1/9. Repetitive sleep starts in neurologically impaired children: an unusual non-epileptic manifestation in otherwise epileptic subjects. Sleep starts, also called hypnagogic or hypnic jerks, are bilateral, sometimes asymmetric, usually single, brief body jerks that coincide with sleep onset. We describe sleep starts occurring repetitively in three epileptic children with spastic-dystonic diplegia and mental retardation. Repetitive sleep starts began at age 18 months in two children and at 9 months in the third. All three children had had feto-neonatal asphyxia; two presented with spastic and one with dystonic tetraparesis. One had West syndrome and two had partial motor seizures in the first year of life. seizures were controlled in all three patients by antiepileptic drug therapy. Video/EEG recordings of all the children during the afternoon nap revealed clusters of sleep starts during the transition between wakefulness and sleep. Cluster lasted 4-15 min and comprised from twenty to twenty-nine contractions. The EEG counterpart of the event sometimes showed an arousal response, at times inducing complete awakening. Repetitive sleep starts should be recognized and clearly differentiated from epileptic seizures, especially if they appear in epileptic subjects. In neurologically compromised patients, they could represent an intensification of an otherwise normal event, due to the lack of strong inhibitory influence of the pyramidal tract resulting from the pyramidal lesion. ( info) |
| quinine is universally used for the very common symptom of night leg cramps. patients may not mention it among their medicines, since it is so commonly used and they regulate it themselves. A 68-year-old man suddenly developed extensive bleeding due to severe thrombocytopenia. The diagnosis was initially thought to be a recurrence of idiopathic thrombocytopenic purpura (ITP) that had initially occurred in 1992 and had required splenectomy. Drug-induced thrombocytopenia was also considered, and he was told to stop all of his medicines. Only after three subsequent episodes of severe, symptomatic thrombocytopenia over the next four weeks did he say, upon repeat questioning, that he had continued to take quinine for night leg cramps. Even after a strict warning, he took another quinine tablet that evening, which triggered his fifth episode of severe thrombocytopenia, and confirmed the etiology of quinine-induced thrombocytopenia. The diagnosis thrombocytopenia caused by common drugs can be difficult, requiring persistent, explicit questions. ( info) |
3/9. An unusual case of rhythmic movement disorder. Rhythmic movement disorder is one of the sleep-wake transition disorders listed in the International classification of sleep disorders. According to this classification, the condition commonly occurs in infants and toddlers, and persistence beyond 4 years of age is unusual. Recently, we encountered a case in which rhythmic movement disorder persisted up until the age of 12 years with spikes registering on the sleep electroencephalogram. Epileptic seizure was ruled out because of the characteristic rolling movement, absence of any other epileptic symptoms (e.g. vocalization and tonic-clonic seizure) and cessation as a result of removal of the blanket. ( info) |
4/9. Antecedent control of sleep-awakening disruption. The sleep-awakening disruption of an adolescent with developmental disabilities was treated using an antecedent control intervention that identified his consistent time of wake-up, provided the presence of a preferred staff in his bedroom, and prompted social interaction from staff before challenging behaviors occurred. Positive findings were documented using a combined reversal and multiple baseline across settings design, with results maintained through a 9-month follow-up. A partial component analysis of the intervention plan suggested that the presence of preferred staff was the influential antecedent variable. ( info) |
5/9. Propriospinal myoclonus at the sleep-wake transition: a new type of parasomnia. STUDY OBJECTIVES: To describe the clinical, neurophysiological, and polysomnographic characteristics of propriospinal myoclonus (PSM) at the sleep-wake transition. DESIGN: patients referred for insomnia due to myoclonic activity arising during relaxed wakefulness preceding sleep, or complaining of muscular jerks also during intrasleep wakefulness and upon awakening in the morning were considered. SETTING: All patients underwent EEG-EMG recordings during wakefulness and night sleep. back-averaging of the EEG activity preceding the jerks was performed. Somatosensory evoked potentials (SEPs), transcranial magnetic stimulation (TMS) and spinal and cranial MRI were also done. PARTICIPANTS: Four patients were studied all affected with involuntary jerks arising when falling asleep, and one with jerks also during sleep and upon awakening in the morning. INTERVENTIONS: N/A. MEASUREMENTS AND RESULTS: Polysomnographic investigations revealed jerks arising during the sleep-wake transition period. Myoclonic activity was neurophysiologically documented to be of the propriospinal type. SEPs, TMS and MRI were normal CONCLUSIONS: PSM may have a peculiar relationship with the state of vigilance and represent a sleep-wake transition disorder. In this regard we consider PSM a new type of parasomnia. ( info) |
6/9. Rhythmic movement disorder: polysomnographic study and summary of reported cases. Rhythmic movement disorder (RMD) is classified as a sleep-wake transition disorder. However, some RMD patients show rhythmic movements during rapid-eye-movement (REM) sleep, during which muscle activity is completely absent. In order to determine the sleep stages in which episodes of RMD occur, we investigated two children with RMD by means of polysomnography, and also summarized the polysomnographic reports on patients with RMD. We also quantified the REM sleep atonia in our patients using the tonic and phasic inhibition indices (TII and PII). In addition, to examine the involvement of the basal ganglia in RMD patients, we studied the frequency of gross movements (GMs) during sleep in each sleep stage. Both patients showed rhythmic movements in all sleep stages, i.e. including REM sleep. Few rhythmic movements occurred during sleep-wake transition periods. Both patients showed normal TII and PII scores as well as a normal pattern for the sleep stage-dependent modulation of GMs during sleep. Eighteen of the 33 reported RMD patients, including ours, experienced episodes during REM sleep, while the other 15 patients had no episodes during REM sleep. Among the 18 patients who had episodes during REM sleep, eight experienced the episodes exclusively during REM sleep. It is unlikely that the neuronal mechanisms that underlie RMD episodes were the same in the 15 patients who had no RMD episodes during REM sleep and the eight who had them only during REM sleep. We propose that RMD can be divided into several subgroups according to the differences in the underlying neuronal mechanisms. ( info) |
| BACKGROUND: dementia with lewy bodies (DLB) is often associated with REM sleep behavior disorders (RBD) characterized, in contrast to the usual paralysis of REM sleep, by violent motor and verbal activity. patients AND methods: The pharmacological management of RBD was investigated in three DLB patients treated with clonazepam, a benzodiazepine used as an antiepileptic, or donepezil, a cholinesterase inhibitor. RESULTS: All three patients had marked improvement. The pharmacodynamic mechanisms underlying the efficacy of the two drugs might be due to facilitator effect on the pedunculopontine nucleus, a key structure in the physiology of REM sleep. ( info) |
| BACKGROUND: quinine is commonly prescribed to the elderly for the treatment of benign nocturnal cramps, yet its use is not without complications. OBJECTIVE: This article presents a case of quinine toxicity producing bilateral blindness, followed by a review of the adverse reactions associated with quinine use and its efficacy in treating benign nocturnal muscular cramps. DISCUSSION: Visual loss has been associated with quinine serum concentrations above 10 microg/mL (therapeutic range 2-5 microg/mL). Other adverse reactions include neurological symptoms, haemolysis, acute renal failure and arrhythmia. There is conflicting evidence for the efficacy of quinine for leg cramps in randomised controlled studies, however, meta-analysis of these studies suggests some benefit. Although severe side effects are rare at therapeutic doses, the possibility of overdose needs to be considered when prescribing and an individual risk benefit analysis needs to be made. Benefits and adverse reactions should be closely monitored and medication ceased if appropriate. ( info) |
| This report describes a case of quinine-induced acute renal failure as a result of a combination of haemolytic uraemic syndrome and rhabdomyolysis with disseminated intravascular coagulation. The abrupt onset of symptoms occurred after ingestion of 300 mg of quinine along with atorvastatin. The patient recovered with supportive management, suggesting that plasma exchange may not be necessary in this situation. The possibility of a drug interaction contributing to rhabdomyolysis is raised. The proposed mechanism is through quinine inhibition of the cytochrome P450 isoenzyme 3A4, which may increase systemic levels of atorvastatin by reducing its first-pass metabolism. ( info) |