BACKGROUND AND OBJECTIVES:The functional outcome and mortality of patients with poststroke epilepsy (PSE) have not been assessed in a prospective study. Previous reports have suggested that patients with PSE may suffer from prolonged functional deterioration after a seizure. In this study, we prospectively investigated the functional outcome and mortality of patients with PSE and analyzed the effect of seizure recurrence on the outcomes. METHODS:This is part of the Prognosis of Post-Stroke Epilepsy study, a multicenter, prospective observational cohort study, where 392 patients with PSE (at least 1 unprovoked seizure more than 7 days after the onset of the last symptomatic stroke) were followed for at least 1 year at 8 hospitals in Japan. This study included only PSE patients with a first-ever seizure and assessed their functional decline and mortality at 1 year. Functional decline was defined as an increase in modified Rankin Scale (mRS) score at 1 year compared with baseline, excluding death. The associations between the seizure recurrence and the outcomes were analyzed statistically. RESULTS:A total of 211 patients (median age of 75 years; median mRS score of 3) were identified. At 1 year, 50 patients (23.7%) experienced seizure recurrence. Regarding outcomes, 25 patients (11.8%) demonstrated functional decline and 20 (9.5%) had died. Most patients died of pneumonia or cardiac disease (7 patients each), and no known causes of death were directly related to recurrent seizures. Seizure recurrence was significantly associated with functional decline (odds ratio [OR] 2.96, 95% CI 1.25-7.03, p = 0.01), even after adjusting for potential confounders (adjusted OR 3.26, 95% CI 1.27-8.36, p = 0.01), but not with mortality (OR 0.79, 95% CI 0.25-2.48, p = 0.68). Moreover, there was a significant trend where patients with more recurrent seizures were more likely to have functional decline (8.7%, 20.6%, and 28.6% in none, 1, and 2 or more recurrent seizures, respectively; p = 0.006). DISCUSSION:One-year functional outcome and mortality of patients with PSE were poor. Seizure recurrence was significantly associated with functional outcome, but not with mortality. Further studies are needed to ascertain whether early and adequate antiseizure treatment can prevent the functional deterioration of patients with PSE.

Background: In native Japanese speakers, diagnosing progressive non-fluent aphasia (PNFA) often depends on the presence of apraxia of speech (AOS), not on the presence of agrammatism in language production. This is because Japanese is a kind of agglutinative language and it is often difficult to detect agrammatism in spontaneous speech or usual examinations such as Western Aphasia Battery (WAB). However, there also exists a syndrome of primary progressive apraxia of speech (PPAOS), which is characterized by progressive pure AOS without any signs of aphasia including agrammatism. Hence diagnosing PNFA upon the presence of AOS inevitably has a substantial limitation, and some sensitive measure to detect agrammatism in Japanese is required.

Background: Neoplastic plexopathies is relatively uncommon and sometime fail to notice especially in patients without history of cancer. Plural nerves disturbance of focal area in nerve conduction studies(NCS) may chance of diagnosis for neoplastic plexopathies.

We investigated apparent diffusion coefficient (ADC) and fractional anisotropy (FA) values using diffusion weighted imaging (DWI) MRI in multiple system atrophy (MSA) and Parkinson's disease (PD) to assess the differential diagnostic values. Differentiation of MSA from PD is clinically important because these disorders manifest dissimilar treatment response and prognosis. Recently, quantitative analysis of DWI associated values was reported to be useful for detecting the early imaging changes of MSA. We measured ADC and FA values in the pons, bilateral middle cerebellar peduncle and putamen comparing MSA and PD patients. We placed circular shaped regions of interest (ROI) in the each area. Subjects were 10 probable MSA patients (mean age 66 years, mean disease duration 5.5 years) and 15 Parkinson's disease patients (mean age 70 years, mean disease duration 8.1 years). Normal Heart/ Mediastinum ratio of cardiac MIBG scintigraphy was applied to confirm the further supporting evidence of MSA. ADC values in the all ROIs of pons, middle cerebellar peduncle, and putamen indicated significantly higher in MSA than PD. ADC values averaging all ROIs seemed best to discriminate MSA from PD. ADC values tend to be higher correlating with longer disease duration and severer clinical symptoms in MSA patients. We found no significant correlation with disease duration nor severity in PD patients. FA values in MSA patients showed lower in the pons and middle cerebellar peduncle, but in the putamen compared with PD. Lower FA values in the pons and middle cerebellar peduncle were detected as longer duration and severer symptoms in MSA. ADC and FA values in the brainstem and putamen of MSA patients were revealed to differ from PD. The changes of these values tended to be evident in the more advanced MSA patients, suggesting these quantitative manners might reflect pathological progression in MSA. DWI appears to be a useful diagnostic tool for differential diagnosis of MSA from PD.

Purpose: Dystonic posturing (DP) is one of the most reliable lateralizing indicators for temporal lobe epilepsy (TLE). We evaluated the ictal hyperperfusional areas in patients with DP by using ictal-interictal subtraction single-photon emission computed tomography (SPECT).Methods: Ninety-seven patients were treated surgically for intractable TLE, and 39 patients underwent ictal and interictal SPECT studies with the same isotope. These patients were divided into three groups: group I with DP of the contralateral side extended to the epileptogenic focus, group II with elevated muscle tonus but without DP, and group III without DP or alteration of muscle tonus. Ictal, interictal SPECT and thin-slice magnetic resonance imaging (MRI) were overlaid by using the automatic multimodality registration program to construct ictal-interictal subtracted images of SPECT on MRI.Results: Thirteen patients belonged to group I; 14, to group II; and 12, to group III. A statistically significant difference in hyperperfusion rate was observed in the putamen (10 patients in group I, three in group II and two in group III; p < 0.01) and mesial temporal lobe (10 patients in group I, seven in group II, and two in group III; p < 0.05) on the ipsilateral side of the epileptogenic focus. No statistically significant difference was observed for other ictal symptoms except ipsilateral upper-limb automatism (eight patients in group I, three in group II, and none in group III p < 0.01).Conclusions: A strong correlation between DP and hyperperfusion in the putamen and mesial temporal lobe was demonstrated. Some patients showed a wide hyperperfusion area extending from the mesial temporal lobe to putamen, which may correspond to the propagation of epileptic discharges. Our results suggest a correlation between hyperperfusion of putamen and contralateral dystonic posturing.

We described two patients, a mother and daughter, of Stormorken's syndrome. The syndrome is characterized clinically by autosomal dominant inheritance, congenital miosis, thrombocytopenia, asplenia and muscle weakness. Both patients had bleeding tendency, ichthyosis of arms, and muscle weakness. The daughter additionally had short stature (146 cm), low body weight (32 kg) and muscle cramp. Neurological findings of the patients included migraine-like headache, cognitive dysfunction, limitation of upward and lateral gaze, and amydriasis. Femoral muscle MRI of the daughter demonstrated decreased volume with patchy high intensity areas in the hamstrings. A muscle biopsy from the daughter showed myogenic changes with muscle fiber necrosis and regeneration, variation in fiber size, tubular aggregates in approximately 5% of fibers, and fibrous tissue proliferation. Dystrophin, dystrophin-associated proteins and dysferlin were normally expressed. Although both patients had elevated creatine kinase levels and generalized muscle wasting, muscle weakness was mild with slow progression. A certain membrane defect in the platelet and muscle fiber might be responsible for the pathogenesis of this syndrome.

Purpose: We describe a patient with complex partial seizure with unidirectional olfactory aura associated with ipsilateral unruptured aneurysm.Methods and Results: The patient felt a sweet pleasant smell coming from behind her light side every time before the attack. Cranial magnetic resonance imaging (MRI) and three-dimensional computed tomography (CT) angiography revealed a large aneurysm at the bifurcation of the right middle cerebral artery and compression of the right orbitofrontal cortex. Small spikes were recorded from the right orbitofrontal and superior temporal gyri and from the uncus by the cortical electrodes during clipping of the aneurysm.Conclusions: The orbitofrontal cortex may have a function related to the ipsilateral directional olfactory sensation.

Oral administration of ethyl O-[N-(p-carboxyphenyl-carbamoyl]-mycophenolate (CAM), a derivative of mycophenolic acid (MPA) and an inosine monophosphate dehydrogenase inhibitor, dose-dependently suppressed acute experimental allergic encephalomyelitis in Lewis rats without exerting any serious adverse effects. A daily dose of 50 mg/kg of CAM almost completely abolished both the clinical disease and the inflammation in the CNS. In the CAM-treated rats, a weight loss and fluctuations of peripheral lymphocyte subsets were minimized. The CAM treatment was effective when started at the time of sensitization but ineffective when deferred till day 10. Furthermore, CAM reduced the percentage of CD4+CD45RC- cells in the peripheral blood. The only detectable adverse effect was moderate anemia but it was rapidly improved after withdrawal of the drug. This drug could be a useful adjunct for the long-term immunosuppressive therapy for inflammatory diseases of the CNS.