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1/30. allopurinol for refractory aggression and self-inflicted behaviour.

    The impairment of the purinergic system, characterized by reduced adenosinergic activity, has been implicated in the neurobiology of aggressive behaviour. Since there are no direct adenosine agonists available for human use, inhibition of purine degradation by allopurinol was conceived as a possible strategy. We report two cases of adults with refractory aggressive behaviour due to a neurological condition (one mainly with self-inflicted behaviour) with dramatic response to therapy with allopurinol, 300 mg/day p.o. These preliminary results reinforce the involvement of the purinergic system in the neurobiology of aggression, warranting further testing of allopurinol as a new treatment for aggressive and self-inflicted behaviours.
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2/30. Neuropsychological consequences of cerebellar tumour resection in children: cerebellar cognitive affective syndrome in a paediatric population.

    Acquired cerebellar lesions in adults have been shown to produce impairments in higher function as exemplified by the cerebellar cognitive affective syndrome. It is not yet known whether similar findings occur in children with acquired cerebellar lesions, and whether developmental factors influence their presentation. In studies to date, survivors of childhood cerebellar tumours who demonstrate long-term deficits in cognitive functions have undergone surgery as well as cranial irradiation or methotrexate treatment. Investigation of the effects of the cerebellar lesion independent of the known deleterious effects of these agents is important for understanding the role of the cerebellum in cognitive and affective development and for informing treatment and rehabilitation strategies. If the cerebellar contribution to cognition and affect is significant, then damage in childhood may influence a wide range of psychological processes, both as an immediate consequence and as these processes fail to develop normally later on. In this study we evaluated neuropsychological data in 19 children who underwent resection of cerebellar tumours but who received neither cranial irradiation nor methotrexate chemotherapy. Impairments were noted in executive function, including planning and sequencing, and in visual-spatial function, expressive language, verbal memory and modulation of affect. These deficits were common and in some cases could be dissociated from motor deficits. Lesions of the vermis in particular were associated with dysregulation of affect. Behavioural deficits were more apparent in older than younger children. These results reveal that clinically relevant neuropsychological changes may occur following cerebellar tumour resection in children. Age at the time of surgery and the site of the cerebellar lesion influence the neurobehavioural outcome. The results of the present study indicate that the cerebellar cognitive affective syndrome is evident in children as well as in adults, and they provide further clinical evidence that the cerebellum is an essential node in the distributed neural circuitry subserving higher-order behaviours.
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ranking = 0.25
keywords = behaviour
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3/30. Rule-abstraction deficits following a basal ganglia lesion.

    The cognitive profile of a patient, PM, who had damage to the right basal ganglia as the result of a stroke was investigated. Whilst most cognitive functions were intact, she showed specific neuropsychological deficits, most notably a difficulty in developing, through abstraction of the relevant information, a higher-level rule by which to guide behaviour. The types of rule affected were those based upon an attentional set (attending to a particular dimension of stimulus features, such as 'shape') or a response strategy (continuing to apply a previously successful pattern of responses). The learning of lower-level rules based on stimulus-reward values was spared, as was the ability to apply an instructed rule and to discontinue use of rules which were no longer appropriate. These data provide evidence for the dissociability of cognitive functions within the basal ganglia.
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ranking = 0.125
keywords = behaviour
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4/30. chondroblastoma of the lumbar spine with cauda equina syndrome.

    STUDY DESIGN: Case report. OBJECTIVE: To describe the clinical presentations, radiological features and clinical progress of a rare case of chondroblastoma of the lumbar spine. SETTING: Regional Hospital, hong kong, china. METHOD: A 54-year-old male patient presented with low back pain and left sciatica. X-ray and MRI revealed tumour infiltration of the fifth lumbar vertebrae and left paraspinal muscles, which was found to be a chondroblastoma by repeated open biopsies. The tumour was removed surgically by combined anterior and posterior approaches, followed by spinal fusion and instrumentation. RESULTS: The anterior tricortical bone graft was complicated with fracture and nonunion. Surgical re-exploration confirmed local recurrence of tumour macroscopically and histologically. The patient developed symptoms and signs of cauda equina syndrome gradually despite repeated surgery and irradiation. The patient eventually died of complications of local recurrence and neurological deficit at 3 years and 8 months after the first operation. CONCLUSION: This is the first case report of chondroblastoma of the lumbar spine. The clinical profile of this patient and the evidence from the literature review suggests that spinal chondroblastoma has a very aggressive behaviour with high recurrence and mortality rate.
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ranking = 0.125
keywords = behaviour
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5/30. Long term effects of bilateral subthalamic nucleus stimulation on cognitive function, mood, and behaviour in Parkinson's disease.

    BACKGROUND: Long term effects of subthalamic nucleus (STN) stimulation on cognition, mood, and behaviour are unknown. OBJECTIVE: This study evaluated the cognitive, mood, and behavioural effects of bilateral subthalamic nucleus deep brain stimulation (STN DBS) in patients with Parkinson's disease (PD) followed up for three years. methods: A consecutive series of 77 PD patients was assessed before, one, and three years after surgery. Mean (SD) age at surgery was 55 (8). Seven patients died or were lost for follow up. Neuropsychological assessment included a global cognitive scale, memory, and frontal tests. depression was evaluated using the Beck depression inventory. Assessment of thought disorders and apathy was based on the unified Parkinson's disease rating scale. Reports of the behavioural changes are mainly based on interviews done by the same neuropsychologist at each follow up. RESULTS: Only two cognitive variables worsened (category fluency, total score of fluency). Age was a predictor of decline in executive functions. depression improved whereas apathy and thought disorders worsened. Major behavioural changes were two transient aggressive impulsive episodes, one suicide, four suicide attempts, one permanent apathy, one transient severe depression, four psychoses (one permanent), and five hypomania (one permanent). CONCLUSIONS: Comparing baseline, one year, and three year postoperative assessments, STN stimulation did not lead to global cognitive deterioration. apathy scores mildly increased. depression scores mildly improved. Behavioural changes were comparatively rare and mostly transient. Single case reports show the major synergistic effects of both medication and stimulation on mood and behaviour, illustrating the importance of a correct postoperative management.
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ranking = 1.125
keywords = behaviour
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6/30. Status dissociatus after surgery for tegmental ponto-mesencephalic cavernoma: a state-dependent disorder of motor control during sleep.

    After surgery for a tegmental ponto-mesencephalic cavernoma, a patient developed sleep-related excessive fragmentary myoclonus, diffuse myoclonic jerks, simple quasipurposeful movements of the limbs, and rapid eye movement (REM) sleep behaviour disorder as motor features of status dissociatus, a condition in which elements of one state of being (wake, NREM and REM sleep) pathologically intrude into another.
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ranking = 0.125
keywords = behaviour
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7/30. Case report: colonic manometry reveals abnormal propulsive behaviour after anterior resection of the rectum.

    Anterior resection of the rectum is a frequent surgical procedure. However, abnormal bowel habits following this procedure are frequently reported. The functional evaluation of these patients is usually limited to the anorectal area. By means of colonic manometry, we have evaluated a patient with frequent urge for defecation and increased bowel frequency following anterior resection of the rectum with straight coloanal anastomosis and almost normal anorectal function. Analysis of the tracing revealed a reduction of contractile segmental activity and much more high-amplitude propagated contractions than which occur in healthy subjects. These high-amplitude propagated contractions, representing the manometric equivalent of mass movements, were always in association with urge for defecation and, sometimes, with loose stools. High-amplitude simultaneous contractions were also observed. We feel that the surgical resection of a potential physiological brake may be responsible for these observations.
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ranking = 0.5
keywords = behaviour
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8/30. Long-term behavioural changes after unilateral stereotactic cingulotomy in a case of therapy-resistant alcohol dependence.

    We report the case of a female patient who had undergone a unilateral stereotactical anterior cingulotomy (AC) 20 years previously because of refractory alcohol dependence. After AC, the drinking behaviour switched from a chronic to an episodic one with fast losses of control accompanied by abnormal oral impulse-control behaviour. Relapses were stress- but not cue-induced and followed by long-term intervals of abstinence. In addition to an intended lesion of the left dorsal anterior cingulate cortex (ACC), MR images showed additional lesions within the left caudate body and the dorsal medial thalamic nucleus. Applying a neuropsychological test battery, we found disturbed divided attention and impaired executive function. From this, we conclude that the complex lesion pattern may contribute to some of the behavioural changes seen in our patient after AC. This hypothesis is supported by the neuropsychological deficits and the fact that the neuronal circuits, impaired by the lesions, are involved in addiction-specific behaviour. This case report further emphasizes the key role of the ACC and its connections in the maintenance of dependent behaviour.
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ranking = 1.125
keywords = behaviour
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9/30. Hypomania as an adverse effect of subthalamic nucleus stimulation: report of two cases.

    Mania following subthalamic nucleus (STN) deep brain stimulation (DBS) is well described and obvious to both the patient and their physician. The authors describe two patients who developed hypomania following STN-DBS but were unaware of their mood disturbance. Two Parkinson's patients with no previous mood disorders received bilateral STN electrodes. Both experienced dramatic improvement in their motor function and neither complained of any side effects. Their families reported detrimental hypomanic behaviour. Readjusting the stimulation parameters resolved the hypomania with continued motor benefits. The authors draw attention to potential adverse effects of STN-DBS that might be neglected by patients.
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ranking = 0.125
keywords = behaviour
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10/30. Psychogenic seizures in brain injury: diagnosis, treatment and case study.

    Psychogenic seizures are a functional phenomena in which a person experiences a paroxysmal event that may be interpreted as epileptiform in nature. By definition, psychogenic seizures imply a sudden episode of change in behaviour or psychic state that is not associated with an identifiable process, either vasculogenic or neurogenic, and during which there is an absence of characteristic epileptiform changes on the electroencephalogram. prevalence rates range from 5% to 50% in outpatient populations seen in epilepsy clinics. However, approximately 20% of true seizure patients also have psychogenic seizures. As psychogenic seizures are not a pathophysiological phenomena, pharmacological interventions do not alter their aetiology and can cloud the cognitive skills necessary to ameliorate the psychogenic seizure behaviour. In non-aphasic true seizure patients, as well as psychogenic seizure patients, self-control relaxation paradigms have been successful where pharmacological intervention has failed. This case involved an aphasic brain-injured patient who had both psychogenic and true seizures. For this patient, the self-control paradigm was modified to include use of gesture and prosody to achieve similar psychotherapeutic effects. Additionally, family therapy was instituted to provide a constructive means for the patient's family to participate in the reduction of psychogenic seizures. As seizures are a common sequelae of brain injury, the differential diagnosis of seizures and knowledge of standard and alternative treatment is essential for rehabilitation professionals.
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ranking = 0.25
keywords = behaviour
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