1/16. Dyspraxia in a patient with corticobasal degeneration: the role of visual and tactile inputs to action.OBJECTIVES: To investigate the roles of visual and tactile information in a dyspraxic patient with corticobasal degeneration (CBD) who showed dramatic facilitation in miming the use of a tool or object when he was given a tool to manipulate; and to study the nature of the praxic and neuropsychological deficits in CBD. methods: The subject had clinically diagnosed CBD, and exhibited alien limb behaviour and striking ideomotor dyspraxia. General neuropsychological evaluation focused on constructional and visuospatial abilities, calculation, verbal fluency, episodic and semantic memory, plus spelling and writing because impairments in this domain were presenting complaints. Four experiments assessed the roles of visual and tactile information in the facilitation of motor performance by tools. Experiment 1 evaluated the patient's performance of six limb transitive actions under six conditions: (1) after he described the relevant tool from memory, (2) after he was shown a line drawing of the tool, (3) after he was shown a real exemplar of the tool, (4) after he watched the experimenter perform the action, (5) while he was holding the tool, and (6) immediately after he had performed the action with the tool but with the tool removed from his grasp. Experiment 2 evaluated the use of the same six tools when the patient had tactile but no visual information (while he was blindfolded). Experiments 3 and 4 assessed performance of actions appropriate to the same six tools when the patient had either neutral or inappropriate tactile feedback-that is, while he was holding a non-tool object or a different tool. RESULTS: Miming of tool use was not facilitated by visual input; moreover, lack of visual information in the blindfolded condition did not reduce performance. The principal positive finding was a dramatic facilitation of the patient's ability to demonstrate object use when he was holding either the appropriate tool or a neutral object. Tools inappropriate to the requested action produced involuntary performance of the stimulus relevant action. CONCLUSIONS: Tactile stimulation was paramount in the facilitation of motor performance in tool use by this patient with CBD. This outcome suggests that tactile information should be included in models which hypothesise modality specific inputs to the action production system. Significant impairments in spelling and letter production that have not previously been reported in CBD have also been documented.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
2/16. Rapid growth in a cavernoma.The availability of magnetic resonance imaging has greatly increased the detection of cavernous malformations of the central nervous system in both symptomatic and asymptomatic patients. These lesions may be responsible for previously unexplained neurological events or may even have been incorrectly diagnosed. We describe a patient presenting with focal neurological signs in whom an initial diagnosis of malignant glioma had been made. Following excision, the pathological diagnosis was cavernoma. This vascular lesion has continued to show rapid growth and aggressive behaviour despite multiple surgical resections. The indications for operative and non-operative intervention will be discussed.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
3/16. Involuntary hand levitation associated with parietal damage: another alien hand syndrome.The alien hand syndrome (AHS) usually consists of an autonomous motor activity perceived as an involuntary and purposeful movement, with a feeling of foreignness of the involved limb, commonly associated with a failure to recognise ownership of the limb in the absence of visual clues. It has been described in association to lesions of the frontal lobes and corpus callosum. However, parietal damage can promote an involuntary, but purposeless, hand levitation, which, sometimes, resembles AHS. In the present study, four patients (cortico-basal ganglionic degeneration - n=2; Alzheimer's disease - n=1 and parietal stroke - n=1) who developed alien hand motor behaviour and whose CT, MRI and/or SPECT have disclosed a major contralateral parietal damage or dysfunction are described. These results reinforce the idea that parietal lobe lesions may also play a role in some patients with purposeless involuntary limb levitation, which is different from the classic forms of AHS.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
4/16. Extra-pyramidal parkinsonism complicating organophosphate insecticide poisoning.We present a 1 7-year-old female with acute extra-pyramidal parkinsonism complicating a suicidal attempt with the organophosphate insecticide chlorpyrifos, who was initially suspected to have developed severe depression or psychosis. On admission she was stupurous, with diarrhoea and massive salivation lapsing into respiratory failure and coma. Following atropine and toxogonin treatment along with mechanical ventilation she developed overt extrapyramidal parkinsonism and encephalopathy, characterized by impaired sensorium and agitation, mask facies along with a muffled voice and swallowing impairment, a resting tremor with cogwheel rigidity switching to bradykinetic choreoathetotic movements. Once a parkinsonian syndrome was diagnosed, she was given amantadine therapy with complete recovery. The patient is presently maintained on amantadine therapy; there was mild worsening of her extrapyramidal signs following unplanned discontinuation of this medication, and on follow-up assessments after 9 months she is virtually asymptomatic. A parkinsonian extrapyramidal syndrome, complicating organophosphate intoxication, should therefore also be taken into account in any patient with organophosphate poisoning, presenting with marked behavioural alterations, rigidity or akinetic mutism, and beneficial response to amantadine.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
5/16. Vulnerability to neuroleptic side effects in frontotemporal lobar degeneration.BACKGROUND: frontotemporal lobar degeneration (FTLD) is commonly associated with behavioural disturbances such as disinhibition and aggression; these often result in the use of neuroleptic medication. methods: All available case notes of patients attending a specialist cognitive disorders clinic with a diagnosis of FTLD were selected. This gave 100 subjects (62 male, 38 female). RESULTS: In 61 patients significant behavioural disturbances were present. Of these patients, 24 had been prescribed neuroleptics. Significant extrapyramidal side effects were reported in eight patients (33%); in five patients these were severe enough to cause severe mobility problems and in one patient resulted in impaired consciousness. In some instances the extrapyramidal side effects took weeks to wear off. CONCLUSION: These results suggest that patients with FTLD may, as in Lewy body dementia, be particularly sensitive to the extrapyramidal side effects of neuroleptics. We suggest that neuroleptics should be used cautiously in FTLD and treatment should be started at low doses avoiding depot preparations until further prospective studies have been performed.- - - - - - - - - - ranking = 2keywords = behaviour (Clic here for more details about this article) |
6/16. Apraxia of eyelid opening in a case of atypical corticobasal degeneration.Apraxia of eyelid opening (AEO) occurs in several clinical conditions, even in the absence of any other neurological sign; nonetheless, in most of the cases AEO has been reported in association with basal ganglia diseases, such as corticobasal degeneration (CBD). We describe a patient with a clinical diagnosis of frontotemporal dementia who, later, developed parkinsonian signs and AEO. We suggest that the finding of AEO in patients with a frontotemporal syndrome could be a helpful expedient for the early diagnosis of atypical clinical findings of CBD, characterised by behavioural and cognitive aspects at first.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
7/16. hysteria following brain injury.Of 167 patients referred to a unit treating severe behaviour disorders after brain injury, 54 showed clinical features closely resembling those of gross hysteria as described by Charcot. Close correlation was found with very diffuse insults (hypoxia and hypoglycaemia), but not with severity of injury or with family or personal history of hysterical or other psychiatric disorder. The findings may have implications for the understanding of the nature of hysteria.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
8/16. Adverse effects of dopamine potentiation by long-term treatment with selegiline.A patient with triosephosphate isomerase (TPI) deficiency exhibited worsening of abnormal involuntary movements of the dystonic type and developed psychiatric symptoms while on selegiline. When selegiline was stopped after 9 years of treatment, abnormal involuntary movements improved to pretreatment level and psychiatric behaviour returned to normal. monoamine oxidase-B platelet activity was low in this patient.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
9/16. Subacute dementia and imaging correlates in a case of Fahr's disease.We report a case of idiopathic bilateral basal ganglia calcinosis, or Fahr's disease (FD) in a 50 year old patient who developed rapidly progressive behavioural abnormalities and severe neuropsychological impairments, but no movement disorder. Neuropsychological deficits included a severe dysexecutive syndrome, anterograde amnesia, and attentional impairment. Neuropsychiatric features comprised apathy with intermittent disinhibition, anxiety, irritability, frequent mood changes, ritualistic and antisocial behaviour, and psychosis. Fluorodeoxyglucose positron emission tomography showed a massive reduction of glucose metabolism in the basal ganglia and the frontal brain. The observed abnormalities possibly result from a disruption of frontostriatal circuits, presumably at the basal ganglia level. This case indicates that FD may cause exclusively behavioural alterations and that the associated hypometabolism in certain frontal areas is closely related to the clinical picture.- - - - - - - - - - ranking = 3keywords = behaviour (Clic here for more details about this article) |
10/16. Fahr's syndrome presenting with pure and progressive presenile dementia.Fahr's syndrome involves calcification of basal ganglia and dentate nuclei of the cerebellum. Clinically it may present with an array of movement disorders, dementia and other behavioural disturbances. Sporadic and familial cases have been reported with or without calcium/phosphorus metabolism. A rare form of frontotemporal dementia with neurofibrillary tangles and Fahr-type calcifications (DNTC) has been observed mainly in japan. We report the singular case of a 50-year-old woman with progressive dementia but neither extrapyramidal symptoms nor a metabolic disorder. brain CT showed Fahr-type calcifications in the basal ganglia, cerebellum and centrum semiovale as well as temporal atrophy; MRI showed diffuse atrophy predominantly in parietotemporal regions. The clinical and radiological features of our patient point to this uncommon form of dementia.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
| | Next -> |