Efficacy of modafinil on excessive daytime sleepiness in Prader-Willi syndrome.

Excessive daytime sleepiness is a frequent and a highly disruptive symptom to the daily routine of children with Prader-Willi Syndrome (PWS) and their families. The objective of the study was to evaluate the efficacy of modafinil, a central stimulant, on excessive daytime sleepiness in children and adolescents with PWS. The efficacy of modafinil was evaluated in this open label pilot study comparing the Epworth sleepiness scale before and after treatment. Ten patients with molecularly confirmed PWS and a complaint of excessive daytime sleepiness underwent a night-time sleep recording and multiple sleep latency tests. One patient was excluded because of severe obstructive sleep apnea syndrome. Nine patients (4 males) with median age of 16 years (8-21) received modafinil at a starting dose of 100 mg/day. We found that all patients had excessive daytime sleepiness with an Epworth sleepiness scale at 14 (11-20) and mean sleep latency on multiple sleep latency tests at 5 (3-6) minutes. Moreover, six patients had at least two sleep-onset rapid eye movement periods showing a narcolepsy-like phenotype. Modafinil significantly improved sleepiness in all patients on the Epworth sleepiness scale from 14 (11-20) to 4 (3-12), (P = 0.007). Body mass index of the patients did not change significantly under treatment. No side effects were reported, and the drug was well-tolerated. We posit that this open label case series shows good efficacy of modafinil in nine children and adolescents with PWS.

[How to manage daytime sleepiness associated with Parkinson's disease]. / Comment prendre en charge la somnolence associée à la maladie de Parkinson ?

Excessive daytime sleepiness (EDS) is frequent in Parkinson's disease. It should be explored by an Epworth sleepiness scale, a nighttime sleep recording and multiple sleep latency tests. EDS can be secondary to disturbed nighttime sleep that should be explored first. The main reasons for nighttime sleep disturbances are pain, nocturia, restless legs syndrome, obstructive sleep apnea syndrome and depression. They should be treated step by step. EDS can also be secondary to antiparkinsonian dopaminergic treatments that can induce nighttime insomnia and/or daytime sleepiness sometimes with sleep attacks. Treatment modifications and, when indicated, deep brain stimulation can improve these symptoms. Furthermore, EDS can be secondary to the disease itself modifying the sleep wake regulation systems. When the treatment of disturbed nighttime sleep and adjustments of the dopaminergic treatments are not sufficient to improve EDS, wake promoting treatments can be used. Their efficacy is variable but new hopeful drugs are coming soon.

Case-control study of writer's cramp.

Task-specific focal dystonias are thought to be due to a combination of individual vulnerability and environmental factors. There are no case-control studies of risk factors for writer's cramp. We undertook a case-control study of 104 consecutive patients and matched controls to identify risk factors for the condition. We collected detailed data on medical history and writing history as part of hobbies or occupation. Cases had a college or university degree more frequently than controls [OR = 4.6 (1.3-20.5), P = 0.01]. The risk of writer's cramp increased with the time spent writing each day (P-trend = 0.001) and was also associated with an abrupt increase in the writing time during the year before onset (OR = 5.7, 95% CI = 1.3-33.9, P = 0.02). Head trauma with loss of consciousness [OR = 3.5 (1.0-15.7), P = 0.05] and myopia [OR = 4.1 (1.7-12.0), P = 0.0009] were both associated with the condition but it was not significantly associated with peripheral trauma, left-handedness, constrained writing, writing in stressful situations or the choice of writing tool. The dose-effect relationship between writer's cramp and the time spent handwriting each day, and the additional burden of acute triggers such as an abrupt increase in the writing time in the year before onset, point to a disruptive phenomenon in predisposed subjects. Homeostatic regulation of cortical plasticity may be overwhelmed, resulting in dystonia.