1/64. melatonin treatment of non-epileptic myoclonus in children.Oral melatonin (MLT) has been used by our Vancouver research group in the treatment of paediatric sleep disorders since 1991; slightly over 200 children, mainly with multiple disabilities, who frequently had seizures, have been treated. Three children with markedly delayed sleep onset due to recurring myoclonus were also referred for MLT treatment: two had non-epileptic, and one had epileptic and non-epileptic myoclonus. Low doses of oral MLT (3 to 5 mg) unexpectedly abolished their myoclonus and allowed them to sleep. There were no adverse effects. It appears that certain types of myoclonus, which might be resistant to conventional anticonvulsant medications, may respond to MLT but the mechanism of action is unclear. Further research on this novel treatment is urgently needed.- - - - - - - - - - ranking = 1keywords = sleep (Clic here for more details about this article) |
2/64. Idiopathic rolandic epilepsy with "interictal" facial myoclonia and oromotor deficit: a longitudinal EEG and PET study.PURPOSE: The prognosis of benign epilepsy with centrotemporal spikes (BECTS) is always favorable as far as the epilepsy is concerned. However, some data suggest that affected children may be at risk for minor cognitive impairment. We report here the longitudinal study of a young girl demonstrating that BECTS also may be associated with severe motor disturbances. methods: BECTS (rare left oromotor seizures, right rolandic spike-waves activated during sleep) started at the age of 3 years 6 months in a girl with normal initial psychomotor development. Her clinical, neuropsychological, and EEG status was assessed every 3-6 months. Regional cerebral glucose metabolism was measured by using the [18F]fluorodeoxyglucose-positron emission tomography (FDG-PET) method. RESULTS: Between the age of 5 and 6 years, the girl had (a) increased seizure frequency; (b) brief perioral and palpebral myoclonic jerks, concomitant with the spike component of interictal spike-waves, and (c) persistent but fluctuating oromotor deficits (drooling, dysarthria, dysphagia). The EEG showed a marked increase in abundance and amplitude of wake and sleep interictal abnormalities, which became bilateral. Awake FDG-PET revealed a bilateral increase of glucose metabolism in opercular regions. A complete and definitive EEG and clinical remission occurred at age 5 years 11 months and has persisted since (present age, 7 years 9 months). CONCLUSIONS: This case confirms that during BECTS, epileptiform dysfunctions within rolandic areas may induce "interictal" positive or negative oromotor symptoms, independent of classic seizures.- - - - - - - - - - ranking = 0.66666666666667keywords = sleep (Clic here for more details about this article) |
3/64. Disappearance of rhythmic involuntary movements during sleep in a case of olivopontocerebellar atrophy.We report on a 54-year-old woman with an 8 or so year history of olivopontocerebellar atrophy associated with the rhythmic involuntary movements of the left upper and lower limbs, and cervical region. Surface electromyogram of the left upper limb revealed rhythmicity (about 3 Hz) and reciprocity between antagonistic muscles, which disappeared on polysomnography at all sleep stages including rapid eye movement sleep without atonia. These were characterized by the co-existence of rhythmic skeletal myoclonus and parkinsonian tremor. These findings suggest that a disturbance of the striatonigral system as well as the dentato-rubro-olivary circuit may be involved in these movements. It also seems that their fate is dependent on the level of wakefulness and that the ascending reticular activating system also plays a role in the development of these movements.- - - - - - - - - - ranking = 2keywords = sleep (Clic here for more details about this article) |
4/64. myoclonus secondary to the concurrent use of trazodone and fluoxetine.A 39-y-o male with a history of human immunodeficiency virus infection and depression was admitted for diagnosis and treatment of tuberculosis and pneumocystis carinii pneumonia infections. Prior to admission, he was on 50 mg trazodone every evening for 2 mo for depression. He was admitted with a 2-w history of fever chills and fatigue and on admission had hand tremors which disappeared at rest. Four days post-admission the trazodone dose was increased to 100 mg and 20 mg fluoxetine was initiated. He became increasingly anxious and his hand tremor worsened 3 d after initiation of the regimen. To rule out drug induced tremor, both trazodone and fluoxetine were discontinued and symptoms resolved in 7 d. Clinicians should be aware of the potential for excessive seratonergic activities secondary to trazodone fluoxetine interactions causing a worsening myoclonus adverse event.- - - - - - - - - - ranking = 0.0011811557294654keywords = initiation (Clic here for more details about this article) |
5/64. 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.- - - - - - - - - - ranking = 4.1007843093747keywords = sleep, insomnia (Clic here for more details about this article) |
6/64. Familial nocturnal facio-mandibular myoclonus mimicking sleep bruxism.A mother and son presented with a multi-decade history of nocturnal tongue biting and bleeding. In both patients, video polysomnographic recordings documented bursts of electromyographic activity originating in the masseter and spreading to orbicularis oris and oculi muscles, present only during sleep. Faciomandibular myoclonic activity during sleep mimics sleep bruxism and may be familial.- - - - - - - - - - ranking = 2.3333333333333keywords = sleep (Clic here for more details about this article) |
7/64. Friedreich's ataxia with chorea and myoclonus caused by a compound heterozygosity for a novel deletion and the trinucleotide GAA expansion.Friedreich's ataxia (FRDA) is the most common hereditary ataxia, affecting about 1 in 50,000 individuals. It is caused by mutations in the frataxin gene; 98% of cases have homozygous expansions of a GAA trinucleotide in intron 1 of the frataxin gene. The remaining 2% of patients are compound heterozygotes, who have a GAA repeat expansion in one allele and a point mutation in the other allele. FRDA patients with point mutation have been suggested to have atypical clinical features. We present a case of compound heterozygotes in a FRDA patient who has a deletion of one T in the start codon (ATG) of the frataxin gene and a GAA repeat expansion in the other allele. The patient presented with chorea and subsequently developed FRDA symptoms. The disease in this case is the result of both a failure of initiation of translation and the effect of the expansion. This novel mutation extends the range of point mutations seen in FRDA patients, and also broadens the spectrum of FRDA genotype associated with chorea.- - - - - - - - - - ranking = 0.0011811557294654keywords = initiation (Clic here for more details about this article) |
8/64. unconsciousness, automatisms, and myoclonic jerks with diffuse rhythmic 9-Hz discharges.A 25-year-old man presented with seizures characterized by unconsciousness, automatic behavior, and myoclonic jerks lasting 0.5 to 8 sec. Videotape analysis of 300 simultaneously recorded electroencephalographic and clinical attacks revealed 8- to 9-Hz 150 to 200 muV sharp waves beginning in both medial temporal areas during eyelid flutters. When discharges spread diffusely on both sides and lasted 2 to 3 sec, 20 degrees forward flexion of the head, unconsciousness, pupil dilation, and staring became evident. Asymmetrical myoclonic jerks invariably appeared. When paroxysms were greater than 3 sec, automatisms and tonic posturing occurred. The electroencephalographic and clinical seizures appeared during the awake and relaxed states, i.e., with normal alpha rhythm. They were suppressed during physical and mental activities and during sleep, i.e., in the absence of alpha rhythm. Our studies suggest that these seizures associated with diffuse electroencephalographic alpha-like activities are subcortical in origin and should be tested in their responsivity to alpha suppressants and inducers.- - - - - - - - - - ranking = 0.33333333333333keywords = sleep (Clic here for more details about this article) |
9/64. Childhood-onset epilepsy associated with polymicrogyria.To study the electroclinical characteristics of patients with childhood-onset epilepsy who showed polymicrogyria (PMG) on MRI, we classified 15 patients according to the location of PMG on MRI. The composition of the subjects was as follows: four patients with PMG in both hemispheres; three with localized PMG in one hemisphere associated with other lesions such as porencephaly; and eight with only localized PMG in one hemisphere. We investigated the electroclinical characteristics of the epileptic syndromes associated with these different types of PMG. Four patients suffered from infantile spasms during their clinical course. Five patients suffered from epilepsy with electrical status epilepticus during slow sleep (ESES) and ESES-related epilepsy. The other six patients had only localization-related epilepsy throughout their clinical course. patients with PMG in both hemispheres, and localized PMG in one hemisphere associated with other lesions tended to have early-onset intractable seizures, especially infantile spasms. On the other hand, patients with only localized PMG in one hemisphere had ESES and ESES-related epilepsy or localization-related epilepsy, and their seizure prognosis was relatively favorable. These findings are useful in predicting the outcome of patients with PMG.- - - - - - - - - - ranking = 0.33333333333333keywords = sleep (Clic here for more details about this article) |
10/64. epilepsia partialis continua associated with widespread gliomatosis cerebri.We report an uncommon association of intractable epilepsia partialis continua that was the main presentation of widespread gliomatosis cerebri in two females. Both children had a preceding prolonged secondary generalized seizure 2-4 months before the evolution of epilepsia partialis continua, including recurrent clusters of left-sided myoclonic twitching and sensory impairment. During these events, the children remained fully alert. These seizures were corroborated by prolonged focal epileptic spike/wave discharges evident on the electroencephalograms. Cerebral magnetic resonance imaging in the first patient demonstrated a wide area of increasing signals over the right frontocentral regions, along with diffuse cortical-subcortical infiltration impinging on the left hemisphere. In the second patient a cortical lesion was suspected. Evaluation for Rasmussen's encephalitis, focal cortical dysplasia, or a gliomatous process was conducted; the patients underwent a stereotactic brain biopsy in which the histologic findings were compatible with gliomatosis cerebri with diffuse widespread infiltration of glioma cells with no constitution of a circumscribed tumor mass. The first patient was treated with cranial radiation, chemotherapy, steroids, and combined antiepileptic therapy. The focal seizures gradually but markedly decreased in frequency, and sensory impairment abated within 18 months after establishment of the diagnosis and ensuing therapy. cognition remains intact. The second female died 2 years after presentation despite massive chemotherapy and antiepileptic medications. Although rare, gliomatosis cerebri should be taken into account in the differential diagnosis of epilepsia partialis continua in children to facilitate a rapid diagnosis and initiation of prompt treatment of this rare disorder that may respond to a concurrent effective combination of cranial radiation, chemotherapy, and antiepileptic medications.- - - - - - - - - - ranking = 0.0011811557294654keywords = initiation (Clic here for more details about this article) |
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