1/16. Threshold concentration of unbound bilirubin to induce neurological deficits in a patient with type I crigler-najjar syndrome.Based on the clinical course of a 16-year-old boy with type I crigler-najjar syndrome, we estimated the threshold concentration of unbound bilirubin, as assayed by the horseradish peroxidase method, that apparently induces toxicity to the brain. Before the age of 15, the patient did not manifest any neurological or behavioural dysfunction despite increased bilirubin in serum. The binding affinity and the binding capacity of the patient's serum albumin for bilirubin determined when he was about 14 years old were 10(8)(mol/L)-1 and 1.01 to 1.04 mol/L, respectively. These values were nearly the same as those of normal controls reported in the literature. The total bilirubin binding capacity was greater than the patient's total bilirubin concentration, showing that his serum albumin was not saturated with bilirubin. The reserve bilirubin binding capacity (RBBC) was estimated to be 158 mumol/L and the unbound bilirubin concentration to be 15.1 nmol/L. Concentration of unbound bilirubin peaked at 21.7 nmol/L at the age of 15 years and 11 months, i.e. 2 months before the onset of difficulties in walking and speaking. At this time, the RBBC was estimated as -64 mumol/L. A peak concentration of total bilirubin, 811 mumol/L, was observed during the period of rapid loss of the ability to walk or speak. At the age of 16 years and 1 month the RBBC decreased to -98 mumol/L and the unbound bilirubin concentration to 18.8 nmol/L. Following phototherapy, the patient's neurological state returned to normal; he could speak and walk normally. At the age of 16 years and 2 months the RBBC returned to 105 mumol/L and unbound bilirubin decreased to 16.6 nmol/L. These results suggest that maintaining the concentration of unbound bilirubin at < 20 nmol/L and the total bilirubin concentration at lower than the binding capacity of serum albumin is important for prevention of neurological deficits in crigler-najjar syndrome. The upper limit of unbound bilirubin in such an older patient was nearly the same as that reported for newborns.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
2/16. The neurobehavioural consequences of St. Louis encephalitis infection.BACKGROUND: St. Louis encephalitis (SLE) is a relatively common cause of neurological illness, yet little is known about its cognitive and psychosocial consequences. PURPOSE: To describe the cognitive, emotional, psychophysiological, and psychosocial consequences of SLE infection. METHOD: A comprehensive neuropsychological and psychophysiological evaluation of a high functioning woman 6 weeks and 1 year after acute SLE infection is presented. The focus and course of rehabilitation is also examined. RESULTS: The primary cognitive consequences of SLE infection involved attention, working memory, speed of processing, and cognitive efficiency. depression was also observed. Psychometric testing suggested that these deficits largely resolved after 1 year. CONCLUSIONS: SLE produces neurocognitive deficits which are reflected in both psychometric and psychophysiologic measures and functional status. Psychometric and vocational improvement were observed over 1 year. However, the normal vocational return came at a significant psychosocial cost. This case emphasizes the importance of a comprehensive neuropsychological evaluation and illustrates the importance of an integrated rehabilitation programme.- - - - - - - - - - ranking = 4keywords = behaviour (Clic here for more details about this article) |
3/16. Prolonged sensory or visceral symptoms: an under-diagnosed form of non-convulsive focal (simple partial) status epilepticus.Four patients had prolonged, sensory, simple partial seizures (SPS), lasting up to several days, without associated behavioural impairment. In three patients, the SPS often occurred as a prolonged "aura" before a more overt seizure. Descriptions included: "butterflies", rising epigastric sensation; "a thought in the stomach", and an olfactory sensation. Seizure localisation was frontal in one case, temporal in two cases and uncertain in one case. These sensations may represent an under-reported form of continuous, focal seizure activity, which arises from various cerebral regions.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
4/16. rabies. A second Australian case, with a long incubation period.OBJECTIVE: The description of a second case of rabies in australia, stressing the clinical features and that long incubation periods are possible. CLINICAL FEATURES: A 10-year-old Vietnamese girl presented with fever, shoulder pain, subcutaneous emphysema, swallowing difficulty and agitation. After a period of maniacal behaviour all peripheral and central nervous system function was lost. INTERVENTION AND OUTCOME: Despite maximal intensive care, the patient died. The diagnosis of rabies was made at autopsy. CONCLUSIONS: rabies occurs in australia and needs to be considered in the differential diagnosis of acute encephalitis and/or the guillain-barre syndrome. Incubation periods of more than six years can occur.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
5/16. McLeod syndrome: life-long neuropsychiatric disorder due to a novel mutation of the XK gene.A 50-year-old man presented with worsening, virtually lifelong, chorea and progressive behavioural disturbance, involving disinhibition and hoarding, over 10 years. Clinical assessment revealed chorea, dysarthria, areflexia, an inappropriately jovial, impulsive manner and neuropsychological evidence of frontosubcortical dysfunction. Investigation results included an elevated creatine kinase, caudate atrophy and hypoperfusion, acanthocytes in the peripheral blood and the McLeod phenotype. dna studies demonstrated a single-base deletion at position 172 in exon 1 of the XK gene, giving rise to a premature stop codon at position 129 in exon 2.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
6/16. Homeopathic approach in the treatment of patients with mental disability.The author describes his experience assisting mentally disabled patients with homeopathy. In these patients' anamneses, common traits shared by some syndromes, pathologies and behaviour, were taken into consideration, mainly to choose the most characteristic symptoms in each case. The study includes 58 cases of patients suffering from this pathology: 28 females and 30 males, ages ranging from 1 to 49 years (mean 20). Forty-seven had some improvement. homeopathy is a useful alternative to relieve pathologies associated with mental disability. In those cases in which there were similarities between remedy and whole symptomatology, improvements in adaptation skills and in overall health were observed.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
7/16. A patient with severe neurologic symptoms and acetoacetyl-CoA thiolase deficiency.A detailed kinetic characterization of the cytosolic acetoacetyl-CoA thiolase in liver, subcutaneous adipose tissue, and cultured fibroblasts of a patient with severe neurologic symptoms showed this enzyme to be much more sensitive to inhibition by CoA than that enzyme of normal human liver. The activity of the cytosolic acetoacetyl-CoA of this patient (14.0 mu mol-min-1-g (w/w) was in the lower range of normal (48.7 /- 20.4 mu mol-min-1 g (w/w), n=6), but the ratio of the mitochondrial thiolase over the cytosoli thiolase was significantly increased (14.9 versus 4.54 /- 2.92, n =6). The increased inhibition of the cytosolic acetoacetyl-CoA thiolase by CoA shows up as a 2-fold increase of the apparent Km for acetoacetyl-CoA at physiologic concentrations of CoA (64 micrometer for the enzyme of the patient verus 35 micrometer for the normal enzymes), resulting in a decreased utilization of the substrate. Aberrant kinetic behaviour of the mitochondrial thiolases of the patient has also been observed, but this has not been analysed further. Morphologic studies of the liver showed greatly increased deposition of glycogen and the occurrence of lipofuschin-like granules.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
8/16. Disturbance of rhythm sense following right hemisphere damage.In a patient with right temporal lobe and additional right basal ganglia damage following a stroke, recognition and reproduction of simple rhythmical Gestalten were examined and found grossly undisturbed. In contrast to this undisturbed perception and production of rhythm, the patient could not tap or move rhythmically in beat with an auditory pacer such as a metronome or marching band music, yet he could do so to rhythmically presented light or touch stimuli. Thus, the impairment seems to be a previously undescribed, modality specific disturbance of auditorily paced predictive motor behaviour affecting, for example, walking, dancing, singing and speech.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
9/16. Two cases of schistosomiasis.Two patients with neuroschistosomiasis are reported. In both patients diagnostic problems were encountered. The first case began with an aspecific allergic reaction of facial oedema and abnormal behaviour. The symptoms were followed by generalized convulsive seizures, dysphasia and hemiparesis. The disease characteristics, paraclinical findings and remarkable improvement on antischistosomal drug therapy strongly suggested cerebral schistosomiasis. The second case history starts with a cauda syndrome while in the end the diagnosis of transverse myelitis was confirmed.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
10/16. multiple system atrophy with neuronal intranuclear hyaline inclusions. Report of a case and review of the literature.A 31/2-year-old girl presented with frequent falls. She had an unsteady gait, delayed behavioural development absent tendon reflexes and in the legs decreased strength tone and equivocal plantar responses. She then developed ataxia, nystagmus, choreoathetosis, cranial nerve palsies, diminished strength and tone in the arms, sensory deficit in the limbs and autonomic nervous system dysfunction. She became progressively less responsive and succumbed at the age of 63/4 years. Examination of the central, peripheral and autonomic nervous system showed ubiquitous neuronal intranuclear hyaline inclusions and neuronal loss in several sites.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
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