Primary lateral sclerosis mimicking atypical parkinsonism
Identifieur interne : 001407 ( Istex/Corpus ); précédent : 001406; suivant : 001408Primary lateral sclerosis mimicking atypical parkinsonism
Auteurs : Ibrahim M. Norlinah ; Kailash P. Bhatia ; Karen Stergaard ; Robin Howard ; Gennarina Arabia ; Niall P. QuinnSource :
- Movement Disorders [ 0885-3185 ] ; 2007-10-31.
English descriptors
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. © 2007 Movement Disorder Society
Url:
DOI: 10.1002/mds.21645
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<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. © 2007 Movement Disorder Society</div>
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<keywordGroup xml:lang="en" type="author"><keyword xml:id="kwd1">primary lateral sclerosis</keyword>
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<supportingInformation><p> This article includes supplementary video clips, available online at <url href="http://www.interscience.wiley.com/jpages/0885-3185/suppmat"> http://www.interscience.wiley.com/jpages/0885‐3185/suppmat </url>
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<caption>Case 1There is slowness of finger taps but with no definite fatiguing or decrement. There is obvious wasting of the left first dorsal interosseus.Case 2Segment 1. Repeated finger taps are slow, but with no definite fatiguing or decrement.Segment 2. The patient walks with a narrow‐based, stiff gait. There is stooped posture with reduced arm swing bilaterally. There is postural instability on the pull test.</caption>
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<p>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. © 2007 Movement Disorder Society</p>
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<!--Version 0.6 générée le 4-12-2015--><mods version="3.6"><titleInfo lang="en"><title>Primary lateral sclerosis mimicking atypical parkinsonism</title>
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<titleInfo type="abbreviated" lang="en"><title>PLS Mimicking Atypical Parkinsonism</title>
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<titleInfo type="alternative" contentType="CDATA" lang="en"><title>Primary lateral sclerosis mimicking atypical parkinsonism</title>
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<name type="personal"><namePart type="given">Ibrahim M.</namePart>
<namePart type="family">Norlinah</namePart>
<namePart type="termsOfAddress">MB</namePart>
<affiliation>Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, London, United Kingdom</affiliation>
<affiliation>Neurology Unit, Department of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur, Malaysia</affiliation>
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<name type="personal"><namePart type="given">Kailash P.</namePart>
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<affiliation>Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, London, United Kingdom</affiliation>
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<name type="personal"><namePart type="given">Karen</namePart>
<namePart type="family">Østergaard</namePart>
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<affiliation>Department of Neurology, Aarhus University Hospital, Aarhus C, Denmark</affiliation>
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<name type="personal"><namePart type="given">Robin</namePart>
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<affiliation>Department of Molecular Neuroscience, Institute of Neurology, London, United Kingdom</affiliation>
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<name type="personal"><namePart type="given">Gennarina</namePart>
<namePart type="family">Arabia</namePart>
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<name type="personal"><namePart type="given">Niall P.</namePart>
<namePart type="family">Quinn</namePart>
<namePart type="termsOfAddress">MD</namePart>
<affiliation>Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, London, United Kingdom</affiliation>
<description>Correspondence: Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, Queen Square, London WC1N 3BG, United Kingdom</description>
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<abstract 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. © 2007 Movement Disorder Society</abstract>
<subject lang="en"><genre>Keywords</genre>
<topic>primary lateral sclerosis</topic>
<topic>atypical parkinsonism</topic>
<topic>akinesia</topic>
<topic>extrapyramidal</topic>
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<note type="content"> This article includes supplementary video clips, available online at http://www.interscience.wiley.com/jpages/0885‐3185/suppmat .Supporting Info Item: Case 1There is slowness of finger taps but with no definite fatiguing or decrement. There is obvious wasting of the left first dorsal interosseus.Case 2Segment 1. Repeated finger taps are slow, but with no definite fatiguing or decrement.Segment 2. The patient walks with a narrow‐based, stiff gait. There is stooped posture with reduced arm swing bilaterally. There is postural instability on the pull test. - </note>
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<identifier type="ISSN">0885-3185</identifier>
<identifier type="eISSN">1531-8257</identifier>
<identifier type="DOI">10.1002/(ISSN)1531-8257</identifier>
<identifier type="PublisherID">MDS</identifier>
<part><date>2007</date>
<detail type="volume"><caption>vol.</caption>
<number>22</number>
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<number>14</number>
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