olly
Extremely helpful member
- Joined
- Jan 10, 2008
- Messages
- 2,743
- Reason
- PALS
- Diagnosis
- 11/2007
- Country
- uk
- State
- uk
- City
- uk
Primary lateral sclerosis: further clarification.Le Forestier N, Maisonobe T, Spelle L, Lesort A, Salachas F, Lacomblez L, Samson Y, Bouche P, Meininger V.
Fédération de Neurologie Mazarin Professeurs Delattre et Meininger, Hôpital de la Salpêtrière, 47 Boulevard de l'Hôpital, 75013, Paris, France.
Does primary lateral sclerosis exist? A study of 20 patients and a review of the literature.Le Forestier N, Maisonobe T, Piquard A, Rivaud S, Crevier-Buchman L, Salachas F, Pradat PF, Lacomblez L, Meininger V.
Fédération Mazarin de Neurologie Professeurs Delattre et Meininger, Hôpital de la Salpêtrière, Paris, France.
The question of whether primary lateral sclerosis (PLS) is a nosological entity distinct from amyotrophic lateral sclerosis (ALS) has been the subject of controversy since it was first described in the nineteenth century. PLS has been defined as a rare, non-hereditary disease characterized by progressive spinobulbar spasticity, related to the selective loss of precentral pyramidal neurones, with secondary pyramidal tract degeneration and preservation of anterior horn motor neurones. In the recent clinical literature, the frontier between ALS and neurodegenerative disease remains poorly defined. We studied 20 patients with a diagnosis of PLS. We carried out a variety of tests in order to determine the presence of a more diffuse neurodegenerative process. We also performed a longitudinal electrophysiological evaluation. Our clinical, electrophysiological and pathological investigations provide evidence that the disease has a heterogeneous clinical presentation and that degeneration is not restricted to the central motor system.
PMID: 11571217 [PubMed - indexed for MEDLINE]
Primary lateral sclerosis (PLS) has been defined as a rare. Non-hereditary disease characterized by progressive spinobulbar spasticity, related to the exclusive involvement of precentral pyramidal neurons, with secondary pyramidal tract degeneration and a preservation of anterior horn motor neurons, the latter allowing PLS to be distinguish from amyotrophic lateral sclerosis (ALS). However, a clear distinction between the two diseases remains a subject of debate. With this in mind, we assessed patients with meeting the previously published criteria for PLS in a prospective, longitudinal study.At regular intervals, we analyzed various clinical and electrophysiological parameters in nine patients with a diagnosis of PLS. We made a deltoid muscle biopsy and PET study.Our results provide evidence that degeneration in PLS is not restricted to the upper motor neurons but also affects the lower motor neurons. The distinction between ALS and PLS is related to the degree and stability of lower motor neuron involvement.In view of the similarities with ALS, we consider that PLS may represent a slowly progressive syndrome closely related to this disease.
PMID: 11311289 [PubMed - indexed for MEDLINE]
Fédération de Neurologie Mazarin Professeurs Delattre et Meininger, Hôpital de la Salpêtrière, 47 Boulevard de l'Hôpital, 75013, Paris, France.
Does primary lateral sclerosis exist? A study of 20 patients and a review of the literature.Le Forestier N, Maisonobe T, Piquard A, Rivaud S, Crevier-Buchman L, Salachas F, Pradat PF, Lacomblez L, Meininger V.
Fédération Mazarin de Neurologie Professeurs Delattre et Meininger, Hôpital de la Salpêtrière, Paris, France.
The question of whether primary lateral sclerosis (PLS) is a nosological entity distinct from amyotrophic lateral sclerosis (ALS) has been the subject of controversy since it was first described in the nineteenth century. PLS has been defined as a rare, non-hereditary disease characterized by progressive spinobulbar spasticity, related to the selective loss of precentral pyramidal neurones, with secondary pyramidal tract degeneration and preservation of anterior horn motor neurones. In the recent clinical literature, the frontier between ALS and neurodegenerative disease remains poorly defined. We studied 20 patients with a diagnosis of PLS. We carried out a variety of tests in order to determine the presence of a more diffuse neurodegenerative process. We also performed a longitudinal electrophysiological evaluation. Our clinical, electrophysiological and pathological investigations provide evidence that the disease has a heterogeneous clinical presentation and that degeneration is not restricted to the central motor system.
PMID: 11571217 [PubMed - indexed for MEDLINE]
Primary lateral sclerosis (PLS) has been defined as a rare. Non-hereditary disease characterized by progressive spinobulbar spasticity, related to the exclusive involvement of precentral pyramidal neurons, with secondary pyramidal tract degeneration and a preservation of anterior horn motor neurons, the latter allowing PLS to be distinguish from amyotrophic lateral sclerosis (ALS). However, a clear distinction between the two diseases remains a subject of debate. With this in mind, we assessed patients with meeting the previously published criteria for PLS in a prospective, longitudinal study.At regular intervals, we analyzed various clinical and electrophysiological parameters in nine patients with a diagnosis of PLS. We made a deltoid muscle biopsy and PET study.Our results provide evidence that degeneration in PLS is not restricted to the upper motor neurons but also affects the lower motor neurons. The distinction between ALS and PLS is related to the degree and stability of lower motor neuron involvement.In view of the similarities with ALS, we consider that PLS may represent a slowly progressive syndrome closely related to this disease.
PMID: 11311289 [PubMed - indexed for MEDLINE]
