Corpus callosum

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Corpus callosum
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Search for Corpus callosum in Gray's.

The corpus callosum (callosal commissure) is the major grouping of nerve fibres that connect together the two cerebral hemispheres of the brain. In human adults it is the largest fibre tract in the brain and contains about 200 million axons. While this function is important in highest function of the mature brain, which evolves laterality of function, it is known that some of those with congenital agenesis of the corpus callosum are fully functional adults, due to axonal plasticity. It has been observed that Albert Einstein had a particularly well developed corpus callosum[1]. Surgical division of the corpus callosum (callosotomy) can be of benefit in some forms of refractory epilepsy and such surgical division leads to subtle problems in:

  • Communication Between brain hemispheres
  • Eye movement and visual stereopsis
  • Maintenance of arousal and attention
  • Tactile localization

Dysgenesis of the corpus callosum

Congenital agenesis and dysgenesis of the corpus callosum (callosal commissure), (also known ACC or ACC syndrome) is found in a range of developmental syndromes but perhaps 1 in 3000 humans who have agenesis of the corpus callosum are fully functional. However it is apparently associated with some subtle neuroendocrine disorders (eg causing chronic hypernatraemia[2], hyponatraemia or hypothermia[3]) and epilepsy[4] as well as resulting in interesting perceptual disorders in adults such as difficulties with visual stereosis (depth perception) and changes in pain perception. Laterality such as handiness is unlikely. Due to axonal plasticity some of the functions of the corpus callosum could be substituted by the anterior commissure, posterior commissure, and hippocampal commissure but with presumably considerably less neurological reserve. Others with such agenesis can have marked neurodisablity and the condition is associated with a number of developmental abnormalities due to genetic conditions and environmental toxin/infection exposure between 12 to 20 weeks of foetal development.

The ability with modern imaging techniques to detect the condition in utero means the prognosis of isolated needs to be better understood. One series showed that 88% had essentially normal childhood development or only mild disabilities by 8 years. For this normal 88% IQ is within normal range with lower scores in verbal comprehension, social judgment, executive functions[5]. Another series supplemented by genetic testing that resulted in selective termination of pregnancy suggested about two thirds were essentially normal[6].

The possible functional impairments in apparently normal humans with agenesis of the corpus callosum are:

  • Perception of pain decreased[7][8]
  • Motor coordination impairment[9][8]
  • Poor depth perception (visual stereosis)[8]
  • Social interactions (tendency to isolation)[10]
  • Memory process[12] (note that IQ is low in over 12% [4])
    • Less able to engage lateralised brain systems specialised for particular memory material
      • Less supramarginal activations for verbal material
      • Less fusiform activations for face processing
      • Absence of right-predominant activations during retrieval
  • Contralateral visual stimuli processing[13]
  • Disordered sleep[14]
  • Epilepsy (15-20%)[15][4]
A-75 year old with intact corpus callosum
B-75 year old with agenesis corpus callosum first diagnosed at time of CT head done because of isolated short term memory loss and failure to mobilise well after repair of a fractured neck of femur. There was intact sense of smell 2 years after presentation with continuing short term memory recall problems without apraxia or incoordination. A retrospective history was obtained of high pain threshold, introvert personality, and three transient right sided hemiparetic events over 20 year period. The patient had had chronic hyponatraemia for over 10 years.
C-71 year old with normal MRI scan head
B-Clinical details as for CT scan above although this MR scan done 1 year later also showed bilateral hippocampal atrophy

Some of the known conditions associated with it are:

References

  1. Men W, Falk D, Sun T, Chen W, Li J, Yin D, Zang L, Fan M. The corpus callosum of Albert Einstein's brain: another clue to his high intelligence? Brain : a journal of neurology. 2014 Apr; 137(Pt 4):e268.(Print-Electronic) (Link to article – subscription may be required.)
  2. Bannister P. Hypernatraemia and agenesis of the corpus callosum. Clinical endocrinology. 1985 Sep; 23(3):319.(Print) (Link to article – subscription may be required.)
  3. Page SR, Nussey SS, Jenkins JS, Wilson SG, Johnson DA. Hypothalamic disease in association with dysgenesis of the corpus callosum. Postgraduate medical journal. 1989 Mar; 65(761):163-167.(Print) (Link to article – subscription may be required.)
  4. a b c Margari L, Palumbi R, Campa MG, Operto FF, Buttiglione M, Craig F, Matricardi S, Verrotti A. Clinical manifestations in children and adolescents with corpus callosum abnormalities. Journal of neurology. 2016 Oct; 263(10):1939-1945.(Print-Electronic) (Link to article – subscription may be required.)
  5. Folliot-Le Doussal L, Chadie A, Brasseur-Daudruy M, Verspyck E, Saugier-Veber P, Marret S. Neurodevelopmental outcome in prenatally diagnosed isolated agenesis of the corpus callosum. Early human development. 2017 Oct; 116:9-16.(Print-Electronic) (Link to article – subscription may be required.)
  6. des Portes V, Rolland A, Velazquez-Dominguez J, Peyric E, Cordier MP, Gaucherand P, Massardier J, Massoud M, Curie A, Pellot AS, Rivier F, Lacalm A, Clément A, Ville D, Guibaud L. Outcome of isolated agenesis of the corpus callosum: A population-based prospective study. European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society. 2017 Sep.(Print-Electronic) (Link to article – subscription may be required.)
  7. Demopoulos C, Arroyo MS, Dunn W, Strominger Z, Sherr EH, Marco E. Individuals with agenesis of the corpus callosum show sensory processing differences as measured by the sensory profile. Neuropsychology. 2015 Sep; 29(5):751-758.(Print-Electronic) (Link to article – subscription may be required.)
  8. a b c Moes P, Schilmoeller K, Schilmoeller G. Physical, motor, sensory and developmental features associated with agenesis of the corpus callosum. Child: care, health and development. 2009 Sep; 35(5):656-672.(Print-Electronic) (Link to article – subscription may be required.)
  9. Mueller KL, Marion SD, Paul LK, Brown WS. Bimanual motor coordination in agenesis of the corpus callosum. Behavioral neuroscience. 2009 Oct; 123(5):1000-1011.(Print) (Link to article – subscription may be required.)
  10. Anderson LB, Paul LK, Brown WS. Emotional Intelligence in Agenesis of the Corpus Callosum. Archives of clinical neuropsychology : the official journal of the National Academy of Neuropsychologists. 2017 May; 32(3):267-279.(Print) (Link to article – subscription may be required.)
  11. Wegiel J, Flory M, Kaczmarski W, Brown WT, Chadman K, Wisniewski T, Nowicki K, Kuchna I, Ma SY, Wegiel J. Partial Agenesis and Hypoplasia of the Corpus Callosum in Idiopathic Autism. Journal of neuropathology and experimental neurology. 2017 Mar; 76(3):225-237.(Print) (Link to article – subscription may be required.)
  12. Siffredi V, Spencer-Smith MM, Barrouillet P, Vaessen MJ, Leventer RJ, Anderson V, Vuilleumier P. Neural correlates of working memory in children and adolescents with agenesis of the corpus callosum: An fMRI study. Neuropsychologia. 2017 Nov; 106:71-82.(Print-Electronic) (Link to article – subscription may be required.)
  13. Okajima M, Futamura A, Honma M, Kawamura M, Yotsumoto Y. Interhemispheric cortical connections and time perception: A case study with agenesis of the corpus callosum. Brain and cognition. 2017 Oct; 117:12-16.(Print-Electronic) (Link to article – subscription may be required.)
  14. Ingram DG, Churchill SS. Sleep Problems in Children With Agenesis of the Corpus Callosum. Pediatric neurology. 2017 Feb; 67:85-90.(Print-Electronic) (Link to article – subscription may be required.)
  15. Romaniello R, Marelli S, Giorda R, Bedeschi MF, Bonaglia MC, Arrigoni F, Triulzi F, Bassi MT, Borgatti R. Clinical Characterization, Genetics, and Long-Term Follow-up of a Large Cohort of Patients With Agenesis of the Corpus Callosum. Journal of child neurology. 2017 Jan; 32(1):60-71.(Print-Electronic) (Link to article – subscription may be required.)
  16. Marsh AP, Heron D, Edwards TJ, Quartier A, Galea C, Nava C, Rastetter A, Moutard ML, Anderson V, Bitoun P, Bunt J, Faudet A, Garel C, Gillies G, Gobius I, Guegan J, Heide S, Keren B, Lesne F, Lukic V, Mandelstam SA, McGillivray G, McIlroy A, Méneret A, Mignot C, Morcom LR, Odent S, Paolino A, Pope K, Riant F, Robinson GA, Spencer-Smith M, Srour M, Stephenson SE, Tankard R, Trouillard O, Welniarz Q, Wood A, Brice A, Rouleau G, Attié-Bitach T, Delatycki MB, Mandel JL, Amor DJ, Roze E, Piton A, Bahlo M, Billette de Villemeur T, Sherr EH, Leventer RJ, Richards LJ, Lockhart PJ, Depienne C. Mutations in DCC cause isolated agenesis of the corpus callosum with incomplete penetrance. Nature genetics. 2017 Apr; 49(4):511-514.(Print-Electronic) (Link to article – subscription may be required.)
  17. Marsh APL, Edwards TJ, Galea C, Cooper HM, Engle EC, Jamuar SS, Méneret A, Moutard ML, Nava C, Rastetter A, Robinson G, Rouleau G, Roze E, Spencer-Smith M, Trouillard O, Billette de Villemeur T, Walsh CA, Yu TW, Heron D, Sherr EH, Richards LJ, Depienne C, Leventer RJ, Lockhart PJ. DCC mutation update: Congenital mirror movements, isolated agenesis of the corpus callosum, and developmental split brain syndrome. Human mutation. 2017 Oct.(Print-Electronic) (Link to article – subscription may be required.)
  18. Hong M, Krauss RS. Ethanol itself is a holoprosencephaly-inducing teratogen. PloS one. 2017 ; 12(4):e0176440.(Electronic-eCollection) (Link to article – subscription may be required.)
  19. Alby C, Boutaud L, Bonnière M, Collardeau-Frachon S, Guibaud L, Lopez E, Bruel AL, Aral B, Sonigo P, Roth P, Vibert-Guigue C, Castaigne V, Carbonne B, Joyé N, Faivre L, Cordier MP, Bernabe Gelot A, Clementi M, Mammi I, Vekemans M, Razavi F, Gonzales M, Thauvin-Robinet C, Attié-Bitach T. In utero ultrasound diagnosis of corpus callosum agenesis leading to the identification of orofaciodigital type 1 syndrome in female fetuses. Birth Defects Res. 2017 Nov 28. doi: 10.1002/bdr2.1154.
  20. Coman D, Fullston T, Shoubridge C, Leventer R, Wong F, Nazaretian S, Simpson I, Gecz J, McGillivray G. X-Linked Lissencephaly With Absent Corpus Callosum and Abnormal Genitalia: An Evolving Multisystem Syndrome With Severe Congenital Intestinal Diarrhea Disease. Child neurology open. 2017 Jan-Dec; 4:2329048X17738625.(Electronic-eCollection) (Link to article – subscription may be required.)
  21. Tan L, Bi B, Zhao P, Cai X, Wan C, Shao J, He X. Severe congenital microcephaly with 16p13.11 microdeletion combined with NDE1 mutation, a case report and literature review. BMC Med Genet. 2017 Dec 1;18(1):141. doi: 10.1186/s12881-017-0501-9
  22. Guen VJ, Edvardson S, Fraenkel ND, Fattal-Valevski A, Jalas C, Anteby I, Shaag A, Dor T, Gillis D, Kerem E, Lees JA, Colas P, Elpeleg O. A homozygous deleterious CDK10 mutation in a patient with agenesis of corpus callosum, retinopathy, and deafness. American journal of medical genetics. Part A. 2017 Nov.(Print-Electronic) (Link to article – subscription may be required.)
  23. Rüland AM, Gloning KP, Albig M, Kagan KO, Hammer R, Schälike M, Berg C, Gembruch U, Geipel A. The Incidence of Chromosomal Aberrations in Prenatally Diagnosed Isolated Agenesis of the Corpus Callosum. Ultraschall in der Medizin (Stuttgart, Germany : 1980). 2017 Sep.(Print-Electronic) (Link to article – subscription may be required.)
  24. Gacio S, Lescano S. Foetal Magnetic Resonance Images of Two Cases of Aicardi Syndrome. Journal of clinical and diagnostic research : JCDR. 2017 Jul; 11(7):SD07-SD09.(Print-Electronic) (Link to article – subscription may be required.)
  25. Chaudhry A, Chung BH, Stavropoulos DJ, Araya MP, Ali A, Heon E, Chitayat D. Agenesis of the corpus callosum, developmental delay, autism spectrum disorder, facial dysmorphism, and posterior polymorphous corneal dystrophy associated with ZEB1 gene deletion. American journal of medical genetics. Part A. 2017 Jul.(Print-Electronic) (Link to article – subscription may be required.)
  26. Stevens SJ, van Essen AJ, van Ravenswaaij CM, Elias AF, Haven JA, Lelieveld SH, Pfundt R, Nillesen WM, Yntema HG, van Roozendaal K, Stegmann AP, Gilissen C, Brunner HG. Truncating de novo mutations in the Krüppel-type zinc-finger gene ZNF148 in patients with corpus callosum defects, developmental delay, short stature, and dysmorphisms. Genome medicine. 2016 Dec; 8(1):131.(Electronic) (Link to article – subscription may be required.)