1/122. Persistent kluver-bucy syndrome after bilateral thalamic infarction.OBJECTIVE: To describe a patient who exhibited a partial kluver-bucy syndrome after small bilateral ischemic lesions in the thalami. BACKGROUND: Previously reported patients with kluver-bucy syndrome had very large, mostly bilateral lesions in the limbic system and could not provide sufficient information about its anatomo-functional correlate. METHOD: Behavioral assessments and clinical examinations, including magnetic resonance imaging and positron emission tomography, were conducted. RESULTS: The patient was severely amnestic, distractible, hyperoral, and affectively dyscontrolled, and she behaved socially inappropriately. magnetic resonance imaging showed bilateral infarctions in the territories of both thalamoperforating arteries, and positron emission tomography revealed bilaterally decreased fluorodeoxyglucose uptake in the anterior parts of the ventral thalami and, to a lesser extent, in the fronto-temporal cortices. CONCLUSIONS: This behavioral syndrome has not yet been reported with isolated diencephalic lesions, but it has been observed after bilateral temporal lobe lesions. The authors conjecture that this syndrome resulted from a disruption of the pathways connecting the dorsomedial thalami with the prefrontal cortices and with other limbic areas, systems essential for memory and the regulation of impulses and emotions.- - - - - - - - - - ranking = 1keywords = motion (Clic here for more details about this article) |
2/122. Acute confusion and unreal experiences in intensive care patients in relation to the ICU syndrome. Part II.The intensive care unit syndrome (ICU syndrome) is defined as an altered emotional state occurring in a highly stressful environment, which may manifest itself in various forms such as delirium, confusion, crazy dreams or unreal experiences. The purpose of this part of a study of patients' experiences is to describe and illuminate patients' experiences of acute confusion, disorientation, wakefulness, dreams and nightmares during and after their stay in the ICU. The data were obtained from 19 ventilated patients, who were interviewed twice and had stayed at least 36 hours in the ICU, the first interview being about one week after discharge from the ICU, and the second 4-8 weeks later. The hermeneutic approach used when interpreting and analysing the text from the interviews revealed that patients' experiences of unreal experiences were often associated with intense fear. Intense or continuous unbearable fear seems to result in frightening unreal experiences, which further increase the level of fear. Care actions or caring relationships with relatives or nurses can reduce this fear, which can help to prevent the occurrence and/or duration and intensity of the unreal experiences. trust and confidence in nurses or significant others and feelings of self-control or trust in self-control seemed to reduce the risk of unreal experiences so that adverse stimuli might only trigger a mild confusion.- - - - - - - - - - ranking = 1keywords = motion (Clic here for more details about this article) |
3/122. Rigid spine syndrome. Two case-reports.Rigid spine syndrome is characterized by massive spinal rigidity, usually most marked in the cervical region. Stiffness of the peripheral joints is sometimes present. We report two cases. Patient 1 was a 12-year-old boy diagnosed at three years of age with Duchenne's muscular dystrophy because of delayed onset of walking. contracture of the Achilles tendons, flexion contracture of the elbows, and loss of motion of the cervical spine were the main findings during the current evaluation. Radiographs of the affected joints were normal. An electrocardiogram showed an incomplete left bundle branch block. Muscle enzyme activities were moderately elevated. A myopathic pattern was seen on the electromyogram. A muscle biopsy showed muscle fiber atrophy with peri- and endomysial fibrosis. Patient 2 was a 39-year-old man with a five-year history of isolated rigidity of the cervical spine thought to be due to a spondylarthropathy. Extension was the only movement possible at the cervical spine. The peripheral joints showed no motion range limitation. Findings were normal from radiographs of the spine and sacroiliac joints, an erythrocyte sedimentation rate determination, an electromyogram, and muscle enzyme activity assays. A muscle biopsy showed muscle fiber atrophy with peri- and endomysial fibrosis. DISCUSSION: Rigid spine syndrome is rare in rheumatological practice and can simulate a number of other muscle and joint diseases. Peri- and endomysial fibrosis may be strongly suggestive, although nonpathognomonic. Involvement of the heart governs the prognosis.- - - - - - - - - - ranking = 2keywords = motion (Clic here for more details about this article) |
4/122. Late-onset central hypoventilation with hypothalamic dysfunction: a distinct clinical syndrome.Idiopathic central hypoventilation has occasionally been reported in previously well children after infancy. The relationship between this late-onset central hypoventilation syndrome (LO-CHS) and congenital central hypoventilation syndrome (CCHS) has not been established. Both CCHS and LO-CHS have been associated with neural crest tumors, such as ganglioneuroblastoma and ganglioneuroma, and they generally occur in the presence of a histologically normal central nervous system. At least 10 case reports of idiopathic LO-CHS featured evidence of hypothalamic dysfunction (HD), including hyperphagia, hypersomnolence, thermal dysregulation, emotional lability, and endocrinopathies. We report on a case of LO-CHS/HD successfully treated by nasal intermittent positive pressure ventilation (NIPPV). Despite the commonalties with CCHS, we propose that LO-CHS/HD is a distinct clinical syndrome. In addition to the markedly different age at presentation, features of hypothalamic dysfunction are not seen in CCHS. review of the literature was undertaken to further clarify the full spectrum of the disease.- - - - - - - - - - ranking = 1keywords = motion (Clic here for more details about this article) |
5/122. Oral self-mutilation in a patient with rhombencephalosynapsys.Rhombencephalosynapsis (RS) is a rare cerebellar malformation. Its essential features are the absence of the incisura cerebelli posterior, fusion of the cerebellar hemispheres, the absence of the velum medullare anterius and nuclei fastigii, and fusion of the dentate nuclei, which are shifted towards the mid-line. Clinically, affected patients present with signs of cerebellar and motor disturbances. The present report describes a new patient affected by RS. The subject first presented at the age of 22 years because of a psychiatric symptomatology which was characterized by obsessive oral self-mutilation associated with an intellectual disability. Objective evaluation documented dysmorphic features, while neurological examination showed only a slight truncal ataxia. The subject's IQ was 74 on the Wechsler Scale (verbal IQ = 79, performance IQ = 74). Psychiatric evaluation with DSM-IV criteria documented an obsessive-compulsive personality disorder associated with emotional instability and oral self-mutilation. The typical picture of rhombencephalosynapsis was evident on magnetic resonance imaging. Both chromosomal analysis and routine biochemical investigations were normal. The relationship between oral self-injurious behaviour and cerebellar malformations is discussed with particular regard to the behavioural aspects of cerebellar congenital pathology in affective disorders and in autism.- - - - - - - - - - ranking = 1keywords = motion (Clic here for more details about this article) |
6/122. Spondylocarpotarsal synostosis syndrome and cervical instability.Spondylocarpotarsal synostosis syndrome is a recently delineated autosomal recessive condition comprising short stature with short trunk, failure of normal spine segmentation resulting in block vertebrae and fusion of posterior elements, carpal and/or tarsal coalition, scoliosis, lordosis, pes planus, dental enamel hypoplasia, decreased range of motion or dislocation of the elbow, renal anomalies, and hearing loss. The vertebral segmentation defects may involve noncontiguous areas of the cervical, thoracic, and lumbar spine. Odontoid hypoplasia was noted in two cases. We report on a sporadic case of spondylocarpotarsal synostosis in a 5-year-old girl with hypoplasia of C1 and odontoid and subluxation of C2 upon C3. This brings the number of well-documented cases of spondylocar- potarsal synostosis to 19, and is the first documenting cervical spine instability. Careful evaluation for this complication should be considered in other cases.- - - - - - - - - - ranking = 1keywords = motion (Clic here for more details about this article) |
7/122. ALS-Plus syndrome. A clinical and neuropathological case study.ALS-Plus syndrome occurs rarely and usually presents typical ALS phenotype associated with dementia, Parkinsonism or both. We reported a case of sporadic, definite ALS with pseudobulbar palsy, emotional lability and selective cognitive deficit in the presence of frontal lobe dementia. neuropsychological tests predominantly demonstrated perserveration and dynamic apraxia, CT and MRI scans showed widened subarachnoideal spaces in the frontal and temporal regions. The neuropathological findings confirmed ALS processes i.e. atrophy of motor nuclei in brainstem and anterior horns of cervical spinal cord and showed mild atrophy and status spongiosus in the frontal lobes. These findings suggest the co-occurrence of sporadic ALS and frontal lobe dementia: ALS-Plus syndrome.- - - - - - - - - - ranking = 1keywords = motion (Clic here for more details about this article) |
8/122. VATER association: is it recognised by rheumatologists?The authors describe an adult patient with history of chronic low back pain and recurrent prostatitis, marked limitation of lumbar spine motion and a radiograph demonstrating fused lumbar vertebrae, which suggest a diagnosis of spondylarthropathy. However, the absence of radiographic evidence of sacroilitis, the nature of the vertebral defects and a history of imperforate anus pointed towards the diagnosis of VATER association, rather than a spondylarthropathy. Although most patients with VATER association are diagnosed during infancy, the musculoskeletal anomalies can be overlooked while the potentially life-threatening problems are under treatment. These anomalies may become evident later in life. Therefore, in a rheumatologic practice, when evaluating patients with back pain and vertebral anomalies, one should become familiar with the varied manifestations of VATER association.- - - - - - - - - - ranking = 1keywords = motion (Clic here for more details about this article) |
9/122. Transient left ventricular apical ballooning without coronary artery stenosis: a novel heart syndrome mimicking acute myocardial infarction. angina pectoris-myocardial infarction Investigations in japan.OBJECTIVES: To determine the clinical features of a novel heart syndrome with transient left ventricular (LV) apical ballooning, but without coronary artery stenosis, that mimics acute myocardial infarction, we performed a multicenter retrospective enrollment study. BACKGROUND: Only several case presentations have been reported with regard to this syndrome. methods: We analyzed 88 patients (12 men and 76 women), aged 67 /- 13 years, who fulfilled the following criteria: 1) transient LV apical ballooning, 2) no significant angiographic stenosis, and 3) no known cardiomyopathies. RESULTS: Thirt-eight (43%) patients had preceding aggravation of underlying disorders (cerebrovascular accident [n = 3], epilepsy [n = 3], exacerbated bronchial asthma [n = 3], acute abdomen [n = 7]) and noncardiac surgery or medical procedure (n = 11) at the onset. Twenty-four (27%) patients had emotional and physical problems (sudden accident [n = 2], death/funeral of a family member [n = 7], inexperience with exercise [n = 6], quarreling or excessive alcohol consumption [n = 5] and vigorous excitation [n = 4]). Chest symptoms (67%), electrocardiographic changes (ST elevation [90%], Q-wave formation [27%] and T-wave inversion [97%]) and elevated creatine kinase (56%) were found. After treatment of pulmonary edema (22%), cardiogenic shock (15%) and ventricular tachycardia/fibrillation (9%), 85 patients had class I New York heart association function on discharge. The LV ejection fraction improved from 41 /- 11% to 64 /- 10%. Transient intraventricular pressure gradient and provocative vasospasm were documented in 13/72 (18%) and 10/48 (21%) of the patients, respectively. During follow-up for 13 /- 14 months, two patients showed recurrence, and one died suddenly. CONCLUSIONS: A novel cardiomyopathy with transient apical ballooning was reported. Emotional or physical stress might play a key role in this cardiomyopathy, but the precise etiologic basis still remains unclear.- - - - - - - - - - ranking = 2keywords = motion (Clic here for more details about this article) |
10/122. Augmentative and alternative communication methods in locked-in syndrome.Locked-in syndrome is a neurological condition due to a brain disease or an injury affecting the brain stem. The symptoms are tetraplegia, double-sided facial paresis, anarthria/dysarthrophonia, dysphagia and reactive involuntary laughing and crying. Vertical eye movements are the only commonly remaining voluntary motor function. Although the linguistic abilities as well as intellectual and emotional functions as a whole remain intact, all the motor abilities of self expression are lost. Seventeen chronic locked-in syndrome patients referred to Kapyla rehabilitation Centre between 1979-2000 are reported. The multidisciplinary rehabilitation team developed an individual alternative communication method for all patients and trained them to use it by minor movements of e.g. thumb, chin or head. An alternative communication method enabled most of the patients to interact with other people using practical as well as theoretical thinking and decision making.- - - - - - - - - - ranking = 1keywords = motion (Clic here for more details about this article) |
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