1/75. Neurofibromatosis, stroke and basilar impression. Case report.Neurofibromatosis type 1 (NF1) can virtually affect any organ, presenting most frequently with "cafe au lait" spots and neurofibromas. Vasculopathy is a known complication of NF1, but cerebrovascular disease is rare. We report the case of a 51-year-old man admitted to the hospital with a history of stroke four months before admission. On physical examination, he presented various "cafe au lait" spots and cutaneous neurofibromas. neurologic examination demonstrated right-sided facial paralysis, right-sided hemiplegia, and aphasia. Computed tomography scan of head showed hypodense areas in the basal ganglia and centrum semiovale. Radiographs of cranium and cervical spine showed basilar impression. angiography revealed complete occlusion of both vertebral and left internal carotid arteries, and partial stenosis of the right internal carotid artery. A large network of collateral vessels was present (moyamoya syndrome). It is an uncommon case of occlusive cerebrovascular disease associated with NF1, since most cases described in the literature are in young people, and tend to spare the posterior cerebral circulation. Basilar impression associated with this case may be considered a pure coincidence, but rare cases of basilar impression and NF1 have been described.- - - - - - - - - - ranking = 1keywords = aphasia (Clic here for more details about this article) |
2/75. Is there a syndrome of tuberothalamic artery infarction? A case report and critical review.Short-term post-acute neuropsychological, neurological, and neuroradiological test results and a 16-month follow-up of a 65-year-old patient with a right hemisphere ischemic lesion in the tuberothalamic area of vascular supply are reported. During a 6-week period of examinations the originally left- but trained right-handed patient exhibited fluctuating neuropsychological disorders including aphasia, visuo-perceptive and visuoconstructive disorders, and memory and attention deficits. In the follow-up examination the patient exhibited no aphasia and significant improvements in most neuropsychological tasks. Based on three-dimensional reconstruction of MRI, lesion topography and involvement of thalamic nuclei were established. We discuss the neuropsychological and neurological symptoms of the present case against the background of the 'syndrome of unilateral tuberothalamic artery territory infarction' proposed by Bogousslavsky and coworkers (1986) and the neuropsychological literature on unilateral ischemic anterior/anterolateral thalamic infarction.- - - - - - - - - - ranking = 2keywords = aphasia (Clic here for more details about this article) |
3/75. The corticobasal degeneration syndrome overlaps progressive aphasia and frontotemporal dementia.OBJECTIVE: To provide evidence for the hypothesis that the corticobasal degeneration syndrome (CBDs) overlaps significantly with primary progressive aphasia and frontotemporal dementia, and that CBDs is part of the Pick complex. BACKGROUND: Corticobasal degeneration has been mainly described as a movement disorder, but cognitive impairment is also increasingly noted. methods: Thirty-five cases of clinically diagnosed CBDs were followed-up with clinical, neuropsychological, and neuroimaging investigations. Twenty-nine patients were seen prospectively in movement disorder and cognitive neurology clinics; five of these came to autopsy. Six other autopsied cases that fulfilled the clinical criteria of CBDs were added with retrospective review of records. RESULTS: All 15 patients presenting with movement disorders developed behavioral, cognitive, or language deficits shortly after onset or after several years. Patients presenting with cognitive problems (n = 20), progressive aphasia (n = 13), or frontotemporal dementia (n = 7) developed the movement disorder subsequently. Eleven cases with autopsy had CBD or other forms of the Pick complex. CONCLUSIONS: There is a clinical overlap between CBD, frontotemporal dementia, and primary progressive aphasia. There is also a pathologic overlap between these clinical syndromes. The recognition of this overlap will facilitate the diagnosis and avoid consideration of CBD as "heterogenous."- - - - - - - - - - ranking = 7keywords = aphasia (Clic here for more details about this article) |
4/75. Selective impairment of verb processing associated with pathological changes in Brodmann areas 44 and 45 in the motor neurone disease-dementia-aphasia syndrome.We report six patients with clinically diagnosed and electrophysiologically confirmed motor neurone disease (MND), in whom communication problems were an early and dominant feature. All patients developed a progressive non-fluent aphasia culminating in some cases in complete mutism. In five cases, formal testing revealed deficits in syntactic comprehension. comprehension and production of verbs were consistently more affected those that of nouns and this effect remained stable upon subsequent testing, despite overall deterioration. The classical signs of MND, including wasting, fasciculations and severe bulbar symptoms, occurred over the following 6-12 months. The behavioural symptoms ranged from mild anosognosia to personality change implicating frontal-lobe dementia. In three cases, post-mortem examination has confirmed the clinical diagnosis of MND-dementia. In addition to the typical involvement of motor and premotor cortex, particularly pronounced pathological changes were observed in the Brodmann areas 44 (Broca's area) and 45. The finding of a selective impairment of verb/action processing in association with the dementia/aphasia syndrome of MND suggests that the neural substrate underlying verb representation is strongly connected to anterior cortical motor systems.- - - - - - - - - - ranking = 6keywords = aphasia (Clic here for more details about this article) |
5/75. Transient posterior encephalopathy induced by chemotherapy in children.The cases of three children, 16, 12, and 12 years of age, who suffered sudden confusional state and cortical blindness lasting 12 to 30 minutes while under treatment with high-dose methotrexate, cyclophosphamide, and dactinomycin for a lower limb osteosarcoma are reported. Transient neuropsychologic deficits arose after the acute phase of treatment: left hemispatial neglect and constructive apraxia (Patient 1); constructive apraxia (Patient 2); and constructive apraxia and alexia without aphasia (Patient 3). The three patients recovered completely from all their deficits within the time frame of 3 hours to 2 weeks. Arterial hypertension and hypomagnesemia were found during the acute phase in all patients. In Patients 2 and 3, magnetic resonance imaging revealed increased parieto-occipital T(2) signal involving gray and white matter. In Patients 1 and 2, HmPAO-SPECT revealed parieto-occipital hypoperfusion that resolved a few days later. The alterations detected by neuroimaging were concurrent with the appearance and disappearance of the clinical symptoms. Such transient acute episodes have been named occipital-parietal encephalopathy. On the basis of our clinical, laboratory, and neuroimaging findings, an explanation for the origin of this syndrome, a migrainelike mechanism, triggered by chemotherapy-induced hypomagnesemia, is proposed.- - - - - - - - - - ranking = 1keywords = aphasia (Clic here for more details about this article) |
6/75. frontotemporal lobar degeneration: a study in japan.frontotemporal lobar degeneration is the most common form of cortical dementia occurring in the presenium after Alzheimer's disease. We analyzed two types of frontotemporal dementia (FTD) and semantic dementia (SD) selected from a consecutive series of outpatients based on neuropsychological symptoms, psychiatric symptoms and abnormal behavior. In our series of 134 patients with primary degenerative dementia, there were 16 cases of FTD and 6 cases of SD. Patients with subgroups of FTD and patients with SD were distinguishable only by the presence of aphasia in the latter group. They were not distinguishable from one another by other neuropsychological examinations, behavioral abnormalities or psychiatric symptoms assessed with the Neuropsychiatric Inventory.- - - - - - - - - - ranking = 1keywords = aphasia (Clic here for more details about this article) |
7/75. Subacute encephalopathy with epileptic seizures in alcoholism (SESA): case report.The case of a 66-year-old patient is reported in view of the rarity of his condition: a case of subacute encephalopathy with seizures in alcoholics (SESA syndrome), described first in 1981 by Niedermeyer, et al. Wernicke-type aphasia, epileptic seizures (generalized tonic-clonic) and PLEDs EEG pattern dominated the neurological picture, in addition to hepatomegaly and rhabdomyolysis. This condition differs from all other known CNS complications in chronic alcoholism and is withdrawal-independent. It is prognostically favorable as far as the syndrome as such is concerned.- - - - - - - - - - ranking = 1keywords = aphasia (Clic here for more details about this article) |
8/75. Acute stroke syndrome with fixed neurological deficit and false-negative diffusion-weighted imaging.diffusion-weighted imaging (DWI) is sensitive for the detection of acute ischemic stroke. However, a negative DWI study of the brain does not always exclude a patient from the possibility of acute cerebral ischemia. The authors report 1 case in which the patient presented with a fixed ischemic neurological deficit (National Institute of health stroke Scale score = 22) that included global aphasia, right hemiparesis, and a right visual field neglect. The initial DWI of the brain within 27 hours of symptom onset was negative. The deficit persisted, and a repeat magnetic resonance imaging study 7 days later showed a large area of restricted diffusion involving the gray matter of the entire left middle cerebral artery and anterior cerebral artery distribution, indicating a large area of cortical stroke.- - - - - - - - - - ranking = 1keywords = aphasia (Clic here for more details about this article) |
9/75. Continuous focal spikes during REM sleep in a case of acquired aphasia (landau-kleffner syndrome).We report a girl 3 years and 6 months old with onset of aphasia at age 3 years and 3 months. There was no evidence of brain damage and there were no seizures. The neuropsychological evaluation showed that the girl tended to be right-handed, that aphasia was global and that other higher cortical functions seemed to be preserved. Isolated spikes and spikes-and-wave were recorded during wake over the right temporal region with rare independent contralateral abnormalities. During polysomnography (PSG), the physiological patterns of sleep were preserved and right temporal epileptiform discharges were significantly increased in all sleep stages. Maximal activation was obtained at sleep onset and during rapid eye movement (REM) sleep periods, when focal abnormalities became continuous and spread contralaterally. Repeat PSGs showed that the activation profile retained this particular trait, although subclinical discharges tended to increase during slow wave sleep (SWS). This pattern of subclinical temporal status epilepticus during REM sleep differs from the characteristic activation profile found in the syndrome of continuous spikes-and-waves during SWS. However, this profile was transient and all epileptiform changes disappeared during clinical recovery at 18 months of follow-up.- - - - - - - - - - ranking = 6keywords = aphasia (Clic here for more details about this article) |
10/75. A role of autoimmunity in the etiopathogenesis of landau-kleffner syndrome?Four children, 3 boys and 1 girl, with the syndrome of acquired aphasia Landau-Kleffner were followed up during the last 10 years. In 3 children an episodic form of the disease with a good response to corticosteroid treatment and with a favourable prognosis was observed. In the other 1 no improvement of speech function was observed and also epileptic fits were reduced only with difficulty. A positive autoimmune reaction to central (MOZAN) and peripheral (LISAN) myelin was observed repeatedly during the attacks of clinical worsening. On the contrary, during the periods of improvement of language disturbances during the treatment with corticosteroids this hypersensitivity to central as well as peripheral myelin disappeared. Possible changes in myelinization and the role of autoimmune reactions in the etiopathogenesis of the disease are discussed.- - - - - - - - - - ranking = 1keywords = aphasia (Clic here for more details about this article) |
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