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Progressive extrapyramidal muscular rigidity

MedGen UID:
867222
•Concept ID:
C4021580
•
Finding
Synonym: Progressive extrapyramidal rigidity
 
HPO: HP:0007158

Definition

A progressive degree of muscular rigidity (continuous contraction of muscles with constant resistance to passive movement). [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVProgressive extrapyramidal muscular rigidity

Recent clinical studies

Etiology

Alpha-lipoic acid supplementation corrects pathological alterations in cellular models of pantothenate kinase-associated neurodegeneration with residual PANK2 expression levels.
Talaverón-Rey M, Álvarez-Córdoba M, Villalón-García I, Povea-Cabello S, Suárez-Rivero JM, Gómez-Fernández D, Romero-González A, Suárez-Carrillo A, Munuera-Cabeza M, Cilleros-Holgado P, Reche-López D, Piñero-Pérez R, Sánchez-Alcázar JA
Orphanet J Rare Dis 2023 Apr 12;18(1):80. doi: 10.1186/s13023-023-02687-5. PMID: 37046296Free PMC Article
Extrapyramidal signs in the primary progressive aphasias.
Kremen SA, Mendez MF, Tsai PH, Teng E
Am J Alzheimers Dis Other Demen 2011 Feb;26(1):72-7. doi: 10.1177/1533317510391239. PMID: 21282281Free PMC Article
Do extrapyramidal features in Alzheimer patients treated with acetylcholinesterase inhibitors predict disease progression?
Brodaty H, Sachdev P, Berman K, Gibson L, Kemp NM, Cullen B, Burns A
Aging Ment Health 2007 Jul;11(4):451-6. doi: 10.1080/13607860601086439. PMID: 17612809
Review of 23 patients affected by the stiff man syndrome: clinical subdivision into stiff trunk (man) syndrome, stiff limb syndrome, and progressive encephalomyelitis with rigidity.
Barker RA, Revesz T, Thom M, Marsden CD, Brown P
J Neurol Neurosurg Psychiatry 1998 Nov;65(5):633-40. doi: 10.1136/jnnp.65.5.633. PMID: 9810930Free PMC Article
Cumulative risks of developing extrapyramidal signs, psychosis, or myoclonus in the course of Alzheimer's disease.
Chen JY, Stern Y, Sano M, Mayeux R
Arch Neurol 1991 Nov;48(11):1141-3. doi: 10.1001/archneur.1991.00530230049020. PMID: 1953398

Diagnosis

Parkinsonism may aggravate dysphagia in myotonic dystrophy type 1: two case reports.
Stano S, Barp A, Bacchin R, Zuccarino R
Acta Myol 2023;42(1):31-34. Epub 2023 Mar 31 doi: 10.36185/2532-1900-247. PMID: 37091528Free PMC Article
Pattern of extrapyramidal signs in Alzheimer's disease.
Tosto G, Monsell SE, Hawes SE, Mayeux R
J Neurol 2015 Nov;262(11):2548-56. Epub 2015 Sep 4 doi: 10.1007/s00415-015-7886-1. PMID: 26338814Free PMC Article
Progressive supra-nuclear palsy: frequency of cardinal extrapyramidal features at first presentation.
Pradhan S, Tandon R
Postgrad Med J 2015 May;91(1075):274-7. Epub 2015 May 11 doi: 10.1136/postgradmedj-2014-132696. PMID: 25964368
Review of 23 patients affected by the stiff man syndrome: clinical subdivision into stiff trunk (man) syndrome, stiff limb syndrome, and progressive encephalomyelitis with rigidity.
Barker RA, Revesz T, Thom M, Marsden CD, Brown P
J Neurol Neurosurg Psychiatry 1998 Nov;65(5):633-40. doi: 10.1136/jnnp.65.5.633. PMID: 9810930Free PMC Article
The neurologic syndrome of severe Alzheimer's disease. Relationship to functional decline.
Franssen EH, Kluger A, Torossian CL, Reisberg B
Arch Neurol 1993 Oct;50(10):1029-39. doi: 10.1001/archneur.1993.00540100024010. PMID: 8215960

Therapy

A Concise Review of Common Plant-derived Compounds as a Potential Therapy for Alzheimer's Disease and Parkinson's Disease: Insight into Structure-Activity-Relationship.
Nishal S, Phaugat P, Bazaad J, Dhaka R, Khatkar S, Khatkar A, Khayatkashani M, Alizadeh P, Haghighi SM, Mehri M, Kashani HRK
CNS Neurol Disord Drug Targets 2023;22(7):1057-1069. doi: 10.2174/1871527321666220614110616. PMID: 35702799
New insight into the therapeutic role of the serotonergic system in Parkinson's disease.
Ohno Y, Shimizu S, Tokudome K, Kunisawa N, Sasa M
Prog Neurobiol 2015 Nov;134:104-21. Epub 2015 Oct 9 doi: 10.1016/j.pneurobio.2015.09.005. PMID: 26455457
Progressive supra-nuclear palsy: frequency of cardinal extrapyramidal features at first presentation.
Pradhan S, Tandon R
Postgrad Med J 2015 May;91(1075):274-7. Epub 2015 May 11 doi: 10.1136/postgradmedj-2014-132696. PMID: 25964368
Neuroleptic-induced catatonia in two hospitalized patients.
Gugger JJ, Saad M, Smith C
J Pharm Pract 2012 Apr;25(2):250-4. Epub 2011 Nov 2 doi: 10.1177/0897190011420159. PMID: 22048928
Do extrapyramidal features in Alzheimer patients treated with acetylcholinesterase inhibitors predict disease progression?
Brodaty H, Sachdev P, Berman K, Gibson L, Kemp NM, Cullen B, Burns A
Aging Ment Health 2007 Jul;11(4):451-6. doi: 10.1080/13607860601086439. PMID: 17612809

Prognosis

Segmental progression of cardinal motor symptoms in Parkinson's disease: a pilot study suggesting a practical approach to rate disease course in the early stages.
Picillo M, Amboni M, Erro R, Vitale C, Longo K, Pellecchia MT, Cozzolino A, Moccia M, Allocca R, Barone P
Parkinsonism Relat Disord 2013 Dec;19(12):1143-8. Epub 2013 Sep 2 doi: 10.1016/j.parkreldis.2013.08.015. PMID: 24041938
Do extrapyramidal features in Alzheimer patients treated with acetylcholinesterase inhibitors predict disease progression?
Brodaty H, Sachdev P, Berman K, Gibson L, Kemp NM, Cullen B, Burns A
Aging Ment Health 2007 Jul;11(4):451-6. doi: 10.1080/13607860601086439. PMID: 17612809
Neonatal rigid-akinetic syndrome and dentato-olivary dysplasia.
Raspall M, Ortega-Aznar A, del Toro M, Roig M, Macaya A
Pediatr Neurol 2006 Feb;34(2):132-4. doi: 10.1016/j.pediatrneurol.2005.08.003. PMID: 16458826
Review of 23 patients affected by the stiff man syndrome: clinical subdivision into stiff trunk (man) syndrome, stiff limb syndrome, and progressive encephalomyelitis with rigidity.
Barker RA, Revesz T, Thom M, Marsden CD, Brown P
J Neurol Neurosurg Psychiatry 1998 Nov;65(5):633-40. doi: 10.1136/jnnp.65.5.633. PMID: 9810930Free PMC Article
Cumulative risks of developing extrapyramidal signs, psychosis, or myoclonus in the course of Alzheimer's disease.
Chen JY, Stern Y, Sano M, Mayeux R
Arch Neurol 1991 Nov;48(11):1141-3. doi: 10.1001/archneur.1991.00530230049020. PMID: 1953398

Clinical prediction guides

New insight into the therapeutic role of the serotonergic system in Parkinson's disease.
Ohno Y, Shimizu S, Tokudome K, Kunisawa N, Sasa M
Prog Neurobiol 2015 Nov;134:104-21. Epub 2015 Oct 9 doi: 10.1016/j.pneurobio.2015.09.005. PMID: 26455457
Pattern of extrapyramidal signs in Alzheimer's disease.
Tosto G, Monsell SE, Hawes SE, Mayeux R
J Neurol 2015 Nov;262(11):2548-56. Epub 2015 Sep 4 doi: 10.1007/s00415-015-7886-1. PMID: 26338814Free PMC Article
Progressive supra-nuclear palsy: frequency of cardinal extrapyramidal features at first presentation.
Pradhan S, Tandon R
Postgrad Med J 2015 May;91(1075):274-7. Epub 2015 May 11 doi: 10.1136/postgradmedj-2014-132696. PMID: 25964368
The neurologic syndrome of severe Alzheimer's disease. Relationship to functional decline.
Franssen EH, Kluger A, Torossian CL, Reisberg B
Arch Neurol 1993 Oct;50(10):1029-39. doi: 10.1001/archneur.1993.00540100024010. PMID: 8215960
Cumulative risks of developing extrapyramidal signs, psychosis, or myoclonus in the course of Alzheimer's disease.
Chen JY, Stern Y, Sano M, Mayeux R
Arch Neurol 1991 Nov;48(11):1141-3. doi: 10.1001/archneur.1991.00530230049020. PMID: 1953398

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