1/31. akinetic mutism and mixed transcortical aphasia following left thalamo-mesencephalic infarction.A 54-year-old man developed somnolent akinetic mutism and acute mixed transcortical aphasia following a left thalamo-mesencephalic infarction. He also exhibited behavioural changes, namely apathy, slowness, lack of spontaneity, disinhibition, perseveration, gait apraxia and incontinence consistent with frontal lobe dysfunction. Presumably the akinetic mutism and language dysfunction were due to the thalamic stroke. All the manifestations could be related to interruption of the frontal-subcortical circuitry.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
2/31. Forced hyperphasia and environmental dependency syndrome.A distinctive, language related fragment of the environmental dependency syndrome is described: compulsive, involuntary, environmentally dependent speaking. Because this syndrome represents the observe of aphasia, it is named forced hyperphasia. An 84 year old woman with acute left frontal infarction was admitted to hospital with gait disturbance, forced grasp reflex, and striking imitation behaviour. After 2 weeks her imitation behaviour disappeared, but an equally striking new behaviour emerged. In the presence of others she would call out the names of objects in the room, and also call out the actions and gestures of people in the room, even though she was not asked to do so, and even though she was asked to stop. For example, if the doctor scratched his nose, she said, "The doctor is scratching his nose." brain CT, MRI, and SPECT showed cerebral atrophy and a left superior frontal subcortical infarct. It is suggested that "forced hyperphasia" is a clinical fragment of the environmental dependency syndrome and that her compulsive, impulsive, involuntary release of spoken language resulted from the release of frontal inhibition of the complex reflex linking environmental cues to the set of motor, limbic, spatial, and linguistic associations underlying spoken language.- - - - - - - - - - ranking = 3keywords = behaviour (Clic here for more details about this article) |
3/31. Childhood epilepsy with neuropsychological regression and continuous spike waves during sleep: epilepsy surgery in a young adult.We describe the case of a man with a history of complex partial seizures and severe language, cognitive and behavioural regression during early childhood (3.5 years), who underwent epilepsy surgery at the age of 25 years. His early epilepsy had clinical and electroencephalogram features of the syndromes of epilepsy with continuous spike waves during sleep and acquired epileptic aphasia (landau-kleffner syndrome), which we considered initially to be of idiopathic origin. seizures recurred at 19 years and presurgical investigations at 25 years showed a lateral frontal epileptic focus with spread to Broca's area and the frontal orbital regions. Histopathology revealed a focal cortical dysplasia, not visible on magnetic resonance imaging. The prolonged but reversible early regression and the residual neuropsychological disorders during adulthood were probably the result of an active left frontal epilepsy, which interfered with language and behaviour during development. Our findings raise the question of the role of focal cortical dysplasia as an aetiology in the syndromes of epilepsy with continuous spike waves during sleep and acquired epileptic aphasia.- - - - - - - - - - ranking = 2keywords = behaviour (Clic here for more details about this article) |
4/31. Non-convulsive status epilepticus with marked neuropsychiatric manifestations and MRI changes after treatment of hypercalcaemia.We describe a 77-year-old woman who developed a confusional state, cognitive impairment, behavioural abnormalities and dysphasia after treatment of hypercalcaemia. Repeated EEG recording revealed rhythmic sharp-wave activity over the right parietal-occipital lobe. magnetic resonance imaging (MRI) showed marked hyperintense signal changes bilaterally. The diagnosis of a non-convulsive status epilepticus (NCSE) was made. With antiepileptic treatment the patient improved and MRI as well as EEG changes were almost all reversible. NCSE is an important differential diagnosis of patients with neuropsychiatric symptoms and can develop after rapid lowering of serum calcium levels in hypercalcaemia.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
5/31. 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 = 1keywords = behaviour (Clic here for more details about this article) |
6/31. Direct and indirect therapy for word sound deafness.This paper evaluates two therapy programmes conducted with PK, an aphasic individual with word sound deafness. The first aimed directly to improve discrimination skills, using minimal pair tasks supported with lip reading. Disappointingly, there were no changes on discrimination tests after this therapy, even when PK could benefit from lip reading information. The second, indirect, programme of therapy aimed to change the communication behaviours of PK's wife. Strategies such as writing and simplifying information were modelled and practiced. A detailed information booklet, outlining the target strategies and explaining PK's main strengths and weaknesses supported the programme. Evaluation of this therapy involved pre- and post-therapy analysis of interactions between PK and his wife, with biographical questions forming the basis of the interactions. There were several changes after therapy on this measure. The number and length of communication breakdowns were reduced, and more questions were answered accurately. This study suggests that, in some cases of severe word sound deafness, indirect therapies may be most effective.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
7/31. The many faces of crossed aphasia in dextrals: report of nine cases and review of the literature.A substantial body of the aphasia literature has been devoted to the topic of crossed aphasia in dextrals (CAD) over the past century but still no theory exists that explains the anomalous organization of neurocognitive functions in this population. However, if strict selection criteria are applied only some cases of vascular CAD are reported in which the correlation between neurocognitive disturbances and the locus of the brain lesion is studied. This study describes nine new cases of vascular CAD who underwent in-depth neurolinguistic and neuropsychological investigations. Our analysis shows the semiological variability of CAD phenomena and the many faces of the lesion-behaviour relationships of this exceptional neurobiological condition.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
8/31. frontotemporal dementia: a clinical-pathological study.We report a 44-year-old female patient without any familial history of dementia presenting with increasing disturbances in behaviour and language followed by a progressive cognitive deterioration. Neuropsychological evaluation revealed a significant impairment on frontal lobe tests. A brain PET scan disclosed a severe frontal hypometabolism. The tentative diagnosis of frontotemporal dementia was made. Her condition rapidly worsened and she died 2 years after the beginning of her disease. Gross examination of the brain showed a selective symmetrical atrophy of both frontal and anterior part of the temporal lobes. Microscopical examination revealed severe neuronal loss in the frontal and anterior temporal cortex associated with gliosis and microvascular spongiosis in the superficial cortical layers in the absence of any specific neuronal or glial inclusions. These neuropathological findings were consistent with the diagnosis of dementia lacking distinctive histology. We discuss the nosology of the frontotemporal dementias, the diagnostic value of PET scan, the recent genetical developments which strongly support the pathogenic role of tau and we emphasize the importance of immunohistochemical examination for a definite neuropathological diagnosis.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
9/31. [Transcortical aphasia and echolalia; problems of speech initiative]Transcortical aphasia accompanied by echolalia occurs with malacias involving the postero-median part of the frontal lobe which includes the supplementary motor field of Penfield and is nourished by the anterior cerebral artery. The syndrome manifests itself in such cases even in fine detials in the same form as does in Pick's atrophy. The same also holds true for cases in which a tumour involves the region mentioned. Sentences or fragments of sentences are echolalised; tendency to perseveration is very marked. It is hardly, if at all, possible to evaluate the verbal understanding of these patients. Analysis of their behaviour supports the assumption that they have not lost the adaptation to some situations. echolalia is often associated with forced grasping and other compulsory phenomena. Therefore, it may be interpreted as a sign of disinhibition of the acusticomotor reflex present during the development of the speech. Competition between the intentionality and the appearance of compulsory phenomena greatly depends on the general condition of the patient, particularly on the clarity of consciousness. The integrity of the postero-median part of the frontal lobe is indespensable for a normal reaction by speech to stimuli received from the sensory areas. The influence of the supplementary motor field on speech intention seems to be linked to the dominant hemisphere. In case lesions of the territory of the anterior cerebral artery and the cortico-bulbar neuron system are coexisting in the dominant hemisphere, the speech disturbance shifts to complete motor aphasia. In such cases the pathomechanism is analogous to that of the syndrome of Liepmann, i.e., right-sided hemiparesis with left-sided apraxia. So-called transcortical motor aphasia without echolalia can be caused by loss of stimuli from the sensory fields.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
10/31. Strategic infarcts of the thalamus in vascular dementia.Strategic infarcts or focal hemorrhages involving the paramedian nuclei of the thalamus may alter consciousness and produce complex neuropsychological symptoms such as impairment of memory, attention and motivation. Lesions disrupting the thalamo-prefrontal circuits lead to severe subcortical dementia. We analysed here the clinical, neuropsychological and neuroimaging data of 19 patients with cerebrovascular lesions in the thalamus. In six patients with bilateral paramedian infarcts, and in two with anterior thalamic infarcts, vascular dementia and severe personality changes developed. SPECT findings did not correlate with the neuropsychological symptoms. Transcortical sensory and motor aphasia was observed in four patients with thalamic hemorrhages and infarcts. In these patients SPECT detected hypoperfusion in adjacent cortical areas. Clinical symptoms and outcome of four patients are reported in detail. The clinico-morphological correlations of the thalamo-cortical circuits are reviewed and the possible causes of multiple cognitive and behavioural consequences of vascular thalamic lesions are discussed.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
| | Next -> |