmo/tratamento farmacológico , Espasmo/etiologia , Rigidez Muscular Espasmódica/diagnóstico , Rigidez Muscular Espasmódica/tratamento farmacológico , Ácido gama-Aminobutírico
8.
Rinsho Shinkeigaku ; 51(10): 770-3, 2011 Oct.
Artigo em Japonês | MEDLINE | ID: mdl-22019870

RESUMO

A 40 year-old man with migraine presented cerebral ischemic attacks several times in one year. He had no risk factors for cerebrovascular disease including hypertension, but had strong family history suggesting autosomal dominant inheritance. A brain MRI on T(2) weighted and FLAIR images revealed patchy and confluent hyper intensity areas in the subcortical white matters and bilateral external capsules, while no anterior temporal pole lesions characteristic of CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy) were detected. His skin biopsy demonstrated granular osmiophilic materials (GOM) on the basement membrane of the vascular smooth muscle cells in dermis as shown by an electron microscope. The following mutational analysis of the Notch3 gene disclosed a missense mutation of p.Arg133Cys in exon 3. Molecular diagnosis of CADASIL may be time consuming because Notch3 is a huge gene and mutations may occur at multiple sites. GOM on skin biopsy is diagnostic especially in cases where anterior temporal pole involvement on MRI is negative.


Assuntos
CADASIL/diagnóstico , Pele/patologia , Adulto , Biópsia , CADASIL/patologia , Análise Mutacional de DNA , Humanos , Imageamento por Ressonância Magnética , Masculino
9.
Rinsho Shinkeigaku ; 51(5): 330-3, 2011 May.
Artigo em Japonês | MEDLINE | ID: mdl-21706829

RESUMO

A 49-year old woman noticed her skin rash several days after taking supplements containing Spirulina, a planktonic blue-green alga. Her skin rash was spreading over large parts of her body, even after stop ingestion two months later. Five months later, she developed muscle weakness of neck flexor and left proximal upper extremity. On admission, creatine kinase (CK) was elevated to 1,268 IU/ml in the serum. A muscle specimen revealed many necrotizing muscle fibers and the infiltration of mononuclear cells in the peri- and endomysium including a lot of eosinophils. Immunohistochemical staining showed the infiltration of CD4 positive cells in the peri- and endomysium and that of CD20 positive B cells in the perivascular regions. She was diagnosed as having inflammatory myopathy with widely skin rash. Therapy with administration of prednisolone and cyclophosphamide followed by methyl-prednisolone pulse improved her clinical symptoms. There is a similar report describing a case of dermatomyositis after ingestion of Spirulina, which is known to have immune-stimulating property such as accelerating tumor necrosis factor (TNF)-alpha production. Also, TNF-alpha single nucleotide polymorphisms (TNF-308A) was demonstrated to have strong association with onset of myositis in Caucasians. The use of Spirulina could result in inflammatory myopathy under some specific conditions.


Assuntos
Suplementos Nutricionais/efeitos adversos , Exantema/etiologia , Miosite/etiologia , Spirulina , Feminino , Humanos , Pessoa de Meia-Idade
10.
Rinsho Shinkeigaku ; 61(9): 635-639, 2021 Sep 28.
Artigo em Japonês | MEDLINE | ID: mdl-34433747

RESUMO

A 62-year-old woman suffering from pulmonary Mycobacterium avium complex (MAC) disease was admitted to our hospital with fever, visual impairment, and lower limb weakness. MRI detected lesions in the optic chiasm and spinal cord extending the length of 6 vertebrae. The anti-aquaporin 4 (AQP4) antibody titer determined by ELISA was elevated to 8.3 IU/l. On the basis of these findings, the patient was diagnosed as having neuromyelitis optica (NMO), when chest CT also demonstrated exacerbation of pulmonary lesions. Methylprednisolone pulse therapy and double-filtered plasma exchange ameliorated the symptoms, and the EDSS score improved from 8.5 to 6.5. Six months later, visual impairment recurred, although ELISA showed that the anti-AQP4 antibody titer had become undetectable. Also, the CSF interleukin-6 (IL-6) level was elevated to 34.8 pg/ml. There have been few reports of NMO associated with pulmonary MAC disease. An increase of IL-6 is considered to exacerbate the clinical picture of NMO, whereas it may suppress progression of the pulmonary MAC disease. Exacerbation of the pulmonary MAC disease and the following internal counteraction with IL-6 may have resulted in a NMO relapse. The present patient was therefore administered eculizumab but not satralizumab, a humanized anti-IL-6 receptor antibody, for prevention of NMO recurrence.


Assuntos
Infecção por Mycobacterium avium-intracellulare , Neuromielite Óptica , Feminino , Humanos , Interleucina-6 , Pessoa de Meia-Idade , Complexo Mycobacterium avium , Infecção por Mycobacterium avium-intracellulare/complicações , Infecção por Mycobacterium avium-intracellulare/diagnóstico , Infecção por Mycobacterium avium-intracellulare/tratamento farmacológico , Transtornos da Visão
11.
Rinsho Shinkeigaku ; 61(7): 477-481, 2021 Jul 30.
Artigo em Japonês | MEDLINE | ID: mdl-34148937

RESUMO

A 75-year-old man was found lying prostrate in a hot room in the middle of summer. On admission, he had high fever, dehydration, and multiple decubitus, in addition to right hemiparesis and total aphasia. Brain CT showed subacute ischemic stroke in the territory of left middle cerebral artery. Brain MRI diffusion-weighted imaging (DWI) 4 days after admission detected high signal intensity lesions in the left pyramidal tract from the midbrain cerebral peduncle to the lower pons, indicating early Wallerian degeneration. The lesions were found to extend to the contralateral pyramidal decussation by MRI DWI day 12, but they had disappeared on day 28. On the other hand, brain MRI FLAIR images detected the lesions clearly day 44. Also, diffusion tensor tractography detects fewer left cerebral pyramidal tracts. No previous reports have documented the time course of such long Wallerian degeneration. This case suggests that dehydration may promote the onset of early and long Wallerian degeneration.


Assuntos
AVC Isquêmico , Degeneração Walleriana , Idoso , Isquemia Encefálica , Desidratação/patologia , Humanos , AVC Isquêmico/complicações , AVC Isquêmico/diagnóstico por imagem , AVC Isquêmico/patologia , Masculino , Tratos Piramidais/patologia , Degeneração Walleriana/diagnóstico por imagem , Degeneração Walleriana/etiologia , Degeneração Walleriana/patologia
12.
Rinsho Shinkeigaku ; 60(12): 869-873, 2020 Dec 26.
Artigo em Japonês | MEDLINE | ID: mdl-33229837

RESUMO

An 84-year-old man was admitted to our hospital with new-onset refractory status epilepticus of unclear etiology. On the third day, diffusion-weighted brain MRI demonstrated lesions in the right medial temporal and parietal lobes. As a CSF sample showed pleocytosis, paraneoplastic limbic encephalitis (PLE) associated with small cell lung cancer (SCLC) was suspected. The patient was also positive for anti-gamma aminobutyric acid (GABA)B receptor antibody in the CSF, which has recently been reported in elderly patients with SCLC-related PLE. Methylprednisolone pulse therapy ameliorated the symptoms. It is noteworthy that immune therapy often improves the symptoms of PLE with anti-GABAB receptor antibody, even though radical therapy for the lung cancer may be difficult.


Assuntos
Autoanticorpos/líquido cefalorraquidiano , Encefalite Límbica/complicações , Encefalite Límbica/imunologia , Neoplasias Pulmonares/complicações , Receptores de GABA-B/imunologia , Carcinoma de Pequenas Células do Pulmão/complicações , Estado Epiléptico/etiologia , Idoso de 80 Anos ou mais , Encéfalo/diagnóstico por imagem , Imagem de Difusão por Ressonância Magnética , Humanos , Imunoterapia/métodos , Encefalite Límbica/tratamento farmacológico , Masculino , Metilprednisolona/administração & dosagem , Pulsoterapia , Estado Epiléptico/diagnóstico por imagem , Estado Epiléptico/tratamento farmacológico
13.
Rinsho Shinkeigaku ; 60(8): 565-568, 2020 Aug 07.
Artigo em Japonês | MEDLINE | ID: mdl-32641630

RESUMO

A 49-year-old woman was admitted to our hospital with suspected hypertensive encephalopathy. On the basis of MRI showing leptomeningeal enhancement and Class V cytology of the CSF, she was diagnosed as having leptomeningeal carcinomatosis. Although no primary site was detected, a few melanin granules were observed at the third CSF examination. The atypical cells in the CSF demonstrated immunoreactivity for HMB-45 and S-100, which are specific markers of malignant melanoma. There have been few reports of meningeal melanomatosis in Japan. This case illustrates that immunostaining is diagnostically useful in patients with leptomeningeal carcinomatosis from neoplasms with unknown primary sites.


Assuntos
Biomarcadores Tumorais/líquido cefalorraquidiano , Líquido Cefalorraquidiano , Citodiagnóstico/métodos , Melanoma/líquido cefalorraquidiano , Melanoma/diagnóstico , Carcinomatose Meníngea/líquido cefalorraquidiano , Carcinomatose Meníngea/diagnóstico , Neoplasias Meníngeas/líquido cefalorraquidiano , Neoplasias Meníngeas/diagnóstico , Coloração e Rotulagem/métodos , Idoso , Feminino , Humanos , Antígenos Específicos de Melanoma/líquido cefalorraquidiano , Neoplasias Primárias Desconhecidas , Proteínas S100/líquido cefalorraquidiano , Antígeno gp100 de Melanoma
14.
Rinsho Shinkeigaku ; 60(10): 677-681, 2020 Oct 24.
Artigo em Japonês | MEDLINE | ID: mdl-32893239

RESUMO

We report a 74-year-old man with a 2-year history of proximal limb pain, body weight loss of 15 kg, and muscle weakness. Muscle atrophy was evident in the limbs and trunk, as well as the tongue. He was admitted to our hospital with suspected amyotrophic lateral sclerosis (ALS). Although he had no physical manifestations of Basedow disease such as palpitations, hyperhidrosis, hand tremor, exophthalmos, and an enlarged thyroid, he was diagnosed as having thyrotoxic myopathy as laboratory examinations indicated hyperthyroidism and positivity for TSH receptor antibody. The serum level of soluble IL-2 receptor was also elevated. Despite the severe muscle atrophy, the serum CK level was normal. A biopsy from the left quadriceps muscle revealed Type 1 fibers atrophy. Administration of anti-thyroid drugs normalized his thyroid function and the level of soluble IL-2 receptor, leading to improvement of the generalized muscle atrophy.


Assuntos
Doença de Graves/complicações , Doença de Graves/diagnóstico , Hipertireoidismo/diagnóstico , Hipertireoidismo/etiologia , Músculo Esquelético , Atrofia Muscular/diagnóstico , Atrofia Muscular/etiologia , Doenças Musculares/diagnóstico , Doenças Musculares/etiologia , Língua , Idoso , Antitireóideos/uso terapêutico , Autoanticorpos/sangue , Biomarcadores/sangue , Diagnóstico Diferencial , Doença de Graves/tratamento farmacológico , Humanos , Hipertireoidismo/tratamento farmacológico , Masculino , Debilidade Muscular/etiologia , Atrofia Muscular/tratamento farmacológico , Doenças Musculares/tratamento farmacológico , Receptores de Interleucina-2/sangue , Receptores da Tireotropina/imunologia , Solubilidade , Resultado do Tratamento
15.
Rinsho Shinkeigaku ; 59(5): 264-267, 2019 May 28.
Artigo em Japonês | MEDLINE | ID: mdl-31061304

RESUMO

A 52-year-old man was admitted to our hospital because of two episodes of bacterial meningitis within a 6-month period. CSF examination showed neutrophilic pleocytosis with marked elevation of protein and hypoglycorrhachia, but the inflammatory reaction was mild and blood and CSF cultures were negative. At the time of the second admission, intermittent watery nasal discharge caused by CSF rhinorrhea was evident. CT and MR imaging revealed a tiny clival bone defect, and transnasal endoscopic repair was performed successfully. The pathological diagnosis was chordoma based on immunohistochemical staining for brachyury. Although chordoma presenting as recurrent bacterial meningitis occurs extremely rare, asking patients detailed questions about the CSF rhinorrhea must be essential for disclosing unclear infection sources.


Assuntos
Rinorreia de Líquido Cefalorraquidiano/etiologia , Cordoma/complicações , Meningites Bacterianas/etiologia , Neoplasias da Base do Crânio/complicações , Cordoma/diagnóstico por imagem , Cordoma/patologia , Fossa Craniana Posterior/diagnóstico por imagem , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Recidiva , Neoplasias da Base do Crânio/diagnóstico por imagem , Neoplasias da Base do Crânio/patologia , Tomografia Computadorizada por Raios X
16.
Rinsho Shinkeigaku ; 48(10): 746-9, 2008 Oct.
Artigo em Japonês | MEDLINE | ID: mdl-19086432

RESUMO

A 75-year-old woman developed loss of vision and decreased ocular motility in all directions. She exhibited a left orbital apex syndrome, accompanied by sphenoiditis and hypertrophic pachymeningitis. Voriconazole treatment was initiated on the basis of clinical suspicion, although use of the serum beta-D glucan had negative results and a biopsy was not performed. Five days later, the left eye movements started to improve, and at that time the use of the serum aspergillus galactomannan antigen proved to have positive results. Six months later, the patient was neurologically intact and stable, except for a lack of visual acuity in counting fingers. Earlier prognoses of invasive sino-orbital aspergillosis were dismal, especially when corticosteroid therapy was done before diagnosis. This case suggests the usefulness of antifungal agents during the diagnostic procedure even when localized invasive aspergillosis is not ruled out.


Assuntos
Antifúngicos/administração & dosagem , Aspergilose/complicações , Aspergilose/tratamento farmacológico , Transtornos da Motilidade Ocular/tratamento farmacológico , Transtornos da Motilidade Ocular/etiologia , Doenças Orbitárias/tratamento farmacológico , Doenças Orbitárias/etiologia , Pirimidinas/administração & dosagem , Triazóis/administração & dosagem , Transtornos da Visão/tratamento farmacológico , Transtornos da Visão/etiologia , Idoso , Aspergilose/diagnóstico , Feminino , Humanos , Síndrome , Resultado do Tratamento , Voriconazol
17.
Rinsho Shinkeigaku ; 58(9): 565-569, 2018 Sep 28.
Artigo em Japonês | MEDLINE | ID: mdl-30175810

RESUMO

A 42-year-old woman with bronchial asthma was admitted to our hospital due to sensory dominant mononeuritis multiplex lasting for more than 6 months. At that time, her eosinophil count was 761/µl and her sural nerve biopsy showed no findings suggestive of vasculitis. Four months later, she experienced sudden convulsions and right hemiparesis due to left lobular parietal subcortical hemorrhage, when her eosinophil count was elevated to 3,257/µl. Numerous microbleeds and small infarctions were also detected in the intracerebral areas of different regions with MRI. Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic necrotizing vasculitis of the small vessels, commonly affecting the peripheral nerves. Subarachnoid hemorrhage in patients with EGPA is extremely rare. Steep elevation of the eosinophil count may release certain cytokines, causing cerebral hemorrhage.


Assuntos
Hemorragia Cerebral/etiologia , Eosinofilia/etiologia , Eosinofilia/patologia , Granuloma Eosinófilo/complicações , Granuloma Eosinófilo/patologia , Eosinófilos/patologia , Granulomatose com Poliangiite/complicações , Granulomatose com Poliangiite/patologia , Adulto , Hemorragia Cerebral/diagnóstico por imagem , Hemorragia Cerebral/tratamento farmacológico , Ciclofosfamida/administração & dosagem , Feminino , Humanos , Imageamento por Ressonância Magnética , Metilprednisolona/administração & dosagem , Prednisolona/administração & dosagem , Pulsoterapia , Tomografia Computadorizada por Raios X , Resultado do Tratamento
18.
Rinsho Shinkeigaku ; 57(7): 387-390, 2017 07 29.
Artigo em Japonês | MEDLINE | ID: mdl-28637937

RESUMO

A 73-year-old woman presented with sudden onset of right hemiparesis and was diagnosed as having cerebral infarction on the basis of diffusion-weighted brain MRI, which demonstrated lesions in the left parietal cortex. On the 3rd day, the patient developed right upper limb myoclonus, aphasia, and disturbance of consciousness with high fever. On the 6th day, she was transferred to our hospital with suspected viral encephalitis, and treatment with acyclovir was started. By the 6th day, the lesions detected by MRI had expanded to the gyrus cinguli, insula and thalamus, but not to the temporal lobe. At that time, the CSF cell count was 8/µl, and this later increased to 17/µl by the 13th day. Although herpes simplex virus DNA was detected in the CSF on the 6th day, there was no evidence of CSF pleocytosis or temporal lobe abnormalities demonstrable by brain MRI throughout the whole follow-up period. This was very atypical case of herpes simplex encephalitis characterized by a stroke-like episode, atypical MRI findings, and absence of cerebrospinal fluid pleocytosis. It is important to be mindful that herpes simplex encephalitis (HSE) can have an atypical presentation, and that sufficient acyclovir treatment should be initiated until HSE can be ruled out.


Assuntos
Encéfalo/diagnóstico por imagem , Encefalite por Herpes Simples/complicações , Encefalite por Herpes Simples/diagnóstico por imagem , Imageamento por Ressonância Magnética , Neuroimagem , Acidente Vascular Cerebral/diagnóstico por imagem , Acidente Vascular Cerebral/etiologia , Aciclovir/administração & dosagem , Idoso , Antivirais/administração & dosagem , Biomarcadores/líquido cefalorraquidiano , Clonazepam/administração & dosagem , DNA Viral/líquido cefalorraquidiano , Quimioterapia Combinada , Encefalite por Herpes Simples/líquido cefalorraquidiano , Encefalite por Herpes Simples/tratamento farmacológico , Feminino , Humanos , Leucocitose/líquido cefalorraquidiano , Levetiracetam , Metilprednisolona/administração & dosagem , Piracetam/administração & dosagem , Piracetam/análogos & derivados , Simplexvirus/genética , Acidente Vascular Cerebral/líquido cefalorraquidiano , Acidente Vascular Cerebral/tratamento farmacológico
19.
J Neurol Sci ; 247(1): 17-20, 2006 Aug 15.
Artigo em Inglês | MEDLINE | ID: mdl-16631797

RESUMO

OBJECTIVE: To elucidate the long-term therapeutic efficacy and safety of low-dose FK506 (tacrolimus) in patients with myasthenia gravis (MG). PATIENTS AND METHODS: We treated nine patients with MG (all women: age range: 35-83 years (mean: 51.1 years); MGFA classification: 4 type IIa, 4 type IIb, and 1 type IVb patients) with FK506 for more than 24 months (observation period: 24-46 months). All the patients had undergone extended thymectomy before FK506 treatment; two patients (22.2%) had noninvasive thymoma and six (66.7%) had thymic hyperplasia. We evaluated total Quantitative MG (Q-MG) score, anti-acetylcholine receptor (AChR) antibody titer in the blood, interleukin 2 (IL-2) production in peripheral blood mononuclear cells (PBMCs), administration dosage of prednisolone (PSL), and adverse effects of FK506. RESULTS: A reduction in steroid dosage of 50% without worsening of the symptoms was observed 1 year after FK506 administration in three out of six steroid-dependent MG patients (50.0%). The total Q-MG scores (range: 0-39 points) at 6 months and 1 year after FK506 administration improved by 3 points or more in six (66.7%) and seven (77.8%) out of nine patients, respectively. The efficacy of FK506 was maintained for more than 2 years. Although adverse effects were observed in three patients (33.3%), these were not serious. CONCLUSIONS: Our study indicates that low-dose FK506 treatment may be efficacious not only in controlling intractable myasthenic symptoms, but also in reducing steroid dosage, and that FK506 is safe as an adjunctive drug to PSL for MG treatment for a maximum of 3 years.


Assuntos
Imunossupressores/uso terapêutico , Miastenia Gravis/tratamento farmacológico , Tacrolimo/uso terapêutico , Adulto , Idoso de 80 Anos ou mais , Quimioterapia Combinada , Feminino , Humanos , Imunossupressores/administração & dosagem , Imunossupressores/efeitos adversos , Pessoa de Meia-Idade , Prednisolona/administração & dosagem , Prednisolona/uso terapêutico , Estudos Prospectivos , Tacrolimo/administração & dosagem , Tacrolimo/efeitos adversos , Fatores de Tempo
20.
Rinsho Shinkeigaku ; 55(2): 111-4, 2015.
Artigo em Japonês | MEDLINE | ID: mdl-25746075

RESUMO

A 62-year-old woman with one-year history of type 1 diabetes mellitus was admitted to our hospital with progressive weakness in the lower extremities and urinary dysfunction following high fever. On admission, she had rigidity and myoclonus in the upper extremities with sensory ataxia. Cerebrospinal fluid examination revealed mild pleocytosis and oligoclonal band. Glutamic acid decarboxylase (GAD) antibodies were detected at high titer in serum, but antibodies to glycine receptor (GlyR), thyroid peroxidase, mitochondrial M2, and GM1 were also detected. She was diagnosed with progressive encephalomyelitis with rigidity and myoclonus (PERM), which probably developed on the basis of polyglandular autoimmune syndromes. The clinical symptoms began to improve after initiation of intravenous high-dose methylprednisolone. Muscle weakness might be related to GM1 antibodies. This is the first report of PERM, in which GM1 antibodies were detected with GAD and GlyR antibodies.


Assuntos
Autoanticorpos/sangue , Encefalomielite/etiologia , Encefalomielite/imunologia , Gangliosídeo G(M1)/imunologia , Glutamato Descarboxilase/imunologia , Rigidez Muscular/etiologia , Rigidez Muscular/imunologia , Mioclonia/etiologia , Poliendocrinopatias Autoimunes/complicações , Poliendocrinopatias Autoimunes/imunologia , Receptores de Glicina/imunologia , Biomarcadores/sangue , Encefalomielite/diagnóstico , Feminino , Humanos , Pessoa de Meia-Idade , Rigidez Muscular/diagnóstico , Poliendocrinopatias Autoimunes/diagnóstico
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