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ObjectiveTo investigate whether the presence of autonomic dysfunction is associated with white matter and functional connectivities and the level of cognitive performance in patients with de novo Parkinson disease (PD).MethodsSeventy-five patients with de novo PD underwent a comprehensive autonomic function test and were classified into 2 groups according to the Composite Autonomic Severity Score (CASS; 30 with moderate to severe autonomic dysfunction [CASS 4–10, PD-AUT+] and 45 without significant autonomic dysfunction [CASS 0–3, PD-AUT−]). Network-based statistics and a graph theoretical analysis were performed to assess the interregional white matter connectivity using diffusion tensor imaging. We also performed analyses of resting-state functional connectivity and compared cognitive performance between the 2 groups.ResultsThere were no significant differences in demographic characteristics and vascular risk factors between the PD-AUT+ and PD-AUT− groups. The PD-AUT+ group showed poorer cognitive performance on frontal/executive function than the PD-AUT− group. The PD-AUT+ group exhibited severely disrupted white matter connectivity in both fronto-subcortical and posterior cortical regions, which was well correlated with the severity of autonomic dysfunction assessed by the CASS. In addition, functional connectivity within the executive control network and dorsal attention network negatively correlated with the CASS.ConclusionsOur results suggest that autonomic dysfunction is associated with disrupted white matter and functional brain connectivity as well as cognitive impairment in de novo patients with PD.

Objective: Hemiconvulsion-hemiplegia-epilepsy (HHE) syndrome is an uncommon consequence of prolonged febrile convulsive seizures in infancy and early childhood. Delayed epilepsy in HHE syndrome is frequently intractable to medical treatment. The present study evaluated the role and prognosis of surgical treatment in patients with delayed epilepsy of HHE syndrome. Methods: We included 26 consecutive patients who were diagnosed with HHE syndrome and underwent surgical treatment for delayed epilepsy at Seoul National University Hospital. The multidisciplinary presurgical evaluations included brain MRI, video-EEG monitoring, FDG-PET, and ictal SPECT. Anterior temporal lobectomy (ATL), cortical resection, functional hemispherectomy, and callosotomy were performed according to the results of presurgical evaluations. Results: Patients were grouped into either the temporal lobe epilepsy (TLE) group (n = 12) or the neocortical/multifocal epilepsy group (n = 14) according to the results of presurgical evaluations. Patients were included in the TLE group if there was strong evidence that the mesial temporal lobe was the only ictal-onset area. The other patients were included in the neocortical/multifocal group. There were no significant differences in demographic characteristics between the two groups. All patients in the TLE group became seizure-free after ATL, but only four patients became seizure-free, and additional two patients showed improvement after various surgical procedures in the neocortical/multifocal group ( p = 0.002). Conclusion: Surgical intervention may be helpful in patients with delayed epilepsy in hemiconvulsion-hemiplegia-epilepsy (HHE) syndrome, especially if the mesial temporal lobe appears to be the only epileptogenic area, regardless of the presence of additional abnormalities seen with neuroimaging. Therefore, surgical treatment should be considered for selected patients with delayed epilepsy in HHE syndrome.

A 40-year-old woman who received a liver transplantation developed nystagmus, downward gaze palsy of her right eye, and gradual mental deterioration. CT and MRI (figure) showed signal change in medial thalami and hemorrhage in the bilateral inferior colliculi, suggesting Wernicke encephalopathy. Swelling around the aqueduct resulted in hydrocephalus. After …

Although surgical resection has been an important alternative treatment for patients with intractable epilepsy related to focal cortical dysplasia (FCD), the prognostic relevance of the degree of pathologic severity is controversial and there has been only limited information regarding the prognostic factors involved in the surgical treatment of refractory epilepsy in patients with FCD.We undertook the present study to assess whether the pathologic subtypes of FCD affect surgical outcomes in patients with drug-resistant epilepsy. We also studied the prognostic roles of clinical factors and various diagnostic modalities in the surgical treatment.A total of 166 consecutive patients were included. By univariate analysis, incomplete resection of epileptogenic area (p0.001), mild pathologic features (p = 0.01), and the presence of secondary tonic clonic seizures (2GTCS) (p = 0.05) were associated with poor surgical outcomes. There was a strong tendency for patients with severe pathologic features to have MRI abnormalities (p0.001). Incomplete resection of epileptogenic area (p0.001) and mild pathologic features (p = 0.02) were poor independent outcome predictors on multivariate analysis. The results of MRI, scalp EEG, fluorodeoxyglucose-PET, and ictal SPECT were not associated with surgical outcomes.Our study shows that there is a strong tendency for patients with severe pathologic features to have MRI abnormalities, and patients with incomplete resection, mild pathologic features, or the presence of secondary tonic clonic seizures have a high chance of a poorer surgical outcome.

Thin-section noncontrast CT (NCT) can detect thrombi in large arteries and can provide a measure of thrombus composition based on Hounsfield Units (HU). A study using polyethylene tubes as a model of intracranial arteries concluded that the HUs of platelet-rich thrombi are lower than those of erythrocyte-rich thrombi. Thrombus HUs were measured by NCT in 34 patients with acute ischemic stroke before thrombolysis. Thrombi with lower HU counts were resistant to thrombolytics.