BACKGROUND: Fatigue is a common and disabling symptom of multiple sclerosis (MS). Previous studies reported that damage of the corticostriatothalamocortical circuit is critical in its occurrence. OBJECTIVE: To investigate the relationship between fatigue in MS and regional cortical and subcortical gray matter atrophy. DESIGN: Case-control study. SETTING: National Institutes of Health. PARTICIPANTS: Twenty-four patients with MS and 24 matched healthy volunteers who underwent 3.0-T magnetic resonance imaging and evaluations of fatigue (Modified Fatigue Impact Scale) and depression (Center for Epidemiologic Studies Depression Scale). MAIN OUTCOME MEASURES: Relationship between thalamic and basal ganglia volume, cortical thickness of frontal and parietal lobes, and, in patients, T2 lesion volume and normal-appearing white matter volume and the extent of fatigue. RESULTS: Patients were more fatigued than healthy volunteers (P = .04), while controlling for the effect of depression. Modified Fatigue Impact Scale score correlated with cortical thickness of the parietal lobe (r = -0.50, P = .01), explaining 25% of its variance. The posterior parietal cortex was the only parietal area significantly associated with the Modified Fatigue Impact Scale scores. CONCLUSIONS: Cortical atrophy of the parietal lobe had the strongest relationship with fatigue. Given the implications of the posterior parietal cortex in motor planning and integration of information from different sources, our preliminary results suggest that dysfunctions in higher-order aspects of motor control may have a role in determining fatigue in MS.

OBJECTIVES: We undertook to determine whether paced vocal fold adduction can check aspiration in patients with various neurologic conditions. METHODS: Five patients with fluoroscopically documented aspiration and repeated pneumonias were enrolled. Two previously reported patients with hemispheric stroke were compared to 3 additional subjects with brain stem-basal ganglia and cerebellar stroke, cerebral palsy, and multiple sclerosis. A modified Vocare stimulator was implanted subcutaneously and linked to the ipsilateral recurrent laryngeal nerve via perineural electrodes. Vocal fold adduction and glottic closure were effected with pulse trains (42 Hz; 1.2 mA; 188 to 560 micros) and recorded with Enhanced Image J. Fluoroscopy results with and without stimulation were assessed by 2 independent blinded reviewers. Pneumonia rates were compared before, during, and after the 6- to 12-month enrollment periods. RESULTS: There was statistically significant vocal fold adduction (p < 0.05) for all patients, further verified with bolus arrest (p < 0.05 for thin liquids, thick liquids, and puree depending on the speech-language pathologist). Pneumonia was prevented in 4 of the 5 patients during enrollment. In the fifth patient, who had brain stem-basal ganglia and cerebellar stroke, we were unable to completely seal the glottis and open the cricopharyngeus enough to handle his secretions. CONCLUSIONS: Vocal fold pacing for aspiration pneumonia from a variety of neurologic insults appears to be appropriate as long as the glottis can be sealed. It is not sufficient when the cricopharyngeus must be independently opened.

The 13513G>A mutation in the ND5 gene of mitochondrial DNA (mtDNA) is usually associated with mitochondrial encephalomyopathy with lactate acidosis and stroke-like episodes (MELAS), or Leigh syndrome (LS). In this study, we describe three young Chinese patients with MELAS/LS overlap syndrome who carried the m.13513G>A mutation. Clinical and MRI features were characteristic of both MELAS and LS. Interestingly, the clinical presentation of this overlap syndrome could be variable depending on the degree of relative contribution of MELAS and LS, that is, MELAS as the initial presenting syndrome, LS as the predominant syndrome, or both MELAS and LS appearing at the same time. The final brain MRI showed findings characteristic of both MELAS and LS, with asymmetrical lesions in the cortex and subcortical white matter of the occipital, temporal, and frontal lobes (MELAS), and bilateral and symmetrical lesions in the basal ganglia and brainstem (LS). Brain autopsy in one case revealed infarct-like lesions in the cerebral cortex, basal ganglia and brainstem, providing further insight into the distribution of the pathological lesions in MELAS/LS overlap syndrome. This is the first report of the brain pathological changes in a patient with m.13513G>A mutation. The spatial distribution of infarct-like lesions in the brain could explain the symptoms in MELAS/LS overlap syndrome.

PURPOSE: Current rabbit stroke models often depend on symptoms as endpoints for embolization and produce wide variation in location, size, and severity of strokes. In a further refinement of an angiographic embolic stroke model, localized infarctions were correlated to neurologic deficits with the goal to create a rabbit model for long-term studies of therapies after stroke. MATERIALS AND METHODS: New Zealand White rabbits (4-5 kg; N = 71) had selective internal carotid artery (ICA) angiography and a single clot was injected. At 24 hours, neurologic assessment score (NAS) was measured on an 11-point scale (0, normal; 10, dead). Brains were removed and stained to identify stroke areas. All animals with single strokes (n = 31) were analyzed by specific brain structure involvement, and NAS values were correlated. RESULTS: Stroke incidence differed by location, with cortex, subcortical, and basal ganglia regions highest. The middle cerebral artery (MCA), at 52%, and anterior cerebral artery (ACA), at 29%, were most commonly involved, with the largest stroke volumes in the ACA distribution. Brainstem and cerebellum strokes had disproportionately severe neurologic deficits, scoring 2.25 +/- 1.0 on the NAS, which represented a significant (P < .02) difference versus cortex (0.5 +/- 0.2), subcortical (1.3 +/- 0.4), and basal ganglia (0.5 +/- 0.3), all in the frontal or parietal regions. CONCLUSIONS: MCA and ACA distributions included 81% of strokes. These sites were relatively silent (potentially allowing longer-term survival studies) whereas others in the posterior circulation produced disproportionately severe symptoms. Symptoms were not reliable indicators of stroke occurrence, and other endpoints such as imaging may be required. These are important steps toward refinement of the rabbit stroke model. Copyright © 2010 SIR. Published by Elsevier Inc. All rights reserved.

We report the case of a patient suffering from sudden apathy and pathological gambling-like behaviour after bilateral ischemic lesions involving the dorsal portion of the head of the caudate nuclei and adjacent anterior limb of the internal capsules. This is the first report of the association of an apathy and abnormal gambling behaviour following a stroke affecting sub-cortical structures. Although the location of the lesions, affecting the dorsal striatum, may explain the emergence of an apathetic state, it is, however, at first sight, not easy to explain the gambling behaviour because the patient was normal in tests evaluating sensitivity to reward, and no radiological abnormality was found in the cortical-sub-cortical system of reward. It is proposed that, for this patient, the mechanism of maladaptive gambling behaviour was the development of a routine behaviour related to the patient’s cognitive inertia, a mechanism different from the changes in reward sensitivity observed after damage to the orbital ventral prefrontal-ventral striatum system or in dopamine dysregulation syndrome in Parkinson’s disease.

OBJECTIVE:: Recent technical developments have led to an extension of perfusion computed tomography (PCT) scan range to cover nearly the entire brain and to reconstruct time resolved (4d) CT-angiography (CTA) datasets from the PCT data. The purpose of this study was to compare the results of simulated standard PCT and extended PCT with 4d-CTA. MATERIALS AND METHODS:: Extended multimodal stroke CT (unenhanced cranial CT, CTA, and PCT) was acquired in 72 patients. PCT images with a scan coverage of 9.6 cm in the z-axis, simulated 2 cm PCT images at the level of the basal ganglia comparable to standard PCT, standard supra-aortic CTA, and 4d-CTA images were reconstructed. Two readers assessed the PCT image quality as well as pathologic findings in extended and simulated PCT, CTA, and 4d-CTA. The brain was divided into 4 axial segments. The independent samples t test was applied to test differences between data for significance. RESULTS:: In 75.0% of all patient exams, pathologic findings were observed in the PCT; these were located in 138 brain segments. In 24.1% of all 54 exams with pathologic PCT findings, the pathology would have been missed on standard PCT. The longer scan coverage resulted in a different final diagnosis in 34.7% of all exams. Quality of the PCT parameter maps was on average very good both for the supratentoric and the infratentoric brain areas (4.28 and 4.18, respectively, on a 5-point scale). In 90% of all exams with pathologic changes in the CTA, these abnormalities were also noted on 4d-CTA. In only 2.8% of all cases, the additional time resolution of the 4d-CTA provided additional information. CONCLUSION:: Extending the scan coverage of PCT from 2 cm to 9.6 cm led to an augmentation of clinically important information in the imaging of acute stroke.

Celiac disease (CD) is manifested by a variety of clinical signs and symptoms that may begin either in childhood or adult life. Neurological symptoms without signs of malabsorption have been observed for a long time in CD. In this report, an 8-year-old girl with CD presented with rarely seen dilated cardiomyopathy and stroke. The girl was admitted with left side weakness. Her medical history indicated abdominal distention, chronic diarrhea, failure to thrive, and geophagia. On physical examination, short stature, pale skin and a grade 2 of 6 systolic murmur were detected. Muscle strength was 0/5 on the left side, and 5/5 on the right side. Coagulation examinations were normal. Tests for collagen tissue diseases were negative. Factor V Leiden and prothrombin GA20210 mutations were negative. Tandem mass spectrophotometry and blood carnitine profiles were normal. Brain magnetic resonance imaging and cerebral angiography showed an infarction area at the basal ganglia level. Examinations of serologic markers and intestinal biopsy revealed CD. We emphasize that in differential diagnosis of ischemic stroke, CD should be kept in mind.