RESUMO
Some studies have hypothesized that primary progressive apraxia of speech (ppAOS) consists of heterogeneous symptoms that can be sub-classified; however, no study has classified stroke-induced AOS (sAOS) and ppAOS according to common criteria. The purpose of this study was to elucidate the symptoms and relevant brain regions associated with sAOS and ppAOS for sub-classification. Participants included 8 patients with sAOS following lesions in the left precentral gyrus and/or underlying white matter, and 3 patients with ppAOS. All patients with sAOS could be classified into three subtypes: type I, with prominent distorted articulation; type II, with prominent prosodic abnormalities or type III, with similarly distorted articulation and prosodic abnormalities. This sub-classification was consistent with the subtypes of ppAOS proposed in previous reports. All patients with ppAOS were classified as type III, and exhibited three characteristics distinguishable from those of sAOS. First, they showed prominent lengthened syllables compared with the segmentation of syllables. Second, they could not always complete the production of multi-syllabic single words in one breath. Finally, they showed dysfunctional lesions in the bilateral supplementary motor area. We conclude that sAOS and ppAOS can be sub-classified and are universal symptoms that are common between the English and Japanese populations.
Assuntos
Afasia Primária Progressiva , Apraxias , Doenças Neurodegenerativas , Distúrbios da Fala , Acidente Vascular Cerebral , Idoso , Idoso de 80 Anos ou mais , Afasia Primária Progressiva/classificação , Afasia Primária Progressiva/etiologia , Afasia Primária Progressiva/patologia , Afasia Primária Progressiva/fisiopatologia , Apraxias/classificação , Apraxias/etiologia , Apraxias/patologia , Apraxias/fisiopatologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Doenças Neurodegenerativas/complicações , Doenças Neurodegenerativas/patologia , Distúrbios da Fala/classificação , Distúrbios da Fala/etiologia , Distúrbios da Fala/patologia , Distúrbios da Fala/fisiopatologia , Acidente Vascular Cerebral/complicações , Acidente Vascular Cerebral/patologiaRESUMO
Anti-aquaporin-4 (anti-AQP-4) antibody-positive neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune disorder resulting in severe, recurrent optic neuritis, transverse myelitis, brain stem syndrome, and other types of neurological involvement. Its median age of onset has been reported to be around 40 years. We report herein a case of very-late-onset NMOSD (76 years of age) and try to promote its awareness as a type of neurological deterioration in elder patients. A 76-year-old woman suffering from Parkinson disease was admitted to our hospital because of consciousness disturbance. Cranial magnetic resonance imaging revealed the presence of fluid-attenuated inversion recovery high-signal-intensity lesions in the right peri- and intralateral ventricle. Part of this lesion and the meninges showed gadolinium enhancement. Physical examination revealed the presence of a tumor in the right breast, which was later diagnosed as invasive ductal carcinoma. In addition, laboratory examinations led to the detection of anti-AQP-4 antibodies in her serum; consequently, the patient was diagnosed as having NMOSD. She received initial pulsed steroid therapy, followed by right mastectomy. Although the patient's consciousness improved significantly, she developed abrupt-onset bilateral leg weakness and multiple longitudinal spinal cord lesions. Additional steroid therapy ameliorated the patient's leg weakness and reduced the swelling of the spinal cord.
RESUMO
Typical absence seizures and isolated myoclonic seizures are both classified as age-related generalized seizures and are considered to be benign neurological manifestations. Concomitance of the two types of seizure is considered benign if it does not accompany other types of seizures or other neurological problems. We followed up a ten-year-old girl with isolated absence and myoclonic seizures whose family history of mental and neurological signs was initially not disclosed. After several years, the family history of neurological and mental problems was finally disclosed, and the diagnosis of dentato-rubro-pallido-luysian atrophy (DRPLA) was confirmed. The patient's clinical course was slowly progressive, and by age 21 she was in a nearly vegetative state. We would like to alert clinicians to consider DRPLA when diagnosing patients with absence and/or myoclonic seizures, even when they present the clinical features of benign epilepsies in the early stage.
Assuntos
Encefalopatias/complicações , Encefalopatias/patologia , Tronco Encefálico/patologia , Cerebelo/patologia , Epilepsias Mioclônicas/etiologia , Córtex Cerebral/patologia , Progressão da Doença , Eletroencefalografia/métodos , Epilepsias Mioclônicas/patologia , Saúde da Família , Feminino , Humanos , Estudos Longitudinais , Imageamento por Ressonância Magnética/métodos , Adulto JovemRESUMO
We report a 60-year-old woman with toxoplasma encephalopathy. She was being treated with prednisolone and methotrexate for rheumatoid arthritis that was diagnosed at the age of 40. In a preoperative examination of her left fifth finger ganglion, pericardial effusions, cardiomegaly, and a right atrial mass were detected. In addition, brain MRI showed nodular shadows in the right thalamus, bilateral globus pallidus, and left dentate nucleus of the cerebellum. T1 and T2 weighted images showed high intensities within those shadows; however, a T1 gadolinium enhancement image showed no contrast enhancement in the lesions. There were no positive neurological findings. Examination of the cerebrospinal fluid and cultivation tests showed nothing particular. The right atrial mass was subsequently diagnosed as malignant lymphoma and treated with radiation therapy. Toxoplasma gondii antibody titers were increased in both serum and cerebrospinal fluid. Based on IgG avidity index and nested PCR, we diagnosed toxoplasma encephalopathy with chronic T. gondii infection. The T. gondii gene product was also detected in cerebrospinal fluid by nested PCR. We consider that IgG avidity index and nested PCR were useful for the diagnosis of toxoplasma encephalopathy.
Assuntos
Anticorpos Antiprotozoários/sangue , Encefalite/diagnóstico , Encefalite/parasitologia , Genoma de Protozoário , Imunoglobulina G/sangue , Imageamento por Ressonância Magnética , Reação em Cadeia da Polimerase , Proteínas de Protozoários/líquido cefalorraquidiano , Testes Sorológicos , Toxoplasmose , Animais , Anticorpos Antiprotozoários/líquido cefalorraquidiano , Doença Crônica , Feminino , Humanos , Imunoglobulina G/líquido cefalorraquidiano , Pessoa de Meia-Idade , Toxoplasma/genética , ZoonosesRESUMO
OBJECTIVES: The aim of the present study was to determine the relationships of drooling with motor symptoms and nigrostriatal neuron loss in drug-naïve patients with Parkinson's disease (PD). We therefore examined the relationships of drooling with motor symptoms and striatal dopamine transporter (DAT) binding measured by [123-Iodine]-fluoropropyl-2beta-carbomethoxy-3beta-(4-iodophenylnortropane) dopamine transporter single-photon emission computed tomography(123I-FP-CIT SPECT). PATIENTS AND METHODS: Thirty-five untreated PD patients (14 men and 21 women with a mean age of 71.9⯱â¯7.2 years) were included in this study. The patients were divided into a drooler group and non-drooler group. They underwent clinical assessments and 123I-FP-CIT SPECT imaging. Motor symptoms were assessed using Unified Parkinson's Disease Rating Scale (UPDRS). RESULTS: The results showed that UPDRS motor score (pâ¯=â¯0.002) and akinetic-rigid score (pâ¯=â¯0.008) were higher and that striatal DAT availability (pâ¯=â¯0.03) was lower in the drooler group than in the non-drooler group. However, tremor score, age, and duration of PD showed no significant differences between the drooler group and non-drooler group. CONCLUSIONS: Drooling in untreated PD is related to an increase in motor symptoms (especially bradykinesia and axial symptoms) and to reduction of striatal DAT availability.
Assuntos
Corpo Estriado/diagnóstico por imagem , Proteínas da Membrana Plasmática de Transporte de Dopamina/metabolismo , Doença de Parkinson/complicações , Sialorreia/complicações , Idoso , Idoso de 80 Anos ou mais , Corpo Estriado/metabolismo , Corpo Estriado/patologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Doença de Parkinson/metabolismo , Doença de Parkinson/patologia , Sialorreia/metabolismo , Sialorreia/patologia , Tomografia Computadorizada de Emissão de Fóton ÚnicoRESUMO
OBJECTIVES: The aim of the present study was to determine the relations of clinical symptoms with nigrostriatal neuron loss in drug-naïve patients with Parkinson's disease (PD). We examined the severity of motor symptoms and freezing of gait (FOG), falls and overactive bladder (OAB) in PD patients and their relations with striatal dopamine transporter (DAT) binding. PATIENTS AND METHODS: Thirty-two untreated PD patients (14 men and 18 women with a mean age of 71.4⯱â¯7.2 years) were included in this study. Clinical assessments were performed by using Unified Parkinson's Disease Rating Scale (UPDRS) and overactive bladder symptom score (OABSS), and striatal dopamine transporter (DAT) binding was measured by123I-FP-CIT SPECT. RESULTS: The results showed that striatal DAT availability was significantly lower in the high UPDRS motor score group, high akinetic-rigid score group, FOG group, and OAB group than in the low UPDRS motor score group, low akinetic-rigid score group, non-FOG group, and non-OAB group. However, the results also showed that there was no significant difference in striatal DAT availability between the high tremor score group and low tremor score group or between the faller group and non-faller group. CONCLUSIONS: The severity of bradykinesia and axial symptoms and the existence of FOG and OAB in untreated PD patients are related to a decrease in striatal DAT availability. Severity of tremors and occurrence of falls are not related to a decrease in striatal DAT availability. The mechanisms underlying the clinical symptoms of PD involve not only dopaminergic pathways but also non-dopaminergic pathways.
Assuntos
Encéfalo/diagnóstico por imagem , Proteínas da Membrana Plasmática de Transporte de Dopamina/metabolismo , Doença de Parkinson/diagnóstico por imagem , Tremor/diagnóstico por imagem , Idoso , Idoso de 80 Anos ou mais , Encéfalo/metabolismo , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Doença de Parkinson/metabolismo , Índice de Gravidade de Doença , Avaliação de Sintomas , Tomografia Computadorizada de Emissão de Fóton Único , Tremor/metabolismoRESUMO
OBJECTIVE: The objective of this study was to determine the incidence of anti-NMDAR encephalitis in patients in whom a teratoma was removed. As far as we know, there has been no report on the incidence of anti-NMDAR encephalitis in patients in whom a teratoma was removed. METHODS: This study was a single-institutional observational study. We enrolled patients who were diagnosed with teratoma in the Department of Pathology, Sapporo City General Hospital during a nine-year period between January 2008 and December 2016. RESULTS: In Sapporo City General Hospital, 6 NMDAR encephalitis cases were detected during the 9-year period. In the same 9-year period, a pathological diagnosis of teratoma was made in 343 cases in the hospital. Anti-NMDAR encephalitis patients with a teratoma accounted for only 1.17% of all teratoma patients. Three of the 4 anti-NMDAR encephalitis patients with a teratoma underwent second removal of a teratoma, and no nervous tissue was detected pathologically. CONCLUSIONS: In this study, we determined the association between teratoma with anti-NMDAR encephalitis and teratoma without anti-NMDAR encephalitis in cases in a single institution. As far as we know, this report is the first report on the incidence of anti-NMDAR encephalitis in teratoma patients.
Assuntos
Encefalite Antirreceptor de N-Metil-D-Aspartato/diagnóstico , Encefalite Antirreceptor de N-Metil-D-Aspartato/epidemiologia , Hospitais Gerais/tendências , Neoplasias Ovarianas/diagnóstico , Neoplasias Ovarianas/epidemiologia , Teratoma/diagnóstico , Teratoma/epidemiologia , Adolescente , Adulto , Feminino , Humanos , Incidência , Neoplasias Ovarianas/cirurgia , Teratoma/cirurgia , Adulto JovemRESUMO
To explore non-motor comorbidities of myasthenia gravis (MG), we present two cases of thymoma-associated MG patients. Alopecia, pure red cell aplasia, and thymoma- associated multiorgan autoimmunity were observed in Case 1, and alopecia, thrombocytopenia, hypogammaglobulinemia and nephrotic syndrome were observed in Case 2. In both cases, autoreactive T lymphocytes inappropriately stimulated by thymus tissue may have played key roles in generating the various autoimmune-associated symptoms. Consequently, systemic immunological involvement due to the thymoma-associated breakdown of immunoregulations in both motor and non-motor systems should be considered in MG patients.
Assuntos
Miastenia Gravis/complicações , Timoma/complicações , Neoplasias do Timo/complicações , Agamaglobulinemia/complicações , Alopecia/complicações , Autoimunidade/fisiologia , Feminino , Humanos , Pessoa de Meia-Idade , Aplasia Pura de Série Vermelha/complicações , Linfócitos T/imunologia , Trombocitopenia/complicaçõesRESUMO
To date, the only instance of tick-borne encephalitis (TBE) in Japan was reported from the southern part of Hokkaido Island in 1993; no other cases have been reported since then. We herein report the first case of TBE reported in the central part of Hokkaido Island, and describe the fatal clinical course of a patient who presented with meningoencephalomyelitis, which partly involved the nerve root. Magnetic resonance imaging (MRI) of the patient's cranium and spine revealed characteristic central nervous system involvement. Our case report is extremely relevant to efforts to protect public health and for precautions against TBE pandemics.
Assuntos
Vírus da Encefalite Transmitidos por Carrapatos/isolamento & purificação , Encefalite Transmitida por Carrapatos/diagnóstico por imagem , Encefalite Transmitida por Carrapatos/mortalidade , Crânio/diagnóstico por imagem , Coluna Vertebral/diagnóstico por imagem , Adulto , Animais , Evolução Fatal , Humanos , Japão , Imageamento por Ressonância Magnética , MasculinoRESUMO
A 27-year-old woman with optic neuritis and cervical myelitis developed hypertrophic demyelinating polyneuropathy. It was hypothesized that the diagnosis was combined central and peripheral demyelination. A hypertrophic nerve was observed subcutaneously, and magnetic resonance imaging demonstrated marked hypertrophy of the nerve roots. The patient was negative for anti-aquaporin 4 antibodies. Her anti-neurofascin 155 antibody levels was slightly elevated, but it was not definitely positive. Pulsed steroid therapy and the administration of immunoglobulin ameliorated her symptoms. Molecules in both the peripheral and central nervous systems might be target antigens, but further investigations will be needed to clarify the precise pathogenic mechanisms.
Assuntos
Hipertrofia/diagnóstico por imagem , Esclerose Múltipla/complicações , Mielite/complicações , Pescoço/diagnóstico por imagem , Neurite Óptica/complicações , Polineuropatias/diagnóstico , Polineuropatias/etiologia , Radiculopatia/diagnóstico por imagem , Adulto , Feminino , Humanos , Hipertrofia/fisiopatologia , Imageamento por Ressonância Magnética , Mielite/fisiopatologia , Neurite Óptica/fisiopatologia , Polineuropatias/tratamento farmacológico , Polineuropatias/fisiopatologia , Radiculopatia/fisiopatologia , Esteroides/uso terapêutico , Resultado do TratamentoRESUMO
OBJECTIVES: The aim of the present study was to determine the relation of urinary dysfunction with motor symptoms and nigrostriatal neuron loss in drug-naïve patients with Parkinson's disease (PD). We therefore examined the relation of overactive bladder (OAB) symptoms with motor symptoms and striatal dopamine transporter (DAT) binding measured by [123-Iodine]-fluoropropyl-2beta-carbomethoxy-3beta-(4-iodophenylnortropane) dopamine transporter single-photon emission computed tomography (123I-FP-CIT SPECT). PATIENTS AND METHODS: Thirty-one untreated PD patients (12 men and 19 women with a mean age of 71.2⯱â¯6.7 years) were included in this study. Patients were evaluated with overactive bladder symptom score (OABSS) and divided into an OAB group and Non-OAB group. They underwent clinical assessments and 123I-FP-CIT SPECT imaging. Motor symptoms were assessed using Unified Parkinson's Disease Rating Scale (UPDRS). RESULTS: The results showed that UPDRS motor score (pâ¯=â¯0.01) and akinetic-rigid score (pâ¯=â¯0.002) were higher and that striatal DAT availability (pâ¯=â¯0.01) was lower in the OAB group than in the Non-OAB group. However, tremor score, age, and duration of PD showed no significant differences between the OAB group and Non-OAB group. CONCLUSIONS: Urinary dysfunction in untreated PD is related with increase in motor symptoms (especially bradykinesia and axial symptoms) and reduction of striatal DAT availability.
Assuntos
Corpo Estriado/metabolismo , Proteínas da Membrana Plasmática de Transporte de Dopamina/farmacocinética , Discinesias/fisiopatologia , Doença de Parkinson/metabolismo , Doença de Parkinson/fisiopatologia , Índice de Gravidade de Doença , Bexiga Urinária Hiperativa/fisiopatologia , Idoso , Idoso de 80 Anos ou mais , Corpo Estriado/diagnóstico por imagem , Discinesias/etiologia , Humanos , Pessoa de Meia-Idade , Doença de Parkinson/complicações , Doença de Parkinson/diagnóstico por imagem , Tomografia Computadorizada de Emissão de Fóton Único , Tropanos , Bexiga Urinária Hiperativa/etiologiaRESUMO
The patient was a woman without hypertension who had previously experienced intracranial hemorrhage twice at 48 and 56 years of age. At 59 years of age, she was diagnosed with neuromyelitis optica spectrum disorder (NMOSD) based on the presence of a brain stem lesion and the detection of anti-aquaporin 4 (AQP4) antibodies. After 5 months of continuous treatment with prednisolone (15 mg/day), she presented with optic neuritis and intracranial bleeding. A recurrent attack of NMOSD and intracranial hemorrhage were concurrently diagnosed. We herein report a case of NMOSD with recurrent intracranial hemorrhage, which indicates an association between NMOSD and cerebellar vascular dysfunction.
Assuntos
Hemorragias Intracranianas/etiologia , Neuromielite Óptica/complicações , Aquaporina 4/imunologia , Autoanticorpos/imunologia , Feminino , Humanos , Pessoa de Meia-Idade , Neuromielite Óptica/tratamento farmacológico , Prednisolona/uso terapêuticoRESUMO
OBJECTIVES: The aim of the present study was to determine the associations of motor symptoms in untreated Parkinson's disease (PD) with urinary dysfunction. We therefore examined the association between the overactive bladder symptom score (OABSS) and Unified Parkinson's Disease Rating Scale (UPDRS). PATIENTS AND METHODS: Thirty-one untreated PD patients without dementia (12 men and 19 women with a mean age of 72.0±6.7years) were included in this study. Their urinary tract dysfunctions were rated using the OABSS. The motor symptoms of all patients were also assessed using UPDRS. RESULTS: OABSS had significant correlations with UPDRS motor score (Spearman's rho=0.39, p=0.03) and akinetic-rigid score (Spearman's rho=0.47, p=0.01). However, OABSS showed no significant correlation with tremor score, age, or duration of PD. CONCLUSIONS: Higher OABSS was consistently associated with increase in severity of motor disorders, especially akinetic-rigid score, in the study participants.
Assuntos
Movimento/fisiologia , Doença de Parkinson/complicações , Bexiga Urinária Hiperativa/etiologia , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Índice de Gravidade de Doença , Estatística como Assunto , Estatísticas não Paramétricas , Inquéritos e QuestionáriosRESUMO
In rare instances, recipients of organ transplants from human T-lymphotropic virus type I- (HTLV-I-) positive donors reportedly developed neurologic symptoms due to HTLV-I-associated myelopathy (HAM). We present herein two cases of HAM associated with renal transplantation from HTLV-I seropositive living-donors. The first patient was a 42-year-old woman with chronic renal failure for twelve years and seronegative for HTLV-I. She underwent renal transplantation with her HTLV-I seropositive mother as the donor, and she developed HAM three years after the transplantation. The second patient was a 65-year-old man who had been suffering from diabetic nephropathy. He was seronegative for HTLV-I and underwent renal transplantation one year previously, with his HTLV-I seropositive wife as the donor. He developed HAM eight months after renal transplantation. Both cases showed neurological improvements after the immunomodulating therapies. We tried to shed some light on the understanding of immunological mechanisms of transplantation-associated HAM, focusing on therapeutic strategies based on the immunopathogenesis of the condition.
RESUMO
We present a very rare case of adult onset subacute sclerosing panencephalitis (SSPE), and explain the characteristic sequential clinico-radiological findings. The patient, a twenty three-year-old man, had noticed unsteadiness in walking about two months previously. Although inosine pranobex and intrathecal interferon were administered, symptoms worsened insidiously and he became bedridden after four months. The levels of serum and CSF anti-measles IgG antibodies have not changed. Initially, supratentorial cortical atrophy was observed, especially of the left temporal lobe, but there were no other MRI signal alterations at the time. After three months, the supratentorial cortices produced low-signal intensities in T1-weighted images and in T2-weighted MRI, the cortical margin was very unclear and white matter signal intensities had become higher. Furthermore, cortices became thinner and ventricular size increased, especially for the lateral and third ventricles. SPECT examinations showed a marked reduction in cerebral blood flow and the perfusion deficits observed seemed to be closely correlated with the abnormal MRI signal patterns. Pathological examinations of biopsy samples revealed infiltration of inflammatory cells around the small vessels. As for immunohistochemical findings, CD68 positive cells were frequently observed, and this result implied the activation of microglia. Further studies are necessary to elucidate the pathogenic mechanisms of SSPE.