1/6. Creutzfeldt-Jakob disease : report of 10 cases from North india.Creutzfeldt-Jakob disease (CJD) is increasingly being reported over the last three decades as a result of heightened awareness of the disease. Various studies have reported annual incidence of 0.5-1.5 cases of CJD per million of general population. In india, the disease is still under reported. Over the period spanning from 1968-1997, National Institute of mental health and neurosciences (NIMHANS), Bangalore recorded 69 cases of CJD from different parts of india in the CJD registry. This paper describes the clinical experience with cases of CJD managed at the Department of neurology, G.B. Pant Hospital, New Delhi from 1990-1998. In this series, the mean age of the patients was 53.80 ( /- 7.32) years and there were 5 females and 5 males. myoclonus was present in all the cases and abnormal behaviour with or without other features was the presenting complaint in 7 of the 10 patients, while one patient of CJD had cerebellar ataxia as the presenting feature. One patient with occipital variant of CJD presented with acute onset cortical blindness and myoclonic jerks. One of the patients had acute psychosis precipitated by emotional stress at the onset. Extrapyramidal features were noted in 7 of the 10 patients before death. The mean duration of symptoms from the onset of disease to death was 6.6 ( /- 6.11) months. Classical EEG changes were observed in all the patients, except in one possible case of occipital variant of CJD, where we did not have access to EEG record. brain biopsy could be undertaken in 3 patients, and in 2 patients the features of subacute spongiform encephalopathy (SSE) were noted.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
2/6. Semiology of typical and atypical Rolandic epilepsy: a video-EEG analysis.Since the first descriptions of Rolandic epilepsy or benign epilepsy with centrotemporal spikes (BECTS), typical and atypical forms have been reported. Indeed, classical focal seizures are sometimes associated with various atypical ictal symptoms and cognitive or behavioural disorders. In an effort to define early clinical and EEG criteria allowing early distinction between typical and atypical forms, we recently conducted a prospective study in a cohort of children with Rolandic epilepsy. The results of this study have been reported elsewhere. We now discuss the semiological characteristics, and comment on the video-EEG data collected during this study. Symptoms were classified into three major categories: "classical focal seizures"; "spike and wave related symptoms"; and "paraictal symptoms". Classical focal seizures constitute the electroclinical expression of the development and the propagation of a focal cortical neuronal discharge. "Spike and wave related symptoms" are brief neurological or neuropsychological phenomena having a relatively strict temporal relation with individual components of isolated focal or generalized spikes and waves. "Paraictal symptoms" consist of acquired progressive and fluctuating motor or cognitive deficits and are not directly correlated with Todd paralysis. We present detailed video-EEG material of selected cases and discuss the usefulness of such distinctions in terminology. We suggest that variability in clinical expression probably reflects the implication of different pathophysiological mechanisms, which in turn could explain differences in sensitivity to treatment. (Published with videosequences.)- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
3/6. Benign early infantile reflex absence seizures.This case report, with videotape, focuses on reflex epileptic seizures of a girl who started to have seizures, provoked by tactile stimuli, at the age of twelve months. They were described as myoclonic jerks predominantly in the upper part of the body and the arms. During video/EEG monitoring at the age of fifteen months, seizures provoked by the same tactile stimuli were recorded. For clinical and electroencephalographic reasons, these are best described as absence seizures (persistence of minimal myoclonic jerking). To our knowledge, such seizures at this age have not been documented before. Treatment with antiepileptic agents was not initiated. Six months later the seizures stopped spontaneously and have not recurred. Two years later, psychomotor and behavioural development is normal [published with video- sequences].- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
4/6. subacute sclerosing panencephalitis presenting with unilateral periodic myoclonic jerks.BACKGROUND: subacute sclerosing panencephalitis (SSPE) is a rare complication of measles virus infection. The disease is characterized by behavioural abnormalities, intellectual deterioration, motor weakness, and generalized myoclonic jerks progressing to coma and death in one to two years in 80% of the cases. The myoclonic jerks are associated with characteristic generalized slow periodic complexes on electroencephalography (EEG). The symptoms and signs of SSPE are frequently quite variable. The clinical course is equally variable and difficult to predict. The characteristic periodic myoclonus can rarely occur unilaterally particularly in the early stages of the disease. As well, the periodic EEG complexes have been reported unilaterally in up to 3% of cases. CASE REPORT: A 12-year-old boy, who was seen at a later stage with atypical manifestation of myoclonic body jerks confined entirely unilaterally, combined with contralateral periodic EEG complexes. One could assume clinically that the more diseased hemisphere was responsible for generating the jerks. However, brain magnetic resonance imaging revealed asymmetric hemispheric changes suggesting that the less neurologically damaged hemisphere is responsible for generating the unilateral myoclonic jerks. This has led to the interpretation that the more severely damaged hemisphere has lost the neuronal connectivity required to generate these periodic myoclonic jerks. CONCLUSIONS: subacute sclerosing panencephalitis may have asymmetric hemispheric involvement, not only early, but also in the advanced stages of the disease, which can result in unilateral periodic myoclonic jerks.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
5/6. hyperalgesia and myoclonus in terminal cancer patients treated with continuous intravenous morphine.Eight cancer patients in the terminal stages of the disease treated with high doses of intravenous morphine developed hyperalgesia. All cases were retrospectively sampled from three different hospitals in Copenhagen. Five patients developed universal hyperalgesia and hyperesthesia which in 2 cases were accompanied by myoclonus. In 3 patients a pre-existing neuralgia increased to excruciating intensity and in 2 of these cases myoclonus occurred simultaneously. Although only few clinical descriptions of the relationship between hyperalgesia/myoclonus and high doses of morphine are available, experimental support from animal studies indicates that morphine, or its metabolites, plays a causative role for the observed behavioural syndrome. The possible mechanisms are discussed and treatment proposals given suggesting the use of more efficacious opioids with less excitatory potency in these situations.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
6/6. Is development of hyperalgesia, allodynia and myoclonus related to morphine metabolism during long-term administration? Six case histories.BACKGROUND: Recently, clinical reports have suggested a relationship between the occurrence of hyperalgesia, allodynia and/or myoclonus and treatment with high doses of morphine in humans. Although few clinical descriptions of these phenomena are available, experimental work supports the notion that high doses of morphine may play a pathogenetic role in the observed behavioural syndrome. methods: Six patients, four with malignant and two with chronic, non-malignant pain conditions, treated with moderate to high doses of oral, continuous intravenous infusion or intrathecal morphine developed hyperalgesia, allodynia and/or myoclonus. When the side-effects occurred, blood or CSF samples were taken and analyzed for contents of morphine, morphine-6-glucuronide (M-6-G) and morphine-3-glucuronide (M-3-G). RESULTS: When comparing the plasma and CSF concentrations from these patients with data from available literature obtained from patients not suffering from these side-effects, it was demonstrated that the values deviated in five patients. In all six patients, the side-effects disappeared after substituting morphine with other opioid agonists or after lowering the daily dose of morphine. CONCLUSION: These results may indicate that elevated concentrations of M-3-G in plasma as well as the plasma and CSF M-3-G/M-6-G ratios may play a pathogenetic role in the development of hyperalgesia, allodynia and myoclonus.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |