Diagnostics and current care of myasthenia gravis.

Myasthenia gravis (MG) is the most common neuromuscular transmission disorder, causing weakness of skeletal muscles on exertion. The course of the disease is highly variable, symptoms and signs may change rapidly due to infection or pregnancy. MG is classified using serological, electrophysiological and pharmaceutical tools.

Decreased Aerobic Capacity in ANO5-Muscular Dystrophy.

Background: Anoctaminopathies are muscle diseases caused by recessive mutations in the ANO5 gene. The effects of anoctaminopathy on oxidative capacity have not previously been studied in a controlled setting.

Objective: To characterize oxidative capacity in a clinically and genetically well-defined series of patients with anoctaminopathy.

Predicting Obstructive Sleep Apnea with Periodic Snoring Sound Recorded at Home.

Study Objectives: The cost-effectiveness of diagnosing obstructive sleep apnea (OSA) could be improved by using a preliminary screening method among subjects with no suspicion of other sleep disorders. We aimed to evaluate the diagnostic value of periodic snoring sound recorded at home.

Methods: We included 211 subjects, aged 18-83 (130 men), who were referred to our laboratory for suspicion of OSA, and had a technically successful overnight polygraphy, measured with the Nox T3 Sleep Monitor (Nox Medical, Iceland) with a built-in microphone.

Progressive cranial nerve involvement and grading of facial paralysis in gelsolin amyloidosis.

Introduction: Hereditary gelsolin amyloidosis (GA) is a rare condition caused by the gelsolin gene mutation. The diagnostic triad includes corneal lattice dystrophy (type 2), progressive bilateral facial paralysis, and cutis laxa. Detailed information on facial paralysis in GA and the extent of cranial nerve injury is lacking.

Intravenous lipid emulsion given to volunteers does not affect symptoms of lidocaine brain toxicity.

Intravenous lipid emulsion has been suggested as treatment for local anaesthetic toxicity, but the exact mechanism of action is still uncertain. Controlled studies on the effect of lipid emulsion on toxic doses of local anaesthetics have not been performed in man. In randomized, subject-blinded and two-phase cross-over fashion, eight healthy volunteers were given a 1.

Frontal electroencephalogram variables are associated with the outcome and stage of hepatic encephalopathy in acute liver failure.

Acute liver failure (ALF) and hepatic encephalopathy (HE) can lead to an elevated intracranial pressure (ICP) and death within days. The impaired liver function increases the risks of invasive ICP monitoring, whereas noninvasive methods remain inadequate. The purpose of our study was to explore reliable noninvasive methods of neuromonitoring for patients with ALF in the intensive care unit (ICU) setting; more specifically, we wanted to track changes in HE and predict the outcomes of ALF patients treated with albumin dialysis.

Cyclic alternating pattern is associated with cerebral hemodynamic variation: a near-infrared spectroscopy study of sleep in healthy humans.

The cyclic alternating pattern (CAP), that is, cyclic variation of brain activity within non-REM sleep stages, is related to sleep instability and preservation, as well as consolidation of learning. Unlike the well-known electrical activity of CAP, its cerebral hemodynamic counterpart has not been assessed in healthy subjects so far. We recorded scalp and cortical hemodynamics with near-infrared spectroscopy on the forehead and systemic hemodynamics (heart rate and amplitude of the photoplethysmograph) with a finger pulse oximeter during 23 nights in 11 subjects.

Screening of snoring with an MP3 recorder.

Purpose: Snoring patients seeking medical assistance represent a wide range of clinical and sleep study findings from nonsleepy nonapneic snoring to severe obstructive sleep apnea syndrome. The prevalence of snoring is high and it significantly impacts quality of life. Its objective diagnosis usually requires a sleep study.

Spontaneous hemodynamic oscillations during human sleep and sleep stage transitions characterized with near-infrared spectroscopy.

Understanding the interaction between the nervous system and cerebral vasculature is fundamental to forming a complete picture of the neurophysiology of sleep and its role in maintaining physiological homeostasis. However, the intrinsic hemodynamics of slow-wave sleep (SWS) are still poorly known. We carried out 30 all-night sleep measurements with combined near-infrared spectroscopy (NIRS) and polysomnography to investigate spontaneous hemodynamic behavior in SWS compared to light (LS) and rapid-eye-movement sleep (REM).

Recovery of brachial plexus lesions resulting from heavy backpack use: a follow-up case series.

Background: Brachial plexus lesions as a consequence of carrying a heavy backpack have been reported, but the typical clinical course and long-term consequences are not clear. Here we evaluated the clinical course and pattern of recovery of backpack palsy (BPP) in a large series of patients.

Methods: Thirty-eight consecutive patients with idiopathic BPP were identified from our population of 193,450 Finnish conscripts by means of computerised register.

Progressive Stroke-Like Symptoms in a Patient with Sporadic Creutzfeldt-Jakob Disease.

Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare neurodegenerative disorder in which accumulation of a pathogenic isoform of prion protein (PrP(Sc)) induces neuronal damage with distinct pathologic features. The prognosis of sCJD is devastating: rapid clinical decline is followed by death generally within months after onset of symptoms. The classic clinical manifestations of sCJD are rapidly progressing dementia, myoclonus, and ataxia.

[Update on current care guidelines. The treatment of status epilepticus].

Status epilepticus is a medical emergency. Most epileptic seizures last for 1-4 minutes and seizures lasting over five minutes, should be treated as status epilepticus. EEG is essential for diagnostics and the monitoring of treatment effect.

[EEG examination in emergency medicine and analysis of unconciousness].

For a long time, the EEG examination has been used as a laboratory examination especially in the diagnostics of epilepsy, but both technical difficulties and problems in interpretation have limited its use in emergency medicine outside special neurological intensive care units. Development of monitoring technology and easier data transfer is making EEG monitoring possible also in the emergency area. The greatest need focuses on the diagnostics and treatment of status epilepticus underlying an unclear unconsciousness.

Severe ataxia with neuropathy in hereditary gelsolin amyloidosis.

Hereditary gelsolin amyloidosis (AGel amyloidosis) is a systemic disorder caused by a G654A or G654T gelsolin mutation, reported from Europe, North America, and Japan. Principal clinical signs are corneal lattice dystrophy, cutis laxa and cranial neuropathy, often deleterious at advanced age. Peripheral neuropathy, if present, is usually mild.

Hypothermia-treated cardiac arrest patients with good neurological outcome differ early in quantitative variables of EEG suppression and epileptiform activity.

Objective: To evaluate electroencephalogram-derived quantitative variables after out-of-hospital cardiac arrest.

Design: Prospective study.

Setting: University hospital intensive care unit.

Prediction of poor outcome using detector of epileptiform EEG in ICU patients resuscitated after cardiac arrest.

Assessing the brain status of patients admitted to intensive care unit (ICU) after out-of-hospital cardiac arrest is challenging. We had earlier found wavelet subband entropy (WSE) to be a useful tool for quantifying the epileptiform content of EEG during anesthesia. In this paper, WSE was applied for EEG of ICU patients to study its prognostic value.

Dysphonia and dysphagia after anterior cervical decompression.

Object: In this paper, the authors investigate the effects of anterior cervical decompression (ACD) on swallowing and vocal function.

Methods: The study comprised 114 patients who underwent ACD. The early group (50 patients) was examined immediately pre- and postoperatively, and the late group (64 patients) was examined at only 3 to 9 months postoperatively.

Severe ataxia with neuropathy in hereditary gelsolin amyloidosis: a case report.

Hereditary gelsolin amyloidosis (AGel amyloidosis) is a systemic disorder caused by a G654A or G654T gelsolin mutation, reported from Europe, North America, and Japan. Principal clinical signs are corneal lattice dystrophy, cutis laxa and cranial neuropathy, often deleterious at advanced age. Peripheral neuropathy, if present, is usually mild.

Smoking and improvement after nasal surgery in snoring men.

Background: The aim of this study was to compare subgroups of smokers and nonsmokers undergoing nasal surgery and to evaluate improvement of nasal stuffiness, snoring, and symptoms related to sleep-disordered breathing after nasal surgery.

Methods: A cross-sectional prospective study was performed. The study population included 40 consecutive snoring men scheduled for surgical treatment of nasal obstruction.

Entropy is more resistant to artifacts than bispectral index in brain-dead organ donors.

Objective: To evaluate the usefulness of entropy and the bispectral index (BIS) in brain-dead subjects.

Design And Setting: A prospective, open, nonselective, observational study in the university hospital.

Patients And Participants: 16 brain-dead organ donors.

Snoring is not relieved by nasal surgery despite improvement in nasal resistance.

Objectives: In the present study, we evaluated the effect of nasal surgery on snoring time, snoring intensity, and sleep-disordered breathing. The role of abnormal cephalometry in treatment outcome was assessed.

Design: A cross-sectional prospective study.

Patient- and bed partner-reported symptoms, smoking, and nasal resistance in sleep-disordered breathing.

Objectives: Nocturnal and daytime symptoms are important determinants in clinical decision making in patients suspected of having sleep-disordered breathing (SDB). We compared patients' and bed partners' reporting of symptoms associated with SDB in a clinical sample of snoring men. The bed partners' view on snoring disturbance was assessed.