Elderly-Onset Multiple System Atrophy with Lewy Body Pathology: A Case Report
An 81-year-old woman presented with a 2-year history of progressive dysarthria and gait disturbance. Subsequently, she developed orthostatic hypotension, obstructive sleep apnea, right-sided resting tremor, and rigidity. Together with characteristic findings of imaging studies, she was diagnosed wit...
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| Veröffentlicht in: | Case Reports in Neurology 2021-09, Vol.13 (3), p.613-619 |
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| creator | Fukushima, Kazuhiro Nakamura, Akinori Takei, Yo-ichi Oguchi, Kenya Itagaki, Hiroko Ohara, Shinji Yamada, Mitsunori |
| description | An 81-year-old woman presented with a 2-year history of progressive dysarthria and gait disturbance. Subsequently, she developed orthostatic hypotension, obstructive sleep apnea, right-sided resting tremor, and rigidity. Together with characteristic findings of imaging studies, she was diagnosed with multiple system atrophy (MSA). Despite progressive dysphagia and repeated choking episodes, the patient elected not to use artificial feeding or tracheostomy. She died suddenly at age 91 after 12 years of illness. The autopsy revealed neuropathological features of both MSA and of Parkinson’s disease. The peripheral autonomic ganglia revealed both pre- and postganglionic involvement by synucleinopathy, which may have underscored the sudden death of the patient. The patient survived 10 years after onset, despite the presence of multiple poor prognostic factors in MSA including the onset of old age and early appearance of orthostatic hypotension and falls, in addition to the complication of PD pathology found by autopsy. Multidisciplinary team approach and her preserved cognitive function may have been contributory to the long-term survival. |
| doi_str_mv | 10.1159/000515973 |
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Subsequently, she developed orthostatic hypotension, obstructive sleep apnea, right-sided resting tremor, and rigidity. Together with characteristic findings of imaging studies, she was diagnosed with multiple system atrophy (MSA). Despite progressive dysphagia and repeated choking episodes, the patient elected not to use artificial feeding or tracheostomy. She died suddenly at age 91 after 12 years of illness. The autopsy revealed neuropathological features of both MSA and of Parkinson’s disease. The peripheral autonomic ganglia revealed both pre- and postganglionic involvement by synucleinopathy, which may have underscored the sudden death of the patient. The patient survived 10 years after onset, despite the presence of multiple poor prognostic factors in MSA including the onset of old age and early appearance of orthostatic hypotension and falls, in addition to the complication of PD pathology found by autopsy. Multidisciplinary team approach and her preserved cognitive function may have been contributory to the long-term survival.</description><identifier>ISSN: 1662-680X</identifier><identifier>EISSN: 1662-680X</identifier><identifier>DOI: 10.1159/000515973</identifier><identifier>PMID: 34703451</identifier><language>eng</language><publisher>Basel, Switzerland: S. Karger AG</publisher><subject>Age ; Airway management ; Atrophy ; Autopsies ; Blood pressure ; Cardiovascular disease ; Care and treatment ; Case reports ; Complications and side effects ; Degeneration (Pathology) ; Diagnosis ; Dopamine ; Dosage and administration ; Gait ; Health aspects ; Hypertension ; Lewy body disease ; Magnetic resonance imaging ; multiple system atrophy ; neurodegeneration ; Neurons ; Neuropathology ; Orthostatic hypotension ; Ostomy ; Parkinson's disease ; Pathology ; Patient outcomes ; Physiological aspects ; Pramipexole ; Risk factors ; Single Case – General Neurology ; Single Case − General Neurology ; Sleep apnea ; Sleep apnea syndromes ; Tracheotomy ; α-synucleinopathy</subject><ispartof>Case Reports in Neurology, 2021-09, Vol.13 (3), p.613-619</ispartof><rights>2021 The Author(s). Published by S. Karger AG, Basel</rights><rights>COPYRIGHT 2021 S. Karger AG</rights><rights>2021 The Author(s). Published by S. Karger AG, Basel . This work is licensed under the Creative Commons Attribution – Non-Commercial License http://creativecommons.org/licenses/by-nc/3.0/ (the “License”). Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.</rights><rights>Copyright © 2021 by S. Karger AG, Basel 2021</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><orcidid>0000-0002-8381-4274</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC8460921/pdf/$$EPDF$$P50$$Gpubmedcentral$$Hfree_for_read</linktopdf><linktohtml>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC8460921/$$EHTML$$P50$$Gpubmedcentral$$Hfree_for_read</linktohtml><link.rule.ids>230,314,725,778,782,862,883,2098,27618,27907,27908,53774,53776</link.rule.ids></links><search><creatorcontrib>Fukushima, Kazuhiro</creatorcontrib><creatorcontrib>Nakamura, Akinori</creatorcontrib><creatorcontrib>Takei, Yo-ichi</creatorcontrib><creatorcontrib>Oguchi, Kenya</creatorcontrib><creatorcontrib>Itagaki, Hiroko</creatorcontrib><creatorcontrib>Ohara, Shinji</creatorcontrib><creatorcontrib>Yamada, Mitsunori</creatorcontrib><title>Elderly-Onset Multiple System Atrophy with Lewy Body Pathology: A Case Report</title><title>Case Reports in Neurology</title><addtitle>Case Rep Neurol</addtitle><description>An 81-year-old woman presented with a 2-year history of progressive dysarthria and gait disturbance. Subsequently, she developed orthostatic hypotension, obstructive sleep apnea, right-sided resting tremor, and rigidity. Together with characteristic findings of imaging studies, she was diagnosed with multiple system atrophy (MSA). Despite progressive dysphagia and repeated choking episodes, the patient elected not to use artificial feeding or tracheostomy. She died suddenly at age 91 after 12 years of illness. The autopsy revealed neuropathological features of both MSA and of Parkinson’s disease. The peripheral autonomic ganglia revealed both pre- and postganglionic involvement by synucleinopathy, which may have underscored the sudden death of the patient. The patient survived 10 years after onset, despite the presence of multiple poor prognostic factors in MSA including the onset of old age and early appearance of orthostatic hypotension and falls, in addition to the complication of PD pathology found by autopsy. Multidisciplinary team approach and her preserved cognitive function may have been contributory to the long-term survival.</description><subject>Age</subject><subject>Airway management</subject><subject>Atrophy</subject><subject>Autopsies</subject><subject>Blood pressure</subject><subject>Cardiovascular disease</subject><subject>Care and treatment</subject><subject>Case reports</subject><subject>Complications and side effects</subject><subject>Degeneration (Pathology)</subject><subject>Diagnosis</subject><subject>Dopamine</subject><subject>Dosage and administration</subject><subject>Gait</subject><subject>Health aspects</subject><subject>Hypertension</subject><subject>Lewy body disease</subject><subject>Magnetic resonance imaging</subject><subject>multiple system atrophy</subject><subject>neurodegeneration</subject><subject>Neurons</subject><subject>Neuropathology</subject><subject>Orthostatic hypotension</subject><subject>Ostomy</subject><subject>Parkinson's disease</subject><subject>Pathology</subject><subject>Patient outcomes</subject><subject>Physiological aspects</subject><subject>Pramipexole</subject><subject>Risk factors</subject><subject>Single Case – General Neurology</subject><subject>Single Case − General Neurology</subject><subject>Sleep apnea</subject><subject>Sleep apnea syndromes</subject><subject>Tracheotomy</subject><subject>α-synucleinopathy</subject><issn>1662-680X</issn><issn>1662-680X</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2021</creationdate><recordtype>article</recordtype><sourceid>M--</sourceid><sourceid>ABUWG</sourceid><sourceid>AFKRA</sourceid><sourceid>BENPR</sourceid><sourceid>CCPQU</sourceid><sourceid>DOA</sourceid><recordid>eNptkUtv1DAQgCNERUvhwJ2DpZ44pPj94IC0rFqotG1RAYmbNUmcbJZsnDpeqvz7GlKtWqnyYayZbz7NaLLsHcGnhAjzEWMsUlTsRXZEpKS51Pj3y0f_w-z1OG4wlkZI_io7ZFxhxgU5yi7PusqFbsqv-9FFdLnrYjt0Dv2Yxui2aBGDH9YTumvjGq3c3YS--GpC3yGufeeb6RNaoCWMDt24wYf4JjuooRvd24d4nP06P_u5_Javrr9eLBervOTaxLwsFKV1DUxSkIKbSjsMShKQDoQ0WheMltgUAoTWTAkwmBmsnZa1EhI0O84uZm_lYWOH0G4hTNZDa_8nfGgshNiWnbNUYIKpJrwihmOliqLSJTCOOaHSSEiuz7Nr2BVbV5WujwG6J9Knlb5d28b_tZpLbChJgpMHQfC3OzdGu_G70Kf9LZUkrSOY4Yk6nakG0lRtX_skK9Or3LYtfe_qNuUXUmulFMUmNXyYG8rgxzG4ej8Swfbf2e3-7Il9P7N_IDQu7Ml9-eTZ8vLmaibsUNXsHmKlsE0</recordid><startdate>20210914</startdate><enddate>20210914</enddate><creator>Fukushima, Kazuhiro</creator><creator>Nakamura, Akinori</creator><creator>Takei, Yo-ichi</creator><creator>Oguchi, Kenya</creator><creator>Itagaki, Hiroko</creator><creator>Ohara, Shinji</creator><creator>Yamada, Mitsunori</creator><general>S. 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Subsequently, she developed orthostatic hypotension, obstructive sleep apnea, right-sided resting tremor, and rigidity. Together with characteristic findings of imaging studies, she was diagnosed with multiple system atrophy (MSA). Despite progressive dysphagia and repeated choking episodes, the patient elected not to use artificial feeding or tracheostomy. She died suddenly at age 91 after 12 years of illness. The autopsy revealed neuropathological features of both MSA and of Parkinson’s disease. The peripheral autonomic ganglia revealed both pre- and postganglionic involvement by synucleinopathy, which may have underscored the sudden death of the patient. The patient survived 10 years after onset, despite the presence of multiple poor prognostic factors in MSA including the onset of old age and early appearance of orthostatic hypotension and falls, in addition to the complication of PD pathology found by autopsy. 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| subjects | Age Airway management Atrophy Autopsies Blood pressure Cardiovascular disease Care and treatment Case reports Complications and side effects Degeneration (Pathology) Diagnosis Dopamine Dosage and administration Gait Health aspects Hypertension Lewy body disease Magnetic resonance imaging multiple system atrophy neurodegeneration Neurons Neuropathology Orthostatic hypotension Ostomy Parkinson's disease Pathology Patient outcomes Physiological aspects Pramipexole Risk factors Single Case – General Neurology Single Case − General Neurology Sleep apnea Sleep apnea syndromes Tracheotomy α-synucleinopathy |
| title | Elderly-Onset Multiple System Atrophy with Lewy Body Pathology: A Case Report |
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