Primary lateral sclerosis mimicking atypical parkinsonism.
Identifieur interne : 001D97 ( Ncbi/Checkpoint ); précédent : 001D96; suivant : 001D98Primary lateral sclerosis mimicking atypical parkinsonism.
Auteurs : Ibrahim M. Norlinah [Royaume-Uni] ; Kailash P. Bhatia ; Karen Ostergaard ; Robin Howard ; Gennarina Arabia ; Niall P. QuinnSource :
- Movement disorders : official journal of the Movement Disorder Society [ 0885-3185 ] ; 2007.
English descriptors
- KwdEn :
- Aged, Dopamine Plasma Membrane Transport Proteins (metabolism), Female, Humans, Magnetic Resonance Imaging (methods), Male, Middle Aged, Motor Neuron Disease (diagnosis), Motor Neuron Disease (radionuclide imaging), Parkinson Disease (physiopathology), Tomography, Emission-Computed, Single-Photon (methods).
- MESH :
- chemical , metabolism : Dopamine Plasma Membrane Transport Proteins.
- diagnosis : Motor Neuron Disease.
- methods : Magnetic Resonance Imaging, Tomography, Emission-Computed, Single-Photon.
- physiopathology : Parkinson Disease.
- radionuclide imaging : Motor Neuron Disease.
- Aged, Female, Humans, Male, Middle Aged.
Abstract
Primary lateral sclerosis (PLS), the upper motor neurone variant of motor neurone disease, is characterized by progressive spinal or bulbar spasticity with minimal motor weakness. Rarely, PLS may present with clinical features resembling parkinsonism resulting in occasional misdiagnosis as one of the atypical parkinsonian syndromes. Here we describe five patients initially referred with a diagnosis of levodopa-unresponsive atypical parkinsonism (n = 4) or primary progressive multiple sclerosis (n = 1), but subsequently found to have features consistent with PLS instead. Onset age varied from 49 to 67 years. Unilateral limb slowness or clumsiness was the initial complaint in four, and bulbar symptoms in one. Repeated finger/foot tapping was slow in all five, but without fatiguing or decrement. Spasticity with hyperreflexia, exaggerated jaw jerk and extensor plantar responses were eventually seen in all patients. Anterior horn cell involvement developed in three cases. Early gait disturbances resulting in falls were seen in all patients and none of them responded to dopaminergic medications. Two patients underwent dopamine transporter (DaT) SPECT scanning with normal results. Other features included emotional lability (n = 5) and cognitive impairment involving frontal subcortical systems (n = 1). In conclusion, these cases represent a subgroup of PLS patients in whom pyramidal slowness may be mistaken for akinesia, and spasticity misconstrued as rigidity, leading to an erroneous diagnosis of atypical parkinsonism. However, the absence of fatiguing and decrement on repeated finger/foot tapping should help to distinguish these patients from the true atypical parkinsonian syndromes.
DOI: 10.1002/mds.21645
PubMed: 17702034
Affiliations:
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<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en">Primary lateral sclerosis mimicking atypical parkinsonism.</title><author><name sortKey="Norlinah, Ibrahim M" sort="Norlinah, Ibrahim M" uniqKey="Norlinah I" first="Ibrahim M" last="Norlinah">Ibrahim M. Norlinah</name><affiliation wicri:level="3"><nlm:affiliation>Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, London, United Kingdom.</nlm:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, London</wicri:regionArea><placeName><settlement type="city">Londres</settlement><region type="country">Angleterre</region><region type="région" nuts="1">Grand Londres</region></placeName></affiliation></author><author><name sortKey="Bhatia, Kailash P" sort="Bhatia, Kailash P" uniqKey="Bhatia K" first="Kailash P" last="Bhatia">Kailash P. Bhatia</name></author><author><name sortKey="Ostergaard, Karen" sort="Ostergaard, Karen" uniqKey="Ostergaard K" first="Karen" last="Ostergaard">Karen Ostergaard</name></author><author><name sortKey="Howard, Robin" sort="Howard, Robin" uniqKey="Howard R" first="Robin" last="Howard">Robin Howard</name></author><author><name sortKey="Arabia, Gennarina" sort="Arabia, Gennarina" uniqKey="Arabia G" first="Gennarina" last="Arabia">Gennarina Arabia</name></author><author><name sortKey="Quinn, Niall P" sort="Quinn, Niall P" uniqKey="Quinn N" first="Niall P" last="Quinn">Niall P. Quinn</name></author></titleStmt><publicationStmt><idno type="wicri:source">PubMed</idno><date when="2007">2007</date><idno type="doi">10.1002/mds.21645</idno><idno type="RBID">pubmed:17702034</idno><idno type="pmid">17702034</idno><idno type="wicri:Area/PubMed/Corpus">002562</idno><idno type="wicri:Area/PubMed/Curation">002562</idno><idno type="wicri:Area/PubMed/Checkpoint">002623</idno><idno type="wicri:Area/Ncbi/Merge">001D97</idno><idno type="wicri:Area/Ncbi/Curation">001D97</idno><idno type="wicri:Area/Ncbi/Checkpoint">001D97</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en">Primary lateral sclerosis mimicking atypical parkinsonism.</title><author><name sortKey="Norlinah, Ibrahim M" sort="Norlinah, Ibrahim M" uniqKey="Norlinah I" first="Ibrahim M" last="Norlinah">Ibrahim M. Norlinah</name><affiliation wicri:level="3"><nlm:affiliation>Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, London, United Kingdom.</nlm:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, London</wicri:regionArea><placeName><settlement type="city">Londres</settlement><region type="country">Angleterre</region><region type="région" nuts="1">Grand Londres</region></placeName></affiliation></author><author><name sortKey="Bhatia, Kailash P" sort="Bhatia, Kailash P" uniqKey="Bhatia K" first="Kailash P" last="Bhatia">Kailash P. Bhatia</name></author><author><name sortKey="Ostergaard, Karen" sort="Ostergaard, Karen" uniqKey="Ostergaard K" first="Karen" last="Ostergaard">Karen Ostergaard</name></author><author><name sortKey="Howard, Robin" sort="Howard, Robin" uniqKey="Howard R" first="Robin" last="Howard">Robin Howard</name></author><author><name sortKey="Arabia, Gennarina" sort="Arabia, Gennarina" uniqKey="Arabia G" first="Gennarina" last="Arabia">Gennarina Arabia</name></author><author><name sortKey="Quinn, Niall P" sort="Quinn, Niall P" uniqKey="Quinn N" first="Niall P" last="Quinn">Niall P. Quinn</name></author></analytic><series><title level="j">Movement disorders : official journal of the Movement Disorder Society</title><idno type="ISSN">0885-3185</idno><imprint><date when="2007" type="published">2007</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Aged</term><term>Dopamine Plasma Membrane Transport Proteins (metabolism)</term><term>Female</term><term>Humans</term><term>Magnetic Resonance Imaging (methods)</term><term>Male</term><term>Middle Aged</term><term>Motor Neuron Disease (diagnosis)</term><term>Motor Neuron Disease (radionuclide imaging)</term><term>Parkinson Disease (physiopathology)</term><term>Tomography, Emission-Computed, Single-Photon (methods)</term></keywords><keywords scheme="MESH" type="chemical" qualifier="metabolism" xml:lang="en"><term>Dopamine Plasma Membrane Transport Proteins</term></keywords><keywords scheme="MESH" qualifier="diagnosis" xml:lang="en"><term>Motor Neuron Disease</term></keywords><keywords scheme="MESH" qualifier="methods" xml:lang="en"><term>Magnetic Resonance Imaging</term><term>Tomography, Emission-Computed, Single-Photon</term></keywords><keywords scheme="MESH" qualifier="physiopathology" xml:lang="en"><term>Parkinson Disease</term></keywords><keywords scheme="MESH" qualifier="radionuclide imaging" xml:lang="en"><term>Motor Neuron Disease</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Aged</term><term>Female</term><term>Humans</term><term>Male</term><term>Middle Aged</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Primary lateral sclerosis (PLS), the upper motor neurone variant of motor neurone disease, is characterized by progressive spinal or bulbar spasticity with minimal motor weakness. Rarely, PLS may present with clinical features resembling parkinsonism resulting in occasional misdiagnosis as one of the atypical parkinsonian syndromes. Here we describe five patients initially referred with a diagnosis of levodopa-unresponsive atypical parkinsonism (n = 4) or primary progressive multiple sclerosis (n = 1), but subsequently found to have features consistent with PLS instead. Onset age varied from 49 to 67 years. Unilateral limb slowness or clumsiness was the initial complaint in four, and bulbar symptoms in one. Repeated finger/foot tapping was slow in all five, but without fatiguing or decrement. Spasticity with hyperreflexia, exaggerated jaw jerk and extensor plantar responses were eventually seen in all patients. Anterior horn cell involvement developed in three cases. Early gait disturbances resulting in falls were seen in all patients and none of them responded to dopaminergic medications. Two patients underwent dopamine transporter (DaT) SPECT scanning with normal results. Other features included emotional lability (n = 5) and cognitive impairment involving frontal subcortical systems (n = 1). In conclusion, these cases represent a subgroup of PLS patients in whom pyramidal slowness may be mistaken for akinesia, and spasticity misconstrued as rigidity, leading to an erroneous diagnosis of atypical parkinsonism. However, the absence of fatiguing and decrement on repeated finger/foot tapping should help to distinguish these patients from the true atypical parkinsonian syndromes.</div></front></TEI><affiliations><list><country><li>Royaume-Uni</li></country><region><li>Angleterre</li><li>Grand Londres</li></region><settlement><li>Londres</li></settlement></list><tree><noCountry><name sortKey="Arabia, Gennarina" sort="Arabia, Gennarina" uniqKey="Arabia G" first="Gennarina" last="Arabia">Gennarina Arabia</name><name sortKey="Bhatia, Kailash P" sort="Bhatia, Kailash P" uniqKey="Bhatia K" first="Kailash P" last="Bhatia">Kailash P. Bhatia</name><name sortKey="Howard, Robin" sort="Howard, Robin" uniqKey="Howard R" first="Robin" last="Howard">Robin Howard</name><name sortKey="Ostergaard, Karen" sort="Ostergaard, Karen" uniqKey="Ostergaard K" first="Karen" last="Ostergaard">Karen Ostergaard</name><name sortKey="Quinn, Niall P" sort="Quinn, Niall P" uniqKey="Quinn N" first="Niall P" last="Quinn">Niall P. Quinn</name></noCountry><country name="Royaume-Uni"><region name="Angleterre"><name sortKey="Norlinah, Ibrahim M" sort="Norlinah, Ibrahim M" uniqKey="Norlinah I" first="Ibrahim M" last="Norlinah">Ibrahim M. Norlinah</name></region></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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