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Home > Journals > European Journal of Physical and Rehabilitation Medicine > Past Issues > Europa Medicophysica 2007 December;43(4) > Europa Medicophysica 2007 December;43(4):505-9



A Journal on Physical Medicine and Rehabilitation after Pathological Events

Official Journal of the Italian Society of Physical and Rehabilitation Medicine (SIMFER), European Society of Physical and Rehabilitation Medicine (ESPRM), European Union of Medical Specialists - Physical and Rehabilitation Medicine Section (UEMS-PRM), Mediterranean Forum of Physical and Rehabilitation Medicine (MFPRM), Hellenic Society of Physical and Rehabilitation Medicine (EEFIAP)
In association with International Society of Physical and Rehabilitation Medicine (ISPRM)
Indexed/Abstracted in: CINAHL, Current Contents/Clinical Medicine, EMBASE, PubMed/MEDLINE, Science Citation Index Expanded (SciSearch), Scopus
Impact Factor 2,063

Frequency: Bi-Monthly

ISSN 1973-9087

Online ISSN 1973-9095


Europa Medicophysica 2007 December;43(4):505-9


Do bulbar-onset amyotrophic lateral sclerosis patients have an earlier respiratory involvement than spinal-onset amyotrophic lateral sclerosis patients?

Pinto S. 1,2, Pinto A. 1,2, De Carvalho M. 1,3

1 Neuromuscular Unit Institute of Molecular Medicine, Faculty of Medicine University of Lisbon, Lisbon, Portugal
2 Department of Rehabilitation Hospital de Santa Maria, Lisbon, Portugal
3 Neuromuscular Unit Department of Neurology Hospital de Santa Maria, Lisbon, Portugal

Aim. Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease involving limbs and bulbar muscles. The major cause of death in these patients is respiratory insufficiency. However, bulbar-onset ALS patients have a shorter survival than those with spinal-onset. It is not established if this difference is related to an earlier respiratory dysfunction in bulbar-onset patients. The aim of the present study was to determine if bulbar-onset patients have an earlier respiratory impairment as compared with the spinal-onset ones.
Methods. From the population of ALS patients followed in our centre, we studied 82 patients (45 spinal-onset and 37 bulbar-onset, group 1 and 2 respectively), all evaluated between 6-18 months after disease onset. We investigated forced vital capacity, maximal inspiratory (PI max) and expiratory (PE max) pressures, mouth occlusion pressure at 100 ms after inspiration, arterial blood gases, percutaneous nocturnal oxymetry, phrenic nerve conduction, needle electromyography (EMG) of the right diaphragm and right internal intercostals muscles and functional ALS scale (ALS-FRS).
Results. No statistical difference was found for gender and disease duration between both groups, but bulbar-onset patients were older. PI max and PE max were statistically lower (P<0.001 and P<0.006, respectively) in group 2. However, no correlation was found between bulbar ALS-FRS subscore and PI max or PE max.
Conclusion. Our results support that PI max and PE max are sensitive measurements of respiratory dysfunction in bulbar patients. Probably, this finding cannot be solely explained by the oro-facial weakness of bulbar-patients. We speculate that respiratory accessory muscles weakness can contribute for this observation.

language: English


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