U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Abnormal corticospinal tract morphology

MedGen UID:
893081
Concept ID:
C4025704
Anatomical Abnormality
Synonyms: Abnormality of the corticospinal tract; Involvement of the corticospinal pathways; Morphological abnormality of the corticospinal tract
 
HPO: HP:0002492

Definition

Abnormality of the corticospinal tract, which is the chief element of the pyramidal system (the principle motor tract) and is the only direct connection between the cerebrum and the spinal cord. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAbnormal corticospinal tract morphology

Conditions with this feature

Infantile-onset ascending hereditary spastic paralysis
MedGen UID:
419413
Concept ID:
C2931441
Disease or Syndrome
ALS2-related disorder involves retrograde degeneration of the upper motor neurons of the pyramidal tracts and comprises a clinical continuum of the following three phenotypes: Infantile ascending hereditary spastic paraplegia (IAHSP), characterized by onset of spasticity with increased reflexes and sustained clonus of the lower limbs within the first two years of life, progressive weakness and spasticity of the upper limbs by age seven to eight years, and wheelchair dependence in the second decade with progression toward severe spastic tetraparesis and a pseudobulbar syndrome caused by progressive cranial nerve involvement. Juvenile primary lateral sclerosis (JPLS), characterized by upper motor neuron findings of pseudobulbar palsy and spastic quadriplegia without dementia or cerebellar, extrapyramidal, or sensory signs. Juvenile amyotrophic lateral sclerosis (JALS or ALS2), characterized by onset between ages three and 20 years. All affected individuals show a spastic pseudobulbar syndrome (spasticity of speech and swallowing) together with spastic paraplegia. Some individuals are bedridden by age 12 to 50 years.

Professional guidelines

PubMed

Lee AY, Choi BY, Kim SH, Chang CH, Jung YJ, Jang SH
Int J Neurosci 2016;126(5):429-35. Epub 2015 Jul 28 doi: 10.3109/00207454.2015.1026966. PMID: 26000805
Bede P, Bokde AL, Byrne S, Elamin M, McLaughlin RL, Kenna K, Fagan AJ, Pender N, Bradley DG, Hardiman O
Neurology 2013 Jul 23;81(4):361-9. Epub 2013 Jun 14 doi: 10.1212/WNL.0b013e31829c5eee. PMID: 23771489Free PMC Article
Filippi M, Agosta F, Abrahams S, Fazekas F, Grosskreutz J, Kalra S, Kassubek J, Silani V, Turner MR, Masdeu JC; European Federation of Neurological Societies
Eur J Neurol 2010 Apr;17(4):526-e20. Epub 2010 Feb 2 doi: 10.1111/j.1468-1331.2010.02951.x. PMID: 20136647Free PMC Article

Recent clinical studies

Etiology

Foesleitner O, Nenning KH, Traub-Weidinger T, Feucht M, Bonelli S, Czech T, Dorfer C, Prayer D, Kasprian G
AJNR Am J Neuroradiol 2018 Aug;39(8):1530-1535. Epub 2018 Jun 28 doi: 10.3174/ajnr.A5715. PMID: 29954815Free PMC Article
Lo Giudice T, Lombardi F, Santorelli FM, Kawarai T, Orlacchio A
Exp Neurol 2014 Nov;261:518-39. Epub 2014 Jun 20 doi: 10.1016/j.expneurol.2014.06.011. PMID: 24954637
Fink JK
Acta Neuropathol 2013 Sep;126(3):307-28. Epub 2013 Jul 30 doi: 10.1007/s00401-013-1115-8. PMID: 23897027Free PMC Article
Martin JJ
Handb Clin Neurol 2012;103:475-91. doi: 10.1016/B978-0-444-51892-7.00030-9. PMID: 21827908
Fink JK
Neurol Clin 2002 Aug;20(3):711-26. doi: 10.1016/s0733-8619(02)00007-5. PMID: 12432827

Diagnosis

Pasquini J, Firbank MJ, Ceravolo R, Silani V, Pavese N
Mov Disord 2022 Oct;37(10):1963-1984. Epub 2022 Aug 29 doi: 10.1002/mds.29195. PMID: 36036378Free PMC Article
Kunam VK, Velayudhan V, Chaudhry ZA, Bobinski M, Smoker WRK, Reede DL
Radiographics 2018 Jul-Aug;38(4):1201-1222. doi: 10.1148/rg.2018170178. PMID: 29995620
Morgello S
Handb Clin Neurol 2018;152:3-19. doi: 10.1016/B978-0-444-63849-6.00002-5. PMID: 29604982
Martin JJ
Handb Clin Neurol 2012;103:475-91. doi: 10.1016/B978-0-444-51892-7.00030-9. PMID: 21827908
Fink JK
Neurol Clin 2002 Aug;20(3):711-26. doi: 10.1016/s0733-8619(02)00007-5. PMID: 12432827

Therapy

Gong M, Liang W, He C, Shen Y, Zhang Z, Lou M, Xu Z
Neurosci Lett 2023 Feb 6;796:137057. Epub 2023 Jan 5 doi: 10.1016/j.neulet.2023.137057. PMID: 36621586
Pasquini J, Firbank MJ, Ceravolo R, Silani V, Pavese N
Mov Disord 2022 Oct;37(10):1963-1984. Epub 2022 Aug 29 doi: 10.1002/mds.29195. PMID: 36036378Free PMC Article
Wong JC, Walsh K, Hayden D, Eichler FS
J Inherit Metab Dis 2018 Jul;41(4):647-656. Epub 2018 Feb 26 doi: 10.1007/s10545-018-0152-9. PMID: 29484516
Wang AC, Ibrahim GM, Poliakov AV, Wang PI, Fallah A, Mathern GW, Buckley RT, Collins K, Weil AG, Shurtleff HA, Warner MH, Perez FA, Shaw DW, Wright JN, Saneto RP, Novotny EJ, Lee A, Browd SR, Ojemann JG
J Neurosurg Pediatr 2018 Jan;21(1):81-89. Epub 2017 Nov 3 doi: 10.3171/2017.7.PEDS17137. PMID: 29099351
Schiffmann R, Mayfield J, Swift C, Nestrasil I
Mol Genet Metab 2014 Feb;111(2):147-51. Epub 2013 Nov 21 doi: 10.1016/j.ymgme.2013.11.007. PMID: 24332805Free PMC Article

Prognosis

Grijalva RM, Pham NTT, Huang Q, Martin PR, Ali F, Clark HM, Duffy JR, Utianski RL, Botha H, Machulda MM, Weigand SD, Ahlskog JE, Dickson DW, Josephs KA, Whitwell JL
Mov Disord 2022 Apr;37(4):702-712. Epub 2021 Dec 31 doi: 10.1002/mds.28901. PMID: 34970796Free PMC Article
Kunam VK, Velayudhan V, Chaudhry ZA, Bobinski M, Smoker WRK, Reede DL
Radiographics 2018 Jul-Aug;38(4):1201-1222. doi: 10.1148/rg.2018170178. PMID: 29995620
Foesleitner O, Nenning KH, Traub-Weidinger T, Feucht M, Bonelli S, Czech T, Dorfer C, Prayer D, Kasprian G
AJNR Am J Neuroradiol 2018 Aug;39(8):1530-1535. Epub 2018 Jun 28 doi: 10.3174/ajnr.A5715. PMID: 29954815Free PMC Article
Fink JK
Neurol Clin 2002 Aug;20(3):711-26. doi: 10.1016/s0733-8619(02)00007-5. PMID: 12432827
Terry RD
Fed Proc 1978 Dec;37(14):2837-40. PMID: 720637

Clinical prediction guides

Hayakawa K, Tanda K, Nishimura A, Koshino S, Kizaki Z, Ohno K
Pediatr Radiol 2022 Jun;52(7):1356-1369. Epub 2022 Mar 16 doi: 10.1007/s00247-022-05331-9. PMID: 35294621
Grijalva RM, Pham NTT, Huang Q, Martin PR, Ali F, Clark HM, Duffy JR, Utianski RL, Botha H, Machulda MM, Weigand SD, Ahlskog JE, Dickson DW, Josephs KA, Whitwell JL
Mov Disord 2022 Apr;37(4):702-712. Epub 2021 Dec 31 doi: 10.1002/mds.28901. PMID: 34970796Free PMC Article
Chiba E, Kimura Y, Shimizu-Motohashi Y, Miyagawa N, Ota M, Shigemoto Y, Ohnishi M, Nakaya M, Nakagawa E, Sasaki M, Sato N
Neuroradiology 2022 Apr;64(4):825-836. Epub 2021 Oct 25 doi: 10.1007/s00234-021-02836-2. PMID: 34693484
Foesleitner O, Nenning KH, Traub-Weidinger T, Feucht M, Bonelli S, Czech T, Dorfer C, Prayer D, Kasprian G
AJNR Am J Neuroradiol 2018 Aug;39(8):1530-1535. Epub 2018 Jun 28 doi: 10.3174/ajnr.A5715. PMID: 29954815Free PMC Article
Fink JK
Neurol Clin 2002 Aug;20(3):711-26. doi: 10.1016/s0733-8619(02)00007-5. PMID: 12432827

Recent systematic reviews

Matejko AA, Ansari D
Neurosci Biobehav Rev 2015 Jan;48:35-52. Epub 2014 Nov 15 doi: 10.1016/j.neubiorev.2014.11.006. PMID: 25446952
Tir M, Delmaire C, Besson P, Defebvre L
Rev Neurol (Paris) 2014 Apr;170(4):266-76. Epub 2014 Mar 20 doi: 10.1016/j.neurol.2013.10.013. PMID: 24656811
Loureiro JL, Brandão E, Ruano L, Brandão AF, Lopes AM, Thieleke-Matos C, Miller-Fleming L, Cruz VT, Barbosa M, Silveira I, Stevanin G, Pinto-Basto J, Sequeiros J, Alonso I, Coutinho P
JAMA Neurol 2013 Apr;70(4):481-7. doi: 10.1001/jamaneurol.2013.1956. PMID: 23400676

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Consumer resources

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...