103 Atypical Progressive Bulbar Palsy presenting with Dropped Head

Introduction: Typical amyotrophic lateral sclerosis (ALS) presents on neurological examination with specific signs of upper and lower motor neuron degeneration (Brooks et al, 1995), which can account for 85% of patients with ALS (Turner and Talbot, 2013). There are different types of clinical presen...

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Autores:
Carvajal-González, Alexander
Iglesias, Antonio H.
Tipo de recurso:
Article of journal
Fecha de publicación:
2020
Institución:
Universidad El Bosque
Repositorio:
Repositorio U. El Bosque
Idioma:
eng
OAI Identifier:
oai:repositorio.unbosque.edu.co:20.500.12495/3398
Acceso en línea:
http://hdl.handle.net/20.500.12495/3398
https://doi.org/10.1017/S1092852920000218
https://repositorio.unbosque.edu.co
Palabra clave:
Esclerosis amiotrófica lateral
Examen neurológico
parálisis bulbar progresiva
Rights
openAccess
License
Acceso abierto
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network_acronym_str UNBOSQUE2
network_name_str Repositorio U. El Bosque
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dc.title.spa.fl_str_mv 103 Atypical Progressive Bulbar Palsy presenting with Dropped Head
dc.title.translated.spa.fl_str_mv 103 Atypical Progressive Bulbar Palsy presenting with Dropped Head
title 103 Atypical Progressive Bulbar Palsy presenting with Dropped Head
spellingShingle 103 Atypical Progressive Bulbar Palsy presenting with Dropped Head
Esclerosis amiotrófica lateral
Examen neurológico
parálisis bulbar progresiva
title_short 103 Atypical Progressive Bulbar Palsy presenting with Dropped Head
title_full 103 Atypical Progressive Bulbar Palsy presenting with Dropped Head
title_fullStr 103 Atypical Progressive Bulbar Palsy presenting with Dropped Head
title_full_unstemmed 103 Atypical Progressive Bulbar Palsy presenting with Dropped Head
title_sort 103 Atypical Progressive Bulbar Palsy presenting with Dropped Head
dc.creator.fl_str_mv Carvajal-González, Alexander
Iglesias, Antonio H.
dc.contributor.author.none.fl_str_mv Carvajal-González, Alexander
Iglesias, Antonio H.
dc.subject.decs.spa.fl_str_mv Esclerosis amiotrófica lateral
Examen neurológico
parálisis bulbar progresiva
topic Esclerosis amiotrófica lateral
Examen neurológico
parálisis bulbar progresiva
description Introduction: Typical amyotrophic lateral sclerosis (ALS) presents on neurological examination with specific signs of upper and lower motor neuron degeneration (Brooks et al, 1995), which can account for 85% of patients with ALS (Turner and Talbot, 2013). There are different types of clinical presentations, including progressive bulbar palsy (PBP), Limb-onset ALS, progressive muscular atrophy (PMA) and upper motor neuron (UMN) predominant ALS. PBP has mainly brainstem signs. There are a few case reports of dropped head syndrome in ALS, mainly in patients with the limb involvement variant. Methods: Case report Results: A 56 year old right-handed male, presented to the clinic with four months of dysphagia to liquids and solids, neck pain and progressive neck weakness causing constant drop head. No dysarthria or other neurological symptoms, no dyspnea. Neurological examination: Cranial Nerve (CN) CN XII: Nasal voice, bilateral atrophy of the tongue with tremor and fasciculations. Motor: Diffuse atrophy and decreased tone of the sternocleidomastoid and trapezii bilaterally, strength: 2/5 in neck flexors and extensors. Sensory: Hypoesthesia of the tongue. The rest of his neurological examination was normal. Labs: Routine blood work, thyroid function tests, collagen vascular work-up, and protein electrophoresis were normal. Creatine Phosphokinase (CPK) and Acetylcholine Receptor Antibodies (AChR Ab) were negative. Brain and Spinal Cord MRI: Showed mild brainstem, cerebellar and cervical spinal atrophy. Conclusions: Patients with ALS initially present with symptoms localized to the limbs or bulbar muscles. A very small percentage 1-2% of ALS patients had neck muscle weakness with head drop (Jokelainen et al, 1977; Gourie-Devi et al, 2003). However, in all the previously reported cases, the patients had limb involvement at the time of presentation which was absent in this case, and the head drop occurred after the onset of symptoms (Lange et al, 1986; Katz et al; 1996). Dropped head syndrome can be seen in inflammatory myopathies, myasthenia gravis, facioscapulohumeral muscular dystrophy, spinal muscular atrophy, nemaline myopathy and carnitine deficiency (Umapathy et al, 2003) but ALS should also be considered in patients with atypical presentations.
publishDate 2020
dc.date.accessioned.none.fl_str_mv 2020-07-09T21:11:10Z
dc.date.available.none.fl_str_mv 2020-07-09T21:11:10Z
dc.date.issued.none.fl_str_mv 2020
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dc.type.local.none.fl_str_mv Artículo de revista
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dc.type.driver.none.fl_str_mv info:eu-repo/semantics/article
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dc.identifier.issn.none.fl_str_mv 1092-8529
dc.identifier.uri.none.fl_str_mv http://hdl.handle.net/20.500.12495/3398
dc.identifier.doi.none.fl_str_mv https://doi.org/10.1017/S1092852920000218
dc.identifier.instname.spa.fl_str_mv instname:Universidad El Bosque
dc.identifier.reponame.spa.fl_str_mv reponame:Repositorio Institucional Universidad El Bosque
dc.identifier.repourl.none.fl_str_mv https://repositorio.unbosque.edu.co
identifier_str_mv 1092-8529
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url http://hdl.handle.net/20.500.12495/3398
https://doi.org/10.1017/S1092852920000218
https://repositorio.unbosque.edu.co
dc.language.iso.none.fl_str_mv eng
language eng
dc.relation.ispartofseries.spa.fl_str_mv CNS spectrums, 1092-8529, Vol. 25, Nro. 2, 2020, p. 267
dc.relation.uri.none.fl_str_mv https://www.cambridge.org/core/journals/cns-spectrums/article/103-atypical-progressive-bulbar-palsy-presenting-with-dropped-head/C8BA05700BA481661691A76E2800C821
dc.rights.local.spa.fl_str_mv Acceso abierto
dc.rights.accessrights.none.fl_str_mv http://purl.org/coar/access_right/c_abf2
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dc.rights.creativecommons.none.fl_str_mv 2020-04-01
rights_invalid_str_mv Acceso abierto
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2020-04-01
eu_rights_str_mv openAccess
dc.format.mimetype.none.fl_str_mv application/pdf
dc.publisher.spa.fl_str_mv Cambridge University Press
dc.publisher.journal.spa.fl_str_mv CNS spectrums
institution Universidad El Bosque
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spelling Carvajal-González, AlexanderIglesias, Antonio H.2020-07-09T21:11:10Z2020-07-09T21:11:10Z20201092-8529http://hdl.handle.net/20.500.12495/3398https://doi.org/10.1017/S1092852920000218instname:Universidad El Bosquereponame:Repositorio Institucional Universidad El Bosquehttps://repositorio.unbosque.edu.coapplication/pdfengCambridge University PressCNS spectrumsCNS spectrums, 1092-8529, Vol. 25, Nro. 2, 2020, p. 267https://www.cambridge.org/core/journals/cns-spectrums/article/103-atypical-progressive-bulbar-palsy-presenting-with-dropped-head/C8BA05700BA481661691A76E2800C821103 Atypical Progressive Bulbar Palsy presenting with Dropped Head103 Atypical Progressive Bulbar Palsy presenting with Dropped HeadArtículo de revistahttp://purl.org/coar/resource_type/c_6501http://purl.org/coar/resource_type/c_2df8fbb1info:eu-repo/semantics/articlehttp://purl.org/coar/version/c_970fb48d4fbd8a85Esclerosis amiotrófica lateralExamen neurológicoparálisis bulbar progresivaIntroduction: Typical amyotrophic lateral sclerosis (ALS) presents on neurological examination with specific signs of upper and lower motor neuron degeneration (Brooks et al, 1995), which can account for 85% of patients with ALS (Turner and Talbot, 2013). There are different types of clinical presentations, including progressive bulbar palsy (PBP), Limb-onset ALS, progressive muscular atrophy (PMA) and upper motor neuron (UMN) predominant ALS. PBP has mainly brainstem signs. There are a few case reports of dropped head syndrome in ALS, mainly in patients with the limb involvement variant. Methods: Case report Results: A 56 year old right-handed male, presented to the clinic with four months of dysphagia to liquids and solids, neck pain and progressive neck weakness causing constant drop head. No dysarthria or other neurological symptoms, no dyspnea. Neurological examination: Cranial Nerve (CN) CN XII: Nasal voice, bilateral atrophy of the tongue with tremor and fasciculations. Motor: Diffuse atrophy and decreased tone of the sternocleidomastoid and trapezii bilaterally, strength: 2/5 in neck flexors and extensors. Sensory: Hypoesthesia of the tongue. The rest of his neurological examination was normal. Labs: Routine blood work, thyroid function tests, collagen vascular work-up, and protein electrophoresis were normal. Creatine Phosphokinase (CPK) and Acetylcholine Receptor Antibodies (AChR Ab) were negative. Brain and Spinal Cord MRI: Showed mild brainstem, cerebellar and cervical spinal atrophy. Conclusions: Patients with ALS initially present with symptoms localized to the limbs or bulbar muscles. A very small percentage 1-2% of ALS patients had neck muscle weakness with head drop (Jokelainen et al, 1977; Gourie-Devi et al, 2003). However, in all the previously reported cases, the patients had limb involvement at the time of presentation which was absent in this case, and the head drop occurred after the onset of symptoms (Lange et al, 1986; Katz et al; 1996). Dropped head syndrome can be seen in inflammatory myopathies, myasthenia gravis, facioscapulohumeral muscular dystrophy, spinal muscular atrophy, nemaline myopathy and carnitine deficiency (Umapathy et al, 2003) but ALS should also be considered in patients with atypical presentations.Acceso abiertohttp://purl.org/coar/access_right/c_abf2info:eu-repo/semantics/openAccessAcceso abierto2020-04-01ORIGINALAlexander Carvajal-González,Antonio H Iglesias_2020.pdfAlexander Carvajal-González,Antonio H Iglesias_2020.pdfapplication/pdf65883http://18.204.144.38/bitstreams/e84b83bf-964b-45b1-8b76-bb1ba9320d91/download1b6d0252973233f0b9c7e1d02ad81af2MD51LICENSElicense.txtlicense.txttext/plain; charset=utf-81748http://18.204.144.38/bitstreams/ce65af7f-9e7d-4fc2-91f9-9c6650c227b8/download8a4605be74aa9ea9d79846c1fba20a33MD52THUMBNAILAlexander Carvajal-González,Antonio H Iglesias_2020.pdf.jpgAlexander Carvajal-González,Antonio H Iglesias_2020.pdf.jpgimage/jpeg5775http://18.204.144.38/bitstreams/d95a3cc0-91a7-4965-bafa-1dc4c30b3d06/download7210a811635d1799e7c05fee5d259be7MD53TEXTAlexander Carvajal-González,Antonio H Iglesias_2020.pdf.txtAlexander Carvajal-González,Antonio H Iglesias_2020.pdf.txtExtracted texttext/plain5273http://18.204.144.38/bitstreams/168a9a3a-afd6-46d1-a604-76ffe41c3e1a/downloadd7ef371e5329313877562b31915070d3MD5420.500.12495/3398oai:18.204.144.38:20.500.12495/33982024-02-06 22:14:15.193restrictedhttp://18.204.144.38DSpace Pre-instalado Biteca S.A.Sbibliotecas@biteca.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