Progress in sleep disorders in multiple system atrophy / 中华神经科杂志

Multiple system atrophy can have various forms of sleep disorders, including insomnia, rapid eye movement sleep behavior disorder, sleep-disordered breathing, periodic leg movements during sleep and excessive daytime sleepiness. This article will focus on the concept, classification, pathogenesis, clinical features, diagnosis and treatment, aiming to deepen clinicians′ understanding of the disease, which will be helpful for early diagnosis and treatment.

Clinical study of sleep related rhythmic movement disorder / 中华神经科杂志

Objective To discuss the clinical and electrophysiological characteristics of sleep related rhythmic movement disorder ( SRMD).Methods We studied the clinical and electrophysiological characteristics of 3 patients diagnosed as SRMD in the Electroencephalography Monitoring Center of Xijing Hospital, Xi′an, China.The 3 patients accorded with diagnostic criteria of SRMD that international classification of sleep disorders-3 edition recommended and were followed up for more than 1 year.Results These 3 male patients were ranging from 6 to 27 years old.The onset age of the patient 1 was 13 years,and the others were 1 year old.The patient 2 became symptom-free at the age of 7.The patient 3 relieved at 2-year-old, but recurred at the age of 21. There was no epileptic seizure discharge in video-electroencephalography of the 3 patients, but synchronous electromyography changes during the attack were mistaken for slow wave.Video-polysomnography showed that numbers of awakenings and arousals index were high.Two patients were treated with clonazepam.One had an obvious curative effect, the other had marked efficacy until added trazodone.Conclusions SRMD can occur not only in infants, but also in adolescents and adults.Patients who have the problems of the sleep quality should be treated.Clonazepam can obviously relieve symptoms and improve sleep quality.Patients who do not have a good effect with clonazepam can try to add trazodone.Video-electroencephalography monitoring and interpreting it correctly are important to the diagnosis of paroxysmal disease.

Periodic Limb Movements During Sleep Associated with Human T-Lymphotropic Virus Type I-Associated Myelopathy

Periodic limb movements during sleep (PLMS) are frequently observed in the general population, although such movements may be associated with a variety of medical and neurological disorders. Human T-lymphotropic virus type I-associated myelopathy (HAM) is a rare progressive disease in which abnormalities are rarely observed on spinal images. We present the case of a 55-year-old woman with PLMS who was later diag-nosed with HAM. The current case indicates that HAM can be considered a possible cause of PLMS.

Mortality associated with periodic limb movements during sleep in amyotrophic lateral sclerosis patients / Mortalidade associada aos movimentos periódicos de membros durante o sono em pacientes com esclerose lateral amiotrófica

OBJECTIVE: To describe the prevalence and severity of periodic limb movements during sleep in amyotrophic lateral sclerosis patients and to explore this fact as a predictor of severity of the condition with respect to mortality. METHODS: In this case-control study, questionnaire and polysomnographic data were analyzed from 35 amyotrophic lateral sclerosis patients. Controls were matched by age, genre, and body mass index. A Kaplan-Meier curve was used to compare the survival time of patients with periodic limb movements of sleep index below or above 5. RESULTS: The number of amyotrophic lateral sclerosis patients with an index greater than five was higher than controls (19 (53%) versus 4 (11%); p<0.0001), and the mean index was higher (23.55±40.07 versus 3.28±8.96; p=0.0009). Earlier mortality was more common in patients with more than five periodic limb movements per hour of sleep than patients with less than five periodic limb movements per hour of sleep (7/19 (37%) versus 1/16 (6%); p=0.04) in this group of patients that had a mean survival of 33 months. CONCLUSIONS: There were more periodic limb movements of sleep in amyotrophic lateral sclerosis patients than in the control population. The higher number of these movements in amyotrophic lateral sclerosis patients correlates with disease severity and may suggest poor survival.

OBJETIVO:Descrever a prevalência e a severidade dos movimentos periódicos de membros durante o sono nos pacientes com esclerose lateral amiotrófica e explorar isso como um preditor de severidade da doença e mortalidade. MÉTODOS: Estudo caso controle em que foram analisados 35 pacientes por questionários e polissonografia. Os controles foram pareados por idade, gênero, e índice de massa corporal. Uma curva de Kaplan-Meier foi usada para comparar o tempo de sobrevida em pacientes com índice de movimento periódico de membros durante o sono acima e abaixo de 5. RESULTADOS: O número de pacientes com esclerose lateral amiotrófica com índice de movimentos periódicos de membros durante o sono acima de cinco foi maior do que os controles (19 (53%) versus 4 (11%); p<0,0001) e a média do índice de movimentos periódicos de membros durante o sono também foi maior no grupo dos pacientes (23,55±40,07 versus 3,28±8,96; p=0,0009). A mortalidade precoce foi mais comum em pacientes com mais que cinco movimentos durante o sono por hora do que pacientes com menos do que cinco movimentos durante o sono por hora (7/19 (37%) versus 1/16 (6%); p=0,04). Nesse grupo, os pacientes tiveram sobrevida média de 33 meses. CONCLUSÃO:Houve um maior número de movimentos periódicos de membros durante o sono em pacientes com esclerose lateral amiotrófica do que na população controle. O maior número de movimentos periódicos de membros durante o sono em pacientes com esclerose lateral amiotrófica foi correlacionado com severidade da doença e pode sugerir menor sobrevida.

Spinal cord injury as a trigger to develop periodic leg movements during sleep: an evolutionary perspective / Lesão medular como gatilho para desenvolver movimentos periódicos das pernas durante o sono: uma perspectiva evolutiva

The primary trigger to periodic limb movement (PLM) during sleep is still unknown. Its association with the restless legs syndrome (RLS) is established in humans and was reported in spinal cord injury (SCI) patients classified by the American Spinal Injury Association (ASIA) as A. Its pathogenesis has not been completely unraveled, though recent advances might enhance our knowledge about those malfunctions. PLM association with central pattern generator (CPG) is one of the possible pathologic mechanisms involved. This article reviewed the advances in PLM and RLS genetics, the evolution of CPG functioning, and the neurotransmitters involved in CPG, PLM and RLS. We have proposed that SCI might be a trigger to develop PLM.

O gatilho principal para o desenvolvimento de movimentos periódicos dos membros (MPM) durante o sono ainda é desconhecido. A associação entre o MPM e a síndrome das pernas inquietas (SPI) em seres humanos já foi previamente estabelecida e relatada em pacientes com lesão medular (LM), classificados pela American Spinal Injury Association (ASIA) como A. A patogênese do MPM não foi completamente desvendada, apesar de avanços recentes poderem ampliar o conhecimento sobre essas disfunções. Um dos possíveis mecanismos patológicos envolvidos é o gerador de padrão central (GPC). Este artigo revisou os avanços na genética do MPM e da SPI, a evolução do funcionamento do GPC e os neurotransmissores relacionados ao GPC, ao MPM e à SPI. Foi proposta a hipótese de que a LM poderia ser um gatilho para deflagrar os MPM.

Restless legs syndrome:23-cases report / 中华神经科杂志

Objective To investigate the clinical features of restless legs syndrome(RLS),its possible pathogenesis.and the effects of benserazide on the patients with RLS.Methods Twenty-three patients who met the primary diagnostic criteria of RLS were retrospectively analyzed.Results Twenty-three middle-aged or elderly patients aged 56 years in average had an average onset age of 52 years.Insomnia and daytime sleepiness were mostly common complains for these patients.Based on the diagnostic criteria of International RLS study group(IRLSSG),the average IRLSSG score was 25,and 16 cases(69%)of the patients were severe(21-30).Polysomnographic examination showed that 18 cases(78%)had periodic limb movement.in which the PLM index of 11 cases(61%)patients Was moderate(25-49).PLM-arousal index of all patients was increased.that of 16 cases(67%)patients being moderate.After treatment by levodopa/benserazide 100/25 mg at bedtime each night for 4 weeks,most of patients'subjective symptoms improved markedly.and the IRLSSG score Was obviously decreased.with an average score of 13,and 5 patients became completely normal.Among patients with periodic legs movement.the PLM index became normal in 5 patients and became mild in others.In addition.the PLM-arousal index in all patients Was markedly decreased.with that of 11 patients becoming normal.The sleep latency of 19 patients became normal.On the other hand,6 patients(26%)had transient headache,nausea,and lethargy.Conclusions Patients with discomfortable feeling of lower extremity which is mitigated after movement.and legs movement during sleep should be check up as early as possible.Benserazide may be considered as an effeetive medication for patients with RLS.