EMBRYOLOGICAL

DEVELOPMENT
OF
THE CENTRAL NERVOUS SYSTEM

“I don’t get it”

Introduction
• • • • • CNS originates in the ectoderm -----Neural plate, Neural folds, Neural tube. BRAIN + 3 VESICLE
– – – Rhombencephalon (hind brain). Mesencephalon (mid brain). Proencephalon (fore brain).


• • • • •

Rhombencephalon (mesencephalon(medulla) &metencephalon (basal efferent and alar afferent). &cerebellum (coordinating center) + pons (pathway). Mesencephalon (midbrain)+ spinal cord ) (basal efferent & alar afferent) –alar part anterior visual and posterior auditory. Diencephalon (posterior portion of the forebrain (thin roof + thick plate) – thalamus & hypothalamus. Rathke’s pouch – adenohypophysis, intermediate lobe & pass tuberalis. Diencephalon --- posterior lobe (neurohypophysis). Telencephalon (rostral brain vesicle) ---= 2 outpockting (cereberal hemisphere medina portion (lamina terminalis – connects the 2 hemispheres).

 Its lateral edges soon elevate to form the neural folds. the neural plate.Central Nervous System  The central nervous system (CNS) appears at the beginning of the third week as a slippershaped plate of thickened ectoderm. Scanning electron micrograph of a mouse embryo at approximately 18 days . in the middorsal region in front of the primitive node.

 Closure of the cranial neuropore proceeds cranially from the initial closure site in the cervical region and from a site in the forebrain that forms later. • Fusion between neural folds begins in the cervical region and proceeds in cephalic and caudal directions. .  Once fusion is initiated. the open ends of the neural tube form the cranial and caudal neuropores that communicate with the overlying amniotic cavity. the neural plate (thickened ectoderm) forms the neural tube.Central Nervous System • With further development. migrate from the edges of the neural folds and develop into spinal and cranial sensory ganglia (A–C). Cells of the neural crest. Transverse sections through successively older embryos showing formation of the neural groove. A–C. neural tube. and neural crest.

B. Dorsal view of a human embryo at approximately day 23.Dorsal view of a human embryo A. . Dorsal view of a human embryo at approximately day 22. Seven distinct somites are visible on each side of the neural tube. The nervous system is in connection with the amniotic cavity through the cranial and caudal neuropores.

rhombencephalic isthmus. The cephalic flexure in the midbrain region.Central Nervous System  The cephalic end of the neural tube shows three dilations. the primary brain vesicles: 1.  When the embryo is 5 weeks old. the prosencephalon consists of two parts: i. 2. formed by a midportion and two lateral outpocketings. optic stalk. The rhombencephalon. ii. . The cervical flexure at the junction of the hindbrain and the spinal cord. the primitive cerebral hemispheres. or hindbrain. The diencephalon. arrowheads. The mesencephalon. roof of the fourth ventricle. The prosencephalon. characterized by outgrowth of the optic vesicles. o. arrow. Asterisk. 3.or midbrain.  Simultaneously it forms two flexures: A. The telencephalon. outpocketing of the telencephalon. B. or forebrain.

the central canal.Central Nervous System  A deep furrow.  The rhombencephalon also consists of two parts: 1) 2)        The metencephalon. The boundary between these two portions is marked by the pontine flexure. the rhombencephalic isthmus. . The lateral ventricles communicate with the third ventricle through the interventricular foramina of Monro. This lumen becomes very narrow and is then known as the aqueduct of Sylvius. and those of the cerebral hemispheres are the lateral ventricles. that of the diencephalon is the third ventricle. The lumen of the spinal cord. which later forms the pons and cerebellum. The myelencephalon. separates the mesencephalon from the rhombencephalon. The lumen of the mesencephalon connects the third and fourth ventricles. is continuous with that of the brain vesicles. The cavity of the rhombencephalon is the fourth ventricle.

Neural Crest Cells • These neural crest cells are ectodermal in origin and extend throughout the length of the neural tube. . • Crest cells migrate laterally and give rise to sensory ganglia (dorsal root ganglia) of the spinal nerves and other cell types.

. cells of the neural crest differentiate into sympathetic neuroblasts. and mesenchyme of the pharyngeal arches. neuroblasts of the sensory ganglia derived from neural crest cells give rise to the dorsal root neurons. Schwann cells. meninges. • In addition to forming sensory ganglia. odontoblasts.Neural Crest Cells • Hence. pigment cells.

arising from nerve cells in the basal plates (ventral horns) of the spinal cord.Spinal Nerves • Motor nerve fibers begin to appear in the fourth week. • Dorsal nerve roots form as collections of fibers originating from cells in dorsal root ganglia (spinal ganglia). • Almost immediately. spinal nerves divide into dorsal and ventral primary rami. . • These fibers collect into bundles known as ventral nerve roots.

Patient with a large meningomyelocele. A. . Patient with a severe defect in which the neural folds failed to elevate throughout the lower thoracic and lumbosacral regions. resulting in rachischisis. B.16 Lumbosacral region of patients with neural tube defects.

CLINICALCORRELATES Neural Tube Defects • Most defects of the spinal cord result from abnormal closure of the neural folds in the third and fourth weeks of development. neural tube defects (NTDs). muscles. • The resulting abnormalities. and skin. vertebrae. • Severe NTDs involving neural and non-neural structures occur in approximately 1 in 1000 births . may involve the meninges.

It consists of a splitting of the vertebral arches and may or may not involve underlying neural tissue. . 2) Spina bifida cystica is a severe NTD in which neural tissue and/or meninges protrude through a defect in the vertebral arches and skin to form a cyst like sac. Two different types of spina bifida occur: 1) Spina bifida occulta is a defect in the vertebral arches that is covered by skin and usually does not involve underlying neural tissue. It occurs in the lumbosacral region (L4 to S1) and is usually marked by a patch of hair overlying the affected region.Neural Tube Defects • Spina bifida is a general term for NTDs affecting the spinal region.

Neural Tube Defects  Occasionally the neural folds do not elevate but remain as a flattened mass of neural tissue (spina bifida with myeloschisis or rachischisis). .  Hydrocephaly develops in virtually every case of spina bifida cystica because the spinal cord is tethered to the vertebral column.

and the infant is placed back in the uterus.  The baby is exposed by cesarean section. the defect is repaired. improves bladder and bowel control. .Neural Tube Defects/ Dx +Rx A new treatment for the defect is to perform surgery in utero at approximately 28 weeks of gestation.  Preliminary results indicate that this approach reduces the incidence of hydrocephalus. and increases motor development to the lower limbs.

the most caudal of the brain vesicles.RHOMBENCEPHALON HINDBRAIN • The rhombencephalon consists of the myelencephalon. Lateral view of the brain vesicles in an 8-week embryo (crown-rump length approximately 27 mm). The roof plate of the rhombencephalon has been removed to show the intraventricular portion of the rhombic lip. which extends from the pontine flexure to the rhombencephalic isthmus. and the metencephalon. . Note the origin of the cranial nerves.

Note the alar and basal plates in the myelencephalon.Myelencephalon • The myelencephalon is a brain vesicle that gives rise to the medulla oblongata. Dorsal view of the floor of the fourth ventricle in a 6week embryo after removal of the roof plate. A. Intermediate special visceral efferent group. • These nuclei are divided into three groups: 1. The rhombic lip is visible in the B and C. 2. Position and differentiation . similar to that of the spinal cord. 3. Lateral general visceral efferent group . contains motor nuclei. • The basal plate. Medial somatic efferent group.

B.20 A. 2. . allowing a view of its floor. containing nuclei of the trigeminal and facial nerves. 3) Figure 19. which gives rise to the nucleus of the abducens nerve. whose axons supply the submandibular and sublingual glands. • Two new components form: 1.Metencephalon • The metencephalon. Note the choroidal fissure and the lateral and medial apertures in the roof of the fourth ventricle. The pons. Dorsal view of the mesencephalon and rhombencephalon in an 8-week embryo. The special visceral efferent group. Similar view in a 4-month embryo. which innervate the musculature of the first and second pharyngeal arches. Each basal plate of the metencephalon contains three groups of motor neurons: The medial somatic efferent group. similar to the myelencephalon. The general visceral efferent group. The roof of the fourth ventricle has been removed.  1) 2) The cerebellum. is characterized by basaland alar plates.

• In a 12-week embryo. the vermis.Cerebellum • As a result of a further deepening of the pontine flexure. the hemispheres. this plate shows a small midline portion. the rhombic lips compress cephalocaudally and form the cerebellar plate. . and two lateral portions.  flocculonodular lobe is phylogenetically the most primitive part of the cerebellum.

13 weeks. D. . B. 12 weeks (70 mm). A.Sagittal sections through the roof of the metencephalon showing development of the cerebellum. cells of the external granular layer migrate inward to mingle with Purkinje cells and form the definitive cortex of the cerebellum. Note formation of the external granular layer on the surface of the cerebellar plate (B and C). 8 weeks (approximately 30 mm). During later stages. C. 15 weeks. Note the anterior and posterior velum. The dentate nucleus is one of the deep cerebellar nuclei.

They migrate inward from the surface (arrows). A. and the internal granular layer beneath the Purkinje cells. the molecular layer on the surface. . the external granular layer gives rise to various cell types. Basket and stellate cells derive from proliferating cells in the cerebellar white matter. B. Postnatal cerebellar cortex showing differentiated Purkinje cells. 22 Stages in development of the cerebellar cortex.  Basket and stellate cells are produced by proliferating cells in the cerebellar white matter. The external granular layer on the surface of the cerebellum forms a proliferative layer from which granule cells arise.Cerebellum • In the sixth month of development.

2. and epiphysis. . which forms the optic cup and stalk. Telencephalon. hypothalamus.PROSENCEPHALON: FOREBRAIN • The prosencephalon consists of the 1. pituitary. which forms the cerebral hemispheres. thalamus. Diencephalon.

• CLINICALCORRELATES Hypophyseal Defects • Occasionally a small portion of Rathke’s pouch persists in the roof of the pharynx as a pharyngeal hypophysis..  Craniopharyngiomas arise from remnants of Rathke’s pouch. growth failure). diabetes insipidus. They may cause hydrocephalus and pituitary dysfunction (e.g. . They may form within the sella turcica or along the stalk of the pituitary but usually lie above the sella.

CLINICALCORRELATES Cranial Defects • Holoprosencephaly (HPE) refers to a spectrum of abnormalities in which a loss of midline structures results in malformations of the brain and face. . and there is a single nasal chamber along with other midline facial defects. the lateral ventricles merge into a single telencephalic vesicle (alobar HPE). the eyes are fused. • In severe cases.

which may be partially or totally lacking. • . meningoencephalocele. sometimes causing a loss of brain tissue • Meningocele. The most frequently affected bone is the squamous part of the occipital bone. only meninges bulge through it (meningocele). and meningohydroencephalocele are all caused by an ossification defect in the bones of the skull. If the opening of the occipital bone is small.CLINICALCORRELATES Cranial Defects • Schizencephaly is a rare disorder in which large clefts occur in the cerebral hemispheres.

. Various types of brain herniation due to abnormal ossification of the skull.36 A–D.Figure 19.

the vault of the skull does not form. As a result. leaving the malformed brain exposed. leaving a mass of necrotic tissue. Since the fetus lacks the mechanism for swallowing. This defect is called anencephaly. although the brainstem remains intact. since the vault of the skull is absent.CLINICALCORRELATES Cranial Defects • Exencephaly is characterized by failure of the cephalic part of the neural tube to close. Later this tissue degenerates. . the last 2 months of pregnancy are characterized by hydramnios. The abnormality can be recognized on a radiograph.

where it would be resorbed. hydrocephalus in the newborn is due to an obstruction of the aqueduct passing into the fourth ventricle and from there into the subarachnoid space. • This prevents the cerebrospinal fluid of the lateral and third ventricles from ventricular system.CLINICALCORRELATES Cranial Defects • Hydrocephalus is characterized by an abnormal accumulation of cerebrospinal fluid within the of Sylvius (aqueductal stenosis). . In most cases.

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