Amyotrophic Lateral Sclerosis

Dose will be increased by 2mg/day increments every one week to reach a maximum dose of 8 mg/day. Treatment phase will be stable dose for 12 weeks then followed by washout period over 2 weeks.

Fycompa

Johnny S. Salameh

Safety and Tolerability of Perampanel in Amyotrophic Lateral Sclerosis Patients

Incidence and severity of drug-related adverse effects

Efficacy as measured by change in ALS Functional Rating Scale Revised -ALSFRS-R score ALSFRS-R score is of 12 items with total score of 48(each item score on a scale of 4); 0 reflects severe disability and 48 is the normal score.

Johnny Salameh

Associate Professor

American University of Beirut Medical Center

Patients with proven neuromuscular disorders known to be potentially associated with significant diaphragmatic weakness, e. g. ALS, myotonic dystrophy type 1, limb-girdle muscular dystrophy, Duchenne and Becker muscular dystrophy. Patients already receiving home ventilatory support will not be included in the study.

Patient with proven obstructive sleep apnea syndrome prior to CPAP initiation.

Patients with sleep disorders other than sleep-related breathing disorders, e. g. narcolepsy, hypersomnia, parasomnia or sleep-related movement disorders.

Neuromuscular Disorders

All patients answer selected questionnaires on sleep-related symptoms, sleep quality, daytime performance, and health-related quality of life.

patient-filled questionnaires

Measurement of forced vital capacity, maximum inspiratory pressure and maximum expiratory pressure

spiromanometry

University Hospital Münster, Department of Sleep Medicine and Neuromuscular Disorders

Bethanien Hospital

Screening Questionnaire for Respiratory Muscle Weakness and Sleep-disordered Breathing in Neuromuscular Disorders

Validation of a German Language Screening Questionnaire for Symptoms of Respiratory Muscle Weakness and Sleep-disordered Breathing in Patients With Neuromuscular Disorders

Matthias Boentert

MD, Senior Consultant in Neurology and Sleep Medicine

Westfälische Wilhelms-Universität Münster

spinal fluid and blood sample for identification of different factors

lumbar puncture for spinal fluid and blood sample

Aarhus University Hospital

sCD163 in ALS Patients

Inflammation in Amyotrophic Lateral Sclerosis - a Study of Soluble Cluster of Differentiation 163 in the Cerebrospinal Fluid

University of Aarhus

2 x 10mg capsules of melatonin, single dose. Blood sampling and physiological measures (blood pressure, ECG, oxygen saturation) every 30 mins for 6 hours.

3 x 10mg capsules of melatonin, single dose. Blood sampling and physiological measures (blood pressure, ECG, oxygen saturation) every 30 mins for 6 hours.

5 x 10mg capsules of melatonin, single dose. Blood sampling and physiological measures (blood pressure, ECG, oxygen saturation) every 30 mins for 6 hours.

10 x 10mg capsules of melatonin, single dose. Blood sampling and physiological measures (blood pressure, ECG, oxygen saturation) every 30 mins for 6 hours.

Sepsis

Melatonin

University of Aberdeen

Melatonin in Healthy Volunteers

A Dose Escalation Study of Melatonin in Healthy Volunteers as a Potential Treatment for Sepsis

Adverse events : nausea, vomiting, diarrhoea, itching, headache, drowsiness and any other symptoms for 6h immediately after dosing and again after 1 week. Sleeping patterns will also be assessed after 1 week.
Altered heart rate, oxygen saturation, ECG, blood pressure.
Changes in biochemistry (sodium , potassium, urea, AST, creatinine, glucose) or haematology (WBC and differential counts).
All events will be graded according to prospectively defined criteria.

At time of discharge from hospital, 40 ALS patients willbe enrolled in a telesurveillance program (TP) for the management of cought at home.
Two hours of an in-hospital educational training will be provided to patients and caregivers on the use of:
air stacking with Ambu balloon
manual manoeuvres and
in-Exoflator device indications and use

A telesurveillance program for cough assistance will be approched by the use of an in-exoflator device. Measurement of oxygen saturation (SpO2) will be performed by a Nonin 9500 oxymeter (Minneapolis Plymouth USA).

telesurveillance

association of amyotrophic lateral sclerosis patients

amyotrophic lateral sclerosis/cefalàlgia

Safety and Tolerability of Perampanel in Amyotrophic Lateral Sclerosis Patients

Screening Questionnaire for Respiratory Muscle Weakness and Sleep-disordered Breathing in Neuromuscular Disorders

sCD163 in ALS Patients

Melatonin in Healthy Volunteers

Feasibility of Telesurveillance and Home Cough Assistance for Amyotrophic Lateral Patients (ALS)

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