Autonomic nervous system and cardiac involvement in familial amyloidosis, Finnish type (FAF)
Familial amyloidosis, Finnish type (FAF), is a gelsolin-related inherited systemic amyloidosis. We report autonomic nervous system and cardiac findings in a study of 30 FAF patients (18 females, 12 males aged 27–74 years: mean 53.9 years). Cardiovascular reflex tests showed a significant decrease in...
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| Veröffentlicht in: | Journal of the neurological sciences 1994-10, Vol.126 (1), p.40-48 |
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| creator | Kiuru, Sari Matikainen, Esko Kupari, Markku Haltia, Matti Palo, Jorma |
| description | Familial amyloidosis, Finnish type (FAF), is a gelsolin-related inherited systemic amyloidosis. We report autonomic nervous system and cardiac findings in a study of 30 FAF patients (18 females, 12 males aged 27–74 years: mean 53.9 years). Cardiovascular reflex tests showed a significant decrease in heart rate variation in FAF patients compared with healthy controls. Orthostatic hypotension was found in 9 of 28 FAF patients, but only in 3 of 69 controls. Signs of amyloid cardiopathy were rare at clinical examination and in radio-, echocardio- and electrocardiographic examinations. Histological and immunohistochemical studies revealed amyloid deposition and immunoreactivity against the gelsolin-related FAF amyloid subunit in autonomic nervous system structures and in cardiac tissue in 3 autopsied FAF patients. The results show that minor autonomic nervous system dysfunction can be found in FAF, while clinically significant amyloid cardiopathy or autonomic neuropathy is not characteristic of this type of amyloidosis. |
| doi_str_mv | 10.1016/0022-510X(94)90092-2 |
| format | Article |
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We report autonomic nervous system and cardiac findings in a study of 30 FAF patients (18 females, 12 males aged 27–74 years: mean 53.9 years). Cardiovascular reflex tests showed a significant decrease in heart rate variation in FAF patients compared with healthy controls. Orthostatic hypotension was found in 9 of 28 FAF patients, but only in 3 of 69 controls. Signs of amyloid cardiopathy were rare at clinical examination and in radio-, echocardio- and electrocardiographic examinations. Histological and immunohistochemical studies revealed amyloid deposition and immunoreactivity against the gelsolin-related FAF amyloid subunit in autonomic nervous system structures and in cardiac tissue in 3 autopsied FAF patients. The results show that minor autonomic nervous system dysfunction can be found in FAF, while clinically significant amyloid cardiopathy or autonomic neuropathy is not characteristic of this type of amyloidosis.</description><identifier>ISSN: 0022-510X</identifier><identifier>EISSN: 1878-5883</identifier><identifier>DOI: 10.1016/0022-510X(94)90092-2</identifier><identifier>PMID: 7836945</identifier><identifier>CODEN: JNSCAG</identifier><language>eng</language><publisher>Shannon: Elsevier B.V</publisher><subject>Adult ; Aged ; Amyloid cardiopathy ; Amyloid polyneuropathy ; Amyloidosis ; Amyloidosis - complications ; Amyloidosis - genetics ; Amyloidosis - pathology ; Amyloidosis - physiopathology ; Autonomic Nervous System - pathology ; Autonomic Nervous System - physiopathology ; Autonomic neuropathy ; Biological and medical sciences ; Cardiovascular reflexes ; Echocardiography ; Familial amyloidosis, Finnish type ; Female ; Finland ; Gelsolin ; Gelsolin - genetics ; Heart - physiopathology ; Heart Rate ; Hereditary amyloidosis ; Humans ; Hypotension, Orthostatic - etiology ; Male ; Medical sciences ; Metabolic diseases ; Microscopy, Electron ; Middle Aged ; Myocardium - pathology ; Other metabolic disorders ; Point Mutation ; Reflex, Abnormal ; Valsalva Maneuver</subject><ispartof>Journal of the neurological sciences, 1994-10, Vol.126 (1), p.40-48</ispartof><rights>1994</rights><rights>1994 INIST-CNRS</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c481t-1e466599a16129aec877db98d51e867b8cc32f7f2bee0a900332fec21887c9ea3</citedby><cites>FETCH-LOGICAL-c481t-1e466599a16129aec877db98d51e867b8cc32f7f2bee0a900332fec21887c9ea3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://dx.doi.org/10.1016/0022-510X(94)90092-2$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>314,780,784,3550,27924,27925,45995</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=4249239$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/7836945$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Kiuru, Sari</creatorcontrib><creatorcontrib>Matikainen, Esko</creatorcontrib><creatorcontrib>Kupari, Markku</creatorcontrib><creatorcontrib>Haltia, Matti</creatorcontrib><creatorcontrib>Palo, Jorma</creatorcontrib><title>Autonomic nervous system and cardiac involvement in familial amyloidosis, Finnish type (FAF)</title><title>Journal of the neurological sciences</title><addtitle>J Neurol Sci</addtitle><description>Familial amyloidosis, Finnish type (FAF), is a gelsolin-related inherited systemic amyloidosis. We report autonomic nervous system and cardiac findings in a study of 30 FAF patients (18 females, 12 males aged 27–74 years: mean 53.9 years). Cardiovascular reflex tests showed a significant decrease in heart rate variation in FAF patients compared with healthy controls. Orthostatic hypotension was found in 9 of 28 FAF patients, but only in 3 of 69 controls. Signs of amyloid cardiopathy were rare at clinical examination and in radio-, echocardio- and electrocardiographic examinations. Histological and immunohistochemical studies revealed amyloid deposition and immunoreactivity against the gelsolin-related FAF amyloid subunit in autonomic nervous system structures and in cardiac tissue in 3 autopsied FAF patients. The results show that minor autonomic nervous system dysfunction can be found in FAF, while clinically significant amyloid cardiopathy or autonomic neuropathy is not characteristic of this type of amyloidosis.</description><subject>Adult</subject><subject>Aged</subject><subject>Amyloid cardiopathy</subject><subject>Amyloid polyneuropathy</subject><subject>Amyloidosis</subject><subject>Amyloidosis - complications</subject><subject>Amyloidosis - genetics</subject><subject>Amyloidosis - pathology</subject><subject>Amyloidosis - physiopathology</subject><subject>Autonomic Nervous System - pathology</subject><subject>Autonomic Nervous System - physiopathology</subject><subject>Autonomic neuropathy</subject><subject>Biological and medical sciences</subject><subject>Cardiovascular reflexes</subject><subject>Echocardiography</subject><subject>Familial amyloidosis, Finnish type</subject><subject>Female</subject><subject>Finland</subject><subject>Gelsolin</subject><subject>Gelsolin - genetics</subject><subject>Heart - physiopathology</subject><subject>Heart Rate</subject><subject>Hereditary amyloidosis</subject><subject>Humans</subject><subject>Hypotension, Orthostatic - etiology</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Metabolic diseases</subject><subject>Microscopy, Electron</subject><subject>Middle Aged</subject><subject>Myocardium - pathology</subject><subject>Other metabolic disorders</subject><subject>Point Mutation</subject><subject>Reflex, Abnormal</subject><subject>Valsalva Maneuver</subject><issn>0022-510X</issn><issn>1878-5883</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>1994</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp9kE1LAzEQQIMotVb_gUIOIi24mmQ_klyEUqwKBS8KHoSQZmcxspvUZLfQf-_Wlh49TYZ5M5N5CF1SckcJLe4JYSzJKfkYy2wiCZEsYUdoSAUXSS5EeoyGB-QUncX4TQgphJADNOAiLWSWD9HntGu984012EFY-y7iuIktNFi7EhsdSqsNtm7t6zU04Nr-jSvd2NrqGutmU3tb-mjjLZ5b52z8wu1mBXg8n84n5-ik0nWEi30coff549vsOVm8Pr3MpovEZIK2CYWsKHIpNS0okxqM4LxcSlHmFETBl8KYlFW8YksAovtL0z4Fw6gQ3EjQ6Qjd7Oaugv_pILaqsdFAXWsH_UWKc06EyGUPZjvQBB9jgEqtgm102ChK1Faq2hpTW2NKZupPqmJ929V-frdsoDw07S329et9XUej6ypoZ2w8YBnLJEu32x92GPQu1haCisaCM1DaAKZVpbf__-MXmGqTaQ</recordid><startdate>19941001</startdate><enddate>19941001</enddate><creator>Kiuru, Sari</creator><creator>Matikainen, Esko</creator><creator>Kupari, Markku</creator><creator>Haltia, Matti</creator><creator>Palo, Jorma</creator><general>Elsevier B.V</general><general>Elsevier Science</general><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope></search><sort><creationdate>19941001</creationdate><title>Autonomic nervous system and cardiac involvement in familial amyloidosis, Finnish type (FAF)</title><author>Kiuru, Sari ; Matikainen, Esko ; Kupari, Markku ; Haltia, Matti ; Palo, Jorma</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c481t-1e466599a16129aec877db98d51e867b8cc32f7f2bee0a900332fec21887c9ea3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>1994</creationdate><topic>Adult</topic><topic>Aged</topic><topic>Amyloid cardiopathy</topic><topic>Amyloid polyneuropathy</topic><topic>Amyloidosis</topic><topic>Amyloidosis - complications</topic><topic>Amyloidosis - genetics</topic><topic>Amyloidosis - pathology</topic><topic>Amyloidosis - physiopathology</topic><topic>Autonomic Nervous System - pathology</topic><topic>Autonomic Nervous System - physiopathology</topic><topic>Autonomic neuropathy</topic><topic>Biological and medical sciences</topic><topic>Cardiovascular reflexes</topic><topic>Echocardiography</topic><topic>Familial amyloidosis, Finnish type</topic><topic>Female</topic><topic>Finland</topic><topic>Gelsolin</topic><topic>Gelsolin - genetics</topic><topic>Heart - physiopathology</topic><topic>Heart Rate</topic><topic>Hereditary amyloidosis</topic><topic>Humans</topic><topic>Hypotension, Orthostatic - etiology</topic><topic>Male</topic><topic>Medical sciences</topic><topic>Metabolic diseases</topic><topic>Microscopy, Electron</topic><topic>Middle Aged</topic><topic>Myocardium - pathology</topic><topic>Other metabolic disorders</topic><topic>Point Mutation</topic><topic>Reflex, Abnormal</topic><topic>Valsalva Maneuver</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Kiuru, Sari</creatorcontrib><creatorcontrib>Matikainen, Esko</creatorcontrib><creatorcontrib>Kupari, Markku</creatorcontrib><creatorcontrib>Haltia, Matti</creatorcontrib><creatorcontrib>Palo, Jorma</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Journal of the neurological sciences</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Kiuru, Sari</au><au>Matikainen, Esko</au><au>Kupari, Markku</au><au>Haltia, Matti</au><au>Palo, Jorma</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Autonomic nervous system and cardiac involvement in familial amyloidosis, Finnish type (FAF)</atitle><jtitle>Journal of the neurological sciences</jtitle><addtitle>J Neurol Sci</addtitle><date>1994-10-01</date><risdate>1994</risdate><volume>126</volume><issue>1</issue><spage>40</spage><epage>48</epage><pages>40-48</pages><issn>0022-510X</issn><eissn>1878-5883</eissn><coden>JNSCAG</coden><abstract>Familial amyloidosis, Finnish type (FAF), is a gelsolin-related inherited systemic amyloidosis. We report autonomic nervous system and cardiac findings in a study of 30 FAF patients (18 females, 12 males aged 27–74 years: mean 53.9 years). Cardiovascular reflex tests showed a significant decrease in heart rate variation in FAF patients compared with healthy controls. Orthostatic hypotension was found in 9 of 28 FAF patients, but only in 3 of 69 controls. Signs of amyloid cardiopathy were rare at clinical examination and in radio-, echocardio- and electrocardiographic examinations. Histological and immunohistochemical studies revealed amyloid deposition and immunoreactivity against the gelsolin-related FAF amyloid subunit in autonomic nervous system structures and in cardiac tissue in 3 autopsied FAF patients. The results show that minor autonomic nervous system dysfunction can be found in FAF, while clinically significant amyloid cardiopathy or autonomic neuropathy is not characteristic of this type of amyloidosis.</abstract><cop>Shannon</cop><pub>Elsevier B.V</pub><pmid>7836945</pmid><doi>10.1016/0022-510X(94)90092-2</doi><tpages>9</tpages></addata></record> |
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| subjects | Adult Aged Amyloid cardiopathy Amyloid polyneuropathy Amyloidosis Amyloidosis - complications Amyloidosis - genetics Amyloidosis - pathology Amyloidosis - physiopathology Autonomic Nervous System - pathology Autonomic Nervous System - physiopathology Autonomic neuropathy Biological and medical sciences Cardiovascular reflexes Echocardiography Familial amyloidosis, Finnish type Female Finland Gelsolin Gelsolin - genetics Heart - physiopathology Heart Rate Hereditary amyloidosis Humans Hypotension, Orthostatic - etiology Male Medical sciences Metabolic diseases Microscopy, Electron Middle Aged Myocardium - pathology Other metabolic disorders Point Mutation Reflex, Abnormal Valsalva Maneuver |
| title | Autonomic nervous system and cardiac involvement in familial amyloidosis, Finnish type (FAF) |
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