WEDNESDAY, NOVEMBER 29, 2017
Language Processing in the Rhombencephalon
How it is affected by the Joubert Syndrome
Divisions of the Brain
- Proscephalon
- Forebrain
- Mesencephalon
- Midbrain
- Rhomcephalon
- Hindbrain
Images of the Divisions of the Brain
Images of the Divisions of the Brain
Hindbrain
- Links the brain and spinal cord
- Portion of the CNS
- Includes the pons, cerebellum, and medulla oblangata
- Automatic function
- Plays a role in verbal and signing expressions of thoughts and feelings
- Affected by the Joubert syndrome
STRUCTURE
Connects medulla to thalamus
STRUCTURE
Large structure located immediately inferior to the posterior cerebral cortex
FUNCTION
coordinates motor commands and sensory inputs to control movement
communicates with the brainstem, spinal cord, and cerebral cortex by means of superior, middle, and inferior cerebral peduncles
Medulla Oblongata
STRUCTURE
- forms the lowest part of the brain stem located below the pons and above the spinal cord
FUNCTION
- control center that regulates functions such as regulating breathing, heart and blood vessels, digestion, sneezing, and swallowing
JOUBERT SYNDROME
is a rare disorder in part of the hindbrain specifically the cerebellum.
Causes :
- may be changes in genetic
- may be inhertited
- meaning that each parent is a carrier
Characteristics :
- hypotonia
- molar tooth sign
- abnormal breathing patterns
- mental disabilities / developmental delays
- ( some children may not be effected intelgibility wise)
Joubert cont...
To this day there is no known cure
- individuals exhibit expressive and ressesive language impairaments
Genetics
- JS is an autosomal recessive pattern of inheritance. (both parents carry one copy of the gene)
- The genetic strains can vary regionally.
Speech Pathology
- JS is a rare syndrome, because of this a lot of SLP's are not informed of it and need more training and research to better benefit the child's improvement rate.
References
- Braddock, B. A., Farmer, J. E., Deidrick, K. M., Iverson, J. M., & Maria, B. L. (2006). Oromotor and communication findings in joubert syndrome: further evidence of multisystem apraxia. Journal of child neurology, 21(2), 160-163.
- Highnam, C. L., & Bleile, K. M. (2011). Language in the cerebellum. American journal of speech-language pathology, 20(4), 337-347. doi:10.1044/1058-0360(2011/10-0096)
- Michuad, J.L., Ospina, L.H., Rypens, F. (2015). Joubert syndrome in french canadians and identification of mutations in CEP104, 97(5), 744-753. doi:10.1016/j.ajhg.2015.09.009
- Parisi, M. A., Doherty, D., Chance, P. F., & Glass, I. A. (2007). Joubert syndrome (and related disorders) (OMIM 213300). European journal of human genetics, 15(5), 511-521. doi:10.1038/sj.ejhg.5201648
- Seikel, J. A., Drumright, D. G., & King, D. W. (2010). Anatomy & physiology for speech, language, and hearing (5th ed.). Clifton Park, NY: Cengage learning.