Sleep–wake problems in patients with amyotrophic lateral sclerosis: implications for patient management

SUMMARY Sleep–wake problems are frequent, although unrecognized, complications of amyotrophic lateral sclerosis (ALS). Sleep disorders such as insomnia, sleep-disordered breathing and restless legs syndrome have all been reported in patients with ALS, despite the limited number of studies and the small populations investigated so far. Sleep disturbances gradually worsen with disease progression, suggesting a relationship between the severity of disease and the neurodegenerative process. However, poor sleep can also be a consequence of several disturbances such as anxiety, depression, pain, choking, sialorrhea, fasciculations, cramps, nocturia and the inability to get comfortable and move f…

REM sleep behavior disorder in a patient with frontotemporal dementia

We describe a patient with frontotemporal dementia (FTD), a tauopathy, who also showed clinical and polysomnographic features of REM sleep behavior disorder (RBD). The patient is a 78-year-old male with a 1 year history of behavioral dysfunction involving emotion, character and social functioning. Brain imaging and the results of neuropsychological testing were consistent with a diagnosis of FTD. Sleep symptom onset occurred some years before the behavioral changes, and consisted of unpleasant dreams, vocalization, and prominent motor behaviors. A polysomnography confirmed the diagnosis of RBD. Our findings support the hypothesis that RBD, although more frequent in synucleinopathies, might …

Exposure to ototoxic agents and hearing loss: A review of current knowledge

Several experimental and clinical studies have shown that a variety of ototoxic agents (such as drugs, industrial chemicals and noise) can cause sensorineural hearing loss. The most common ototoxic drugs used in clinical practice include: aminoglycoside and macrolide antibiotics, quinoline anti-malarials, platinum analog antineoplastics, loop diuretics, and acetylsalicylic acid. Among chemical agents with potential ototoxic properties are: organic solvents, heavy metals, organotins, nitriles, asphyxiants, and pesticides/herbicides. Acoustic exposure to high intensity and/or prolonged noise can also cause permanent threshold shifts in auditory perception. Ototoxic agents can influence audito…

THORACIC SYRINGOMYELIA IN A PATIENT WITH AMYOTROPHIC LATERAL SCLEROSIS

We report a patient with bulba r - onset, clinically defined, sporadic amyotrophic lateral sclerosis bearing an isolated syringomyelia of the lower thoracic portion of the spinal cord. This is a very unusual association between two rare and progressive disorders, both affecting the spinal motoneurons. Syringomyelia might have acted as a phenotypic modifier in this ALS patient.

FATIGUE, SLEEP, AND NOCTURNAL COMPLAINTS IN PATIENTS WITH AMYOTROPHIC LATERAL SCLEROSIS.

Background and purpose: Fatigue is a common symptom in amyotrophic lateral sclerosis (ALS). Although sleep disturbances are a candidate factor that may interfere with fatigue in patients with ALS, the role of sleep-related abnormalities in determining fatigue in ALS is unknown. Objective: To evaluate the frequency and determinants of fatigue in a group of 91 consecutive patients with ALS, with special attention to the relationship between fatigue and sleep problems. Methods: Measures included the Fatigue Severity Scale (FSS), Pittsburgh Sleep Quality Index (PSQI), Epworth Sleepiness Scale (ESS), ALS Functional Rating Scale- Revised (ALSFRS-R), and Beck Depression Inventory (BDI). Results: T…

A SPG4 GENE MUTATION CAUSES AUTOSOMAL DOMINANT SPASTIC PARAPLEGIA IN AN ITALIAN FAMILY

Cognitive impairment and stroke in elderly patients

Daniele Lo Coco,1 Gianluca Lopez,1 Salvatore Corrao,2,31Neurology and Stroke Unit, 2Department of Internal Medicine, National Relevance and High Specialization Hospital Trust ARNAS Civico, Di Cristina, Benfratelli, Palermo, 3Centre of Research for Effectiveness and Appropriateness in Medicine (C.R.E.A.M.), Di.Bi.M.I.S., University of Palermo, Palermo, Italy Abstract: We reviewed current knowledge about the interaction between stroke and vascular risk factors and the development of cognitive impairment and dementia. Stroke is increasingly recognized as an important cause of cognitive problems and has been implicated in the development of both Alzheimer's disease and vascular dementia…

Sleep quality in caregivers of patients with Alzheimer’s disease and Parkinson’s disease and its relationship to quality of life

ABSTRACTBackground: Knowledge about sleep complaints of caregivers of patients with Alzheimer's disease (AD) and Parkinson's disease (PD) is limited, and we lack information about the relationship between caregivers’ sleep problems and their quality of life (QoL).Methods: We evaluated subjective sleep quality and its relationship to QoL in a group of 80 caregivers of patients with AD (ADCG, n = 40) and PD (PDCG, n = 40), and in 150 controls. Information about night-time complaints was collected using the Pittsburgh Sleep Quality Index (PSQI). QoL was measured using the McGill QoL Questionnaire.Results: Eighteen ADCG (45%), 22 PDCG (55%), and 45 (30%) controls reported poor sleep quality. Me…

URIC ACID LEVELS IN SERUM AND CSF OF ALS PATIENTS

Objective: Urate (UA) is a potent antioxidant that effectively scavenges reactive nitrogen and oxygen radicals, and persons with a high plasma UA level may be at lower risk of some neurodegenerative disorders, as Parkinson’s disease (PD). Low plasma UA level has been observed in Alzheimer’s disease (AD) and vascular dementia, but there is no data on correlations to neuropsychological test results in these patient groups. Amyotrophic Lateral Sclerosis (ALS) is a devastating motor neuron disease, with a highly variable rate of progression and whose diagnosis is chiefly based on clinical and neurophysiological parameters. The etiopathogenesis is unknown, but the oxidative stress seems to play …

Restless legs syndrome in patients with amyotrophic lateral sclerosis

We aimed to evaluate the frequency and determinants of restless legs syndrome (RLS) in a group of 76 patients with amyotrophic lateral sclerosis (ALS) and 100 control subjects. A diagnosis of RLS was made according to the criteria of the International RLS Study Group, and severity was assessed by the RLS severity scale. RLS was significantly more frequent in patients with ALS (ALS/RLS(+)) than in control subjects (25% vs. 8%; P = 0.002). Compared with control subjects, patients with ALS/RLS(+) showed shorter history of RLS complaints and higher frequency of symptoms occurrence. Moreover, compared with those without RLS, patients with ALS/RLS(+) showed increased functional impairment and mor…

Sleep-wake disturbances in patients with amyotrophic lateral sclerosis

Objective To evaluate the frequency, severity and determinants of sleep disturbances in patients with amyotrophic lateral sclerosis (ALS). Methods Information about night-time complaints was collected using a standardised questionnaire, the Pittsburgh Sleep Quality Index (PSQI), and the Epworth Sleepiness Scale (ESS) in a group of 100 patients with ALS and in 100 control subjects matched for age and sex. Functional disability was assessed using the ALS Functional Rating Scale-Revised (ALSFRS-R). Sleep was studied by overnight polysomnography in 12 patients. Results Fifty-nine patients with ALS and 36 controls reported sleep disturbances. The mean global PSQI score of patients with ALS was s…

The amyotrophic lateral sclerosis functional rating scale predicts survival time in amyotrophic lateral sclerosis patients on invasive mechanical ventilation.

Objective: To determine whether the amyotrophic lateral sclerosis functional rating scale (ALSFRS), which is a validated instrument that assesses the functional status and the disease progression in patients with amyotrophic lateral sclerosis (ALS), predicts hospital length of stay and survival time in ALS patients treated with tracheostomy-intermittent positive-pressure ventilation (TIPPV). Methods: Thirty-three consecutive ALS patients with acute respiratory failure who received therapy with TIPPV were prospectively followed up from their admission to the hospital until death. The association of ALSFRS score at hospital admission with length of hospital stay and survival after TIPPV were …

Inappropriate sexual behaviors associated with donepezil tratment: a case report

We documented a patient with Alzheimer's Disease with profound changes in sexuality after donepezil treatment. We suggested that inappropriate sexual behaviors associated with donepezil, although uncommon adverse event, could develop after donepezil should be taken into consideration by the phisicians prescribing this drug

RESTLESS LEGS SYNDROME IN PATIENTS WITH AMYOTROPHIC LATERAL SCLEROSIS: A CASE-CONTROL STUDY.

Cognitive impairment and stroke in elderly patients.

Daniele Lo Coco,1 Gianluca Lopez,1 Salvatore Corrao,2,31Neurology and Stroke Unit, 2Department of Internal Medicine, National Relevance and High Specialization Hospital Trust ARNAS Civico, Di Cristina, Benfratelli, Palermo, 3Centre of Research for Effectiveness and Appropriateness in Medicine (C.R.E.A.M.), Di.Bi.M.I.S., University of Palermo, Palermo, Italy Abstract: We reviewed current knowledge about the interaction between stroke and vascular risk factors and the development of cognitive impairment and dementia. Stroke is increasingly recognized as an important cause of cognitive problems and has been implicated in the development of both Alzheimer's disease and vascular dementia. Th…

LA DISFUNZIONE RESPIRATORIA NELLA SCLEROSI LATERALE AMIOTROFICA

Noninvasive Positive Pressure Ventilation in Amyotrophic Lateral Sclerosis

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease of unknown origin that affects approximately 1.5 individuals per 100,000 every year; usually, these individuals are between 55 and 75 years of age [1]. ALS causes progressive weakness of voluntary muscle groups, including respiratory ones, and respiratory failure or pneumonia related to respiratory muscle weakness is the most frequent cause of death.

ECHOCARDIOGRAPHIC ASSESSMENT OF 537 DOGS WITH MITRAL VALVE PROLAPSE AND LEAFLET INVOLVEMENT

In this work we investigated which mitral valve leaflet was most often involved in mitral valve prolapse with degenerative mitral valve disease and whether there was an association with breed, age, gender, or weight. Five hundred and thirty-seven dogs with mitral valve prolapse-degenerative mitral valve disease were assessed; the cross-breed dog was the most represented breed (248 dogs, 46.2%). Mitral valve prolapse was more common in male dogs, and the average age was 11.3 +/- 2.8 years. Prolapse of the anterior leaflet was present in 48.4% of dogs, prolapse of the the posterior leaflet in 7.1%, and bileaflet prolapse was present in 44.5%; this distribution is different than that typically…

Clindamycin-induced restless syndrome

Brief description: We presented a case of severe RLS associated with clindamycin (a lincosamide antibiotic frequently used in clinical practice) which was also a cause of a neurological emergency unit admission

INDIVIDUAL AND HEALTH-RELATED QUALITY OF LIFE ASSESSMENT IN AMYOTROPHIC LATERAL SCLEROSIS PATIENTS AND THEIR CAREGIVERS

Abstract: We performed a cross-sectional study aimed to address the quality of life (QoL) and putative associated variables in amyotrophic lateral sclerosis (ALS) patients and their respective caregivers, using both health-related (WHOQOL-BREF) and individual (SEIQoL-DW) QoL instruments. Further, we sought to investigate concordance within patient-caregiver pairs for ratings of respective QoL. Thirty-seven patient-caregiver pairs were included in the study. QoL was rated low by both patients and caregivers, and there was no significant difference between them on scores of overall QoL, even if caregivers showed higher scores on the physical and psychological WHOQOL-BREF domains compared to p…

NONINTENSIVE POSITIVE-PRESSURE VENTILATION IN ALS.

Development of chronic hypoventilation in amyotrophic lateral sclerosis patients

SummaryEarly prediction of respiratory muscle involvement and chronic hypoventilation (CH) in amyotrophic lateral sclerosis (ALS) patients can help to plan mechanical ventilatory aids and palliative care interventions well before respiratory failure occurs. To describe the natural history of the progressive pulmonary dysfunction leading to CH, and to identify potential parameters associated with its development in ALS, we prospectively followed 38 ALS patients up to 26 months, starting from their first presentation at our Clinic. At study entry, median FVC was 87% (interquartile range: 72–104%) and declined by 10% after 6 months (range: 2–49%), showing a very high inter-patient variability.…

Restless legs syndrome in a patient with multifocal motor neuropathy

Restless legs syndrome (RLS) has been frequently reported in association with peripheral neuropathy, and it is especially frequent in some forms of polyneuropathy with preferential involvement of small sensory fibers. Here, we describe a patient with multifocal motor neuropathy, who developed RLS during the course of the disease. Our findings support the notion that RLS may develop in the context of immune-mediated neuropathies and it should be specifically investigated even in those patients with preferentially or exclusive motor involvement.

Amyotrophic Lateral Sclerosis, a Multisystem Pathology: Insights into the Role of TNFα

Amyotrophic lateral sclerosis (ALS) is considered a multifactorial, multisystem disease in which inflammation and the immune system play important roles in development and progression. The pleiotropic cytokine TNFαis one of the major players governing the inflammation in the central nervous system and peripheral districts such as the neuromuscular and immune system. Changes in TNFαlevels are reported in blood, cerebrospinal fluid, and nerve tissues of ALS patients and animal models. However, whether they play a detrimental or protective role on the disease progression is still not clear. Our group and others have recently reported opposite involvements of TNFR1 and TNFR2 in motor neuron dea…

PREDICTORS OF SURVIVAL AFTER NON - INVASIVE POSITIVE - PRESSURE VENTILATION IN ALS PATIENTS

Stroke after tadalafil use

No abstract available

Outcome and attitudes toward home tracheostomy ventilation of consecutive patients: a 10-year experience.

Summary Objectives To describe survival, predictors of long-term outcome and attitudes in patients treated at home by tracheostomy-intermittent positive-pressure ventilation (TIPPV) for respiratory failure during a 10-year period (1995–2004). Methods Seventy-seven consecutive patients were treated by TIPPV at home. Patients were divided into three groups: neuromuscular, pulmonary, and non-pulmonary patients. Effects of TIPPV on survival, factors influencing outcome after TIPPV, and attitudes of patients and caregivers regarding mechanical ventilation were studied. Results Forty-one patients (53%) were neuromuscular, 19 (25%) were affected by pulmonary diseases, and 17 (22%) by non-pulmonary…

Guillain-Barré Syndrome

Background: Guillain–Barré syndrome is a rare disorder in which our body’s immune system attacks nerves determining weakness and tingling of extremities as first symptoms. It can also be associated to respiratory failure and require mechanical ventilation during hospitalization (up to 30% of patients). Nowadays patient’s hyper-reactive immune responses benefits from immunotherapies such as intravenous immunoglobulin (IVIg), therapeutic plasma exchange (TPE) and new biological drugs. Case Report: We report our experience with the case of a 64-year-old woman who presented a symmetric progressive flaccid paralysis after a week of mild cold symptoms. The respiratory and neurological symptoms wo…