Severe childhood encephalopathy with dyskinesia and prolonged cognitive disturbances: evidence for anti-N-methyl-d-aspartate receptor encephalitis.

Aim We report four cases of acquired severe encephalopathy with massive hyperkinesia, marked neurological and cognitive regression, sleep disturbance, prolonged mutism, and a remarkably delayed recovery (time to full recovery between 5 and 18mo) with an overall good outcome, and its association with anti-N-methyl-d-aspartate (anti-NMDA) receptor antibodies. Method We reviewed the four cases retrospectively and we also reviewed the literature. Results Anti-NMDA receptor antibodies (without ovarian teratoma detected so far) were found in the two children tested in this study. Interpretation The clinical features are similar to those first reported in 1992 by Sebire et al.,(1) and rarely recognized since. Sleep disturbance was not emphasized as part of the disorder, but appears to be an important feature, whereas coma is less certain and difficult to evaluate in this setting. The combination of symptoms, evolution (mainly seizures at onset), severity, paucity of abnormal laboratory findings, very slow recovery, and difficult management justify its recognition as a specific entity. The neuropathological substrate may be anatomically close to that involved in encephalitis lethargica, in which the same target functions (sleep and movement) are affected but in reverse, with hypersomnolence and bradykinesia. This syndrome closely resembles anti-NMDA receptor encephalitis, which has been reported in adults and is often paraneoplastic.

The impact of drug-induced dyskinesias on rapid alternating movements in patients with Parkinson's disease

We investigated the likelihood that hypokinesia/bradykinesia coexist with druginduced dyskinesias (DID) in patients with Parkinson's disease (PD). The influence of dyskinesias on rapid alternating movements (RAM) was investigated in ten dyskinetic patients (DPD). Their motor performance was compared to that of ten age/gendermatched non-dyskinetic patients (NDPD) and ten healthy control subjects. Whole-body magnitude (WBM) and fast pronation-supination at the wrist were recorded using 6- degrees of freedom magnetic motion tracker and forearm rotational sensors, respectively. Subjects were asked to pronate-supinate their dominant hand for 10s. Pre- and postmeasures were taken in a neutral position for 20s. RANGE (measure of hypokinesia), DURATION (measure of bradykinesia). VELOCITY (measure of bradykinesia) and IRREGULARITY (measure of fluctuations in movement amplitude) were used to assess RAM performance. Results showed that DPD patients had greater WBM than NDPD and control groups during rest and RAM performance. There were no differences in performance between NDPD and DPD groups for RANGE, DURATION and VELOCITY, despite significant longer disease duration for the DPD group (DPD = 15.5 ± 6.2 years versus NDPD = 6.6 ± 2.6 years). However, both the NDPD and DPD groups showed lower RANGE, longer DURATION, and reduced VELOCITY compared to controls,, suggesting the presence of bradykinesia and hypokinesia. In the case of IRREGULARITY, DPD patients showed clear fluctuations in movement amplitude compared to the NDPD and control groups. However, the lack of correlation between WBM and IRREGULARITY within the DPD group (Spearman's rank order, Rho - 0.31, p > 0.05), suggests that DID was not the primary cause of the fluctuating movementamplitude observed in that group. In conclusion, these findings suggest that DID may coexists with bradykinesia and hypokinesia, but that they are not inevitably accompanied with worsening motor performance.

An educator's perspective of Dr. Feingold's K-P (Kaiser- Permanents) elimination diet for hyperkinetic children and others

Do evaluation of the literature and a regional observational report support Dr. Feingold's claim that the K-P (Kaiser-Permanente) elimination diet improves the behaviours of hyperkinetic children, and others? Dr. Feingold suggests that some hyperkinetic children, and other children as well, are genetically predisposed to intolerance of food additives, particularly food colours and flavours. He claims that the K-P diet, that eliminates salicylates and artificial food colours and flavours, improves the hyperkinetic child's behaviour, muscle co-ordination, and scholastic performance. Public acceptance of the K-P diet has outstripped acceptance in the medical and scientific communities. Evaluation of available data and additional studies are needed to arrive at a conclusion of acceptance or rejection of the K-P diet for hyperkinetic children and others. My interest in the K-P elimination diet for hyperkinetic children is educational. My experience as an elementary school teacher in special education and in the classroom from K-8 has taught me that attentiveness is crucial to learning. Hyperkinesis appears to impair a child's ability to attend. Learning problems appear, followed by behavioural and social problems. l If we accept the possibility of a relationship between diet and attentiveness, and attentiveness and school behaviours, then the diet-behaviour link could be of lay importance. For instance, if a diet such as the K-P diet could do what is claimed, substantial benefits could accrue to the child. One could, for example, improve a child's behaviours. One could identify attending disturbances early in the child's education, possibly minimizing, or eliminating future difficulties in school. Finally, the greatest benefit may be the fulfillment of the basic goal of our Ontario schools, that the eh~ld-,lIla1p.evelop happily and competently within our educational framework. 2 This thesis reports evidence from the literature and from a regional observational investigation to determine the possibility of a link between the behaviours of children and Dr. Feingold's K-P elimination diet. The literature research examines (1) Dr. Feingold's concept of H-LD, (2) his K-P elimination diet, and (3) the response from three sectors, medicine, science, and the public. The regional investigation examines the observed behaviours of nine children in Regional Niagara during a nine-month period on the K-P diet.