-We report on the clinical characteristics of amyotrophic lateral sclerosis (ALS) in Fortaleza (Northeastern Brazil). For this, we analyzed retrospectively (from 1980 to 1999) 78 cases of ALS from the Service of Neurology of the University Hospital of Fortaleza diagnosed clinically and laboratorially (EMG, muscle biopsy, myelography, blood biochemistry, muscle enzymes and cranio-cervical X-ray). The results showed that they were mostly sporadic ALS (76/78), and they were divided into definite (n= 36), probable (n= 20), possible (n= 15) and suspected (n= 7), according to the level of diagnostic certainty. They were also subdivided into juvenile (n= 17), early-onset adult (n= 18), age-specific (n= 39) and late-onset (n= 4) groups. Clinically, they presented as initials symptoms, principally, asymmetrical (30/78) and symmetrical (24/78) weakness of extremities, besides bulbar signs, fasciculations, and atrophy. Curiously, pain as first symptom occurred in an expressive fashion (17/78). The predominant initial anatomic site, in this series, was the spinal cord, and mainly affecting the arms. As to the symptom accrual from region to region, this occurs more quickly in contiguous areas, and fasciculations are predominant when bulbar region was associated.KEY WORDS: ALS, sporadic, early-onset, pain, anatomy, symptom accrual, fasciculations. Esclerose lateral amiotrófica: análise clínica de 78 casos de Fortaleza (Nordeste Brasileiro)RESUMO -Descrevemos as características clínicas da esclerose lateral amiotrófica (ELA) em Fortaleza (Nordeste Brasileiro). Para isso, fizemos uma análise retrospectiva (de 1980 a 1999) de 78 casos de ELA do Serviço de Neurologia do Hospital Universitário de Fortaleza, diagnosticados do ponto de vista clínico e laboratorial (EMG, biópsia de músculo, mielografia, análises hematológica e bioquímica, e raio X da junção crânio-cervical). Os resultados mostraram que esses casos eram principalmente da forma esporádica (76/78), e estes foram divididos, de acordo com o nível de certeza diagnóstica, em definidos (n= 36), prováveis (n= 20), possíveis (n= 15) e suspeitos (n= 7). Eles foram também subdivididos nos grupos juvenil (n= 17), adulto jovem (n= 18), idadeespecífico (n= 39) e tardio (n= 4). Clinicamente, os principais sintomas iniciais foram fraqueza assimétrica (38/ 78) e simétrica (24/78) das extremidades, além de sinais bulbares, fasciculações e atrofia. Curiosamente, dor como sintoma inicial ocorria de maneira expressiva (17/78). O local anatômico inicial predominante foi, nessa série, a medula espinhal, afetando principalmente os braços. O avanço regional de sintomas ocorreu mais rapidamente em áreas contíguas, e as fasciculações eram predominantes quando a região bulbar estava associada. PALAVRAS-CHAVE: ELA, esporádica, início precoce, dor, anatomia, avanço dos sintomas, fasciculações.Amyotrophic lateral sclerosis (ALS) is a condition of undetermined cause, which affects the anterior horn cells and the corticospinal tract.The etiology is manifold so that different causes have been...

-We report on the preliminary clinical and electrophysiological aspects of an in-patient possibly presenting epilepsia partialis continua (Koshevnikov). We discuss the different etiologies and emphasize on the possible idiopathic form in this case.KEY WORDS: epilepsia partialis continua, electrophysiology, symptoms, idiopathic form. Epilepsia parcial contínua (Kojevnikov): relato preliminar de um casoRESUMO -Relatamos os aspectos clínicos e eletrofisiológicos preliminares de um paciente hospitalizado que possivelmente apresenta crises de epilepsia parcial contínua (Kojevnikov). Discutimos as diferentes etiologias dessa condição e salientamos a possível forma idiopática, nesse caso.

Background: Sleep disturbances have been associated with poor glycemic control in differential clinical settings. Both short and long sleep duration, influence insulin resistance and blood glucose in diabetic patients. Pregnancy is an additional risk for reduced sleep quality and quantity, and the presence of hyperglycemia, as a complicating factor, has being increasingly frequent. Different measures of sleep evaluation, both objectively and subjectively, can provide additional information about the influence of sleep in metabolic control in Gestational Diabetes Mellitus (GDM). Objective: To investigate the influence of sleep quality and objective sleep measures on glycated hemoglobin (HBA1C) in patients with GDM. Methodology: This is a cross-sectional study examining patients with GDM from 2nd to 3rd trimester of pregnancy. Clinical data and behavior questionnaires were collected by a face-to-face interview. Self-Rated Sleep Quality was evaluated by Pittsburgh Sleep Quality Index- (PSQI). In order to improve the accuracy of the information, a 14-day sleep log was obtained, and objective sleep measurements were registered by actigraphic record (5 to 7 days). Results: Overall, GDM patients (N=311), aged from 20 to 46 y (33.1±5.6) were evaluated. Sleep duration ≤6 hours/night was found in 43.4%, and 63.9% reported poor sleep quality (PSQI>5). Sleep duration measured by actigraphy was correlated with sleep duration registered by sleep log (r=.45, p=.04), and with PSQI (r=-.33, p=.002). Sleep quality and sleep duration registered by either actigraphy or sleep log were not correlated with Hba1c. Amongst all, Hba1c varied from 4.3 to 7.0 mg/dL (5.9 ±.53). Sleep fragmentation, measured by the length of time patient spends awake after sleep onset (WASO) was correlated withHba1c level in patients with GDM (r=.41, p=0.04). Conclusion: Sleep duration obtained from the sleep log was a reliable measure correlating with objective sleep parameters registered by actigraphy and with sleep quality. In GDM patients, increased wake time after sleep onset was correlated with higher Hba1c.

Introduction: Gestational diabetes mellitus (GDM) is an increasingly frequent complication of pregnancy and its presence is related to the development of adverse maternal and fetal outcomes. Pregnancy is deeply influenced by the circadian rhythm and the misalignment of the maternal rhythm can lead to disturbances in the temporal organization of physiological and metabolic functions. Previously, the association between circadian rhythm disorders and diabetes has been well described in pregnancy. Objective: To investigate the influence of chronotype on the development of maternal and fetal complications during pregnancy in GDM. Methodology: Prospective cohort study of patients with GDM. Clinical data and behavior questionnaires were collected by face- to- face interview. Questionnaires evaluated the chronotype (Morning-Eveningness-Questionnaire- MEQ), sleep quality (Pittsburgh Sleep Quality Index- PSQI), daytime sleepiness (Epworth Sleepiness Scale -ESS), depressive symptoms (Edinburgh Postnatal Depression Scale-EDPS) and insomnia (Insomnia Severity Index- ISI). Sleep diaries (14 days) and actigraphic record (5 to 7 days) were obtained. Results: Overall, 305 2nd to 3rd trimester GDM patients were evaluated. Eighty-nine GDM patients completed the sleep diary and 53 performed a valid actigraphic registry. Amongst these, 49.5% were identified as morning chronotypes (MEQ≥59), 43.6% intermediate types (MEQ from 42 to 58) and 6.9% had evening type preference (MEQ<41). Evening preference patients were younger (p< 0.005), had more unstable marital status (P = 0.04), had worse sleep quality (p = 0.02); latter sleep midpoint (p= 0.01), higher prevalence of insomnia (p< 0.005) and depression (p= 0.004) before and during pregnancy. No differences were found in total sleep duration as recorded by sleep diary (p=0.4) or actigraphic measurements (p=0.83). Evening type preference in GDM was associated with pre-eclampsia (p= 0.02) and neonatal ICU admission (p= 0.03). Conclusion: This study suggests that the presence of the evening-type chronotype in pregnant women with GDM is associated with adverse pregnancy outcomes such as the development of hypertensive syndromes, insomnia and depressive symptoms. In addition, a higher prevalence of newborns admitted to the neonatal ICU was found in the offspring of GDM patients with evening preference.