Cerebellar vermis

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Cerebellar vermis
Sobo 1909 653 Cerebellar vermis.png
Upper surface of cerebellum. The vermis is highlighted in red.
Cerebellar vermis -- animation.gif
Vermis (highlighted in red) on the cerebellum.
Details
Part of Cerebellum
Identifiers
Latin vermis cerebelli
MeSH D065814
NeuroNames 2463
NeuroLex ID birnlex_1106
TA98 A14.1.07.006
TA2 5819
FMA 76928
Anatomical terms of neuroanatomy

The cerebellar vermis (from Latin vermis, "worm") is located in the medial, cortico-nuclear zone of the cerebellum, which is in the posterior fossa of the cranium. The primary fissure in the vermis curves ventrolaterally to the superior surface of the cerebellum, dividing it into anterior and posterior lobes. Functionally, the vermis is associated with bodily posture and locomotion. The vermis is included within the spinocerebellum and receives somatic sensory input from the head and proximal body parts via ascending spinal pathways. [1]

Contents

The cerebellum develops in a rostro-caudal manner, with rostral regions in the midline giving rise to the vermis, and caudal regions developing into the cerebellar hemispheres. [2] By 4 months of prenatal development, the vermis becomes fully foliated, while development of the hemispheres lags by 30–60 days. [3] Postnatally, proliferation and organization of the cellular components of the cerebellum continues, with completion of the foliation pattern by 7 months of life [4] and final migration, proliferation, and arborization of cerebellar neurons by 20 months. [5]

Inspection of the posterior fossa is a common feature of prenatal ultrasound and is used primarily to determine whether excess fluid or malformations of the cerebellum exist. [6] Anomalies of the cerebellar vermis are diagnosed in this manner and include phenotypes consistent with Dandy–Walker malformation, rhombencephalosynapsis, displaying no vermis with fusion of the cerebellar hemispheres, pontocerebellar hypoplasia, or stunted growth of the cerebellum, and neoplasms. In neonates, hypoxic injury to the cerebellum is fairly common, resulting in neuronal loss and gliosis. Symptoms of these disorders range from mild loss of fine motor control to severe intellectual disability and death. Karyotyping has shown that most pathologies associated with the vermis are inherited through an autosomal recessive pattern, with most known mutations occurring on the X chromosome. [1] [7]

The vermis is intimately associated with all regions of the cerebellar cortex, which can be divided into three functional parts, each having distinct connections with the brain and spinal cord. These regions are the vestibulocerebellum, which is responsible primarily for the control of eye movements; the spinocerebellum, involved in fine tune body and limb movement; and the cerebrocerebellum, which is associated with planning, initiation and timing of movements. [8]

Structure

Anterior surface of cerebellum. The vermis is highlighted in red. Sobo 1909 655 Cerebellar vermis.png
Anterior surface of cerebellum. The vermis is highlighted in red.

The vermis is the unpaired, median portion of the cerebellum that connects the two hemispheres. [9] Both the vermis and the hemispheres are composed of lobules formed by groups of folia. There are nine lobules of the vermis: lingula, central lobule, culmen, clivus, folium of the vermis, tuber, pyramid, uvula and nodule. [9] These lobules are often difficult to observe during human anatomy classes and may vary in size, shape and number of folia. It has been shown that folia of the cerebellum exhibit frequent variations in form, number and arrangement between individuals. [9]

Lobe anatomy

Schematic representation of the major anatomical subdivisions of the cerebellum. CerebellumDiv.png
Schematic representation of the major anatomical subdivisions of the cerebellum.

The lingula is the first lobule of the upper portion of the vermis on the superoinferior axis and pertains to the paleocerebellum together with the central lobule, culmen, pyramid and uvula. It is separated from the central lobule by the pre-central fissure. The central lobule is the second lobule of the upper portion of the vermis on the superoinferior axis. The culmen is the third and largest lobule of the upper portion of the vermis on the superoinferior axis. It is separated from the declive by the primary fissure and is related with the anterior quadrangular lobule of the hemisphere. The pyramid is the seventh lobule of the vermis on the superoinferior axis. It is separated from the tuber and uvula by the pre-pyramidal and secondary fissures, respectively. [9] This lobule is related with the biventral lobule of the hemisphere. The uvula is the second largest lobule, following the culmen. It pertains to the paleocerebellum and is separated from the nodule by the posterolateral fissure. [9]

Spinocerebellum

The spinocerebellum receives proprioception input from the dorsal columns of the spinal cord (including the spinocerebellar tract) and from the trigeminal nerve, as well as from visual and auditory systems. It sends fibers to deep cerebellar nuclei that, in turn, project to both the cerebral cortex and the brain stem, thus providing modulation of descending motor systems. [8] This region comprises the vermis and intermediate parts of the cerebellar hemispheres. Sensory information from the periphery and from the primary motor and somatosensory cortex terminate in this region. Purkinje cells of the vermis project to the fastigial nucleus, controlling the axial and proximal musculature involved in the execution of limb movements. [10] Purkinje cells in the intermediate zone of the spinocerebellum project to the interposed nuclei, which control the distal musculature components of the descending motor pathways needed for limb movement. Both of these nuclei include projections to the motor cortex in the cerebrum. [10]

Nuclei

The interposed nucleus is smaller than the dentate nucleus but larger than the fastigial nucleus and functions to modulate muscle stretch reflexes of distal musculature. [9] It is located dorsal to the fourth ventricle and lateral to the fastigial nucleus; it receives afferent neuronal supply from the anterior lobe of the cerebellum and sends output via the superior cerebellar peduncle and the red nucleus. [8]

The fastigial nucleus is the most medial efferent cerebellar nucleus, targeting the pontine and medullary reticular formation as well as the vestibular nuclei. [10] This region deals with antigravity muscle groups and other synergies involved with standing and walking. [11] It is thought that fastigial nuclei axons are excitatory and project beyond the cerebellum, likely using glutamate and aspartate as neurotransmitters. [10]

Pathology

Malformations of the posterior fossa have been recognized more frequently during the past few decades as the result of recent advances in technology. Malformations of the cerebellar vermis were first identified using pneumoencephalography, where air is injected into the cerebrospinal fluid spaces of the cerebellum; displaced, occluded or dysplastic structures could be identified. Upon the advent of computerized tomography (CT) and magnetic resonance imaging (MRI), the resolution of cranial structures including the mid-hindbrain regions improved dramatically. [12]

Joubert syndrome

Joubert syndrome (JS) is one of the most commonly diagnosed syndromes associated with the molar tooth sign (MTS), [13] or hypoplasia/dysplasia of the cerebellar vermis accompanied by brainstem abnormalities. JS is defined clinically by features of hypotonia in infancy with later development of ataxia, developmental delays, mental retardation, abnormal breathing patterns, abnormal eye movements specific to oculomotor apraxia, or the presence of the MTS on the cranial MRI. [14] [15] JS is an autosomal recessive condition with an estimated prevalence of 1: 100,000. [16]

Dandy Walker malformation

Dandy Walker malformation is a relatively common congenital brain malformation with a prevalence of 1:30,000 live births. [17] Dandy Walker malformation is characterized by enlarged posterior fossa and in which the cerebellar vermis is completely absent, or present in a rudimentary form, sometimes rotated accompanied by an elevation of the fourth ventricle. It is also commonly associated with dysplasias of brainstem nuclei. [18] DWM has been reported to be in association with a wide array of chromosomal anomalies, including trisomy 18, trisomy 9, and trisomy 13. Surveys suggest that prenatal exposure to teratogens such as rubella or alcohol are correlated with development of Dandy Walker malformation. [19] [20]

Rhombencephalosynapsis

Rhombencephalosynapsis is an anomaly characterized by the absence or severe dysgenesis of the cerebellar vermis with fusion of the cerebellar hemispheres, peduncles, and dentate nuclei. Diagnostic features include fusion of the midbrain colliculi, hydrocephalus, absence of the corpus callosum other midline structural brain malformations. [21] [22] [23]

Autism spectrum disorders

Hypoplasia and other structural alterations of the vermis have been identified in many patients with autism spectrum disorder (ASD). While the exact nature and extent of the impacts ASD has on the vermis remain in question, it has also been shown that other injuries and malformations of the vermis sometimes produce symptoms closely analogous to ASD. Furthermore, several genetic syndromes known to cause autism (such as fragile X syndrome) have also been shown to cause damage to the vermis. [24]

Damage

Lesions to the vermis commonly give rise to clinical depression, inappropriate emotional displays (e.g. unwarranted giggling) in addition to movement disorders. [ citation needed ]

Comparative anatomy

Early neurophysiologists suggest that retinal and inertial signals were selected for about 450 million years ago by primitive brainstem-cerebellar circuitry because of their relationship with the environment. [25] Microscopically, it is evident that Purkinje cell precursors arose from granule cells, first forming in irregular patterns, then progressively becoming organized in a layered fashion. Evolutionarily, the Purkinje cells then developed extensive dendritic trees that increasingly became confined to a single plane, through which the axons of granule cells threaded, eventually forming a neuronal grid of right angles. [25] The origin of the cerebellum is in close association with that of the nuclei of the vestibular cranial nerve and lateral line nerves, perhaps suggesting that this part of the cerebellum originated as a means of carrying out transformations of the coordinate system from input data of the vestibular organ and the lateral line organs. [26] This suggests that the function of the cerebellum evolved as a mode of computing and representing an image relating to the position of the body in space. The cerebellar vermis evolved in conjunction with the hemispheres; this is seen in lampreys and higher vertebrates. [27]

In fish

In vertebrates, the cerebellar vermis develops between two bilaterally symmetrical formations located dorsal to the upper end of the medulla oblongata, or rhombencephalon. This is the region of termination for the fibers of the vestibular nerve and lateral line nerves; thus, these are the oldest afferent paths to the cerebellum and cerebellar vermis. [27] In bony fish, or teleosts, it has been proposed that the cerebellar auricles, which receive a large amount of input from the vestibulolateral line system, constitute the vestibulocerebellum and are homologues of the flocculonodular lobe of higher vertebrates along with the corpus cerebelli, which receives spinocerebellar and tectocerebellar fibers. The labyrinth and the lateral line organs of lampreys have structural and functional similarity. An important difference between the two structures is that the arrangement of the lateral line organs are such that they are sensitive to relative motion of the fluid surrounding the animal, whereas the labyrinths, having very similar sensing mechanisms, are sensitive to endolymph, providing information concerning the animal's own equilibrium of the body and orientation in space. [27]

See also

Additional images

Related Research Articles

<span class="mw-page-title-main">Cerebellum</span> Structure at the rear of the vertebrate brain, beneath the cerebrum

The cerebellum is a major feature of the hindbrain of all vertebrates. Although usually smaller than the cerebrum, in some animals such as the mormyrid fishes it may be as large as it or even larger. In humans, the cerebellum plays an important role in motor control. It may also be involved in some cognitive functions such as attention and language as well as emotional control such as regulating fear and pleasure responses, but its movement-related functions are the most solidly established. The human cerebellum does not initiate movement, but contributes to coordination, precision, and accurate timing: it receives input from sensory systems of the spinal cord and from other parts of the brain, and integrates these inputs to fine-tune motor activity. Cerebellar damage produces disorders in fine movement, equilibrium, posture, and motor learning in humans.

<span class="mw-page-title-main">Sense of balance</span> Physiological sense regarding posture

The sense of balance or equilibrioception is the perception of balance and spatial orientation. It helps prevent humans and nonhuman animals from falling over when standing or moving. Equilibrioception is the result of a number of sensory systems working together; the eyes, the inner ears, and the body's sense of where it is in space (proprioception) ideally need to be intact.

<span class="mw-page-title-main">Brainstem</span> Posterior part of the brain, adjoining and structurally continuous

The brainstem is the stalk-like part of the brain that interconnects the cerebrum and diencephalon with the spinal cord. In the human brain, the brainstem is composed of the midbrain, the pons, and the medulla oblongata. The midbrain is continuous with the thalamus of the diencephalon through the tentorial notch.

In neuroanatomy, a nucleus is a cluster of neurons in the central nervous system, located deep within the cerebral hemispheres and brainstem. The neurons in one nucleus usually have roughly similar connections and functions. Nuclei are connected to other nuclei by tracts, the bundles (fascicles) of axons extending from the cell bodies. A nucleus is one of the two most common forms of nerve cell organization, the other being layered structures such as the cerebral cortex or cerebellar cortex. In anatomical sections, a nucleus shows up as a region of gray matter, often bordered by white matter. The vertebrate brain contains hundreds of distinguishable nuclei, varying widely in shape and size. A nucleus may itself have a complex internal structure, with multiple types of neurons arranged in clumps (subnuclei) or layers.

<span class="mw-page-title-main">Cerebellar hypoplasia</span> Medical condition

Cerebellar hypoplasia is characterized by reduced cerebellar volume, even though cerebellar shape is (near) normal. It consists of a heterogeneous group of disorders of cerebellar maldevelopment presenting as early-onset non–progressive congenital ataxia, hypotonia and motor learning disability.

<span class="mw-page-title-main">Inferior olivary nucleus</span> Brain structure in the medulla that helps coordinate movement

The inferior olivary nucleus (ION) is a structure found in the medulla oblongata underneath the superior olivary nucleus. In vertebrates, the ION is known to coordinate signals from the spinal cord to the cerebellum to regulate motor coordination and learning. These connections have been shown to be tightly associated, as degeneration of either the cerebellum or the ION results in degeneration of the other.

<span class="mw-page-title-main">Deep cerebellar nuclei</span>

There are four deep cerebellar nuclei embedded in the white matter of the medullary centre. The nuclei are the fastigial, globose, emboliform, and dentate nuclei.

<span class="mw-page-title-main">Inferior cerebellar peduncle</span>

The upper part of the posterior district of the medulla oblongata is occupied by the inferior cerebellar peduncle, a thick rope-like strand situated between the lower part of the fourth ventricle and the roots of the glossopharyngeal and vagus nerves.

<span class="mw-page-title-main">Dentate nucleus</span> Nucleus in the centre of each cerebellar hemisphere

The dentate nucleus is a cluster of neurons, or nerve cells, in the central nervous system that has a dentate – tooth-like or serrated – edge. It is located within the deep white matter of each cerebellar hemisphere, and it is the largest single structure linking the cerebellum to the rest of the brain. It is the largest and most lateral, or farthest from the midline, of the four pairs of deep cerebellar nuclei, the others being the globose and emboliform nuclei, which together are referred to as the interposed nucleus, and the fastigial nucleus. The dentate nucleus is responsible for the planning, initiation and control of voluntary movements. The dorsal region of the dentate nucleus contains output channels involved in motor function, which is the movement of skeletal muscle, while the ventral region contains output channels involved in nonmotor function, such as conscious thought and visuospatial function.

<span class="mw-page-title-main">Fastigial nucleus</span> Grey matter nucleus in the cerebellum

The fastigial nucleus is located in the cerebellum. It is one of the four deep cerebellar nuclei, and is grey matter embedded in the white matter of the cerebellum.

<span class="mw-page-title-main">Flocculus</span>

The flocculus is a small lobe of the cerebellum at the posterior border of the middle cerebellar peduncle anterior to the biventer lobule. Like other parts of the cerebellum, the flocculus is involved in motor control. It is an essential part of the vestibulo-ocular reflex, and aids in the learning of basic motor skills in the brain.

<span class="mw-page-title-main">Dandy–Walker malformation</span> Congenital malformation of the cerebellar vermis

Dandy–Walker malformation (DWM), also known as Dandy–Walker syndrome (DWS), is a rare congenital brain malformation in which the part joining the two hemispheres of the cerebellum does not fully form, and the fourth ventricle and space behind the cerebellum are enlarged with cerebrospinal fluid. Most of those affected develop hydrocephalus within the first year of life, which can present as increasing head size, vomiting, excessive sleepiness, irritability, downward deviation of the eyes and seizures. Other, less common symptoms are generally associated with comorbid genetic conditions and can include congenital heart defects, eye abnormalities, intellectual disability, congenital tumours, other brain defects such as agenesis of the corpus callosum, skeletal abnormalities, an occipital encephalocele or underdeveloped genitalia or kidneys. It is sometimes discovered in adolescents or adults due to mental health problems.

<span class="mw-page-title-main">Cerebellar peduncle</span> Structure connecting the cerebellum to the brainstem

Cerebellar peduncles connect the cerebellum to the brain stem. There are six cerebellar peduncles in total, three on each side:

<span class="mw-page-title-main">Middle cerebellar peduncle</span> Structure in the brain connecting the pons to the cerebellum

The middle cerebellar peduncle is a paired structure of the brain. It connects the pons to the cerebellum, with fibres originating from the pontine nucleus and travelling to the opposite hemisphere of the cerebellar cortex. It is supplied by the anterior inferior cerebellar artery (AICA) and branches from the basilar artery. It conveys information from the cerebrum and the pons to the cerebellum.

<span class="mw-page-title-main">Cerebellar tonsil</span>

The cerebellar tonsil is a rounded lobule on the undersurface of each cerebellar hemisphere, continuous medially with the uvula of the cerebellar vermis and superiorly by the flocculonodular lobe. Synonyms include: tonsilla cerebelli, amygdala cerebelli, the latter of which is not to be confused with the cerebral tonsils or amygdala nuclei located deep within the medial temporal lobes of the cerebral cortex.

<span class="mw-page-title-main">3C syndrome</span> Medical condition

3C syndrome is a rare condition whose symptoms include heart defects, cerebellar hypoplasia, and cranial dysmorphism. It was first described in the medical literature in 1987 by Ritscher and Schinzel, for whom the disorder is sometimes named.

<span class="mw-page-title-main">Anatomy of the cerebellum</span> Structures in the cerebellum, a part of the brain

The anatomy of the cerebellum can be viewed at three levels. At the level of gross anatomy, the cerebellum consists of a tightly folded and crumpled layer of cortex, with white matter underneath, several deep nuclei embedded in the white matter, and a fluid-filled ventricle in the middle. At the intermediate level, the cerebellum and its auxiliary structures can be broken down into several hundred or thousand independently functioning modules or compartments known as microzones. At the microscopic level, each module consists of the same small set of neuronal elements, laid out with a highly stereotyped geometry.

Cerebellar cognitive affective syndrome (CCAS), also called Schmahmann's syndrome is a condition that follows from lesions (damage) to the cerebellum of the brain. It refers to a constellation of deficits in the cognitive domains of executive function, spatial cognition, language, and affect resulting from damage to the cerebellum. Impairments of executive function include problems with planning, set-shifting, abstract reasoning, verbal fluency, and working memory, and there is often perseveration, distractibility and inattention. Language problems include dysprosodia, agrammatism and mild anomia. Deficits in spatial cognition produce visual–spatial disorganization and impaired visual–spatial memory. Personality changes manifest as blunting of affect or disinhibited and inappropriate behavior. These cognitive impairments result in an overall lowering of intellectual function. CCAS challenges the traditional view of the cerebellum being responsible solely for regulation of motor functions. It is now thought that the cerebellum is responsible for monitoring both motor and nonmotor functions. The nonmotor deficits described in CCAS are believed to be caused by dysfunction in cerebellar connections to the cerebral cortex and limbic system.

<span class="mw-page-title-main">Unipolar brush cell</span>

Unipolar brush cells (UBCs) are a class of excitatory glutamatergic interneuron found in the granular layer of the cerebellar cortex and also in the granule cell domain of the cochlear nucleus.

References

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  2. ↑ Cho, K. H.; Rodriguez-Vazquez, J. F.; Kim, J. H.; Abe, H.; Murakami, G.; Cho, B. H. (2011). "Early fetal development of the human cerebellum". Surgical and Radiologic Anatomy. 33 (6): 523–530. doi:10.1007/s00276-011-0796-8. PMID   21380713. S2CID   25451924.
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  13. ↑ Brancati F, Dallapiccola B, Valente EM (2010). "Joubert Syndrome and related disorders". Orphanet Journal of Rare Diseases. 5: 20. doi: 10.1186/1750-1172-5-20 . PMC   2913941 . PMID   20615230.
  14. ↑ J.M. Saraiva; M. Baraitser (1992). "Joubert syndrome: a review". American Journal of Medical Genetics. 43 (4): 726–731. doi:10.1002/ajmg.1320430415. PMID   1341417.
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  20. ↑ S.K. Clarren; J. Alvord; S.M. Sumi (1978). "Brain malformations related to prenatal exposure to ethanol". Journal of Pediatrics. 92 (1): 64–67. doi:10.1016/S0022-3476(78)80072-9. PMID   619080.
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  22. ↑ H. Utsunomiya; K. Takano; T. Ogasawara; T. Hashimoto; T. Fukushima; M. Okazaki (1998). "Rhombencephalosynapsis: cerebellar embryogenesis". American Journal of Neuroradiology. 19 (3): 547–549. PMC   8338264 . PMID   9541316.
  23. ↑ C.L. Truwit; A.J. Barkovich; R. Shanahan; T.V. Maroldo (1991). "MR imaging of rhomboencephalosynapsis: report of three cases and review of the literature". American Journal of Neuroradiology. 12 (5): 957–965. PMC   8333516 . PMID   1950929.
  24. ↑ Hampson, David R.; Blatt, Gene J. (2015). "Autism spectrum disorders and neuropathology of the cerebellum". Frontiers in Neuroscience. 9: 420. doi: 10.3389/fnins.2015.00420 . ISSN   1662-4548. PMC   4635214 . PMID   26594141.
  25. 1 2 Nieuwenhuys, R.; Voogd, J.; van Huijzen, C. (1988). The Human Central Nervous System: A Synopsis and Atlas (3rd ed.). Heidelberg: Springer-Verlag.
  26. ↑ Butler, A.B.; Hodos, W. (1996). "12: The Cerebellum". Comparative Vertebrate Neuroanatomy: Evolution and Adaptation. New York: Wiley-Liss. pp. 180–197.
  27. 1 2 3 Ariens, K.; C.U.; Huber, G.C.; Crosby, E.C. (1960). The Comparative Anatomy of the Nervous System of Vertebrates, Including Man. Vol. 3. New York: Hafner.

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