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Internal Medicine 01/2013; 52(9):1005-6. · 0.94 Impact Factor
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ABSTRACT: We report a case of paradoxical brain embolism mediated through a pulmonary arteriovenous malformation (PAVM) with hereditary hemorrhagic telangiectasia (HHT). A 25-year-old right handed man was admitted to our hospital after sudden headache and visual field abnormality. In neurologic examinations, he had left superior-quadrantanopsia. Laboratory findings showed iron deficiency anemia. Diffusion weighted images disclosed a high-signal-intensity area in the right occipito-temporal lobe, and intraarterial digital subtraction cerebral angiography revealed occlusion of the right posterior cerebral artery. Transesophageal echocardiography revealed continuous right-to-left shunt. We confirmed his history of spontaneous recurrent epistaxis and the first-degree relatives with epistaxis or PAVM. A contrast enhanced CT scan of the chest revealed a PAVM. The diagnosis of paradoxical brain embolism mediated through the PAVM with HHT was, thus, established. The PAVM was occluded by using embolization coils successfully. In Asian countries, the prevalence of PAVM with HHT is thought to be lower than in European countries. We should carefully take medical and family histories, especially epistaxis, in a young stroke patient.
Rinshō shinkeigaku = Clinical neurology. 01/2012; 52(3):161-5.
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ABSTRACT: We describe a case of a 67-year-old man with systemic lupus erythematosus who presented with progressive left hemiplegia. Although the cerebral spinal fluid (CSF) polymerase chain reaction (PCR) for the JC virus was negative, a brain biopsy confirmed the diagnosis of progressive multifocal leukoencephalopathy (PML). The tapering of prednisone and the use of cidofovir could not arrest the disease progression. Administration of mefloquine stopped the extension of the lesion, and resulted in obvious clinical improvement. The CSF nested PCR for the JC virus also became negative. This widely used drug should be tried for the treatment of non-HIV PML.
Internal Medicine 01/2012; 51(10):1245-7. · 0.94 Impact Factor
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Daichi Inoue,
Aiko Kato,
Sumie Tabata,
Takeshi Kitai,
Yoko Takiuchi,
Takaharu Kimura,
Sonoko Shimoji,
Minako Mori,
Yuya Nagai,
Katsuhiro Togami,
Akiko Matsushita,
Kenichi Nagai,
Hayato Maruoka,
Yukihiro Imai,
Minako Beppu, Michi Kawamoto,
Takayuki Takahashi
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ABSTRACT: A 55-year-old woman was diagnosed with POEMS syndrome; however, we could not perform high-dose chemotherapy with autologous peripheral blood stem cell transplantation (auto-PBSCT) because of rapidly progressive congestive heart failure. Thus, we treated the patient with thalidomide plus dexamethasone, which brought about a marked improvement of systolic dysfunction, POEMS syndrome-related symptoms and the serum concentrations of vascular endothelial growth factor. Subsequently, we safely performed high-dose chemotherapy with auto-PBCST. This report strongly suggests that thalidomide is effective for POEMS syndrome, even if complicated by congestive heart failure which is considered to be related to POEMS syndrome.
Internal Medicine 01/2010; 49(5):461-6. · 0.94 Impact Factor
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ABSTRACT: Only three facioscapulohumeral muscular dystrophy (FSHD) patients have been reported to have cardiomyopathy. An asymptomatic 38-year-old man was incidentally found to have electrocardiographic abnormalities. His echocardiogram demonstrated mild dilatation of the left ventricle and poor contractility. Cardiac histopathology indicated hypertrophic cardiomyopathy. Later he developed muscle weakness in the right arm. Scapular winging and asymmetrical facial weakness were evident. Muscle biopsy at the age of 44 years showed myopathic changes consistent with FSHD. His daughter had symptoms of infantile FSHD, which was genetically confirmed. This is the first report of an FSHD patient with biopsy-proven cardiomyopathy.
Neuromuscular Disorders 02/2009; 19(2):140-2. · 2.80 Impact Factor
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ABSTRACT: Influenza-associated encephalopathy (IAE) usually occurs in children aged <5 years. Adult cases of IAE are very rare and, thus far, no definite adult autopsy case has been reported. Here, we present the first definite adult autopsy case of IAE. A 76-year-old man presented with sudden coma a day after the onset of fever caused by infection with influenza type A virus. Soon after admission, his condition was complicated by DIC, shock, and multiple organ failure, and he was diagnosed with IAE. Oseltamivir administration and steroid pulse therapy were performed but these proved to be ineffective. The patient died about 24 hours after the onset of encephalopathy. The autopsy revealed massive brain edema and diffuse increase of amoeboid glias without inflammatory cell infiltration. Influenza type A/Hong Kong virus (H3) was isolated from his lungs. Serum IL-6 level was extremely high (35,800 pg/ml; normal, 0.221-4.62 pg/ml). The clinical course, and the laboratory and pathological findings of this adult case resembled those of a typical childhood-onset IAE, suggesting the same pathogenesis. During the influenza season, IAE should be taken into account for differential diagnosis in adult patients with altered mental status and fever.
Rinsho shinkeigaku = Clinical neurology 10/2008; 48(10):713-20.
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ABSTRACT: Although nonconvulsive status epilepticus (NCSE) is a major neurological emergency, its frequency and clinical course are not well clarified. We investigated the clinical characteristics of status epilepticus focusing on the significance of NCSE. One thousand seven hundred twenty-three patients were admitted as neurological emergency cases in our hospital between October 2003 and September 2006. Of these cases, 94 (5.5%) were diagnosed as status epilepticus of which, 24 (25.5%) were diagnosed with NCSE on admission. Moreover, 8 patients who presented with convulsive status epilepticus on admission had episodes of NCSE during hospitalization. Thus, 32 patients (34.0%) suffered from NCSE during their clinical course. We analyzed the prognostic factors of status epilepticus using the Glasgow Outcome Scale. Poor outcome was significantly correlated with NCSE (p = 0.003) and acute cerebrovascular disease (p = 0.010), independent of age, sex, history of epilepsy, and other etiologies. Our study revealed that NCSE is not a rare condition and results in a poor outcome. Careful EEG evaluation of patients with consciousness disturbance might increase the diagnostic accuracy of NCSE, and aggressive treatment of patients with NCSE should be necessary to improve the prognosis of NCSE.
Rinsho shinkeigaku = Clinical neurology 05/2008; 48(4):242-8.
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ABSTRACT: We investigated clinical features of juvenile patients presenting non-herpetic viral acute encephalitis (4 men and 7 women, aged of onset; 23.7 +/- 3.3 years) without malignancy and immunodeficiency. We divided the patients into two groups according to initial neurological symptoms: psychiatric symptoms mimicking schizophrenia (group P, n=5), seizure (group S, n=6), and compared clinical manifestations among the two groups. Symptoms frequently seen in initial phase of the illness were neck stiffness (4 cases, 36%), involuntary movement (7 cases, 64%) and convulsion (8 cases, 73%). There were no significant difference among the groups except seizure. Patients in group P had more CSF cells and CSF lymphocytes compared with other groups (p < 0.05 and p < 0.01, respectively). Abnormal intensities in T2-weighted magnetic resonance images were found in 4 cases (36%). The term from the onset to leaving hospital of group P (213 +/- 227 days) was longer than that of group S (98 +/- 85 days), although it did not reach a significant difference. These findings indicate that juvenile acute non-herpetic encephalitis initially presenting psychiatric symptoms was serious and had relatively poor prognosis.
Nō to shinkei = Brain and nerve 07/2005; 57(7):599-606.
